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Granulomatosis with polyangiitis
See also in: External and Internal Eye,Oral Mucosal Lesion
Other Resources UpToDate PubMed

Granulomatosis with polyangiitis

See also in: External and Internal Eye,Oral Mucosal Lesion
Contributors: Vivian Wong MD, PhD, Philip I. Song MD, Michael W. Winter MD, Susan Burgin MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed


Granulomatosis with polyangiitis (GPA), formerly known as Wegener granulomatosis, is a multisystem disease of unknown etiology characterized by vasculitis and necrotizing granulomatous inflammation of the small arteries and veins. Classic generalized GPA is a triad of necrotizing granulomas of the upper and lower respiratory tract, systemic necrotizing small vessel granulomatous vasculitis, and glomerulonephritis (usually focal segmental). GPA most often occurs in patients in middle age and of Northern European descent (80%-90% of cases), but cases have been reported in children and older adults.

The disease typically presents with upper airway disease and nonspecific complaints such as malaise, arthralgias, fever, and weight loss. Respiratory tract symptoms include sinusitis, otitis media, rhinorrhea, cough, sputum production, chest pain, dyspnea, and hemoptysis. Ocular involvement occurs in 30%-50% of patients with GPA and can occur without systemic manifestations. Oral lesions can be seen with any of the clinical presentations of GPA and consist of ulcerations and/or "strawberry" gingivitis.

The disease presents variably on a continuum from limited involvement (which may remain limited for variable periods of time) to a more generalized form with involvement of the upper and lower respiratory tract, kidney, skin, and other organs. Untreated, the generalized form is fatal in less than 2 years; however, with the advent of cytotoxic therapy, long-term remissions are possible in more than 90% of patients.

Complications of GPA include pericarditis, coronary arteritis, myocardial infarction, pancarditis, granulomatous valvulitis, mononeuritis multiplex, and polyneuritis. Laryngotracheal stenosis with stridor can be a complication of GPA and initially may be misdiagnosed as asthma.


M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement

195353004 – Wegener's granulomatosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Microscopic polyangiitis (MPA) – Absence of granulomatous inflammation and sparing of upper respiratory tract. Overlap of clinical phenotypes is common.
  • Eosinophilic granulomatosis with polyangiitis – Associated with peripheral eosinophilia and a history of asthma.
  • Leukocytoclastic vasculitis
  • Sarcoidosis
  • Cryoglobulinemia
  • Cryofibrinogenemia
  • Systemic lupus erythematosus (SLE)
  • Polyarteritis nodosa
  • Coccidioidomycosis
  • Cocaine levamisole toxicity can often present with positive antineutrophil cytoplasmic autoantibodies (ANCA) studies and may mimic vasculitides.
  • Drug-induced Vasculitis with positive ANCA studies (propylthiouracil, hydralazine, anti-tumor necrosis factor alpha, sulfasalazine, D-penicillamine, minocycline)
  • Deep fungal and mycobacterial infections (eg, Mucormycosis, Tuberculosis) – May have pulmonary symptoms that mimic GPA. Biopsy should demonstrate the causative organisms.
  • Pyoderma gangrenosum
  • Calciphylaxis
  • Primary hyperoxaluria
  • Ecthyma gangrenosum
  • Chronic meningococcemia
  • Strongyloidiasis
  • Type 2 lepra reaction
  • Immunoglobulin A vasculitis
  • Leukemic Vasculitis
  • Embolism (septic, Fat emboli, Air embolism, Cholesterol emboli, marantic)
  • Hypercoagulable state
  • Disseminated intravascular coagulation
  • Purpura fulminans
  • Warfarin necrosis
  • Deficiency of adenosine deaminase type 2
  • Other collagen vascular diseases or vasculitis (eg, Rheumatoid arthritis, Dermatomyositis)
  • Giant cell arteritis

Best Tests

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Management Pearls

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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Last Reviewed:05/17/2020
Last Updated:12/06/2023
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Granulomatosis with polyangiitis
See also in: External and Internal Eye,Oral Mucosal Lesion
A medical illustration showing key findings of Granulomatosis with polyangiitis (General manifestations) : Chest pain, Cough, Fever, Hematuria, Malaise, Proteinuria, Rhinorrhea, Arthralgia, Dyspnea, Hemoptysis, Myalgia, Pulmonary infiltrates
Clinical image of Granulomatosis with polyangiitis - imageId=153379. Click to open in gallery.  caption: 'Hemorrhagic bullae and vesicles on the distal toes and faint, maroon-colored patches on the dorsal feet.'
Hemorrhagic bullae and vesicles on the distal toes and faint, maroon-colored patches on the dorsal feet.
Copyright © 2024 VisualDx®. All rights reserved.