Clinical and autoantibody profile in systemic sclerosis: baseline characteristics from a West Malaysian cohort.

AIM: To evaluate the clinical and antibody profile of systemic sclerosis (SSc) in a Malaysian cohort. METHODS: Consecutive patients with SSc in University Malaya Medical Centre from March to November 2012 were included in this study. In addition to clinical characterization, all subjects underwent autoantibody testing using Euroline immunoblot assay. The association between clinical features and autoantibody profile was evaluated. RESULTS: There were 31, predominantly Chinese (45.2%), subjects. Limited cutaneous disease was the most common subtype (71%). Raynaud's phenomenon was the most commonly observed feature (83.9%). Nine (29%) had esophageal dysmotility symptoms and 23 (74.2%), including all patients with diffuse SSc, had symptoms of gastro-esophageal reflux disease (GERD). Restrictive pattern on pulmonary function test and evidence of lung fibrosis were seen in more than 70% of patients. Echocardiographic evidence of pulmonary arterial hypertension was seen in 58.1%. Telangiectasia, calcinosis, digital ulcers, digital pulp loss or pitting were seen more commonly in the diffuse subtype. The two most prevalent autoantibodies were anti-Scl-70 and anti-Ro-52. The presence of anti-Scl-70 was significantly associated with restrictive lung disease (P = 0.05). Anti-Ro-52 was associated with control subjects with other autoimmune diseases (P = 0.043). The presence of anti-PM-Scl-75 was associated with overlap syndrome (P = 0.032). Patients with anticentromere antibodies were more likely to have vasculitic rash (P = 0.012). CONCLUSION: In Malaysia, SSc most commonly affects the Chinese. Limited cutaneous is more common than diffuse subtype. Features of CREST (calcinosis, Reynaud disease, esophageal dysmotility, sclerodactyly, telangiectasia) are more commonly observed in the diffuse cutaneous subgroup. Anti-Scl-70 and anti-Ro-52 antibodies are promising biomarkers for pulmonary involvement in SSc.

Open label randomized controlled trial assessing the efficacy of mycophenolate sodium against other conventional immunosuppressive agents in active systemic lupus erythematosus patients without renal involvement.

AIM: Mycophenolate is an immunosuppressive agent which has been used in systemic lupus erythematosus (SLE) patients who have failed conventional therapy. However, the use of mycophenolate sodium in extra-renal SLE involvement has yet to be established. This study aimed to assess the efficacy of mycophenolate sodium in extra-renal SLE. METHODS: A total of 14 SLE patients without renal involvement were randomized either to receive mycophenolate sodium or other immunosuppressive agents. Patients were assessed monthly from baseline until week 16. Assessment parameters included SLE Disease Activity Index (SLEDAI) score, other organ-specific parameters and immunological parameters, including anti-double stranded DNA and C3. Steroid-sparing effect of mycophenolate sodium was also evaluated. RESULTS: Mycophenolate sodium produced a significant reduction in SLEDAI scores (P < 0.05) after 16 weeks of treatment. Mixed responses were detected in terms of organ-specific clinical changes. A positive trend was observed in improvement of immunological parameters and steroid dose reduction. No major adverse events were reported in this study. CONCLUSION: Mycophenolate sodium is a safe alternative therapy in SLE patients with extra-renal involvement. The reduction in SLEDAI scores and the observation of no major safety concerns suggest that a larger prospective study of mycophenolate sodium in non-renal SLE is warranted.