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.

Opportunities and challenges of lupus care in Latin America, the Middle East, and Asia-Pacific: A call to action.

Lupus remains a disease with a low prioritisation in the national agendas of many countries in Latin America, the Middle East, and Asia-Pacific, where there is a dearth of rheumatologists and limited access to new or even standard lupus treatments. There is thus an important need for education, advocacy, and outreach to prioritise lupus in these regions to ensure that patients receive the care they need. This article reviews some of the specific challenges facing the care and management of people with lupus in these regions and suggests strategies for improving patient outcomes. Specifically, we review and discuss (with a focus on the aforementioned regions) the epidemiology of lupus; economic costs, disease burden, and effects on quality of life; barriers to care related to disease assessment; barriers to effective treatment, including limitations of standard treatments, high glucocorticoid use, inadequate access to new treatments, and low adherence to medications; and strategies to improve lupus management and patient outcomes. We hope that this represents a call to action to come together and act now for the lupus community, policymakers, health authorities, and healthcare professionals to improve lupus management and patient outcomes in Latin America, the Middle East, and Asia-Pacific.

Experiences of Dermatologists and Patients Regarding Psoriasis and Its Connection to Psoriatic Arthritis in Saudi Arabia.

Purpose: Psoriasis is a chronic, inflammatory, immune-mediated skin disease that has significant impact on a patient's quality of life, yet it remains challenging for dermatologists to successfully identify and manage. Without effective screening, diagnosis and treatments, psoriasis can potentially progress to psoriatic arthritis. A descriptive, observational cross-sectional study of Saudi Arabian dermatologists and patients with psoriasis was conducted to explore dermatologist and patient perspectives of psoriasis, including diagnosis, management, disease course and unmet needs. Patients and Methods: This study involved a quantitative questionnaire administered to 31 dermatologists and 90 patients with psoriasis at eight medical centers and was analyzed using descriptive statistics. Results: Dermatologists and patients perceived that psoriasis treatment was initiated promptly and that follow-up visits were sufficient. Their perspectives differed in the time to diagnosis and patient reaction, symptom severity, input into treatment goals and educational needs. The dermatologists' concerns about underdiagnosed psoriasis (13%) were primarily related to patient awareness (87%), physician awareness (58%), and the absence of a regular screening program (52%). Only 31% of patients with psoriasis were highly satisfied with their psoriasis treatment, with 78% experiencing unpleasant symptoms of pain or swelling in joints indicative of psoriatic arthritis. However, only 56% of these patients reported these symptoms to their physicians. When dermatologists were made aware of this difference, referrals to a rheumatologist increased. Conclusion: The study highlights the importance of strengthening psoriasis management by enhancing dermatologist referral and screening practices, adopting a multidisciplinary approach to care, and improving education and resources for physicians and patients. These results can help to inform the improvement of psoriasis screening, diagnosis and treatment strategies and ensure that expectations meet treatment outcomes. Further research exploring the dermatologist and patient perspectives of the disease pathway from psoriasis to psoriatic arthritis and tailor-made treatment approaches is recommended.

Cost-effectiveness analysis of upadacitinib as a treatment option for patients with rheumatoid arthritis in the Kingdom of Saudi Arabia.

AIM: To evaluate cost-effectiveness of upadacitinib (targeted synthetic-disease modifying anti-rheumatic drug [ts-DMARD]) as first-line (1 L) treatment versus current treatment among patients with rheumatoid arthritis (RA) in the Kingdom of Saudi Arabia (KSA), who had an inadequate response to prior conventional-synthetic (csDMARDs) and/or biologic-DMARDs (bDMARDs). METHODS: This Excel-based model included patients with moderate (Disease Activity Score [DAS28]: >3.2 to ≤5.1) or severe RA (DAS28 > 5.1). Cost-effectiveness of current treatment (1 L: adalimumab-originator/biosimilar; second-line (2 L): other bDMARDs/tofacitinib) was compared against a new treatment involving two scenarios (1 L: upadacitinib, 2 L: adalimumab-biosimilar [scenario-1]/adalimumab-originator [scenario-2]) for a 10-year time-horizon from societal perspective. Model outcomes included direct and indirect costs, quality-adjusted life-years (QALYs), hospitalization days, number of orthopedic surgeries, and incremental cost-utility ratio (ICUR) per QALY. RESULTS: With the current pathway, estimated total societal costs for 100 RA patients over 10-year period were Saudi Riyal (SAR) 50,450,354 (United States dollars [USD] 13,453,428) (moderate RA) and SAR50,013,945 (USD13,337,052) (severe RA). New pathway (scenario-1) showed that in patients with moderate-to-severe RA, upadacitinib led to higher QALY gain (+8.99 and +15.63) at lower societal cost (cost difference: -SAR2,023,522 [-USD539,606] and -SAR3,373,029 [-USD899,474], respectively). Thus, as 1 L, upadacitinib projects "dominant" ICUR per QALY over current pathway. Moreover, in alternate pathway (scenario-2), upadacitinib also projects "dominant" ICUR per QALY for patient with severe RA (QALY gain: +15.63; cost difference: -SAR 164,536 [-USD43,876]). However, moderate RA was associated with additional cost of SAR1,255,696 (USD334,852) for improved QALY (+8.99) over current pathway (ICUR per QALY: SAR139,742 [USD37,264]). Both scenarios resulted in reduced hospitalization days (scenario-1: -14.83 days; scenario-2: -11.41 days) and number of orthopedic surgeries (scenario-1: -8.36; scenario-2: -6.54) for moderate-to-severe RA over the current treatment pathway. CONCLUSION: Upadacitinib as 1 L treatment in moderate-to-severe RA can considerably reduce healthcare resource burden in KSA, majorly due to reduced drug administration/monitoring/hospitalization/surgical and indirect costs.

The Saudi consensus recommendations for the management of psoriatic arthritis (2023).

Psoriatic arthritis (PsA) is a complex inflammatory disease characterized by musculoskeletal and non-musculoskeletal manifestations. It is a distinct disease entity at the interface between rheumatology and dermatology, making it challenging to manage. The diverse clinical presentation and severity of PsA require a multidisciplinary approach for optimal care. Early diagnosis and management are necessary to improving quality of life for patients. In Saudi Arabia, there is currently no unified national consensus on the best practices for managing PsA. This lack of consensus leads to debate and uncertainty in the treatment of the disease, resulting in over or under prescribing of biological agents. To address this issue, a multidisciplinary work group was formed by the Saudi Ministry of Health. This group, consisting of dermatologists, rheumatologists, and pharmacists, aimed to develop evidence-based consensus recommendations for he use and monitoring of biological therapy in PsA management. The work group conducted five consensus workshops between December 2021 to March 2022. Using the nominal group technique, they discussed various aspects of PsA management, including eligibility criteria for biological treatment, monitoring of disease activity, treatment goals, screening, precautions, and management of PsA with biologic therapies. The group also considered special considerations for patients with comorbidities, pregnant and lactating women, as well as pediatric and adolescent populations. The resulting consensus document provides recommendations that are applicable to the Saudi setting, taking into account international guidelines and the specific needs of PsA patients in the country. The consensus document will be regularly updated to incorporate new data and therapeutic agents as they become available. Key Points • In Saudi Arabia, there is a lack of unified national consensus on the optimal management of PsA, therefore, this article aims to provide up-to-date evidence-based consensus recommendations for the optimal use and monitoring of biologic therapy in the management of PsA in Saudi Arabia. • The consensus development process was undertaken by a multidisciplinary work group of 13 experts, including two dermatologists, six rheumatologists, and five pharmacists. • There is more than one disease activity tool used in PsA disease, depending on the disease domain - peripheral arthritis Disease Activity Index in Psoriatic Arthritis (DAPSA) or Minimal Disease Activity (MDA), axial PsA Ankylosing Spondylitis Disease Activity Score (ASDAS), and dactylitis and enthesitis MDA. • The main goal of therapy in all patients with PsA is to achieve the target of remission, or alternatively, low disease activity in all disease domains and improve quality of life (QoL).

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.

Rituximab treatment in patients with systemic sclerosis and interstitial lung disease.

There is increasing interest in rituximab (RTX) as an alternative to cyclophosphamide for the treatment of interstitial lung diseases (ILDs) associated with systemic sclerosis (SSc). However, no report has addressed its efficacy in Saudi patients with SSc-ILD. To assess the efficacy of RTX treatment in Saudi patients with SSc-ILD, hospital records were reviewed between 2013 and 2016. Four female patients received at least 4 cycles of RTX (I cycle, consisting of two infusions of 1000 mg 2 weeks apart). Pulmonary function tests (PFTs) and chest high-resolution computed tomography (HRCT) were performed before and after treatment to assess the response. HRCT revealed improvement in one patient, stable disease in two patients, and worsening in one patient. Moreover, RTX prevented the further decline of forced vital capacity significantly in PFT. These results provide further evidence that RTX is an effective treatment for SSc-ILD.

The Prevalence and Risk Factors of Osteoporosis among a Saudi Female Diabetic Population.

AIM: This study aimed to assess the prevalence and determinants of osteoporosis [lumbar spine (LS) and femoral neck (FN)] among patients with type 2 diabetes at King Salman Hospital. MATERIALS AND METHODS: One hundred seventy patients with type 2 diabetes were enrolled in this cross-sectional study in the period from the 1st of January until the 1st of July 2015. Patient selection was based on self-report of the previous diagnosis by a physician, being on an antidiabetic agent, or a fasting glucose of 126 mg/dl as per the American Diabetes Association criteria. A dual energy X-ray absorptiometry scan with the bone mineral density (BMD) categorization based on the WHO cut of levels of T-scores and determination of vitamin D levels were performed. A detailed questionnaire was used to collect demographic data. RESULTS: Out of 170 participants, 50 (29.4%) were diagnosed as having osteoporosis, while 68 (40%) were diagnosed with osteopenia. Age was determined as a risk factor for a decreased BMD in patients with osteopenia (odds ratio (OR) = 1.1, 95% confidence interval (CI) = (1.0-1.1), p = 0.039) and osteoporosis (OR = 1.1, CI = 1.0-1.2, p < 0.001). Similarly, oral hypoglycemic agents (OHA) increased the risk of decreased BMD in osteopenia (OR = 2.6; CI = 1.0-6.7; p = 0.023) as well as osteoporosis, (OR = 3.8; CI = 1.3-10.9; p = 0.013), while vitamin D deficiency increased the risk of osteopenia OR = 3.0; CI = 1.2-7.2; p = 0.012). Increased BMI decreased the risk of both osteopenia and osteoporosis (OR = 0.9; CI = 0.9-0.99; p = 0.031 vs. OR = 0.9; CI = 0.80-0.95; p = 0.003). CONCLUSION: Advanced age, OHA and vitamin D deficiency are determinants of decreased BMD in Saudi women with type 2 diabetes, while an increased BMI protects against low BMD.

Depression in Patients with Systemic Lupus Erythematosus: A Multicenter Study.

BACKGROUND AND OBJECTIVE: Neuropsychiatric disorders including depression are common clinical manifestations of systemic lupus erythematosus (SLE). Depression in patients with SLE is under-recognized, although it is a treatable clinical entity. The present study aimed to determine the prevalence of depression and identify the relationship between depression and SLE disease characteristics. PATIENTS AND METHODS: This multicenter cross-sectional study was conducted in the rheumatology clinics of four tertiary referral hospitals in Saudi Arabia between April and September 2014. Patients' demographic data and SLE disease characteristics such as disease duration, severity and drug treatments were collected. A validated Arabic Beck Depression Inventory (BDI) score was used to estimate the prevalence of depression. RESULTS: A total of 68 patients with SLE (64 women, 4 men) were enrolled in the study. Forty-six (67.6%) patients were found to have BDI scores indicating depression; of them, only four patients (8.7%) were receiving antidepressant treatments. Higher prevalence of depression was associated with steroid treatment (P = 0.046). CONCLUSIONS: The study results revealed high prevalence of depression among Saudi patients with SLE. Most of the study population were not adequately treated, suggesting inadequate recognition and treatment of depression in SLE.

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.