Background/Purpose: The published Indian data on Granulomatosis with Polyangiitis (GPA) is sparse and only in the form of few small case series. The purpose of this study was to describe the clinical features, treatment and long term outcome of 105 patients with GPA from a tertiary care centre in north India and compare them with published single center experiences from Europe and America.

Methods: Clinical data of all patients diagnosed to have GPA between April 2005 and April 2016 were retrieved. Clinical details including the clinical presentation, laboratory data, treatment received and outcomes of 105 patients of GPA were obtained. The disease activity at presentation was assessed with Briminghams Vasculitis Activity Score (BVAS v3) and the patients were classified according to EUVAS categories into localized, early systemic, generalized, severe and refractory categories. Our results were compared with published single center experiences from Europe and USA.

Results: A total of 105 patients (60 females and 45 males) were diagnosed to have GPA at a mean age of 40.31 years. The duration of follow up ranged from 0 months to 136 months with a mean duration of follow up of 37.21 months. Ninety four (89.52%) patients had ANCA positivity by either IIF or ELISA and 11 (10.48%) were ANCA negative. Upper respiratory tract involvement was seen in 76.19% of patients. Renal and lung involvement was seen in 51.43% and 67.62% of patients respectively. Cavitating lung lesions were seen in 21.9% of patients and subglottic stenosis was noted in 3 patients. Ocular, ear, CNS, peripheral nervous system, heart, gastrointestinal and skin involvement was seen in 40.95%, 18.1%, 28.57%, 11.43%, 5.71%, 12.38% and 29.52% respectively. Constitutional symptoms were noted in 62.86% of patients and joint symptoms were present in 43.81% of patients. The mean BVAS v3 score was 17.78. Among the EUVAS categories, localized disease was seen in 3 patients, early systemic disease in 44 patients, generalized disease in 38 patients. Seventeen patients had severe disease and one patient had refractory disease. All the patients were treated with steroids at presentation and cyclophosphamide (either oral or intravenous) was used in 90 patients. Rituximab was used as primary remission induction in 6 patients while 25 patients received it as secondary remission induction agent. Plasma exchange was performed in 6 patients and 3 patients received intravenous immunoglobulins. Total 17 patients expired out of which 9 patients expired at first admission and 4 patients expired within 1 year of diagnosis. The cause of death was attributed to disease activity in 11 patients, disease activity plus sepsis in 3 patients and sepsis alone in 3 patients. Twenty six patients experienced relapses during the follow up period which were managed appropriately. Comparison of our findings with single center experiences from Europe and USA is shown in table 1.

Conclusion: Indian population seems to get effected by GPA at an earlier age compared to the western population and also has a lesser renal involvement. The other organ involvement, response to therapy, relapse rates and mortality appear to be similar between the Indian and western population. Table 1: Comparison of clinical features of GPA among European, American and Indian patients

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-features-and-long-term-outcome-of-105-patients-of-granulomatosis-with-polyangiitis-a-single-centre-experience-from-north-india/