Comprehensive description of the prevalence, serological and clinical characteristics, and visceral involvement of systemic sclerosis (scleroderma) in a large cohort from the United Arab Emirates Systemic Sclerosis Registry.

Systemic sclerosis is an autoimmune condition characterized by a wide range of clinical presentations. Registries may serve to expand understanding about systemic sclerosis and aid in patient care and follow-up. The objective of this study was to analyze the prevalence of systemic sclerosis in a large cohort from the United Arab Emirates Systemic Sclerosis Registry and find the significant similarities and differences between the different subsets. All scleroderma patients in the United Arab Emirates were included in this multicenter national retrospective analysis. Data on demographics, comorbidities, serological characteristics, clinical aspects, and treatment were collected and analyzed, highlighting the most common traits identified. A total of 167 systemic scleroderma patients from diverse ethnic backgrounds were enrolled. Overall, 54.5% (91/167) of the patients were diagnosed with diffuse cutaneous systemic sclerosis, and 45.5% (76/167) with limited cutaneous systemic sclerosis. The prevalence of systemic sclerosis was 1.66 per 100,000 for the total registry and 7.78 per 100,000 for United Arab Emirates patients. Almost all patients in the diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis groups tested positive for the immunofluorescence antinuclear antibody. Antibodies against Scl-70 were significantly more associated with diffuse cutaneous systemic sclerosis, whereas anticentromere antibodies were significantly more associated with the limited cutaneous systemic sclerosis group (p < 0.001). Sclerodactyly, shortness of breath, and digital ulcers were more common in diffuse cutaneous systemic sclerosis patients compared with the limited cutaneous systemic sclerosis subtype in terms of clinical symptoms and organ involvement. Telangiectasia was much more common in the limited cutaneous systemic sclerosis group. Furthermore, diffuse cutaneous systemic sclerosis patients had more lung fibrosis (interstitial lung disease) than limited cutaneous systemic sclerosis patients (70.5% vs 45.7%), and pulmonary arterial hypertension was twice as common in limited cutaneous systemic sclerosis patients as it was in diffuse cutaneous systemic sclerosis patients. Local registries are paramount to understanding the clinical/serological characteristics of scleroderma. This study emphasizes the importance of raising disease awareness and distinguishing between the various systemic sclerosis subsets to implement patient-tailored strategies for early detection, better management, and higher quality of care.

What is the best referral strategy for axial spondyloarthritis? A prospective multicenter study in patients with suspicious chronic low back pain.

OBJECTIVE: To assess the value of referral strategies for axial spondyloarthritis (axSpA) in patients with suspicious chronic inflammatory low back pain (LBP), to estimate the value of inflammatory back pain (IBP) for referral, and to identify the predictive factors of the final diagnosis of axSpA in Middle Eastern Arab countries. METHODS: The study was multicentric, prospective, and conducted in LBP first-line clinics (rheumatology, internal, family medicine, orthopedic surgery, neurosurgery, and neurology). Consecutive adult patients aged under 45years were included in case of LBP suspicious of inflammatory nature according to the first-line physician. The rheumatologist's final diagnosis was the gold standard. The diagnostic properties of ten referral strategies (Brandt I, II, III, Hermann, RADAR, RADAR 2/3, MASTER, Braun, CAFASPA, and ASAS) and of IBP were calculated. A multivariable logistic regression identified the clinical predictive factors of axSpA. RESULTS: In 515 referred patients, axSpA was confirmed in 48%, refuted in 43%, and diagnosis remained inconclusive in 9%. The optimal referral strategy was the MASTER (PLR 3.3), which comprises IBP, good response to NSAIDs, positive HLA-B27, and SpA family history. Considering strategies without HLA-B27, the RADAR 2/3 had a PLR of 2.9 (IBP, good response to NSAIDs, any extra-musculoskeletal manifestation). The predictive factors for axSpA were MRI sacroiliitis, positive HLA-B27, high CRP, psoriasis, IBP, and longer symptom duration. Of all patients, 35% were self-referred, 16% were referred by primary care physicians, and 15% by neuro/orthopedic surgeons. CONCLUSION: Optimizing physicians' awareness of these clinical features may enhance referral in axSpA.

Overlap between dermatomyositis and ANCA vasculitides.

We present the second report of the association between antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis with dermatomyositis (DM). A 47-year-old woman suddenly developed rapidly progressive renal failure in the context of (DM). The kidney biopsy showed focal and segmental necrotizing glomerulonephritis with crescent formation. Cyclophosphamide treatment was commenced resulting in a significant recovery of kidney function and maintenance of recovery at 6 months. Although the pathophysiology is unknown, we hypothesize that CD8-T-deficient cells and MPO+ neutrophils in the DM lesions play an important role in the disease process.