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

Clinical and Experimental Medicine
Vineeta ShobhaRavi Prakash

Abstract

Granulomatosis 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%) patients 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...Continue Reading

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Citations

Oct 9, 2018·Diseases of the Esophagus : Official Journal of the International Society for Diseases of the Esophagus·P HoverstenD A Katzka
Jan 24, 2020·Clinical and Experimental Medicine·Miriana d'AlessandroElena Bargagli
May 18, 2018·BMC Research Notes·Omar IrfanAli Bin Sarwar Zubairi
Oct 13, 2021·Rheumatology International·Pratibha BanerjeeSabyasachi Senapati

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Methods Mentioned

BETA
X-ray
biopsy
biopsies

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