Saudi National Clinical Practice Guidelines for Management of Adult Systemic Lupus Erythematosus.

OBJECTIVE: To provide evidence-based clinical practice recommendations for managing Systemic Lupus Erythematosus (SLE) in Saudi Arabia. METHODS: This EULAR-adapted national guideline in which a multidisciplinary task force utilized the modified Delphi method to develop 31 clinical key questions. A systematic literature review was conducted to update the evidence since the EULAR publication. After reaching a consensus agreement, two rounds of voting and group discussion were conducted to generate consolidated recommendations/statements. RESULTS: A significant number of patients in Saudi Arabia experience delays in accessing rheumatologists, highlighting the significance of timely referral to SLE specialists or rheumatologists to ensure accurate diagnosis and prompt treatment. The primary goal of Glucocorticoid (GC) therapy in SLE patients is to establish disease control with a minimum dose and duration. Steroid-sparing agent utilization facilitates steroid-sparing goals. Hydroxychloroquine is recommended for all SLE patients, though physicians must carefully monitor toxicity and prioritize regular medication adherence assessment. SLE management during pregnancy starts from preconception time by assessing disease activity, major organ involvement, hypercoagulability status, and concomitant diseases that may negatively impact maternal and fetal outcomes. Multidisciplinary care with close monitoring may optimize both maternal and fetal outcomes. For patients with antiphospholipid antibodies, low-dose aspirin prophylaxis is recommended. Also, Long-term anticoagulant medications are fundamental to prevent secondary antiphospholipid syndrome due to high thrombosis recurrence. CONCLUSION: This Saudi National Clinical Practice guidelines for SLE management provide evidence-based recommendations and guidance for healthcare providers in Saudi Arabia who are managing patients with SLE. These guidelines will help to standardize healthcare service, improve provider education, and perhaps lead to better treatment outcomes for SLE patients.

Effectiveness of secukinumab in patients with psoriasis and psoriatic arthritis in a Saudi real-world setting.

INTRODUCTION: Psoriasis (PsO) is an immune-mediated chronic inflammatory disease that results in severe outcomes that impact the patient's quality of life and work productivity. We investigated the effectiveness of secukinumab in patients with chronic plaque psoriasis and psoriatic arthritis (PsA) over a 12-month period. METHODS: This was a longitudinal, retrospective study of the medical records of 81 patients with psoriasis and/or psoriatic arthritis who had been treated with secukinumab for at least 12 weeks. RESULTS: The Psoriasis Area Severity Index (PASI), Body Surface Area (BSA) percentage, and Dermatology Quality of Life Index (DLQI) among patients with PsO and PsO-PsA showed a statistically significant decrease from baseline over 12 months by approximately 9.86, 19.3%, and 9.7, respectively (p values < 0.001 for each). Moreover, there was a statistically significant decrease in the overall Disease Activity in Psoriatic Arthritis score (DAPSA) by approximately 22.35 from baseline over 12 months of treatment (p < 0.001). Considering the patients who started secukinumab 12 months or more prior to the study cutoff date, the 12-month retention rate was 85%. CONCLUSION: In a Saudi real-world setting, secukinumab proved to be an efficient medication with high efficacy and retention rates.

Characteristics of lupus nephritis in Saudi lupus patients: A retrospective observational study.

OBJECTIVES: Systemic lupus erythematosus (SLE) is a chronic autoimmune multi-systemic disorder of the connective tissue, characterized mainly by involvement of the skin, joints, kidneys, and serosal membranes. It affects females particularly at childbearing age more commonly than males. Lupus nephritis affects around half of patients with SLE. Data about SLE and lupus nephritis in Saudi Arabia are still scarce. In this study, we aimed to evaluate the prevalence, clinical and laboratory findings of SLE and different histological types of lupus nephritis among Saudi patients at King Fahad Medical City. METHODS: This is a retrospective study for adult patients who have been evaluated at king Fahad medical city between 2014 and 2019 and fulfilled the Systemic Lupus International Collaborating Clinics classification criteria (SLICC). RESULTS: 112 patients, 103 (92%) females and 9 (8%) males, with confirmed diagnoses of SLE were reviewed. Skin rash (69.6%), photosensitivity (61.6%), mucosal ulcerations (45.9%), arthralgia and/or arthritis (44.6%) are the most common clinical features. Ninety seven (86.6%) out of 112 patients had a recorded first visit 24 hour urine protein level, out of those only 26 (23.2) patients presented with significant proteinuria of more than 0.5grams per day. Forty four (39.2%) have undergone kidney biopsy. Class IV and III lupus nephritis are the most common reported biopsy results (43.18% and 27.28% respectively). During the study period, three patients (2.7%) developed end-stage kidney disease requiring dialysis and five (4.5%) had renal transplant. CONCLUSION: Our study provided insight on the demographics, characteristics and presentation of SLE patients and the outcome of lupus nephritis in Saudi Arabia.

Disease burden and treatment challenges of psoriatic arthritis in Africa and the Middle East.

Psoriatic arthritis (PsA) is a chronic, inflammatory arthropathy occurring in up to 30% of patients with psoriasis, and is characterized by multiple manifestations including peripheral arthritis, enthesitis, dactylitis, spondylitis, and psoriatic skin and nail disease. This complex and heterogeneous disease is poorly understood and its diagnosis and treatment are suboptimal, particularly in Africa and the Middle East, where very few studies into the impact of PsA have been carried out. This article aims to highlight the disease burden of PsA in the region as well as to identify unmet clinical needs. A non-systematic review was carried out in the PubMed database and the most relevant publications were selected. Expert rheumatologists practicing in Africa and the Middle East provide an insight into the challenges of treating PsA in daily practice, along with recommendations for improvements.

IgG4-Related Disease coexisting with Behçet's Disease.

We describe a case of Immunoglobulin G4-related disease (IgG4-RD) coexisting with Behçet's disease. A 49-year-old man, with a diagnosis of Behçet's Disease for 15 years who was found to have an acute kidney injury. His investigations revealed an elevated IgG4 level and the abdominal computerized tomography showed a retroperitoneal mass, which was diagnosed to be IgG4-RD based on histology. The patient showed symptomatic and radiological improvement after starting high dose steroid for 1 month followed by a maintenance dose. Our case report suggested that the two diseases arose separately.

Echocardiographic findings in asymptomatic systemic lupus erythematosus patients.

The aim of this study is to use transthoracic echocardiographic (TTE) imaging methods to identify cardiac dysfunction in asymptomatic systemic lupus erythematosus (SLE) patients and to determine the association between echocardiographic findings and serology. This is a prospective cross-sectional study where 50 patients with confirmed diagnoses of SLE were recruited from rheumatology outpatient clinics. Clinical and serological evaluation to confirm the diagnosis of lupus was done in all patients. Fifty SLE patients, 46 (92%) females and 4 (8%) males, were recruited. Anti-double-stranded DNA (Anti-dsDNA), anticardiolipin, lupus anticoagulant, and anti-ß2-glycoproteins were positive in 52.1, 32.6, 13.3, and 15.6%, respectively. Transthoracic echocardiogram revealed mitral regurgitation in 16 patients (32%), pericardial effusion in16 patients (32%), aortic regurgitation in five patients (10%), and tricuspid regurgitation in 10 patients (20%). Eleven patients had left ventricular hypertrophy (22%), and eight patients had ventricular systolic dysfunction (16%). Only four patients had ventricular diastolic dysfunction (8%). A significant association between mitral and tricuspid valve regurgitation and positive anti-dsDNA (p < 0.018, p < 0.006, respectively) was found. Positive anticardiolipin antibodies, lupus anticoagulant, and anti-ß 2 glycoprotein antibodies were also associated with mitral valve regurgitation (p values 0.044, 0.006, and 0.023), respectively. Active disease assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was found to be associated with increased risk of mitral valvular leaflet thickening (p value 0.028). Performing regular transthoracic echocardiogram in asymptomatic SLE patients is important for early detection and appropriate treatment of cardiac lesions. Clinically quiescent but serologically active disease and presence of antiphospholipid antibodies were associated with structural heart abnormalities.

Tocilizumab: a new therapy for large vessel vasculitis.

Large vessel vasculitis represents mainly two main diseases: giant cell arteritis and Takayasu arteritis. Recent advancements in the treatment for different rheumatic diseases refractory to disease-modifying antirheumatic agents with biologic agents have resulted in remarkable efficacy and tolerability. However, treatments for large vessel vasculitis (LVV) with tumor necrosis factor-α inhibitors have yielded conflicting results. Recently, very promising results have been reported in patients treated with tocilizumab. The purpose of this review is to summarize the current treatment strategies of LVV and the recent evidence on the use of tocilizumab in LVV.

Biologic treatments for adult-onset Still's disease.

Adult-onset Still's disease (AOSD) is a systemic inflammatory disorder that is a diagnosis of exclusion. It is characterized by high spiking fevers, arthritis or arthralgia, and an evanescent salmon-coloured rash. Many other systemic manifestations and laboratory test abnormalities may occur. Biologic drugs, TNF-α inhibitors, and IL-1 and IL-6 blockers have been used for the treatment of patients with AOSD refractory to conventional treatment or those with life-threatening manifestations aiming for better disease control. Data on biologic treatments in AOSD are limited and consist mainly of case reports, small case series and retrospective studies. Using biologic agents (anti-TNF-α, anti-IL-1 and anti-IL-6) with traditional immunosuppressive drugs resulted in significant improvement of disease outcomes. IL-1 and IL-6 inhibitors seem to be more efficient than TNF-α inhibitors.

Rheumatic conditions in patients with diabetes mellitus.

Diabetes mellitus (DM) is a chronic systemic disease with a wide range of complications, including complications in the musculoskeletal system. DM is a common disease in nearly all countries and continues to increase in numbers and significance. Approximately 90 % of patients have type 2 DM which is caused by resistance to insulin. Type 1 DM results from an absolute deficiency of insulin due to an autoimmune destruction of insulin-producing beta cells within the pancreas. Diabetic patients suffer from diverse rheumatic conditions that affect their life quality. This article reviews the rheumatic conditions that are associated with DM and the pathophysiologic relationships that might link these conditions; it also summarizes recent advances in the field of diabetes and rheumatic conditions. The rheumatic conditions that are discussed in this review include limited joint mobility, Dupuytren's contracture, flexor tenosynovitis, carpal tunnel syndrome, adhesive capsulitis, diffuse idiopathic skeletal hyperostosis, neuropathic osteoarthropathy, diabetic muscle infarction, crystal induced arthritis, osteoarthritis, and reflex sympathetic dystrophy.

Thrombosis in systemic lupus erythematosus: a review article.

Thrombosis is a well-known clinical entity in systemic lupus erythematosus (SLE), and it is multifactorial. The most important risk factor is the presence of antiphospholipid antibodies (APLAs). However, approximately 40% of adults with SLE who are negative for APL A are diagnosed with thrombosis, indicating the importance of other risk factors. Thus, the thrombosis risk factors should be evaluated extensively and regularly and treated aggressively in every patient with systemic lupus erythematosus.