A vasculitis of small and medium sized vessels characterized by the presence of necrotizing granulomas.
Formerly known as Wegener’s granulomatosis.
GPA because inflammation of the upper respiratory tract resulting in rhinitis, otitis, subglottic stenosis, and sinusitis.
Organ involvement can be variable, leading to delay in diagnosis due to the incongruous nature of symptoms.
Up to 70% of patients with granulomatosis with polyangiitis are chronic nasal carriers of Staphylococcus aureus, with carriers having an eight times increased risk of relapse: considered a type II hypersensitivity reaction.
Acute phase reactants are elevated, and cANCA positivity occurs and 75%-90% of cases.
Diagnostic diagnosis is confirmed with a combination of clinical picture, serpositivity for c-ANCA and biopsy results.
Patients with GPA most commonly have a cytoplasmic ANCA pattern and antibodies directed against proteinase3, located in neutrophils and monocytes.
A small number of patients have a perinucleus staining pattern P-ANCA with antibodies typically directed against myeloperoxidase.33“21
Differential diagnosis includes: Chung-Strauss syndrome, Goodpasture syndrome, and sarcoidosis.
Every patient with suspected granulomatosis with polyangoitid should have cANCA testing, evaluation for sinusitis, and histopathologic examination of biopsy specimens.
Untreated is uniformly fatal.
GPA causes necrotizing granulomatous inflammation in small blood vessels throughout the body, most commonly affecting the capillary beds of the kidneys, lungs, and skin.
Renal and pulmonary involvement can progress rapidly and the diagnosis warrants urgent management.
Rapidly progressive glomerulonephritis occurs in up to 70% the patients with GPA.
commonly affects the lower respiratory tract with lung granuloma, alveolar hemorrhage.
Associated with significantly increased risks of myocardial infarction and ischemic stroke.
Relapses occur in more than 50% of patients.