Granulomatosis with polyangiitis: clinical course and outcome of 60 patients from a single center in South India

AbstractGranulomatosis with polyangiitis (GPA) previously known as Wegener ’s granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study. Disease activity and damage were assessed by Birmingham Vasculitis Activity Score v. 3 (BVAS v. 3) and Vasculitis Damage Index (VDI), respectively. Relapses were defined as recurrence of GPA of sufficient severity to require treatment or increase in the dose of treatment on a patient who was previously stable. Out of 60 patients, initial BVAS evaluation showed that 57 (95%) patie nts had severe disease and 3 (5%) patients had limited disease where median BVAS was 21.5 (range 17–44). Follow-up BVAS evaluation for severe disease showed that 13 (22.8%) patients continued with severe disease of which 9 patients did not survive, 24 (42.3%) had remission, 11 (19.2%) had persiste nt disease, and 9 (15.7%) were lost to follow-up. The mean VDI score was 2.5 ± 2. Renal involvement was established in 42 (70%) patients. Upper and lower respiratory involvement was seen in 38 (63%) patients. Nervous sys...
Source: Clinical and Experimental Medicine - Category: Research Source Type: research