Granulomatosis with polyangiitis: Indian experience

Granulomatosis with polyangiitis (GPA) is now a well-recognised clinical entity in India. The earliest description of GPA in India (then called Wegener's granulomatosis) appeared as a single case in a series of 108 cases of systemic vasculitis published in 1985. Later, several short case-series (mostly from northern India) and a number of case reports were published. The number of published cases of GPA from India till date is about 130 (actual burden of disease is certainly much larger as only a few cases get published). The overall clinical profile, response to treatment and long-term outcome of GPA do not differ significantly from what is described in western literature. A female preponderance has been noted in the Indian series, whereas no gender predilection was seen in western series. Although many cases used to masquerade as pulmonary tuberculosis in the past, the situation has improved considerably, with better awareness about the disease and availability of ANCA as a diagnostic test. High relapse rate and significant organ damage continue to pose a challenge. Cyclophosphamide combined with prednisolone is the standard induction regimen. Rituximab and its two biosimilars are being increasingly used in the management of refractory cases as well as for induction of remission in certain situations.