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Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 27-33

Posters for the 8th Annual Saudi Society for rheumatology conference

Date of Submission07-Mar-2022
Date of Decision15-Mar-2022
Date of Acceptance08-Mar-2022
Date of Web Publication25-May-2022

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

DOI: 10.4103/ara.ara_6_22

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How to cite this article:
. Posters for the 8th Annual Saudi Society for rheumatology conference. Ann Rheumatol Autoimmun 2022;2:27-33

How to cite this URL:
. Posters for the 8th Annual Saudi Society for rheumatology conference. Ann Rheumatol Autoimmun [serial online] 2022 [cited 2022 Dec 10];2:27-33. Available from: http://www.ara.ssr.com/text.asp?2022/2/1/27/345953

  Anxiety, Depression, Disease Disability, and Health-Related Quality of Life in Rheumatoid Arthritis Patients during the COVID-19 Pandemic Top

Shiekha S. AlAujan, Haya M. Almalag, Shahad M. AlShehri, Jumanah M. AlKendi, Mohamad A. AlMansour, Layla N. Alanizy, Mohammed A. Omair

Background: The coronavirus disease 2019 (COVID-19) pandemic has posed a threat to mental health. This study aimed to examine the impact of the pandemic on psychological health and to identify the factors associated with anxiety in patients with rheumatoid arthritis (RA) in Saudi Arabia. Patients and Methods: A cross-sectional study was conducted between September and November 2020 in two tertiary care hospitals. Eligible participants were adults with a confirmed diagnosis of RA. Data collected included sociodemographics, contact history, commitment to COVID-19 precautionary measures, medication-taking behavior, the Hospital Anxiety and Depression Scale (HADS), the European Quality of Life measure (EQ-5D-3L), and the Health Assessment Questionnaire Disability Index (HAQ-DI). Results: Of the 252 invited eligible patients, 204 agreed to participate. The majority were aged 41–50 years (28.2%), were female (86.5%), had missed medications (8.8%), and had psychiatric illnesses or were on psychological medication for the past 3 months (15.4%). Borderline-abnormal anxiety was reported in 32.8% of patients. The mean (standard deviation [SD]) score for HADS depression was 3 (3), for the EQ-5D-3L index was 0.587 (0.378), and for the HAQ-DI was 0.947 (0.887). After adjusting for confounders, higher levels of anxiety were significantly associated with a higher level of depression and RA disability index (P < 0.05), as well as lower levels of health-related quality of life (P < 0.001). Conclusion: Almost one-third of RA patients reported the presence of COVID-19 induced anxiety. Higher anxiety levels were found to be associated with depression, health-related quality of life, and disease disability in RA patients.

Anxiety, COVID-19, depression, disease activity, quality of life, rheumatoid arthritis

  Atypical Childhood Kawasaki Disease with Giant Coronary Aneurysm Presenting with Features Mimicking Other Systemic Vasculitides Top

Mohammed Olfat1, Asmaa Jokhdar1, Banan Al-Ruwaithy1, Saeed Alghamdi2

Departments of 1Pediatric Rheumatology and 2Pediatric Cardiology, Maternity and Children Hospital, King Salman Medical City, MOH, Medina, Saudi Arabia

Background: Kawasaki disease (KD) is a common self-limited vasculitis that is characterized by high-grade fever, conjunctivitis, dry fissured lips, strawberry tongue, unilateral cervical lymphadenopathy, skin rash, and hand and feet changes. Childhood polyarteritis nodosa (PAN) is a rare and often fatal medium- and small-vessel vasculitis that may be indistinguishable from KD, especially when coronary arteries are involved. Childhood Henoch Schonlein pupura (cHSP) is the most frequent small-vessel vasculitis in childhood with distinct clinical features of palpable purpura, arthritis, abdominal pain, and/or renal involvement (hematuria and/or proteinuria). To our knowledge, only few cases of KD presenting initially with features mimicking cHSP or PAN have been reported; some of them were cases of atypical KD with coronary aneurysm presenting with gastrointestinal manifestations and/or purpuric rash, and others were rare cases of cHSP or PAN with coronary aneurysm. Case Report: A 10-year-old boy presented to our hospital with 8-day history of persistent high-grade fever associated with severe colicky abdominal pain, purpuric and hemorrhagic rash over upper and lower extremities, palpable vasculitic lesions at palms and sole, generalized erythematous body rash with target lesions, edema of feet, and ankle joint pain. His systemic review was unremarkable. Physical Examination: He looked very ill, in pain, tachycardic, dehydrated, temperature 40°C, erythema multiforme, purpuric and hemorrhagic rash over his trunk and extremities vasculitic lesions over his palm and sole, edema of the dorsum of the feet, and bilateral ankle arthritis. Abdominal examination revealed generalized tenderness with guarding. Two days later, he had mild nonpurulent conjunctivitis and dusky lips. Other systemic examination was unremarkable. Laboratory Finding: White blood counts (WBC) 17.3/mm3 (4.5–10/mm3), hemoglobin (Hb) 10.8 g/dL (11–14 g/dl), platelet count 880,000/mm3 (150,000–450,000), AST 95 U/L (10–40 U/L), ALT 88 U/L (17–55 U/L), sodium (Na) 129.8 mmol/L (139–146), albumin 24 g/L (38–54), CRP 135, ESR 125, complements C3 25 mg/dL (90–180), and C4 5.8 mg/dL (10–40). Electrocardiogram (ECG) showed sinus tachycardia, and his initial echo was normal, but 2 days later, repeated echo revealed a giant aneurysm of the circumflex artery measuring about 8 mm × 9 mm, ectasia of right coronary artery and left middle coronary artery measuring about 3 mm, and normal left ventricular systolic function. His renal function, urine examination, and infectious workup including hepatitis viruses panel were normal. His chest X-ray, renal Doppler and abdominal ultrasound, and computed tomography were normal. Diagnosis: initially, he was diagnosed with suspected vasculitis (cHSP versus PAN) and treated empirically with a single dose of intravenous immunoglobulin 2 g/kg. He remained to be febrile and tachycardic. After the finding of the giant coronary aneurysm, he was treated as probable PAN with giant aneurysm and started on IVMP and methotrexate. He showed significant improvement and was discharged home on oral prednisone, methotrexate, warfarin, and aspirin 81 mg once daily. He was given outpatient patient follow-up with repeat echo. Management and Follow-up: He was referred to pediatric cardiology for further evaluation and management including catheterization. One-week following discharge, he had peeling of his feet. Physical Examination: He looked well, was afebrile, had peeling of his feet, and resolving purpuric lesions after discharge. Other systemic examination was unremarkable. Laboratory findings 4 weeks after discharge revealed Hb 12.4, platelet 322, WBC 5000, ESR 15, CRP 3, BUN 13.5, creatinine 81, Na 134, albumin 34, AST 15, and ALT 24. Echo showed the same size of giant coronary aneurysm. ECG was normal. Final diagnosis was atypical KD with a giant coronary aneurysm. Discharge medications given were tapering prednisolone dose 40 mg daily, methotrexate 15 mg/week, warfarin and low-dose aspirin, and Vitamin D3 1000 IU daily. During follow-up, he was doing well and went into remission on medication. Discussion: KD is an acute self-limited medium vessels vasculitis with distinct clinical features. Diagnosis of KD according to the European League Against Rheumatism/Paediatric Rheumatology European Society Consensus criteria. The clinical presentation of KD varies over time, and the course of the disease may differ and patients may present with atypical features that may mimic or coexist with other systemic vasculitides such as cHSP or PAN. There are few case reports of atypical KD who presented initially with gastrointestinal symptoms such as abdominal pain and purpuric rash. These patients were at higher risk of developing giant coronary aneurysm. Sarah reported a rate of 43% of coronary aneurysms at the time of diagnosis in KD patients presenting initially with abdominal pain. Ravinder et al. reported giant aneurysm in a 4.5-year-old boy who presented with fever, abdominal pain, and a diffuse pink papular rash on the palms and soles. To our knowledge, there are no reports of peeling of skin in association with cHSP or PAN. Our patient was similar to those cases reported in the literature. He presented initially with persistent fever, abdominal pain, pupuric rash, a rash on the palms and soles, and giant aneurysm. Supported by the appearance of peeling, atypical KD diagnosis was made 2 weeks later. Conclusions: Knowing the wide heterogeneity of clinical manifestation of childhood vasculitis, KD may initially present with features of other systemic vasculitides such as abdominal pain and purpuric rash. Since such a group of children is at increased risk of coronary aneurysm, therefore, patients with suspected systemic vasculitis presenting with persistent fever, abdominal pain, and features mimicking cHSP or PAN must be evaluated for the development of coronary artery abnormalities.

  Behcet's Disease Associated with Advanced Osteonecrosis of Both Hips: A Case Report from Saudi Arabia Top

Nezar A. Bakhsh, Khalaf S. Alghamdi

Case Presentation: A 39-year-old Saudi male from Riyadh diagnosed with Behcet's disease (BD) for the past 7 years based on clinical manifestation and HLA-B51, following by his rheumatologist who started him on colchicine with good response for musculoskeletal manifestation. In 2019, he presented to the emergency department with bleeding per rectum. Computed tomography of the abdomen done showed bowel perforation. Intestinal resection was done for him. Blood culture was positive for ESBL which led us to start meropenem 1 g IV q8 h with methylprednisone 1 mg/kg/day. He responded very well. On discharge, imuran 100 mg po od was initiated. During follow-up in the clinic, he had gastrointestinal manifestation associated with elevated inflammatory markers, which led us to substituted imuran to infliximab 5 mg/kg. In 2020, after several doses of infliximab, he started to developed hypotension with skin allergy during infusion time, which led us to discontinue infliximab and initiated adalimumab. Nine months later, he reported a history of intermittent hip and back pain, progressive associated with inability to walk, and gait disturbance. Clinical examination revealed no muscle weakness, but there was a limitation in range of motion. Magnetic resonance imaging of the pelvis showed osteonecrosis stage IIA on the right hip and stage IIB on the left hip. Orthopedic team has been involved for possible intervention, but no surgical intervention is needed currently, and they will follow him accordingly. As avascular necrosis of the weight-bearing joints is an unusual manifestation of BD and very rarely has been observed according to the previous literature, we report this case as isolated hip osteonecrosis in a confirmed case of BD. Avascular necrosis could be related to either side effects of corticosteroids or the progression of the disease itself.

  Bone Mineral Density by Dual-Energy X-Ray Absorptiometry Scan of Different Stages of Chronic Kidney Disease Top

Hilal Alkalbani, Issa Alsalmi

Introduction: The aim of the study is to evaluate bone mineral density (BMD) determined by dual-energy X-ray absorptiometry (DEXA) and bone turnover markers in chronic kidney disease (CKD) patients. Methods: This was an observational-clinical study of all patients scanned by DEXA scan in 2018. All patients with low bone density or osteoporosis based on World Health Organization (WHO) definition were included. Results: 505 patients with abnormal BMD, 87.3% were in early-stage CKD stage I–II, 8.5% were in CKD stage III–V, and 4.2% did not have renal tests. 95 (18.8%) were male with a mean age of 57.0 years, and 410 (81.2%) were females with a mean age of 55.8 years. Patients of ≥65 years had lower T-score than those younger than 65 years. Among CKD patients, those with late CKD (stage III–V) had less BMD measurements and lower T-score than those with early CKD (stage I–II). A significant positive correlation exists between parathyroid hormone (PTH) level and the lower T-score. Female had a worse T-score at the lumbar region, whereas male had a worse T-score at the femoral region. There was no significant difference between males and females for the T-score at hip region. Conclusion: We observed distribution of abnormal BMD among different age, sex, and CKD groups. Measurements of BMD by DEXA might be a useful test to diagnose osteoporosis in CKD patients. Femoral and total hip areas were more affected; however, DEXA might not be able to detect osteoporosis in the lumbar area of CKD patients. T-scores are lower in patients with more severe CKD and lower in elderly patients. PTH level is associated proportionally with the degree of bone loss. Early intervention and proper management must be implemented early among CKD patients with a multidisciplinary team approach strategy. More studies are needed to determine if DEXA techniques are enough to distinguish the quantity of bone loss between different stages of CKD.

  Coexistence of Vogt-Koyanagi-Harada and Rheumatic Disease: A Case Report Top

Asma Bedaiwi, Mohammed Omair

Introduction: Vogt-Koyanagi-Harada (VKH) disease is a rare disease characterized by bilateral granulomatous panuveitis associated with central nervous system, auditory manifestation, and cutaneous manifestation. It is seen more commonly in genetically predisposed patients and noted to be more common in populations with greater skin pigmentation. The exact etiology and pathogenesis of VKH are unknown; however, current clinical and experimental evidence suggests a cell-mediated autoimmune process driven by T-lymphocytes directed against self-antigens associated with melanocytes of all systems in genetically susceptible individuals. Several autoimmune diseases have been associated with VKH as they share the same pathway or similar genetic mutations, such as rheumatoid arthritis, autoimmune polyglandular syndrome, and autoimmune thyroid disease.[1],[2] Case Presentation: A 60-year-old woman, diagnosed with VKH in 2001 based on acute-onset transient vision loss, complicated by scleromalacia, and secondary uveitic glaucoma, received 7 doses infliximab. In 2014, she complained of 1-year history of multiple bilateral joints pain, swelling, and early morning stiffness that lasted for 15–30 min; laboratories showed elevated inflammatory markers, positive rheumatic factor, and anti-CCP. She was diagnosed with seropositive rheumatic arthritis (RA), followed by family medicine initially, and started on sulfasalazine, referred to the rheumatology department in 2017 as her disease was uncontrolled and started on MTX and adalimumab, then switched to etanercept along with MTX due to failure of adalimumab. Discussion: This case illustrates the association of VKH and RA. Careful follow-up, systemic evaluation, and early referral guarantee early diagnosis, effective management, and complication avoidance.

  Down Syndrome Associated with Myositis and Hypothyrodism Top

Nezar Bakhsh, Lubna Alhosaini

Case Description: A 17-year-old male patient with a case of Down syndrome (DS), mental retardation, and hypothyroidism has presented to the Rheumatology Clinic with an inability to walk, muscle weakness, and difficulty standing that is started for the last 1 month. He reported a history of diffuse joints pain and swollen that prevented him from standing and did his usual activity associated with morning stiffness for more than 1 h. Seen in the endocrine clinic, he started on Vitamin D and calcium supplements without improvement. He did not report any history of shortness of breath, chest pain, oral ulcer, gentle ulcer, or skin rash. Other systemic review was unremarkable. Physical examination disclosed progressive deterioration of walking, proximal muscle weakness, the necessity of full-time use of a wheelchair, and four tender and swollen joints. Laboratories revealed normal complete blood count, electrolyte, Vitamin d, calcium, CRP 6, ESR 121 mm/h, ANA 1:160, Ds-DNA –ve, and CK 1200. Imuran was started with prednisolone 40 mg daily, and plaquenil 400 mg daily was started. Initially, he responded good with decrease dose of steroid, he flare up again, which led us to substitute imuran with methotrexate 15 mg weekly with folic acid. Magnetic resonance imaging of the thigh without contrast showed mild atrophic changes in the posterior compartment muscles and a significant increase of signals in the right gluteal as well as in the vastus intermedius in the left midthigh. The overall appearances suggested a case of myositis with active inflammatory changes in the right gluteal and left vastus intermedius muscles. Our case highlighted the connection between genetic DS disease and diseases such as myositis and thyroid and increased awareness for this severe disease.

  Effects of Ixekizumab Treatment on Structural Changes in the Sacroiliac Joints Based on Magnetic Resonance Imaging Assessments at 16 Weeks in Patients with Nonradiographic Axial Spondyloarthritis Top

Walter P. Maksymowych1, Xenofon Baraliakos2,3, Robert Lambert4,5, Robert Landewé6, David Sandoval7, Hilde Carlier7, Jeffrey Lisse7, Xiaoqi Li7, Maja Hojnik7, Mikkel Østergaard8, Alper Erdogan9

1University of Alberta, Edmonton, Canada, 2Ruhr-University, Bochum, Germany, 3Rheumazentrum Ruhrgebiet, Herne, Germany, 4University of Alberta, Edmonton, Canada, 5Medical Imaging Consultants, Edmonton, Canada, 6University of Amsterdam, Amsterdam, Netherlands, 7Eli Lilly and Company, Indianapolis, USA, 8Copenhagen, Center for Arthritis Research, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark, 9Eli Lilly and Company, Riyadh, Saudi Arabia

Objective: To evaluate the effect of treatment with ixekizumab versus placebo (PBO) on MRI lesions in the sacroiliac joints (SIJs) at 16 weeks in patients with non-radiographic axial spondyloarthritis (nr-axSpA). Methods: Patients with active nr-axSpA who were biologic-naïve (COAST-X, NCT02757352) were randomized 1:1:1 to receive double-blinded ixekizumab 80 mg every 2 (Q2W) or 4 weeks (Q4W), with an 80-mg or 160-mg starting dose at week 0, or PBO. SIJ magnetic resonance imaging (MRI) was assessed by the Spondyloarthritis Research Consortium of Canada (SPARCC) SIJ structural score (SSS). Treatment comparisons were conducted using analyses of covariance based on observed cases. Results: Of 303 randomized patients, 266 patients (Q2W: n = 91, Q4W: n = 85, PBO: n = 90) with an MRI scan available at baseline and week 16 were included in this analysis. Mean SPARCC SSS for PBO and ixekizumab dose groups were similar at baseline. Significant reduction in SPARCC SSS for erosion was observed in both ixekizumab dose groups versus PBO. Worsening of erosion scores occurred in fewer patients in both ixekizumab dose groups versus PBO. Furthermore, improvement in erosion scores was observed for more patients in both ixekizumab dose groups versus PBO. Mean change from baseline in SPARCC SSS was significantly greater for ixekizumab for fat metaplasia (Q4W) and backfill (Q2W and Q4W) versus PBO. Changes in ankylosis were generally not observed. Conclusions: Treatment with ixekizumab versus PBO led to significant reductions in erosions and significant increases in fat metaplasia and backfill in the SIJ in patients with nr-axSpA at 16 weeks of treatment.

  Optic Neuritis following the BNT162b2 mRNA COVID-19 Vaccine in a Patient with Systemic Lupus Erythematosus Uncovering the Diagnosis of Neuromyelitis Optica Spectrum Disorders Top

Bader H. Shirah1, Yasser S. Aladdin2,3,4

1Department of Neuroscience, King Faisal Specialist Hospital and Research Centre, 2Department of Medicine, King Abdulaziz Medical City, 3King Abdullah International Medical Research Center, 4College of Medicine, King Saud bin Abdulaziz University for Health Sciences, Jeddah, Saudi Arabia

Introduction: The COVID-19 pandemic has created a worldwide disruption of the healthcare system. Due to the rapid spread, vaccine development was pursued at an unprecedented speed, and approvals were expedited to encounter this infection. Several complications following the different types of COVID-19 vaccinations were reported previously. Case Report: We describe a rare case of optic neuritis following the BNT162b2 mRNA COVID-19 vaccine in a patient with systemic lupus erythematosus (SLE), uncovering the diagnosis of neuromyelitis optica spectrum disorders (NMOSDs). Conclusion: The most likely explanation is that the patient suffered visual symptoms related to the central systemic illness triggered by the vaccine. We attribute the occurrence of optic neuritis to the BNT162b2 mRNA COVID-19 vaccine due to temporal relationship. The risk of administering the vaccine in a patient with overactive immune system such as this patient may outweigh the benefits of vaccination against COVID-19. Screening for aquaporin-4 antibodies in patients with SLE before vaccination against COVID-19 may be considered to prevent the potentially devastating long-term neurological disability that may be associated with NMOSD disease outbreak.

Keywords: COVID-19, neuromyelitis optica spectrum disorders, Saudi Arabia, systemic lupus erythematosus

  Progressive Multifocal Leukoencephalopathy Misdiagnosed as Neuropsychiatric Systemic Lupus Erythematosus with a Catastrophic Outcome Top

Bader H. Shirah1, Hussein A. Algahtani2,3

1Department of Neuroscience, King Faisal Specialist Hospital and Research Centre, 2Department of Medicine, King Abdulaziz Medical City, 3King Abdullah International Medical Research Center, Jeddah, Saudi Arabia

Introduction: Progressive multifocal leukoencephalopathy (PML) is a rare infection of the central nervous system due to the reactivation of the John Cunningham polyomavirus. It is commonly a progressive fatal disease with worldwide distribution. Case Report: We describe a rare case of PML, misdiagnosed as neuropsychiatric systemic lupus erythematosus (SLE) with a catastrophic outcome due to delay in diagnosis with superadded cyclophosphamide therapy. Conclusion: There are several lessons taught from our case. First, in patients with autoimmune disorders who are strongly immunosuppressed, the new onset of cognitive impairment and seizures should alert the treating physician to look carefully for PML. Second, in cases of SLE where the diagnosis of the cause of cognitive impairment and seizures is not clear, we suggest that immunosuppression should not be intensified until PML has been ruled out. At last, multidisciplinary care in patients with suspected neuropsychiatric SLE, including a neurologist, an infectious diseases consultant, a neuroradiologist, and a rheumatologist, is needed.

Keywords: Cyclophosphamide, progressive multifocal leukoencephalopathy, Saudi Arabia, systemic lupus erythematosus

  Rate of Clinical and Functional Remission in Rheumatoid Arthritis Patients on Biologics under Treat to Target Strategy in a Single-Center Real-Life Data Top

Sultana Abdulaziz, Fatmah Alanazi, Ahlam Zabbani

Introduction: Current rheumatoid arthritis (RA) treatment aims in obtaining treat to target strategy (remission or low-disease activity), measured by patient outcome measures by DAS28, CDAI, SDAI, and functional status measured by health assessment-disability index (HAQ-DI). Objectives: To know remission rates by DAS28, CDAI, SDAI, and HAQ score in patients with RA treated with biologics or JAK inhibitors as monotherapy or in combination with MTX, as well as factors associated with obtaining remission. Methods: This is a cross-sectional retrospective study of patients with RA in a single center, King Fahad Hospital, in Jeddah, from October 2021 to December 2021. Adult patients ≥18 years who fulfilled ACR 1987 criteria or 2010 ACR/EULAR classification criteria of RA are included. Patients' demographic, clinical, and functional disease outcomes were analyzed. Multivariate logistic regression was used to analyze related factors for remission. Results: Of 200 patients analyzed, 138 patients fulfilled the criteria with a mean age of 53 ± 13 years, and women represented 89%, and mean RA duration of 10 years ± 4. Remission rate was 54.3% in our study. 69% of patients using anti-TNF reach remission, followed by rituximab group with 64.3%, JAK inhibitors with 50%, abatacept with 37%, and tocilizumab with 33.3% (P < 0.01). 47.8% of patients reached functional remission (HAQ-DAI <0.5) (P < 0.06). 34 patients (58%) were from anti-TNF group, 12 patients (34%) from abatacept group. Number of tender and swollen joints, patient global assessment of pain, and number of previous biologics were factors associated with remission. Conclusion: Remission rate was 54.3% in our study, which keeps up with previous studies in countries with high prescription rate for biologics. Functional remission (HAQ-DAI <0.5) 47.8% of patients reached reflecting good functional outcome.

Functional remission, patient-reported outcome measures, remission, rheumatoid arthritis, treat of target

  Real-World Effectiveness of Baricitinib in the Swiss Rheumatoid Arthritis Register (SCQM-RA) Top

Benoit Gilbert1, Delphine S. Courvoisier1, Denis Mongin1, Kim Lauper1,2, Clémentine Perrier3, Ruediger Mueller4, Axel Finckh1, Alper Erdogan5

1Division of Rheumatology, Geneva University Hospitals, Geneva, 3Eli Lilly (Suisse) SA, 16 Ch Des Coquelicots, Vernier, 4Division of Rheumatology, Medical University Department, Kantonsspital Aarau, 5001 Aarau, Switzerland, 2Centre for Epidemiology Versus Arthritis, Centre for Musculoskeletal Research, University of Manchester, UK, 5Eli Lilly and Company, Riyadh, Saudi Arabia

Introduction: Rheumatoid arthritis (RA) patients unresponsive to csDMARDs may receive bDMARDs/tsDMARDs such as baricitinib (BARI). BARI has demonstrated efficacy in randomized controlled trials, but limited studies have established comparative effectiveness in real-world settings, especially in b/tsDMARD-naïve patients. Objectives: To analyze BARI's effectiveness versus bDMARDs, assessed by drug maintenance over time and 12-month response rates in a Swiss Clinical Quality Management observational cohort. Methods: All BARI/bDMARDs treatment courses (TCs) initiated 2017-09-01-2020-06-01, with at least 1 follow-up visit, were included. BARI TCs were compared with alternative non-BARI bDMARDs, including all b/tsDMARDs except rituximab. The non-BARI group was subdivided into TNFi and other-mode-of-action bDMARDs (OMA), excluding tsDMARDs. Secondary analysis on b/tsDMARD-naïve patients was conducted. Baseline characteristics were compared using ANOVA/χ2 test. A Cox-model survival analysis assessed drug maintenance. 12-month response rates were estimated with an attrition-corrected, confounder-adjusted approach. CDAI ≤10 defined low-disease activity (LDA) state and ≤2.8 remission. Results: 1218 eligible TCs were initiated (273: BARI, 154: tsDMARD, 473: TNFi, 318: OMA). Drug maintenance was significantly shorter for TNFi than BARI, even after adjustment for HR for drug discontinuation 1.85 (95% confidence interval [CI] [1.40–2.43]; P < 0.001), and shorter for OMA than BARI, but the difference was not significant (HR 1.18 [95% CI (0.87–1.60)]; P = 0.28). These differences were larger when analyzing only bDMARD-naïve patients. The rates of LDA and remission did not differ significantly between the three groups at 12 months. LDA ranged from 63% to 67% (BARI vs. OMA P = 0.87; BARI vs. TNFi P = 0.81) and remission from 19% to 23% (BARI vs. OMA P = 0.30; BARI vs. TNFi P = 0.77). Conclusions: BARI demonstrated significantly higher overall drug maintenance than TNFi and similar drug maintenance to OMA, both in a bDMARD-naïve and in the overall population. The adjusted 12-month response rates did not differ between BARI, TNFi, and OMA, suggesting that prescription of BARI after csDMARD has at least similar outcomes as alternative bDMARDs.

  Refractory Raynaud Phenomena Associated with Systemic Sclerosis Top

Nezar A. Bakhsh, Khalaf S. Alghamdi

Introduction: Systemic sclerosis is an autoimmune disease affecting the skin and other organs of the body, meaning that the body's immune system is causing inflammation and other abnormalities in these tissues. Case: A 78-year-old Jordanian female known case of hypertension, heart failure with ejection fraction (EF) 20%, and ICD treated as heart failure with low EF. Her symptoms started before 20 years; she noticed her skin became tight over hand, feet, and mouth associated with finger color changed to blue discoloration with cold weather. Her primary physician started her on aspirin and Plavix without improvement. She reported decrease in mouth opening and amount of saliva. Further, she reported increase in her reflux with constipation. During this period, no immunosuppression medication was started nor computed tomography (CT) chest. On January 2021, she presented to emergency department with bluish discoloration of her finger and feet associated with active ulceration on tips of her fingers. She was admitted to our hospital as severe Raynaud phenomena and infected ulcer. Proper antibiotics were started associated with aspirin, Plavix, and pentoxifylline. All of these medications failed to improve her symptoms which led us to start prazosin. Proper screening was done for her including malignancy screening, auto-antibody, high-resolution CT chest and echocardiogram. Anti-scl 70 was positive. CT chest had no evidence of ILD. Repeated echocardiogram showed EF 45%.Cellcept 3 g daily was initiated which improve her skin tightness. Currently, her Raynaud phenomena improved and her digits protected from ischemia.

Conclusion: Negligence of systemic sclerosis feature from the beginning which leads to delay initiating proper treatment. Lack of experience in treating Raynaud phenomena in our area which may lead to serious adverse events. Consider prazosin one of the options in severe and refractory Raynaud phenomena.

  The Impact of Rheumatological Diseases on Sexual Function among Men in Saudi Arabia: A Cross-Sectional Study Top

Rahaf Alodaini, Mohammed Omair

Objective: To assess the impact of rheumatic diseases on sexual function among male patients. Methodology: A comparative cross-sectional study design was used to assess sexual dysfunction in 140 eligible candidates; exclusion criteria include age <18, not sexually active candidate, patients diagnosed or treated for any disease-causing sexual dysfunction, or infertility. An International Index of Erectile Function (IIEF-15) questionnaire was filled out by patients with rheumatological disease and healthy controls groups with a mean age of 46.5 ± 14.6 years and 40 ± 10 years, respectively. An Arabic version of this questionnaire was used as most of our targeted patients speak the Arabic language. Results: A total of 140 patients filled the IIEF-15 questionnaire; among the total, 47 (17.7%) diagnosed with rheumatological disease. Healthy participants (67.7%) found it not difficult to maintain erection to completion of intercourse for the past 4 weeks as compared in patients with rheumatological disease (34%), which was statistically significant with P < 0.001. Comparing means of IIEF-15 score among both groups, mean score was significantly higher for healthy participants than diseased participants with P < 0.05. Conclusion: Our results suggest that male patients with rheumatological disease experience an increased prevalence of sexual dysfunction compared to healthy individuals. Relevance to Clinical Practice: This study highlighted that sexual dysfunction is a significant feature among male patients with rheumatological diseases, which suggests that both patients and clinicians should be aware of the potential impact of rheumatological disease on sexual function. Besides, more specific measurement should be combined with the IIEF-15 questionnaire to assess sexual dysfunction in rheumatology patients.

  Case of Spondyloenchondrodysplasia with Monogenic Systemic Lupus Erythematosus Treated with Belimumab: Case Report with 2 Years of Follow-up Top

Deena AlWakeel1, Abdulrahman Asiri1, Abdulaziz AlRowais1, Munira AlMarri

1Department of Pediatric Rheumatology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia


Spondyloenchondrodysplasia (SPENCD) is a very rare skeletal dysplasia due to homozygous mutations in ACP5 gene that impairs endochondral bone growth and creates a type 1 interferon (INF) signature, which leads to distinctive spondylar and metaphyseal dysplasia, and extraskeletal manifestations, such as neurological involvement and immune dysregulation such as systemic lupus erythematosus (SLE). To date, there are only 17 reported cases of SPENCD associated with SLE in the literature. This report presents clinical and laboratory data of one patient, 4-year-old girl diagnosed with SPENCD associated SLE and sensory neural hearing loss (SNHL) who is treated with belimumab. Case Presentation: A 4-year-old Saudi girl, full-term, from first-degree consanguine parent. She had good health until the age of 6 months when she started to have recurrent fever with no specific cause. At the age of 1 year, she was investigated for motor and speech delayed and subtle dysmorphism when she found to have profound bilateral SNHL and lower limb spasticity and hepatosplenomegaly. She was worked up for a possibility of mucopolysaccharidosis. At the age of 1 year 8 months, she developed skin discoloration of purple to black color associated with pain and swelling of foot bilaterally. It was progressive till reached to upper limbs as well. Then, she developed autoimmune hemolytic anemia that was incidentally found. On physical examination, she appeared well and active but pale. Head circumference was on 50th percentile, weight was less than 3rd percentile, and height was on 3rd percentile. There were subtle dysmorphic features in frontal bossing and depressed nasal bridge. Livedo reticularis skin rash involving her lower and upper limbs as in [Figure 1]. Abdominal examination showed hepatosplenomegaly with multiple lymphadenopathies involving neck, axilla, and inguinal region, but other systemic examination was normal. Investigations: WBC: normal, HGB: 6.6 g/L, platelet: 127 × 109, Direct Coomb's test: positive, ANA: 1/2560, dsDNA: 1356 IU/mL, ESR: 137 mm/h, antiphospholipid (antiphosphatidylserine and anticardiolipin): positive. C-reactive protein was within normal limit, complement (CH50, C1Q, C3, and C4), liver and renal function, VLCFA, and CGH microarray were normal. Skeletal Survey: Mild reduced bone density with mild craniofacial disproportion. Reduced vertebral body heights, anterior wedging with coarse trabecular pattern. Metaphyseal cupping of the distal metaphysis of upper and lower extremities [Image 1]. Computed tomography of the brain: Bilateral symmetrical calcifications of the basal ganglia and corticosubcortical region of both cerebral hemispheres as in Image 2. WES: Detected homozygous pathogenic variant in ACP5 gene. Hence, that confirmed the diagnosis of SPENCD. The patient was initially treated with short course of prednisone and hydroxychloroquine with monthly IVIG. She showed some improvement in the form of less febrile attacks, but her skin manifestation was still not improved, so mycophenolate mofetil was added. The patient was kept on the same medications for almost 1 year, with frequent flare-up of her symptoms and frequent courses of steroid therapy. She had a history of painful chilblains on her left hand and tip of the ear [Figure 2]; for that, she was admitted and started on amlodipine, topical tacrolimus, topical mometasone, and methylprednisolone pulse therapy, but there was not much improvement. Then, sildenafil was added. After that, she improved significantly [Figure 3]. For her frequent flare-up, we started our patient on belimumab infusion on a monthly basis, and she showed dramatic improvement after the first dose, with no side effect. After stabilization of her illness, she underwent bilateral cochlear implant, and it was successful procedure, with no complication. Discussion: SPENCD is a rare disorder that was first described by Schorr et al. in 1976 in 2 Iraqi Jewish siblings productive from consanguineous parent. Menger et al. in 1989 were proposed that the disease is transmitted in an autosomal recessive pattern by reporting cases with different phenotypic features from one family with a variable expression. Approximately 80 cases of SPENCD were reported worldwide. There are only 17 reported cases of SPENCD associated with SLE in the literature. The patient with SPENCD has been shared different manifestations such as skeletal dysplasia, platyspondyly and short stature, and neurological manifestation. Moreover, autoimmune dysfunction and predisposition of autoimmune disease might be manifested as well. In addition, vasculopathy is sometimes associated with this disease. Deficiency of tartrate-resistant acid phosphatase activity, which resulted from biallelic mutation of ACP5 gene, plays a critical role in SPENCD. Osteopontine is the primary substrate of TRAP and losing its activity subsequently will increase the production of IFN-1, leading to SLE manifestation. Our patient presented with cutaneous, hematological, neurological, and immunological manifestation of SLE. In addition, she has SNHL, which is never been reported as association with SPENCD in the literature. Belimumab is a monoclonal antibody approved recently by US FDA to be used in SLE, and its efficacy has been reported in cutaneous lupus in the pediatric age group. Up to our knowledge, based on extensive literature review of similar cases, belimumab has never been used in treating patient having SPENCD with SLE. Conclusion: SPENCO is an extremely pleiotropic and rare genetic disorder with distinctive radiographic findings that are helpful for diagnosis. With regard to patients with SLE who have short stature and skeletal abnormalities, the possibility of SPENCO should be considered. Wide variety of manifestations that have been assessed by different experts and clinics without a multidisciplinary team approach will result in a delay in diagnosis. Belimumab is a promising treatment option for similar cases.


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