A Review Article on Granulomatosis with Polyangitis

Abstract

Wegener's granulomatosis, also known as granulomatosis with polyangitis (GPA), is a rare inflammatory illness characterised by vasculitis and granulomatosis. Upper & lower respiratory tract and kidneys are the organ systems that gets affected in GPA. Granulomatosis with polyangitis is characterised by necrotizing granulomas with pauci-immune vasculitis. GPA prevalence in Asians ranges between 0.37/million to 2.1/million populations. Symptoms usually occur depending on organ systems that get affected. Initial symptoms in the respiratory tract may include mucous membrane ulcers with secondary bacterial infection, chronic runny nose, sinus pain, and chronic middle ear infection, resulting in hearing loss. Clinical features along with physical examination, laboratory tests, X-Ray, MRI or CT scan and biopsy (sometimes) together help to prove diagnosis of GPA. The two stages in the treatment of granulomatosis with polyangitis are inducing remission of symptoms and maintenance therapy. Mainstay of treatment is immunosuppressant's and is selected on the basis of severity of the disease.