To describe the clinical profile and compare the long-term outcomes of patients with S-PAN treated with various treatment regimens at our centre in the last 2 decades.
Data regarding clinical presentation, treatment allocation, relapses and outcomes of patients fulfilling American College of Rheumatology (ACR) 1990 criteria for PAN in the last 2 decades were recorded from electronic medical records. Relapse-free survival and predictors were analysed using KM survival statistics and regression analysis.
Altogether, 53 patients including 2 with hepatitis B infection were included. Cutaneous lesions and peripheral neuropathy were the commonest manifestations. Most patients (64.2%) presented with a five-factor score (FFS) of 0. Disease-attributable hypertension and peripheral gangrene were the most common manifestations of severe disease. During a median follow-up period of 53.5 months in 49 patients, 43 (87.8%) attained complete response while 3(6.1%) had a partial response. Nineteen (40.4%) patients relapsed at a median duration of 82 (IQR 36.3-127.7) months. The relapse-free survival in patients who received induction with mycophenolate (n = 26), was comparable to that with cyclophosphamide (n = 21) [adjusted HR : 0.68]. Smoking history was an independent predictor of relapse (HR = 6.28, p= 0.013) while age was protective (HR = 0.94, p= 0.015). FFS and BVAS at 3 months were among the predictors of mortality (total deaths = 5).
In our cohort of S-PAN, relapses were observed in 40.4% of patients. Mycophenolate was similar to cyclophosphamide in maintaining relapse-free survival. Only 10% fatality was recorded. FFS and BVAS at 3 months were predictors of mortality.

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