What is the natural history of Granulomatosis with polyangiitis?
The presentation and natural history of GPA are highly variable. The spectrum of clinical presentation may range from relatively mild disease limited to the upper respiratory tract to fulminant life-threatening involvement of the upper and lower respiratory tract, kidneys, and other end-organs. The disease progression is also variable and protean, including protracted mild disease remaining in the upper respiratory tract despite absence of treatment, widespread but relatively mild and slowly progressive disease, and rapidly progressive pulmonary and renal disease manifesting as alveolar hemorrhage syndrome and rapidly progressive renal failure upon presentation. A further caveat is the observation that relatively mild and limited disease may rapidly progress to more diffuse and active disease at any time during the course of disease in at least 10% of patients. Untreated generalized GPA is uniformly fatal.
