Synonym: Klinger's syndrome; Wegener's granulomatosis
Granulomatosis with polyangiitis (GPA) - formerly known as Wegener's granulomatosis - is a rare form of vasculitis.1 Granulomatosis with polyangiitis It is thought to be an autoimmune inflammatory process affecting endothelial cells. It is a multisystem disease which can affect many parts of the body, categorised by the ELK classification: it most commonly presents with lesions in the upper respiratory tract (E indicating ears/nose/throat, almost 100%), lungs (L most patients) and kidneys (K >75%). Many other areas of the body may also be affected, with joint inflammation occurring in 25-50% of all cases. The sinuses, eyes and skin may also be affected.2 3 4
This is a companion discussion topic for the original article at https://patient.info/doctor/multisystem-diseases/granulomatosis-with-polyangiitis-wegeners-granulomatosis-pro