DOI: 10.36347/sjams.2019.v07i07.015

Pages: 2339-2343

Introduction: Granulomatosis polangitis is a granulomatous vasculitis involving small vessels, the diagnosis is by circulating antibodies against cytoplasmic component of neutrophils (c-ANCA) which have been detected in more than 90% patients with active GPA and histopathological hallmark of the disease small vessel vasculitis. Since the introduction of steroids and cyclophosphamide as a standard immunosuppressive regimen, survival of patients diagnosed with GPA has improved considerably. Despite the ability to successfully induce remission, 50–70% of remissions are later associated with one or more relapses. Case report: 48 year old male was diagnosed with GPA on histopathology and c-ANCA titres, and was treated with steroids and cyclophosamide for 18months and Azathioprine for 3yrs of maintainance therapy and treatment was stopped gradually. After 2 and ½ years off treatment, patient was diagnosed as Relapse of GPA, and as per guideline recommendations, we started our patient on cyclophosphamide with combination of glucocorticoids. Conclusion: Granulomatosis with polyangiitis is a chronic relapsing disease. Its relapse rate rises from 20% at 12 months to almost 60% after 5 years [2]. Each relapse may result in further morbidity; hence recognition of a relapse is essential. In our patient, on follow up after 3 months of treatment, there was significant clinical and radiological improvement of the disease on cyclophosphamide along with glucocorticoids and hence the use of Rituximab can be preserved for refractory cases of relapse not responding to cyclophosphamide.