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 Table of Contents  
Year : 2023  |  Volume : 3  |  Issue : 1  |  Page : 14-21

The 9th annual saudi society for rheumatology conferenceabstracts presented at the 9th SSRC 2023

Date of Submission05-Mar-2023
Date of Decision10-Mar-2023
Date of Acceptance15-Mar-2023
Date of Web Publication31-Mar-2023

Correspondence Address:
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ara.ara_3_23

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How to cite this article:
. The 9th annual saudi society for rheumatology conferenceabstracts presented at the 9th SSRC 2023. Ann Rheumatol Autoimmun 2023;3:14-21

How to cite this URL:
. The 9th annual saudi society for rheumatology conferenceabstracts presented at the 9th SSRC 2023. Ann Rheumatol Autoimmun [serial online] 2023 [cited 2023 Jun 7];3:14-21. Available from: https://www.arajournal.org//text.asp?2023/3/1/14/373352

  Consensus Recommendations on the Use of Biosimilars in the Treatment of Rheumatic Diseases in the Gulf Region Top

K. A. Alnaqbi1,2, N. Al Adhoubi3, S. Al Dallal4, S. Al Emadi5, A. Al-Herz6, A. M. El Shamy7, S. Hannawi8, M. A. Omair9, S. A. Saad10, T. K. Kvien11

1Department of Rheumatology, Tawam Hospital, Al Ain, UAE, 2College of Medicine and Health Sciences, UAE University, Al Ain, UAE

Purpose of the Study: Biosimilars, when compared to high-cost innovator reference products, can reduce financial burden and medication underutilization while remaining effective and safe in treating rheumatic diseases. The objective was to develop evidence-based consensus recommendations and overarching principles aimed at standardizing best practices for the use of biosimilars in treating rheumatic diseases in the Gulf region. Materials and Methods: A scientific committee comprising expert rheumatologists, pharmacists, and healthcare economists reviewed the outputs of a systematic literature review addressing PICO questions related to challenges regarding the use of biosimilars for the treatment of rheumatic diseases in the Gulf region. Overarching principles and recommendations were developed and critically reviewed to assess the quality of evidence and strength of recommendation. Furthermore, these were validated by external stakeholders, including pediatric and adult rheumatologists, nurses, pharmacists, payors, regulators, and patients. Results: The scientific committee and external stakeholders reached consensus on all statements with the required level of agreement of 70% or higher. The recommendations are congruent with previously published guidelines, with adaptations considering the nuances of the healthcare systems within the Gulf region. The overarching principles emphasize the importance of improving the understanding and perception of biosimilars among rheumatologists, patients, and other stakeholders, thereby increasing the adoption of biosimilars into rheumatic disease treatment strategies. Standardizing protocols for real-world evidence generation and the adoption of biosimilars as viable cost-effective treatment options for patients are required. The recommendations advocate for shared treatment decisions between rheumatologists and patients when switching to approved biosimilars. Furthermore, confirmation of efficacy and safety of a biosimilar in a single indication is sufficient for extrapolation to other indications for which the reference product has been approved. National health policies governing biosimilar pharmacovigilance must be developed. Conclusion: These are the first Gulf-focused consensus recommendations based on an elaborate systematic literature review, integrating clinical evidence and expertise to optimize the use of biosimilars for treating rheumatic diseases.

  Real-World Effect of Intravenous Belimumab on Clinical Outcomes in Patients with Systemic Lupus Erythematosus in Saudi Arabia: The Observe Study Top

Ibrahim Al-Homood1, Ibrahim Almaghlouth2, Alhussain Asiri3, Hanan Hamdy4, Ali Alhammad5, Alaa Mustafa6, Mohamed Othman6, Munther Khamashta7, Tamer Elfishawy7, Lindsey Teichman8, Debora dos Santos9, Juliana Queiroz9, Saeed Noibi5

1King Fahad Medical City, Riyadh, Saudi Arabia, 2King Saud University Medical City, King Saud University, Riyadh, Saudi Arabia, 3Asir Central Hospital, Aser, Saudi Arabia, 4King Fahad Specialized Hospital, Tabouk, Saudi Arabia, 5Value Evidence and Outcomes, GSK Saudi Arabia, Jeddah, Saudi Arabia, 6Medical Affairs, GSK Saudi Arabia, Jeddah, Saudi Arabia, 7Medical Affairs, GSK, Dubai, United Arab Emirates, 8Real World Matrix Team, GSK, Dubai, United Arab Emirates, 9Real World Matrix Team, GSK, Rio de Janeiro, Brazil

Introduction: Real-world studies investigating intravenous belimumab effectiveness among patients with systemic lupus erythematosus (SLE) in the Kingdom of Saudi Arabia (KSA) are limited. Understanding clinical outcomes can inform treatment regimens in clinical practice. Objectives: Describe clinical outcomes of patients with SLE in KSA after 6 months of intravenous belimumab use. Methods: Adults with SLE who received ≥1 intravenous belimumab dose during standard care were enrolled in this retrospective, observational study (GSK Study 215349). The primary outcome: physician-assessed clinical response (worse, no improvement, improvement of ≥20% and ≥50%) at 6 months after index (belimumab initiation). Other outcomes (assessed at index and during 6 months pre- and post-index): SLE flares (mild/moderate; severe); C-reactive protein (CRP), anti-dsDNA and C3/C4 levels; steroid use. Results: Overall, 47 patients were included; 44 completed ≥6 months of belimumab treatment. At 6 months post-index, clinical improvements of ≥20% and ≥50% were reported in 97.7% and 79.5% of patients, respectively. During pre-index, among patients with flares recorded (n=19), 57.9% had ≥1 mild/moderate flare and 47.3% had ≥1 severe flare. During post-index, among patients with flares recorded (n=21), 38.1% had ≥1 mild/moderate flare and 9.5% had ≥1 severe flare. Median (interquartile range) CRP levels decreased from 5.5 (22.4) mg/L at index to 3.3 (7.1) mg/L within 6 months post-index; anti-dsDNA levels decreased from 80.0 (140.0) IU/mL to 30.0 (180.7) IU/mL; no changes were observed in C3 levels, C4 levels increased from 0.1 (0.1) to 0.2 (0.1). Mean (standard deviation) steroid dose was 12.7 (9.6) mg/day at pre-index, 10.2 (7.5) mg/day at index, and 6.2 (3.4) mg/day at 6 months post-index. Conclusions: In KSA, patients with SLE receiving belimumab experienced substantial clinical improvements and reductions in anti-dsDNA levels and steroid use.

  Safety Profile of JAK Inhibitors Use in Rheumatic Diseases in Qatar Population, A Four-Year Real-World Data Top

Omar Alsaed, Abdul Razzakh Poil, Mohanad Ahmed, Samar Alemadi

Department of Medicine, Division of Rheumatology, Hamad Medical Corporation, Doha, Qatar

Introduction: Similar to other real-world data reports, the U.S. Corrona RA registry showed Tofacitinib and bDMARD had a similar rate of major adverse cardiovascular events (MACE), malignancy, and venous thromboembolism (VTE). However, ORAL Surveillance, revealed higher rates of MACE and malignancy than tumor necrosis factor inhibitors (TNF-i). Objectives: Explore the rate of malignancy and MACE in patients with autoimmune rheumatic diseases (ARDs) received janus kinase inhibitors (JAK-i) in Qatar population. Methods: Patients with ARDs who received JAK-i (Tofacitinib, Upadacitinib and Baricitinib) from 1st January 2018 to 1st November 2022 were captured electronically from the pharmacy database of Hamad Medical Corporation. The electronic medical records of these patients were reviewed retrospectively to identify demographics, clinical characteristics, MACE, VTE, shingles and malignancy post JAK-i initiation. Results: 188 JAK-i treatment courses were identified from the pharmacy database. Tofacitinib was most common 163 (88.6%) followed by Upadacitinib 13 (7.1%) and Baricitinib 8 (4.3%). Of these, four prescriptions were not dispensed hence excluded from the analysis. Mean (SD) age of the patients was 51 (12) years, females were (75%). Ethnicities distribution was Arab (73%), Asian (21%) and others (5%). Mean (SD) duration of JAK-i exposure was 25(17) months. Mean (SD) duration of ARDs was 9.2 (6.8) years. Rheumatoid arthritis was in 129(70%) patients followed by psoriatic arthritis 28(15%), axial spondyloarthropathy 13(7%) and other diagnosis in 14(7.6%) patients. The comorbidity profile (hypertension, diabetes mellitus, dyslipidemia and coronary artery disease) was 28%, 23%, 25% and 6.5%, respectively. Two patients got shingles, one during Tofacitinib and another one during Upadacitinib. New cancer diagnosis (follicular thyroid) was confirmed in one patient post Tofacitinib and one case developed lymphoma relapse after Tofacitinib initiation. One ischemic cerebrovascular accident (CVA) identified post Tofacitinib initiation. This patient was in atrial fibrillation when CVA occurred. No VTE reported from this cohort. Conclusion: Data from this cohort which had a quite good period of JAK-i exposure showed an extremely low rate of MACE and malignancy rates. A study with a control group is needed for further exploration the association of MACE and malignancy with JAK-i.

  The Role of Macrophages Migration Inhibitory Factor in Ankylosing Spondylitis Activity Top

Yasser Gazar1, Mohammed Hanafy1, Mohammed Elrefi2

1Department of Rheumatology, Faculty of Medicine, Al-Azhar University, Cairo, Egypt, 2Armed Force Hospitals, Cairo, Egypt

Background: Ankylosing spondylitis (AS) has been recognized as part of the spondyloarthropathy group of rheumatic diseases. Macrophages Migration Inhibitory Factor (MIF) is a potent proinflammatory. Cytokine implicated in several diseases. It plays a key role in the development of chronic colitis in mouse models. Elevated serum MIF levels have been reported in AS. Although the role of MIF in bone formation has been a subject of controversy; high levels of MIF transcripts have been found in murine. neonatal calvaria, and CD74- knockout mice show enhanced osteoclastogenesis. Objectives: The purpose of this study is to determine the role of macrophage migration inhibitory factor in ankylosing spondylitis activity and if has a role in prediction of spinal progression. Methods: This cross-sectional case-control study were concluded upon 70 randomly selected people from internal medicine department in Kobry El-Kobba Military Hospital and from rheumatology and rehabilitation department in EL-Hussein university hospital, Cairo, Egypt. The population study were divided into two groups: Group A: 50 Patients with Ankylosing Spondylitis (AS) who met the modified New York criteria for AS. Group B: 20 Healthy controls, not known to be AS, psoriatic, enteropathy, rheumatoid arthritis, SLE, nor any other autoimmune disease. Results: The mean age of all patients was (38.2 ± 8.4) years. Regarding gender of the patients, the majority (65.7%) of patients were males, while (34.3%) were females. the mean disease duration of AS patients was (15.5 ± 6.7) years, while the mean BASDAI score was (3.5 ± 2.17). Regarding smoking, (40%) of AS patients were smokers. We also found that, (18%) od AS patients had HTN, (16%) had DM, (54%) received NSAIDs, (88%) received DMARDs, (14%) received TNFi. Regarding radiological data, (88%) of AS patients had cervical erosion or sclerosis, (46%) had dorsal erosion or sclerosis, (92%) had lumbosacral erosion or sclerosis, with m-SASS score of (11 ± 6.1). Regarding musculoskeletal U/S, (72%) had evidence of inflammation and erosion. highly significant increase in ESR and MIF levels in progressor AS group; compared to nonprogress or AS group; with highly significant statistical difference (p < 0.01 respectively). highly significant increase in m-SASS score, dorsal erosion or sclerosis and effusion & inflammation in (U/S), in progressor AS group; compared to non-progressor. AS group; with highly significant statistical difference (p < 0.05 respectively). By using ROC-curve analysis, MIF level at a cutoff point (>51) predicted patients with progression, with fair accuracy. (74%), sensitivity = 53% and specificity = 94% (p = 0.0056). Conclusion: MIF appears to have the unique ability to drive both inflammation and new bone formation and could play an important role in the pathogenesis of AS. Serum MIF levels were predictive of progressive. spinal damage in AS patients.

  Vascular and Neurological Manifestations of Behçet's Disease in the Eastern Province of Saudi Arabia Top

Khawla Alghanim MD, Hoda Alsomali MD, Ali Alharbi MBBS, Awab Elamin MD, Arulanantham Z. Jebakumar, Hisham Elsayed Shadi

Department of Internal Medicine, Rheumatology Unit, King Fahad Military Medical Complex, Dhahran, Saudi Arabia

Introduction: Behcet's disease (BD) is a vasculitis with multisystem and multiorgan involvement. It can involve nearly all systems of the body with recurrent oral and genital ulcers, as well as ocular, neurological, musculoskeletal, vascular, cardiovascular, and gastrointestinal manifestations. Objectives: We aim to examine manifestations of vascular & neurological involvement in patients with Behçet's disease in Eastern Province of Saudi Arabia as they carry the highest morbidity & mortality among BD patients. Methods: This is a Prospective Study of single center conducted at Rheumatology outpatient clinics of King Fahad Medical Military Complex (KFMMC) in Dhahran, Eastern Province of Saudi Arabia. Data were gathered using the registered information from January 2016 to December 2022. Results: Most of our patients were males (76%) and all of them were Saudi. (24%) had family history of BD. Median age of diagnosis was 34 years old & median disease duration was 3 years. Most common presentation among our patients was oral ulcer (79%) followed by genitalia ulcer (76%). (89%) of our patients developed vascular manifestations and most of them were males. Venous involvements (56%) were more common than arterial involvements (45%). (79%) were HLA-B51 positive with significant P Value of 0.01. (31%) of BD patients had neuro-Behçet with headache being the commonest manifestation (33%). Interestingly we found that most common manifestation of the familial Behçet was arthritis with significant p-value of 0.01. Conclusion: Vascular and neurological manifestations carry significant morbidity and mortality with increased tendency of occurrence towards male patients.

  Nailfold Videocapillaroscopy Patterns and Associated Clinical Features in Systemic Sclerosis in Qatar Top

Neethu Maria Kunjumon, Fiaz Alam, Samar Al Emadi, Karima Becetti

Department of Medicine, Division of Rheumatology, Hamad Medical Corporation, Doha, Qatar

Introduction: The role of nailfold videocapillaroscopy (NVC) in raynaud's phenomenon (RP) is well established. Recent studies in systemic sclerosis (SSc) suggest a prognostic value to NVC. Objectives: The aim of this study was to evaluate the NVC patterns in SSc in Qatar and their association with disease features. Methods: Consecutive SSc patients were recruited. Demographic, laboratory and clinical information, and NVC examination were collected. NVC images were evaluated quantitatively for capillary density (in 1mm) and qualitatively for overall pattern classified into non-severe (normal, non-specific or early) and severe (active or late) patterns. Chi-square and student t tests were used to compare variables between the 2 patterns. Capillary density was correlated with clinical variables. Results: The study included 20 SSc patients with 70% women, mean age of 41.8 + 2.4 years and disease duration of 4.8 + 0.6 years. Diffuse cutaneous SSc was present in 60% of patients. Majority (55%) had Anti-Scl70. RP was reported in 65% of patients with digital ulcers (DUs) in 35%, interstitial lung disease in 75%. On NVC, 71% had severe pattern (44% active and 27% late). The diffuse and limited cutaneous subsets had higher frequency of severe pattern (60% and 100%, respectively, p = 0.049). Severe pattern was more frequent in patients with RP (92% vs 8%, p < 0.01) and DUs (100% vs 0, p = 0.04). Mean density was significantly reduced in those who reported RP (4.1 + 0.6 vs. 6.6 + 0.5, p = 0.02). No associations were observed between NVC pattern or density with other variables. Conclusions: In this SSc cohort, severe NVC patterns were associated with the presence of RP and DUs. A larger sample size will allow for a better evaluation of its association with other clinical features at baseline and follow up.

  The Annual Rate of Sacroiliitis and Disease Detection Using Different Imaging Modalities: A Single Center Experience Top

S. M. Attar1, K. Khashoggi2, S. Alharbi3, A. Alsulami3, E. Faden3, A. Alamuodi3, S. Alkhamisi3, M. Addas3, S. Alqarni3, R. Alothimen3

1Department of Internal Medicine, King Abdulaziz University, Jeddah, Saudi Arabia, 2Department of Radiology, King Abdulaziz University Hospital, Jeddah, Saudi Arabia, 3King Abdulaziz University Hospital, Jeddah, Saudi Arabia

Introduction: Inflammation of the sacroiliac joint (SIJ), termed sacroiliitis, commonly presents as chronic back pain. A definitive diagnosis is made using a variety of radiological techniques that differ in specificity and sensitivity. Objectives: This study aims to estimate the annual incidence of sacroiliitis and to explore the frequency of multiple modalities used in its evaluation. Method: This is a retrospective analytic study that examines patients' records of diagnostic imaging of the sacroiliac joint (MRI, CT, and X-ray) at the Radiology Department at a tertiary Center from January 2012 to June 2016. Results: Out of 388 candidate patients 129 were diagnosed with sacroiliitis. The mean age of sacroiliitis cases was 48.6 ± 15.5 years (mean ± SD), and the mean age of non-sacroiliitis patients was 45.2±15.5 years in which 102 were female and 27 were male (P = 0.005). The data showed a significant risk in the white ethnic group (P = 0.04). From 2012 to 2016, the annual incidence of sacroiliitis increased (28.17%, 31.58%, 31.78%, and 36.05% in 2012, 2013, 2014, 2015, and 2016, respectively). The evaluation of SIJ by MRI in 2014 was found to have an incidence of 16.98%; there was a significant increase in the following year to 31.40%. Conclusion: The annual incidence of sacroiliitis increased over five years (January 2012 - June 2016). MRI was found to have broadly exceeded other radiological modalities in the diagnostic process. Accordingly, the results were supported by existing guidelines that recommend MRI for early detection; however, the techniques of CT and X-ray have yet to be entirely replaced and its diagnostic value should undergo further investigation.

  Public Awareness about Arthritic Diseases in Saudi Arabia: A Systematic Review and Meta-analysis Top

Abdullah A. Ghaddaf1,2, Mohammed S. Alomari1,2, Fahad A. AlHarbi1,2, Mohammed S. Alquhaibi1,2, Jawaher F. Alsharef1,2, Noor K. Alsharef1,2, Ahmed S. Abdulhamid1,2, Dania Shaikh3, Mohammed S. Alshehri1,2,4

1College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia, 2King Abdullah International Medical Research Center, Jeddah, Saudi Arabia, 3Department of Orthopedic Surgery, King Fahad General Hospital, Jeddah, Saudi Arabia, 4Department of Surgery, Orthopedic Section, King Abdulaziz Medical City, Jeddah, Saudi Arabia

Background: Raising the public knowledge and perception would have a discernible impact on providing the optimal care and reducing the burden of the common arthropathic conditions in the community. This systematic review aimed to identify the public knowledge about the common arthritic conditions in Saudi Arabia. Methods: We searched Medline, Embase, and CENTRAL for relevant literature. We included questionnaire-based cross-sectional studies performed in Saudi Arabia, to assess the public perception of general knowledge, causes/risk factors, signs/symptoms, and relieving/management measures of the common arthritic conditions including osteoarthritis (OA), rheumatoid arthritis (RA), and gout. The meta-analysis was performed on outcomes reported in ≥2 studies utilizing random-effects model. Quality assessment was done using AXIS tool. Results: Ten studies were deemed eligible for inclusion in this review. A total of 35 questions were feasible to be included in the meta-analysis (i.e., reported in ≥2 studies). The meta-analysis estimated that 83.51% (95% confidence interval (CI): 81.50% to 85.34%), 54.51% (95%CI: 51.29% to 57.69%), and 80.42% (95%CI: 77.27% to 83.23%) have ever heard or read about OA, RA, and gout. Joint pain and swelling were perceived to be the main signs/symptoms of OA, RA, and gout. Conclusion: The Saudi public perception about the general knowledge and causes/risk factors of the most common arthritic conditions was acceptable. The level of knowledge about other aspects of the common arthritic conditions is still limited and need to be addressed by future educational interventions.

  Phenotype and Disease Course Differences in Monogenic and Sporadic Childhood Lupus Top

Sulaiman M. Al-Mayouf1,2, Fatima Alkhars1, Samia AlHashim1, Alhanouf AlSaleem1

1Department of Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia, 1College of Medicine, Alfaisal University, Riyadh, Saudi Arabia

Objective: To report the differences in phenotypic characteristics, disease course, and outcome in monogenic and SC-lupus from a single tertiary childhood lupus clinic. Methods: A descriptive, observational, cross-sectional study was conducted. Data were retrospectively collected at the last follow-up visit on patients with monogenic lupus proven by genetic variants and SC-lupus seen between 1997 and 2022. SC-lupus patients were selected by systematic sampling from lupus patients presenting to our lupus clinic; the first patient was chosen randomly, and the subsequent patients were chosen at intervals of 3. Data comprised the clinical and laboratory findings, disease activity using the SLEDAI, and damage measured by the pSDI. Results: A total of 54 patients with a median disease duration of 6.8 (IQR 3.5-10.5) years were included. There were 27 patients with monogenic lupus and 27 patients with SC-lupus, with a median age at disease onset of 3.5 (IQR 1.0-6.0), and 9.5 (IQR 7.0-11.8), respectively (p < 0.05). The rate of consanguinity and family history of lupus were higher in monogenic lupus patients. The two groups were comparable. However, monogenic lupus patients showed more gastrointestinal tract symptoms, and failure to thrive (p < 0.05). They also had more infections. The frequency of the autoantibody profile was higher in monogenic lupus patients. Belimumab is more frequently used in monogenic lupus, while rituximab in SC-lupus patients. Monogenic lupus patients had a higher mean SLEDAI, but statistically it was insignificant. Patients with monogenic had greater disease damage, with a higher mean pSDI and a higher mortality rate (p < 0.05). Conclusion: Patients with monogenic lupus are likely to have an early disease onset and a strong family history of lupus; as well as guarded prognosis, which is likely due to the disease's severity and frequent infections. These differences may be related to the high consanguinity rate and underlying genetic variants.

  Quality Indicators for the Process of Care in Juvenile Idiopathic Arthritis Top

Reem Alshammari1, Hend Alkwai2, Hala Lotfy3, Khulood Khawaja4, Muna Almutairi5, Raed Alzyoud6, Rebecca James7, Reem Abdwani8, Soad Hashad9,10, Soamarat Vilaiyuk11, Sumaira Farman12, Swee Ping Tang13, Thaschawee Arkachaisri14,15, Sulaiman M. Al-Mayouf2,16

1Department of Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia, 2Department of Pediatrics, University of Ha'il, Ha'il, Saudi Arabia, 3Department of Pediatric Rheumatology, Cairo University, Cairo, Egypt, 4Division of Pediatric Rheumatology, Shaikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates, 5Department of Pediatrics, Adan Hospital, Kuwait City, Kuwait, 6Allergy, Immunology, and Rheumatology Division, Queen Rania Children's Hospital, Amman, Jordan, 7Department of Pediatric Rheumatology, Queensland Children's Hospital, Brisbane, Australia, 8Department of Pediatrics, Sultan Qaboos University, Muscat, Oman, 9Department of Rheumatology, Tripoli Children Hospital, Tripoli, Libya, 10Department of Rheumatology, University of Tripoli, Tripoli, Libya, 11Department of Pediatrics, Rheumatology Division, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand, 12Department of Rheumatology, National Hospital and Medical Center, Lahore, Pakistan, 13Department of Pediatric Rheumatology, Selayang Hospital, Batu Caves, Malaysia, 14Department of Paediatric Subspecialties, Rheumatology and Immunology Service, KK Women's and Children's Hospital, Singapore, 15Duke-NUS Medical School, Singapore, 16College of Medicine, Alfaisal University, Riyadh, Saudi Arabia

Background and Objective: Juvenile idiopathic arthritis (JIA) is the most common inflammatory arthritis in children. The prognosis of JIA can be significantly improved with prompt diagnosis and care. Assessing the quality of care is an essential component of continuous quality improvement. The objective of this study was to develop a set of quality indicators (QIs) for the care process of children with JIA in the Middle East and Asian-Pacific region. Methods: The modified RAND/UCLA Appropriateness Method was used to develop the QIs. First, a systematic literature review was performed. Second, candidate QIs were extracted to be evaluated by an expert panel. The panel was composed of 12 representatives from the Arab League of Associations for Rheumatology (ArLAR), the African League Against Rheumatism (AFLAR), and the Asia Pacific League of Associations for Rheumatology (APLAR). Finally, the appropriateness of the items was assessed via rating rounds and panelists' discussions. Results: Initially, a candidate list of 113 QIs was extracted after a systematic literature review. This was reviewed by a working group comprised of three members of the panelists. 32 QIs were sent by electronic mail to the panelists for assessment regarding validity and feasibility. Round one of the Delphi surveys resulted in the elimination of nine QIs due to disagreement. In round two, panelists were presented with QIs for which the median absolute deviation was ≥1, for revision and re-rating. Finally, 18 QIs for the process of care in JIA were established. Conclusion: For the assessment of the JIA care process, a set of 18 QIs was formulated. This set of indicators is an initial step toward the important goal of improving the medical care process for children with JIA in the Middle East and Asian-Pacific region.

  Safety and Efficacy of Biologic Agents in Childhood Lupus: A Critical Literature Review Top

Samar Jaber1, Rawan Elshaer1, Nour Odeh1, Lana Arbili1, Sulaiman M. Al-Mayouf1, 2

1College of Medicine, Alfaisal University, Riyadh, Saudi Arabia, 2Department of Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Objective: To collate available evidence related to the efficacy and safety of using biologic agents in c-lupus. Methods: The reporting of the data was guided by the PRISMA checklist. A search was conducted using three databases (PubMed, Cochrane Library, and Scopus) for articles published between January 2005 and September 2022. Articles were screened by title and abstract and included only if they met the following criteria: diagnosis of c-lupus, use of a biologic agent, and available outcome measures obtained at 6- and 12-month follow-up evaluated the treatment's safety and efficacy. Case reports were not included. Four independent reviewers extracted and screened the articles; the discrepancies were solved by the fifth reviewer to reach a consensus. Results: Thirteen articles were finally included; seven retrospective studies, four prospective studies, one clinical trial, and one pilot study. The total number of patients in the included articles was 311; they were treated with biologic agents for severe and/or refractory c-lupus. The most common indication for using biologic agents was lupus nephritis. Rituximab was used in ten studies and belimumab in three studies. The efficacy of biologic agents in c-lupus was evaluated based on the change in standardized scoring systems for SLE disease activity, improvement in laboratory parameters, and a reduction in corticosteroid dose. Most studies show improvement in renal, hematologic, and immunologic parameters and a reduction in corticosteroid dose. Mild adverse effects were common and were mainly infection- and infusion-related reactions. Conclusion: According to the current data, biologic agents, namely belimumab and rituximab, seem to be effective for refractory and severe cases of c-lupus. Overall, disease activity decreased, and a significant number of patients achieved complete or partial remission with a reasonable safety profile. However, further studies are required to allow more definitive conclusions, especially in patients with major organ involvement.

  A Challenging Case with Nephritis and Pancreatitis: Could It Be a Case for the Rheumatologist Top

Njood Nazer1, Mohammed Muzzafer2, Najla Alotaibi3, Mohammed Nashawi3

1Department of Pediatrics, King Fahad Armed Forces Hospital, Jeddah, Saudi Arabia, 2Pediatric Nephrology Center of Excellence, King Abdulaziz University, Jeddah, Saudi Arabia, 3Department of Pediatrics, Pediatric Rheumatology, King Abdulaziz University, Jeddah, Saudi Arabia

Introduction: Sarcoidosis is a multisystem granulomatous disorder of undetermined underlying cause that most often affects the lungs and may cause significant morbidity. Patients with extrapulmonary involvement without lung involvement make up about 8% of patients with the disease. Even though sarcoidosis is a well-known disease, its renal manifestations remain elusive and evidence regarding diagnosis and treatment is scarce. Furthermore, acute pancreatitis as a manifestation of sarcoidosis is extremely rare. Objective: We present a challenging case of acute pancreatitis and kidney dysfunction, which had been diagnosed later to be sarcoidosis. Methodology: Case presentation. Results: A relatively healthy 12-year-old presented with progressively worsening, constant abdominal pain and weight loss for one month with tenderness over the epigastrium. His blood investigations showed elevated amylase and lipase with signs of acute kidney failure. The kidney biopsy showed foci of non-necrotizing granuloma with giant cell resection with the impression of acute interstitial nephritis, interstitial fibrosis, and tubular atrophy. Later during the disease course, the slit lamp exam showed anterior uveitis. Accordingly, sarcoidosis had been diagnosed and the patient started on infliximab, azathioprine, and steroid. During the work-up, a whole exome sequencing is done and showed a compound heterozygous variant in the ACE- Gene, which supports the diagnosis. Conclusion: Our presented case according to our knowledge the only case with pancreatitis and nephritis in pediatric sarcoidosis. Pancreatic involvement in sarcoidosis is very rare in adult patients. 9% of patients had mildly elevated pancreatic amylase levels. The disease is usually manifested by pancreatic glands being infiltrated or compressed pancreas by the enlarged nodes around the pancreas. Ophthalmology assessment is essential in patients with sarcoidosis. Anterior granulomatous uveitis is a common pathology involving the anterior segment of the eye.

  Deficiency of Adenosine Deaminase Type 2 (ADA2) DADA2 masquerade as lupus Top

Bayan Almabadi1, Hesham Alswat1, Hasheema H. Alsulami2, Salma Alkhammash3

1Department of Specialized Internal Medicine, King Abdullah Medical City, Makkah, Saudi Arabia, 2Department of Rheumatology, Department of Specialized Internal Medicine, King Abdullah Medical City, Makkah, Saudi Arabia, 3Department of Allergy and Immunology, Department of Specialized Internal Medicine, King Abdullah Medical City, Makkah, Saudi Arabia

Background: Deficiency of adenosine deaminase type 2 (ADA2) DADA2 is an autoinflammatory autosomal recessive disease resulting from biallelic loss of function mutations in the ADA2 gene. It is considered a rare disease; however, since its initial description in 2014, more than 170 affected individuals have been reported in the literature. Based on allele frequencies of in silico predicted ADA2 damaging variants, the estimated prevalence of DADA2 could be as high as 4:100,000. Initially recognized as a syndrome that manifests with intermittent fevers, polyarteritis nodosa, livedo racemosa, early-onset stroke, and mild immunodeficiency. Clinical presentation and age of onset vary widely even among related patients, and variability of symptoms and severity manifestations include bone marrow failure, autoinflammation, immunodeficiency and vasculitis. Here we describe a case of late onset, a young male patient who presented with fever, lower limbs skin rash, joint pain, and anemia. Case Presentation: This is a 24-year-old male patient, from consanguineous parents. He presented with Raynaud's phenomenon for 1 year. The patient also suffered from dizziness, headache, palpitation, and exertional shortness of breath, and he was found to have anemia of hemoglobin 5.4 mg/dl. In addition, he reported recurrent unexplained fever, associated with arthralgia, morning stiffness and rash involving his lower limbs. He had recurrent herpes zoster. Regarding family history, he has one sister who had recurrent mouth ulcers and was diagnosed with Behçet disease in outside facility, and she suffered from long standing anemia. Unfortunately, she died at age of 27 from ruptured appendix. Upon physical examination, he looked pale, no syndromic features. He had hepatosplenomegaly. He had a maculopapular rash involving his lower limbs bilaterally extending from his legs to thighs along with hyperpigmentation in his ankle regions [Figure 1a and c]. Lower limb pulses were palpable. No signs of arthritis or joint deformities. There were no palpable lymph nodes. Neurological examination was unremarkable. There were no signs of arthritis or ischemic ulcers. Investigations revealed hypochromic microcytic anemia hemoglobin of 5.7 mg/dl [Table 1]. Coombs test was negative and hemolytic workup were normal. He had leukopenia (WBC 2.3 109 /L), lymphopenia (0.29 109 /L) and mild neutropenia (1.65 109 /L). Inflammatory markers ESR and CRP were remarkably elevated >145 mm/h and 6.6 mg/dl respectively. His autoimmune workup showed positive antinuclear antibodies, anti-double stranded DNA, and rheumatoid factor while ANCA and lupus anticoagulant were negative. Pan-CT was done and showed few cervical, axillary, and paratracheal lymphadenopathy and significant hepatosplenomegaly. The patient underwent bone marrow biopsy and revealed pure red cell aplasia. Moreover, he underwent lymph node biopsy that did not show evidence of lymphoma. Skin biopsy revealed perivascular infiltration by neutrophils admixed with eosinophils in the upper dermis, and infiltration of vessel wall by these cells and extravasated red blood cells. These findings were compatible with vasculitis. Genetic testing was requested and revealed two pathogenic variants, c.882-2A>G (Splice acceptor (homozygous), were identified in ADA2. His genetic testing and clinical phenotype confirmed the diagnosis of DADA2, deficiency of ADA2. Anti-TNF has been proved effective in aborting autoinflammatory and vasculitis features and preventing strokes in patients with DADA2. However, it is less effective in hematological and immunological phenotypes. In our case, we started the patient on adalimumab 40 mg subcutaneous every two weeks. His autoinflammatory/vasculitis symptoms (fever, arthralgia, rash, Raynaud's) [Figure 1c] remarkably improved and his inflammatory markers significantly decreased [Table 1]. We also assessed his serum amyloid which is considered as acute-phase protein with proinflammatory properties and is being used in monitoring of monogenic autoinflammatory diseases and in some cases with rheumatoid arthritis, and impressively reduced by nearly 50% after initiating anti-TNF therapy. Surprisingly, his anti-dsDNA, a commonly used marker of lupus activity, did not improve with anti-TNF therapy. This means anti-dsDNA cannot be used as a monitoring marker of autoinflammation/autoimmunity in DADA2 cases. Unfortunately, his anemia persisted (Hgb around 5 mg/dl) and he continued to receive blood transfusion every 2 weeks. Thus, he is currently prepared for hematopoietic stem cell transplantation. Conclusion: DADA2 has an extremely variable clinical phenotype. It was described into three categories: inflammatory/vascular, immune dysregulation, and hematologic. However, the data is scant in describing autoimmunity phenotype in DADA2 and further studies are required to investigate the clinical correlation and presence of autoantibodies. We recommend genetic testing in cases with lupus-like disease especially if there is consanguinity between parents and family history of vasculitis.

  Outcome of Pregnancy in Women with Rheumatological Diseases: Single Center Experience Top


Background: Rheumatic diseases (RD) are challenging during pregnancy. The impact of these diseases on pregnancy will add further burden on the patient, fetus, physician and healthcare system. Objective: To study the outcome of pregnancy in women with RD and the behavior of the disease during pregnancy. Methods: A retrospective cohort study was conducted in King Abdulaziz Medical City (KAMC), Riyadh to compare the outcomes of pregnancy across RD from 2016 to 2021. A total of 128 pregnancies in 107 women with RD were included. Results: There were 55 patients with RA, 44 with SLE, and 8 with primary SS. Most of the patients were in clinical remission before pregnancy. Anti-SSA was positive in 41 patients. Eight SLE patients were labeled as lupus nephritis which was in remission. Previous abortions were found in 63 patients. Vaginal delivery was the most common mode of delivery. Postpartum complications (e.g., infection, bleeding) were noted in 12 (9.38%) pregnancies, and complications during pregnancy were found in 29 (22.66%). RD flares occurred in 10 (7.87%) pregnancies. Out of the 122 babies delivered, 52 were males and 72 were females. Preterm delivery occurred in 25 (20.83%) pregnancies, and 16 (13.22%) of the newborns needed NICU care. Interestingly, congenital heart block (CHB) was found in 5 (12.2%) neonates out of 41 Anti-SSA positive mothers; one out of those five died from heart block. Eleven neonates were delivered with positive serology, and five of them were diagnosed as Infantile Lupus. Conclusion: The outcome of pregnancy in patients with rheumatological disease is favorable. Multidisciplinary team approach and close clinical follow up is the cornerstone for such success. CHB is a concern for pregnant women with positive Anti-SSA.

[TAG:2]Clinical Features and Management of Neuropsychiatric Lupus in a Single Tertiary Center

Shigdar-Rahaf, Mohammed Nashawi, Mohammed Muzaffer

King Abdulaziz University Hospital, Jeddah, Saudi Arabia

Introduction: Systemic lupus erythematosus (SLE) is a complex autoimmune disorder characterized by multisystem involvement, including the nervous system. Approximately one-third of all children with SLE will display neuropsychiatric (NP) manifestations at some point during the disease. Early diagnosis and prompt intervention are critical for a better prognosis. Objectives: The proposed goal of this research is to estimate the prevalence in Saudi Arabia and other Arab countries of neuropsychiatric systemic lupus erythematosus (NPSLE) in children, describe the most common clinical features, compare these with the literature and help establish a clear recommendation in the management. Methods: Data of All children with SLE and evidence of NP manifestations were obtained from the database of King Abdulaziz University Hospital. NPSLE is defined by the presence SLE in addition to at least one of the following: headache, cerebrovascular accident (CVA), movement disorder, seizure, cognitive dysfunction, psychiatric or spinal cord manifestations. Result: The preliminary result of our study showed 9 female patients diagnosed at mean age of 10.7 years. Their clinical features were Headache 4/9 (44%), Seizure disorders 6/9 (66%), CVA 3/9 (33%), Movement disorders 1/9 (11%), Acute confusional state 1/9 (11%), Cognitive dysfunction 2/9 (22%), Mood disorder 2/9 (22%), and GBS 1/9 (11%). ANA titer was positive in all patients while dsDNA was high in 6/8 (75%). MRI showed positive changes for vasculitis in 7/8 (87.5%). Only 4 were treated with pulse steroid therapy, while 3 received cyclophosphamide and one rituximab. Conclusions: This study shows the heterogenicity of NPSLE management in children. A larger study including all regions in Saudi Arabia and other Arab countries is needed to estimate the exact prevalence of the disease and its clinical features. Finally, a consensus treatment recommendation is necessary for better treatment outcome.

  Phenotype and Genotype Characteristics of Interferon Mediated Diseases: A Single Tertiary Hospital Cohort Top

Alhanouf AlSaleem1, Shahad Alansari1, Sulaiman M. Al-Mayouf1,2

1Department of Pediatric Rheumatology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia, 2College of Medicine, Alfaisal University, Riyadh, Saudi Arabia

Background: IFN-mediated diseases, are mendelian innate immune-dysregulatory disorders that present early in life with fevers, sterile organ inflammation and a high type-I IFN-response gene signature in peripheral blood cells. To date, monumental discoveries of novel genetic variants and phenotypic features are recognized. Objective: To describe the clinical disease manifestations and genetic findings in patients with final diagnosis of autoinflammatory interferonopathy. Methods: A single tertiary autoinflammatory disease clinic's medical records were reviewed retrospectively for patients with genetically confirmed autoinflammatory interferonopathy. Patients were reviewed for demographic, clinical, and genetic characteristics. Results: Total of 13 patients (10 female) were included in the study. Majority presented in the first year of life, 50% within the first 6 months. Median age of disease onset was 6 months (IQR: 2-24), and the median age of diagnosis was 4 years (IQR: 2-10). Whole exome sequencing was performed in 11 patients, while 2 had a Leukoencephalopathy genetic panel. 6 patients with Aicardi-Goutières syndrome (RNASEH2A, RNASEH2C, IFIH1), 2 patients with STING-associated vasculopathy with onset in infancy (TMEM173), 1 patient with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperature (PSMB8). 4 patients had rare interferonopathy conditions (1 = SOCS1, 1 = STAT1, 2 = ISG15). Of 13 patients, 6 (46.1%) had novel genetic variants. Fever, skin rash, neurological, and respiratory involvement were the most prevalent features, respectively in 10, 8, 7, and 5 patients. Elevated inflammatory markers were identified in 9 (69.2%) patients, antinuclear antibody positive in 7 (53.8%) patients. Conventional Immunosuppressive treatment was used in more than half of the patients; (4 IVIG, 8 corticosteroids, 6 Jak inhibitor). Most of the patients had partial response to treatment. Mortality rate was zero. Conclusion: Our data represents group of patients with autoinflammatory interferonopathy from a single center in the Arab region. Interestingly, novel variants were identified in almost half of the patients, which is correlated with the identifications of rare interferonopathy conditions.

  Atypical Presentation of Active Systemic Juvenile Idiopathic Arthritis Top

Sarah Nagadi1, Ghufran Alhashmi2, Mohammed Nashawi1

1Department of Paediatrics, King Abdulaziz University Hospital, Jeddah, Saudi Arabia, 2Department of Radiology, King Abdulaziz University Hospital, Jeddah, Saudi Arabia

Introduction: The term juvenile idiopathic arthritis (JIA) refers to a heterogeneous group of chronic childhood arthritis that develop without apparent cause and generally last longer than six weeks. Systemic juvenile idiopathic arthritis (sJIA) is characterized by prominent systemic inflammation. The phenotypes' spectrum of the sJIA is wide and high suspicion is mandatory in atypical cases. Enthesitis and bone marrow oedema in such cases is rare and might represent a highly active disease. Case Presentation: We report a case of a 10-year-old male patient known case of systemic Juvenile idiopathic arthritis diagnosed at the age of 18-monthsmwhere he was started on multiple drug regimens to control the activity of the disease. In June 2022, the patient presented with left foot pain, which was progressive for three months duration. On Assessment, he had polyarthritis supporting the diagnosis of a disease flare-up. Additionally, there was mild tenderness in the dorsal aspect of the left foot. Fracture was excluded by X-ray followed by CT scan. MRI was done and showed diffuse midfoot synovitis, mild peroneus longus tenosynovitis and bone marrow oedema in the second metatarsal bone. Two months later after controlling the disease, the MRI showed improvement. Discussion: Bone marrow oedema (BME) is one of the main imaging characteristics of juvenile idiopathic arthritis (JIA) in children and rheumatoid arthritis (RA) in adults. Bone oedema occurs in various forms of inflammatory and noninflammatory arthritis and it is associated with erosive progression and poor functional outcome. A retrospective study demonstrated that the prevalence of knee BME in JIA was 27.2%. Older children and children with long disease duration had a higher risk for BME, which was commonly a late presentation and more likely involved the weight-bearing surfaces of the joint. The overall prognosis was satisfactory after the standard treatments. Conclusion: BME frequently presents as a late finding, and it is associated with erosive progression and poor functional outcome. Further validation with quantitative MRI techniques is needed for the evaluation of inflammatory and destructive changes in sJIA.

  Does Janus Kinase Inhibitor Increase the Risk of Herpes Zoster in Patients with Rheumatoid Arthritis in Saudi Arabia? A Multicenter Retrospective Cohort Study Top

Ola Abudaowd1, Fida Ahmed2, Reham Kaki2, Jamal Attieh3, Suzan Attar4, Hani Almoallim5

1Department of Medicine, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia, 2Department of Medicine, Department of Infectious Disease and Department of Infection Control and Environmental Health, King Abdulaziz University Hospital, King Abdulaziz University, Jeddah, Saudi Arabia, 3Department of Medicine, Division of Rheumatology, Saudi German Hospital, Asser, Saudi Arabia, 4Division of Rheumatology, King Abdulaziz University Hospital, King Abdulaziz University, Jeddah, Saudi Arabia, 5Division of Rheumatology, Umm Alqura University, Mecca, Saudi Arabia

Background: Currently, there is an advancing interest in the Janus kinase (JAK) intracellular pathway since targeted inhibitors are proving to be excellent in the treatment of Rheumatoid arthritis (RA). Nevertheless, this new therapeutic era of small molecules comes at a cost, JAKi is associated with a heightened risk of reactivation of dormant infections herpes zoster (HZ) virus infection. Furthermore, the incidence of HZ in rheumatoid arthritis using JAKi is higher in eastern Asian countries making this study essential for the assessment of its safety among the middle eastern population in a real-world setting. Methods: Multicenter retrospective cohort study in Saudi Arabia. Rheumatoid arthritis patients aged 18 and above from the period of 2007-2022 were included. Medical records were reviewed, and patients were phone interviewed during 2022 to register an outcome of HZ infection. Chi-square was used to assess for the correlation between various risk factors and HZ infection. Results: From a total of 308 patients, 108 were on JAKi. JAKi didn't significantly increase the risk of HZ development (OR1.97, 95% CI 0.71-4.67). Rather, patients on Etanercept had higher risk of HZ (OR 2.88, 95% CI 1.65-2.83). 3 out of the 7 cases were on chronic corticosteroid. However, it wasn't significantly associated with HZ devolvement. Patients with Asian ethnicity were significantly more predisposed to HZ infection (OR 1.2, 95% CI 0.8-4.37). None of the patients received the vaccine prior to HZ infection. Conclusion: In this multicenter study in Saudi Arabia, JAKi were not associated with the development of HZ among Rheumatoid Arthritis patients in real world setting. Asian ethnicity and etanercept usage were significantly associated with HZ infection.

  Adverse Impact of Systemic Lupus Erythematosus on Pregnancy Outcomes: A Saudi Arabia Prospective Multi-Center Study Top

Hanan Al Rayes1, Norah Aloudah1, Roaa Alsolaimani2,3, Abdulrahman Alharthi4, Alya Alkaff5, Abdulrahman Albeity2, Afnan M. Afifi2, Abdulelah AlQahtani1, Mohammed Attar4, Hassan Daghasi4, Basant Elnady4,6

1Department of Rheumatology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia, 2Department of Medicine, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia, 3Department of Medicine, Faculty of Medicine, King Abdulaziz University, Saudi Arabia, 4Department of Rheumatology, Al Hada Armed Forces Hospital, Taif, Saudi Arabia, 5Department of Obstetrics and Gynecology, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia, 6Department of Rheumatology, Rehabilitation and Physical Medicine, Faculty of Medicine, Benha University, Benha, Egypt

Background: Systemic Lupus Erythematosus (SLE) typically affects females of reproductive age. Pregnancy is a popular concern for individuals with SLE since these women have a normal reproductive rate. SLE and pregnancy have mutually harmful effects. In addition to having short- or long-term negative effects on renal function, pregnancy may also enhance the activity of SLE. SLE can cause difficulties during pregnancy, such as preeclampsia, premature labor, and fetal death, and it can also enhance the activity of the condition. Pregnancy may also have short- or long-term negative effects on renal function. Objective: The current study aimed to determine the pregnancy outcomes and post partum complications in patients with SLE and its association with disease activity in the Saudi population. Methods: This prospective multicenter study included pregnant female patients with SLE from three tertiary centers in Saudi Arabia. The demographics, SLE disease activity index, medication before, during, and after pregnancy, pregnancy-related outcomes, and complications in comparison to age-matched healthy female controls were noted. Results: A total of 66 pregnant patients with SLE and 93 healthy age-matched pregnant controls were included in the study. A total of 86.36% were having SLEDAI-2K ≤ 4 before conception, and 84.85% of pregnancies were planned. Age of conception, cesarean section, miscarriage, and low birth weight were statistically significant (p < 0.05) and higher in SLE patients than in healthy controls. A mongall clinical and laboratory variables positive anti-DNA was statistically associated with adverse pregnancy outcomes (p < 0.05). Conclusion: SLE has a negative impact on pregnancy-related outcomes. A higher serology level of anti-DNA is considered a major risk. Planned pregnancy follow-up is associated with better pregnancy outcomes. The current study supports how pre-gestational counseling and a multidisciplinary approach may help SLE patients have healthy pregnancies. Multidisciplinary management is essential due to the high rate of illness relapse.

  Tocilizumab in Treating COVID 19 Cytokine Release Storm: Single Center Experience Top

Sultana Abdulaziz1, Amin Yousef2, Ahmed Rajab2, Rana Alhilmi3, Rawan Alghamdi3, Wael Bajhamoun4, Abeer K. Alhindi5,6

1Department of Medicine, Division of Rheumatology, King Fahad Hospital, Jeddah, Saudi Arabia, 2Department of Intensive Care, King Fahad Hospital, Jeddah, Saudi Arabia, 3King Fahad Hospital, Jeddah, Saudi Arabia, 4Department of Medicine, Division of Infectious Diseases, King Fahad Hospital, Jeddah, Saudi Arabia, 5College of Medicine, King Saud Bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia, 6King Abdullah International Medical Research Center, Jeddah, Saudi Arabia

Introduction: Severe or critical COVID 19 pneumonias can be complicated by cytokine release syndrome (CRS) causing high rate of mortality. Tocilizumab has been indicated in CRS associated with cancer. chemotherapy and some autoimmune disease. Objectives: The aim of our study is to evaluate the effectiveness of Tocilizumab in COVID 19 related CRS. Methods: A retrospective case control study conducted in King Fahad hospital in severe and critical COVID 19 pneumonia patients with CRS who met the criterion from June to August 2021. Tocilizumab 8mg/kg IV was given to the treatment group initially as recommended by the MOH protocol. Mortality, morbidity, survival rate, clinical response, biomarkers, and safety outcomes were assessed. Results: A total of 68 patients were included, from which 33 had received tocilizumab plus standard of care (TOC) and 35 received standard of care (SOC). Mean age and gender distribution was balanced. between the two groups. The mean temperature and non-invasive respiratory support were significantly. lower in severe TOC group compared to SOC p = 0.003 and P = 0.001, respectively. The mean CRP and invasive respiratory support were significantly lower in severe SOC group compared to TOC P = 0.003 and p = 0.006, respectively. Death was significantly higher p = 0.045 in severe TOC patients compared to SOC group. Critical patients TOC group showed longer survival curve. Conclusion: In this retrospective study involving severe to critical Covid 19 related pneumonia with CRS, the use of tocilizumab did not significantly decrease the mortality rate in severe and critical patients. However, the mean temperature and non-invasive respiratory support were significantly lower in severe. patients treated with tocilizumab. The mean CRP and invasive respiratory support were significantly lower. in severe patients treated with standard of care only. The survival rate was longer in critical patients.

  Atypical HUS Mimicking Systemic Lupus Erythematosus Top

Omar Aljohani1, Banan Alrewaithi2,3, Asmaa Jokhdar2,3, Faten Almutairi, Reda Alarabi, Ahmed Alhujaili, Mohammed Olfat2,3

1Department of Pediatric Rheumatology, King Salman Medical City, Medina, 2Rheumatology Section, 3Department of Pediatrics, King Salman Medical City, Ministry of Health, Medina, Saudi Arabia

Lupus nephritis is one of most common causes of SLE morbidity and mortality, and its non-uncommon early manifestations of lupus, can lead to chronic and irreversible tissue damage, renal involvement in lupus patients' variables from minimal mesangial nephritis to end stage kidney disease (ESKD). Atypical hemolytic uremic syndrome belongs under umbrella of the thrombolytic microangiopathy (TMAs), C3 glomerulopathy, which can be triggered by SLE. - A 10-year-old Indonesian girl, previously healthy Presented with a 2-month history of pallor, headache and auditory hallucinations, few days prior to admission presented with convulsions, vomiting and diarrhea, with no fever. In addition, she reports history of intermittent chest pain most prominent at time of inspiration. On examination she was noticed to has a pallor, small and large joints arthritis, Pericardial rub and pleural rub with ejection. systolic murmur, Mild abdominal distention. Admission complete blood count showed normocytic normochromic anemia, leukocytosis, and normal platelet count and high. ESR 111 CRP 210 with, blood urea nitrogen BUN and creatinine both elevated With Proteinuria and hematuria, Initially ANA positive with low C3 and normal C4 Renal biopsy postponed due to high risk of bleeding, Echocardiogram showed Left ventricular hypertrophy and coronary. Fistula Impression SLE VS Atypical HUS Given pulse steroid therapy and cyclophosphamide. Based on nephritis, arthritis, thrombocytopenia, CNS manifestations and ANA positivity CRRT inserted for 4 days, and subsequently renal profile improved after stopped BUN and creatinine raised again. Result of anti-smith and anti-dsDNA negative Renal biopsy done and showed: Membranoproliferative glomerulonephritis with dominant C3 staining suggestive of C3 glomerulopathy. Here we will present a unique case of newly diagnosed lupus nephritis, presented initially with atypical hemolytic uremic. Syndrome (C3 glomerulopathy) associated with other manifestations of SLE. Renal biopsy, complements levels, and ADAMTS-13 are very helpful for diagnosis of patient with C3 glomerulopathy and to be consider in case of rapidly deteriorating lupus nephritis and in case of cyclophosphamide resistance.


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