Granulomatosis with polyangiitis (GPA) presents a wide spectrum of manifestations from the common respiratory symptoms to infrequent neurological and cardiac complications. The challenge in diagnosis and management makes the rapidly progressive disorder one of the most challenging dilemmas in clinical medicine.The ultimate goal is an improved prognosis through outcome measures which assesses the disease control with minimal adverse effects of intensive immunosuppressive regimens, an integral part of the clinical approach to improve the quality of life of GPA patients.