Clinical profile and long-term outcome of granulomatosis with polyangiitis (GPA): A corporate hospital-based study from northern India

Abstract

ObjectiveTo study the clinical profile and long-term outcome of granulomatosis with polyangiitis (GPA). MethodsRecords of patients with GPA attending our rheumatology clinic between April 2009 and February 2015 were retrieved. Clinical and laboratory data, details of treatment given and outcome were obtained and analysed. Remission and relapse were defined according to British Society for Rheumatology guidelines. Vasculitis damage index (VDI) was used for quantifying organ damage. ResultsThere were 45 patients (26 female, 19 male) with the mean age of 46±12 years and median disease duration at diagnosis of 20 months. Lung, ear, musculoskeletal system, eye, nose and kidney were most frequently involved in that order. Disease was severe in 29 and limited in 16. Miscellaneous presenting features included saddle-nose deformity in 5 patients, digital gangrene in 2 and complete heart block, renal mass and renal infarction in 1 each. Tissue biopsy contributed to diagnosis in 26/30 (86%). Anti-neutrophil cytoplasmic antibody was positive in 33 (73%) patients. Prednisolone+cyclophosphamide (oral in 25, intravenous in 10) and prednisolone+rituximab were initially used for inducing remission in 35 and 4 patients, respectively. Rituximab was also used for induction in 4 patients who failed on cyclophosphamide. Mycophenolate or methotrexate were used as immunosuppressants in limited disease. Azathioprine was used for maintenance of remission. Median follow-up was 49 months. One patient died of intractable diffuse alveolar haemorrhage and stroke within 3 months. 34 patients achieved complete remission. Sustained remission (≥2 years) occurred in 24 (median 3.5 years). Major relapse occurred in 24 (53%). Of these, 3 had a second and 2 had a third relapse. Two patients relapsed after 11 years in remission. Preferred treatment for relapse included rituximab and cyclophosphamide. Mean VDI of patients in this series was 3.02. ConclusionGPA remits with standard treatment but relapses frequently, leading to substantial morbidity. Mortality is uncommon.