ABSTRACT
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.
ABSTRACT
OBJECTIVE: To longitudinally evaluate esophageal dysmotility (ED) in patients with limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). METHODS: We performed a retrospective review of all adult patients with SSc seen between 1995 and 2008. Patients were included if they had undergone 2 or more esophageal transit scintigraphy (ETS) studies at least 1 year apart. Data from 382 ETS studies of 102 patients with SSc were analyzed. Eighty patients had lcSSc and 22 patients had dcSSc. A grading system was used to quantify the degree of esophageal dysfunction, ranging from grade 0 (normal) to grade 3 (severe hypomotility). Change in esophageal motility over time was evaluated and compared between the limited and diffuse subtypes. RESULTS: Sixty-eight patients (66.7%) had an abnormal ETS study at any time. Of patients with dcSSc, 95.4% had an abnormal ETS study, compared to 58.5% of patients with lcSSc. dcSSc and regurgitation were independent risk factors for ED. There was no association between the presence of anticentromere antibodies or antitopoisomerase (anti-Scl-70) antibodies and an abnormal ETS study. Esophageal motility in patients with dcSSc worsened in 96% of cases compared with only 58.8% in those with lcSSc. CONCLUSION: ED is more frequent in patients with dcSSc than in those with lcSSc, and is more likely to deteriorate over time. Given the potential associated risks of erosive esophagitis, Barrett's esophagus, and esophageal cancer in patients with SSc, routine screening and monitoring for ED is advised.
