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

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%) had persistent 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 system involvement was noted in the 15 (25%) patients. Articular manifestations were seen in 16 (27%) patients. Diverse clinical manifestation delay early diagnosis and treatment of this potentially treatable vasculitis. Focused approach could expedite early diagnosis and can reduce the mortality.

Time-dependent risk of mortality and end-stage kidney disease among patients with granulomatosis with polyangiitis

“…We observed that the excess mortality of GPA was extremely high within the first year, particularly the first 6 months after diagnosis, compared to the matched comparison cohort. Previously published clinic-epidemiologic studies regarding GPA in Japan, Korea, and other Asian countries did not report timing of death or mortality risk stratified by follow-up duration due to limited sample size or insufficient tracking time ( 7 , 10 , 15 ). A pooled analysis from randomized clinical trials documented that the causes of death in GPA differ in patients with early death compared to those with late death ( 9 ).…”

Anca Associated Vasculitis: Clinical Course and Outcome of 44 Patients From a Single Center in Turkey

“…Withdrawal of glucocorticoid therapy has been identified as a strong predictor of relapse; therefore, it is common practice to keep patients on low doses of prednisone (or equivalent) as part of the maintenance regimen. AZT, MTX, and mycophenolate mofetil have proven to be as effective as CYC for AAV maintenance therapy in randomized trials (Table 3) (20)(21)(22)(23)(24)(25)(26)(27)(28).…”

Clinical profile and management of granulomatosis with polyangiitis–associated scleritis from a tertiary care hospital in South India

“…[ 15 ] Relapses were defined as recurrence of GPA of sufficient severity that required treatment or increase in the dose of treatment on a previously stable patient. [ 4 ] Diminution of vision was defined as a decrease in visual acuity by two Snellen lines or more. An increase in visual acuity by two Snellen lines or more after resolution of the scleral inflammation clinically was denoted as improvement of vision.…”

“…It mainly affects the upper and lower respiratory tracts and kidneys. [ 3 4 ] Limited GPA usually spares the kidneys. Ocular GPA without respiratory and renal involvement is classified as very limited form of GPA.…”