|Year : 2022 | Volume
| Issue : 1 | Page : 17-21
The 8th Annual Saudi Society for Rheumatology Conference: The Abstracts Young Investigator
|Date of Submission||07-Mar-2022|
|Date of Decision||15-Mar-2022|
|Date of Acceptance||08-Mar-2022|
|Date of Web Publication||25-May-2022|
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
. The 8th Annual Saudi Society for Rheumatology Conference: The Abstracts Young Investigator. Ann Rheumatol Autoimmun 2022;2:17-21
|How to cite this URL:|
. The 8th Annual Saudi Society for Rheumatology Conference: The Abstracts Young Investigator. Ann Rheumatol Autoimmun [serial online] 2022 [cited 2022 Dec 10];2:17-21. Available from: http://www.ara.ssr.com/text.asp?2022/2/1/17/345956
| An Exploratory Investigation of Genetic Polymorphism in CYP2D6 with Risk of Metabolic Syndrome in Systemic Lupus Erythematosus Patients|| |
Lena Hassen, Maha Daghestani
Background: Metabolic syndrome (MetS) is a multifactorial condition prevalent among systemic lupus erythematosus (SLE) patients. Polymorphic cytochrome P450 2D6 (CYP2D6) involved in metabolizing various exogenous and endogenous substances may play a role in inducing metabolic abnormalities related to MetS.
Aim: This study aimed to investigate the association of selected single-nucleotide polymorphisms (SNPs) found in CYP2D6 gene (rs1080985, rs28624811, rs1065852, rs28371725, and rs1135840) with the risk of MetS in lupus patients. Methods: This case–control study was conducted on adults, Saudi SLE patients registered at the National Lupus Prospective Cohort between March 2020 and March 2021. Patients with confirmed SLE and MetS were included in the study. Venous blood was collected for DNA extraction and genotyping using TaqMan technologies. For statistical analysis, genotype and allele frequencies were determined using Chi-square test, and their association with metabolic traits was evaluated using multivariate regression models. Results: A total of 107 subjects were included (57 cases and 50 controls). Overall, the most prevalent variant genotype was rs28624811 (23.4%), and the least prevalent variant genotype was rs28371725 (1.9%). The highest odds ratio was found for rs1135840 (odds ratio = 3.00; 95% confidence interval = 0.71, 12.74; P = 0.13) in lupus patients with MetS compared to those without MetS. However, this variant genotype was negatively associated with MetS measurements (P < 0.05), even after adjusting for age, sex, and respective medications. Meanwhile, the rs1065852 and rs28371725 variants were found of potential risk for hypertension and obesity (P < 0.05), respectively. Conclusion: Lupus patients carrying multiple CYP2D6 SNPs might be considered at a higher risk for the concurrence of MetS. Further studies are needed to investigate the potential yield of these alleles as surrogate markers for increased future risk of cardiovascular diseases.
Keywords: Cytochrome P450, genotyping, lupus, metabolic disorders, risk allele
| Assessment of the Effect of Rheumatological Diseases on the Voiding Function of Female Patients|| |
Rahaf Alodaini, Mohammed Omair
Purpose: The purpose of the study was to assess the impact of rheumatic diseases on voiding function in female patients. Methodology: The study design is a cross-sectional study. We included all female patients with rheumatic diseases following in King Saud University Medical City and King Abdulaziz University Hospital. Exclusion Criteria: Age < 18 years and patients diagnosed or treated for any voiding dysfunction were excluded from the study. Questionnaire that was distributed to the patient is International Prostate symptom Score (IPSS) to assess voiding function and its effect on the quality of life (QoL). Previously validated Arabic version was used as most of our patients speak the Arabic language. We excluded incomplete responses before analysis. Results: A total of 110 female patients filled out the IPSS questionnaire. Among the total, 90 (81.8%) were currently diagnosed with rheumatoid arthritis, overall median (interquartile range) IPSS score was 11 (5–18), 43.6% scored moderately symptomatic, whereas 19.1% scored severely symptomatic. Upon questioning about how often have you had to urinate again in <2 h after you finished urinating for the past 1 month, 18.2% answered almost always. When asked about how often have you found it difficult to postpone urination for the past 1 month, 16.4% answered almost always. Also, when asked if you have discussed this problem with your doctor, 44.5% answered “No.” Conclusion: According to our study, we found that lower urinary tract dysfunction can be a feature among female patients with rheumatological disease, with increased prevalence of frequency and urgency.
| Describing the Association between Rheumatoid Arthritis and Vogt–Koyanagi–Harada Disease. Case Report Followed by a Systematic Review|| |
Asma Bedaiwi1, Rahaf Alodaini1, Mohammed Omair1
1Department of Medicine, Rheumatology Unit, King Saud University Medical City, Riyadh, Saudi Arabia
Two patients are described who developed rheumatoid arthritis (RA) along with Vogt–Koyanagi–Harada (VKH) diseases with an excellent outcome using methotrexate and tumor necrosis factor inhibitors. A systematic review revealed six similar cases. In most cases, VKH preceded RA by years. Treatment outcomes and complications were variable. The current report describes an increasingly recognized association between RA and VKH. There is a need to have a multidisciplinary approach that includes rheumatologists and ophthalmologists in managing these cases.
| Effect of Ultrasound-guided versus Landmark-Guided Local Corticosteroid Injection for Carpal Tunnel Syndrome: A Systematic Review|| |
Abeer Aljindi, Mohammed Alomari, Jawaher Alsharef, Saleh Alzahrani, Mohammed Alshehri
Background: Carpal tunnel syndrome (CTS), the most common neuropathy of the upper limb, can be managed with different therapeutic approaches. Local corticosteroid injection has been adopted widely in clinical practice, as it showed great efficacy in treating CTS. However, the best injection technique continues to be a subject of controversy. Objectives: The aim of this systematic review and meta-analysis was to evaluate the efficacy of ultrasound-guided (US-guided) versus landmark-guided (LM-guided) corticosteroid injection on the clinical and electrophysiological outcomes in patients with CTS. Methods: We performed a systematic literature search in Medline, Embase, and CENTRAL, from which we included randomized controlled trials (RCTs) that compared US-guided and LM-based corticosteroid injection in treating individuals with CTS. We evaluated the following outcomes: Boston Carpal Tunnel Questionnaire-Functional Status Scale (BCTQ-FSS) and Symptom Severity Scale (BCTQ-SSS), and adverse event rate. The standardized mean difference (SMD) was used to represent continuous outcomes, while the risk ratio (RR) was used to represent dichotomous outcome. Results: A total of 8 RCTs that enrolled 500 wrists were deemed eligible. US-guided injection showed a significantly better BCTQ-FSS (SMD = −0.22, 95% confidence interval [CI] 0.39–0.04), BCTQ-SSS (SMD = −0.77, 95% CI:1.22–0.31), and adverse event rate (RR = 0.32, 95% CI: 0.21–0.49) compared to LM-based injection. Conclusion: This meta-analysis showed the superiority of US-guided corticosteroid injection over LM-guided corticosteroid injection in enhancing functional status, improving symptom severity, and reducing the adverse event rate in individuals with CTS.
Carpal tunnel syndrome; corticosteroid injection; in-plane ultrasound; out-plane ultrasound; conservative management
| Exploring Factors Influencing Medication Compliance in Saudi Rheumatoid Arthritis Patients: A Nationwide Cross-Sectional Survey: On Behalf of the COPRA Study|| |
Mohammed A. Omair1, Maha M. Alshehri2, Nouf A Altokhais2, Ghada A Aljanobi3, Maha I. El Dessougi4, Amal AlHarthi4, Maha A. Omair5, Sami M. Bahlas6, Abdullah S. Alfurayj7, Mansour S. Alazmi8, Alhussain M. Asiri9, Mohammed M. AlOmair9, Lobna Aljaffaly2, Haya M. Almalag2
1Department of Medicine, Rheumatology Unit, King Saud University Medical City, 2Department of Clinical Pharmacy, College of Pharmacy, King Saud University, 4Department of Medicine, Rheumatology Unit, Security Forces Hospital Riyadh, 3Department of Medicine, Rheumatology Unit, Qatif Central Hospital, Qatif, 5Department of Operation and Statistics, College of Science, King Saud University, 6Department of Medicine, Rheumatology Unit, King Abdulaziz University, Jeddah, 7Department of Medicine, Rheumatology Unit, Buraidah central hospital, Buraidah, 8Department of Medicine, Rheumatology Unit, Prince Mohammed Medical city, Sakaka-Aljouf, 9Department of Medicine, Rheumatology Unit, Aseer Central Hospital, Abha, Saudi Arabia
*Address for correspondence: Mohammed A. Omair, Associate professor, Department of Medicine, King Saud University Medical City, Riyadh, Saudi Arabia. Phone: +966505270513,
E-mail: [email protected]
Background: Compliance with medication is essential to achieve treatment goals in rheumatoid arthritis (RA) patients. The current study aims to evaluate compliance and related factors in a large, diverse population using a simple validated tool. Methods: RA patients on active treatment were invited through an online survey. The Arabic version of the 5-Item Compliance Questionnaire for Rheumatology (ACQR-5) was used to measure compliance. The RA impact of disease (RAID) was used to assess disability. Patients were subgrouped based on background disease-modifying antirheumatic drugs (DMARDs). Variables associated with high or low compliance were selected for logistic regression analysis. Results: A total of 1241 patients completed the survey and were included in the final analysis. Of those, 1055 (85%) were female with a mean (±SD) age of 47.14 ± 13.71 years. The mean disease duration was 8.77 ± 7.43 years. The mean RAID was 4.4 ± 2.58, with 980 (79%) having an unacceptable level state. Patients with an unacceptable level of disease impact had a lower compliance level (78.8% vs. 85.8%, P = 0.001). Demographics associated with high compliance were female gender and increased age with a reported odds ratio of 1.018 (95% CI: 1.007–1.028) and 1.464 (95% CI: 1.016–2.108), respectively. In addition, higher education with university degree odd ratio 0.595 (95% CI: 0.368–0.962) or postgraduate degree odds ratio of 0.669 (95% CI: 0.479–0.900) were inversely correlated to compliance. Subgroup analysis did not show a statistical difference between patients on Janus kinase inhibitors or biological DMARDs (88.14% vs. 80.83% P = 0.17) and between monotherapy, double, or triple therapy (80% vs. 82.23% vs. 81.32% P = 0.665). Conclusion; A high compliance level was observed in our population. Patients' demographics influenced compliance rather than background therapy. Interventional studies showed a focus on high-risk patients.
| Outcomes of COVID-19 in Inflammatory Rheumatological Diseases: A Retrospective Cohort Study|| |
Moustafa S Alhamadh1,2, Thamer Alhowaish1,2, Abdulrahman Alhabeeb1,2, Shaya Aldosari1,2, Emad Masoudi2,3, Abdulrahman Alrashid2,4
1College of Medicine-Riyadh, King Saud Bin Abdulaziz University for Health Sciences, 2King Abdullah International Medical Research Centre, Ministry of the National Guard Health Affairs, 3Department of Medical Education, College of Medicine at King Saud Bin Abdulaziz University for Health Sciences, 4Department of Medicine, Division of Rheumatology, Ministry of National Guard Health Affairs, Riyadh, Saudi Arabia
Background: Autoimmune connective tissue diseases are chronic inflammatory diseases with a highly complicated pathogenesis that includes excessive production of pro-inflammatory cytokines. Similarly, COVID-19 has been associated with cytokines dysregulation and increased expression of pro-inflammatory cytokines. Although several factors have been associated with poor outcomes, it is still not well known whether patients with inflammatory rheumatological diseases, especially those receiving immunosuppressants, have an increased risk of severe COVID-19. Objective: This study aimed to study the outcomes (as mortality/survival) and length of stay (as not admitted/admitted for ≤ a week/admitted for > a week) of COVID-19 PCR + ve patients with known inflammatory rheumatological diseases. Methods: This was a retrospective cohort study that took place in the National Guard Health Affairs at King Abdulaziz Medical City, Riyadh, Saudi Arabia. All patients with inflammatory rheumatological diseases and documented COVID-19 infection from 2019 to 2021 were included. Patients with noninflammatory diseases such as osteoarthritis were excluded. The following variables were collected: age, gender, body mass index (BMI), primary rheumatological diagnosis, comorbidities, symptoms of COVID, number of COVID infection, steroid dose, immunosuppressants, length of admission (in weeks), length of ICU admission, mechanical ventilation, cytokine storm syndrome (CSS), secondary bacterial infection, and outcome. Due to the small sample size, Fisher's exact test was used instead of Chi-square to calculate the P values. Multivariate logistic regression analysis was performed. P value < 0.05 was considered significant. Results: There were a total of 192 rheumatology patients with COVID-19, only 122 of whom have fulfilled our inclusion criteria. Ninety-nine (81.1%) patients were female and 23 (18.9%) were male. The patients' mean age was 48.3 ± 16 years with an average BMI of 30.8 ± 6.4 kg/m2. Rheumatoid arthritis was the most common primary rheumatological diagnosis. The majority (65.6%) of the patients did not require hospital admission. However, 16.4% required admission for ≤ a week, 11.5% for > a week but < a month, and 6.6% for > a month. The overall mortality rate was 11.5%. On Fisher's exact test, we found that having more than one COVID infection, mechanical ventilation, CSS, secondary bacterial infection, having more than one comorbidity, rituximab use, diabetes mellitus (DM), hypertension (HTN), chronic kidney disease (CKD), and heart failure (HF) were significantly associated with longer hospital stay (P = 0.006, <0.001, 0.006, 0.01, <0.001, 0.046, 0.001, 0.003, 0.003, 0.011, respectively). In addition, HTN, HF, rituximab use, mechanical ventilation, CSS, and secondary bacterial infection were significantly associated with higher mortality rates (P = 0.002, 0.006, 0.001, <0.001, <0.001, 0.006, and 0.001, respectively). In ordinal logistic multivariate regression, we found that high BMI, number of COVID-19 infection, mechanical ventilation, number of comorbidities, and having CKD were significantly associated with higher odds of longer hospitalization (P = 0.005, 0.011, 0.002, 0.003, and 0.006, respectively). In binary logistic multivariate regression, HTN was the only comorbidity that significantly associated with higher odds of mortality (P = 0.022, OR = 5.291). Conclusion: The mortality rate was 11.5%. Number of COVID infection, mechanical ventilation, CSS, secondary bacterial infection, number of comorbidities, rituximab use, DM, HTN, CKD, and HF were significantly associated with longer hospital stay. Moreover, HTN, HF, rituximab use, mechanical ventilation, CSS, and secondary bacterial infection were significantly associated with higher mortality rates. In ordinal logistic multivariate regression, we found that high BMI, number of COVID-19 infection, mechanical ventilation, number of comorbidities, and having CKD were significantly associated with higher odds of longer hospitalization. In binary logistic multivariate regression, the only comorbidity that was significantly associated with higher odds of mortality was HTN.
Keywords: COVID-19, immunosuppressants, inflammatory diseases, inflammatory rheumatic diseases, rheumatology
| Psychosocial and Functional Impact of COVID-19 Pandemic on Rheumatic Patients' Quality of Life in Saudi Arabia|| |
Lena Hassen, Rana Albarrak
Introduction: People's daily lives have been exacerbated due to situational factors in the context of COVID-19 outbreak measures. Aim: This study aims to examine rheumatic patients' psychosocial and functional state during COVID-19 pandemic and assess the impact it had on their quality of life. Methods: Our time-series study included a patient-centered electronic survey sampling adult rheumatic patients living in Saudi Arabia at different time points from March to August 2020. Patient-reported outcomes included physical function, anxiety, depression, fatigue, sleep disturbance, ability to participate in social roles, and pain interference domains were measured using Patient-Reported Outcomes Measurement Information System (PROMIS-29 Profile v2.1). Results: A total of 1278 respondents were enrolled. Results showed significant variation in patient's experiences. Our analyses revealed that the psychological well-being of rheumatic patients was significantly impacted, and such effect has been persistent over time irrespective of public health measures to control COVID outbreaks. Conclusion: These findings consistently demonstrate the need for psychosocial consideration for improvement in rheumatic patients' quality of life and disease outcomes.
Keywords: COVID-19, rheumatic disease, quality of life, mental health, patient-reported outcome
| Rheumatoid Arthritis and the Rheumatic Disease Comorbidity Index: Its Impact on Disease Activity|| |
Sultana A. Abdulaziz1, Suzan M. Attar2, Renad A. Ahmed3, Omar A. Bokhary3, Anas S. Alyazidi3, Abdulelah G. Abumohssin3, Hamza L. Fida3
1Department of Medicine, King Fahad Hospital, 2Department of Medicine, King Abdulaziz University Hospital, 3Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia
Background: Comorbid conditions in the setting of rheumatoid arthritis (RA) play an important role in predicting disease activity and functional impairment. Application of a specific comorbidity index in a clinical setting could be useful in identifying patients with a poor prognosis. The rheumatic disease comorbidity index (RDCI) is a scoring system developed specifically for RA patients that is used to assess their total burden of comorbidity. Objectives: The RDCI scale has yet to be thoroughly investigated in published literature, and the clinical presentations and comorbidities of RA patients have not been adequately described in the local region. Our study aims to assess the relationship between comorbidity and disease activity of RA by use of the RDCI.
Methods: Between 2019 and 2021, medical records and clinical data from the latest rheumatology clinic visit of consecutive RA outpatients above 18 years of age were reviewed in a two-center retrospective study. Data retrieved included demographics, clinical presentation, inflammatory markers, comorbid conditions, and medications. RDCI was calculated for each patient, and disease activity was assessed using DAS28, CDAI, and SDAI scores. The degree of functional impairment was documented using the Health Assessment Questionnaire (HAQ). Results: One hundred ninety-five patients were included in this study. Most of the study population was female, with a mean age of 55 years and an average body mass index (BMI) of 31. The most prevalent comorbidity was hypertension (29.7%). A large portion of patients had an RDCI score of zero (38.5%), with the second-largest group scoring 3 or higher (22.1%). The mean DAS 28 CRP score was 3.34 (±1.2), with 49.3% of patients achieving a good EULAR response, while the mean CDAI score was 13.8 ± 10.3, with only 19 (9.9%) patients achieving the same response. Patients with a higher RDCI score were older (P < 0.001) with a longer duration of disease (P < 0.001) and significantly higher HAQ scores (P = 0.012). However, no association between RDCI scores and disease activity was found. Independent predictors of a good clinical response were decreasing BMI and the use of tumor necrosis factor-inhibiting medications. Conclusion: Although a direct relationship between RDCI score and disease activity could not be established, it did have a significant impact on functional impairment. We conclude that RDCI evaluation could prove useful in identifying multimorbid RA patients with a poor prognosis and help in fine adjustment of treatment goals to restore the quality of life.
Comorbidities, disease activity, rheumatoid arthritis, rheumatic disease comorbidity index
| The Anti-Melanoma Differentiation-associated Gene 5 Antibody-associated Inflammatory Myositis: A Retrospective Multicenter Study from Saudi Arabia|| |
Jude Almasoud1, Abdulrahman Asiri2, Wafa Sewiari1,3, Munirah Almarri2, Khairiyah Alsufyani4, Abdulrahman Alrasheed1,3
1King Abdullah Specialized Children Hospital, Ministry of the National Guard, 2Prince Sultan Military Medical Complex, 3King Saud bin Abdulaziz University for Health Sciences, Riyadh, 4Maternity and Children Hospital, Makkah, Saudi Arabia
Introduction: Juvenile idiopathic inflammatory myopathies (JIIM) are heterogeneous diseases. About 70% of patients with JIIM have positive myositis-specific antibodies (MSA). Anti-MDA5, an MSA, has been reported to be associated with the worst outcome. Objectives: The objective was to describe clinical features, laboratories, treatment, and outcome of patients with anti-MDA5 JIIM. Methods: This was a multicenter retrospective report of patients with MDA5-positive JIIM managed in three tertiary care Hospitals in Saudi Arabia. Our report describes their clinical presentation, laboratory investigations, treatment modalities, complications, and outcomes. Results: Seven patients were identified to have MDA5-positive JIIM; all were Saudi females, the mean age of presentation was 79 months. Common presentations were fever, skin rash, muscle weakness, skin ulcerations, reverse Gottron sign, and arthritis. Two patients had low WBC and one with high liver enzymes and positive anti-smooth-muscle-antibodies. All patients except one had high ferritin. All patients but one were ANA positive and had myositis-associated antibodies with mild elevation in muscle enzymes. They were treated with steroids, IVIG with the addition of DMARD or biologic medications, and two patients were treated with JAK inhibitor with a mean duration of follow-up of 28.4 months. Two patients below 5 years of age developed rapidly progressive interstitial lung disease in the first 4 months after diagnosis leading to rapid deterioration and death in both cases. One patient recovered from macrophage activation syndrome. Conclusion: Anti-MDA5 antibody-associated JIIM has a unique phenotype and complication. Early recognition and aggressive treatment may change disease outcomes.
| The Management Flow of Sepsis in Systemic Lupus Erythematosus Patients: A Single-Center Retrospective Study|| |
Noha K. Khalil1, Mansour altuwaijri2, Mazen Barry1, Rakan Alqahtani3, Abdulaziz Alrabiah4, Ali Alhijji1, Ibrahim almaghlouth5,6, Mohammed A. Omair5,6
1Department of Medicine, Infectious Disease Unit, King Saud University Medical City, 2Department of Medicine, Gastroenterology Unit, King Saud University Medical City, 3Department of Intensive Care, King Saud University Medical City, 4Department of Emergency, King Saud University Medical City, 5Department of Medicine, Rheumatology Unit, King Saud University Medical City, 4Department of Medicine, Rheumatology Unit, King Saud University Medical City, Riyadh, Saudi Arabia
Background: Infection is one of the leading causes of morbidity and mortality in patients with systemic lupus erythematosus (SLE). During emergency room (ER) visits, SLE patients are not prioritized as immunocompromised. The aim of the current study is to explore the impact of different time lags, type of infection, and culture results on treatment outcomes. Methods: This was a single-center retrospective study that included patients with SLE between 2015 and 2021. Descriptive statistics were presented using numbers, percentages, mean, and standard deviation (SD). Univariate analysis was conducted to determine the factor associated with death by using Fischer exact test. Statistical collinearity was measured using the Shapiro–Wilk test. P value of 0.05 was considered statistically significant. Results: This study analyzed 74 SLE patients. The majority (85.1%) were male with a mean age, and disease duration was 37.2 (±14.5) and 8.27 (±7.75) years, respectively. Background medications included prednisolone (75.7%), hydroxychloroquine (79.7%), mycophenolate mofetil (40.5%), cyclophosphamide (8.4%), rituximab (5.4%), and tacrolimus (1.4%). The median hours of triage to ER physician, ER to medicine, presentation to rheumatology, and presentation cultures were 2.0, 5.0, 19.0, and 6.0, respectively. Positive cultures were as follows: blood (25.7%), urine culture (10.8%), and sputum culture (4.1%). Staphylococcus epidermidis and Escherichia coli were the most frequent organisms identified (21.1% and 15.8%, respectively) in blood and E. coli and Klebsiella pneumoniae (37.5%) in urine culture. Six (8.1%) patients died with sepsis and multi-organ failure, the reported causes of death. Univariate analysis identified previous sepsis, delay of intensive care unit admission, high lactate dehydrogenase, and the SLICC index as risk factors (P < 0.05). Conclusion: Sepsis remains an important cause of mortality in SLE patients with identified risk factors. There is a need to develop a fast-track pathway in the ER to manage these patients.
| The Performance of the EULAR/ACR 2019 Classification Criteria for Monogenic Lupus|| |
Sulaiman M. Al-Mayouf1, Lujayn Akbar1, Reem Abdwani2, Giulia Ginesi3, Stefano Volpi3, Reima Bakry4, Samia AlHashim1, Alhanouf Alsaleem1
1King Faisal Specialist Hospital and Research Center, Riyadh, 4???, East Jeddah Hospital, Jeddah, Saudi Arabia, 2Sultan Qaboos, Muscat, Oman, 3IRCCS Istituto Giannina Gaslini, Genova, Italy
Objective: The objective was to evaluate the application of the European League Against Rheumatism/American College of Rheumatology (EULAR/ACR-2019) criteria to monogenic lupus patients and compare its performance against the Systemic Lupus International Collaborating Clinics (SLICC-2012) criteria. Methods: In a multicenter retrospective cohort study, consecutive patients with monogenic lupus from three tertiary lupus clinics were enrolled. The diagnosis of monogenic lupus was based on the expert physician's opinion and fulfilled the SLICC-2012 criteria. In addition, all enrolled patients had genetic variants. A control group of sporadic childhood systemic lupus erythematosus (cSLE) and non-SLE patients including systemic idiopathic arthritis and juvenile dermatomyositis were included. A descriptive data analysis was conducted, and the EULAR/ACR-2019 and SLICC-2012 criteria were applied to both groups. Results: For these analyses, a total of 153 (100 females) patients were available. Forty-nine patients with monogenic lupus with a median age at diagnosis of 6.0 (interquartile range: 3.0–10.8) years and 104 controls (55 patients with cSLE and 49 nonlupus patients with a median age at diagnosis of 10.0 and 5.0, respectively) were included. Forty-four (89.8%) patients with monogenic lupus fulfilled the EULAR/ACR-2019 with a mean score of 22.3 ± 8.9. The most frequent domains were immunologic (93.9%), musculoskeletal and renal (each 57.1%), and mucocutaneous (55.1%). Fifty-four (98.2%) cSLE patients and six (12.2%) nonlupus patients met the EULAR/ACR-2019 criteria, with a mean score of 22.5 ± 9.2 and 8.5 ± 5.2, respectively. The sensitivity of the EULAR/ACR-2019 criteria in monogenic lupus was 95.6% (95% CI: 0.8–0.9), while the specificity was 75.0% (95% CI: 0.5–0.9).Conclusion: This is the first and largest cohort of monogenic lupus patients testing the performance of the 2019-EULAR/ACR criteria. It efficiently classifies monogenic lupus patients, irrespective of the underlying genetic variants. Further studies are needed before these new criteria are adopted worldwide.