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Granulomatosis with polyangiitis - Oral Mucosal Lesion
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Granulomatosis with polyangiitis - Oral Mucosal Lesion

See also in: Overview,External and Internal Eye
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Contributors: Vivian Wong MD, PhD, Philip I. Song MD, Michael W. Winter MD, Susan Burgin MD, Lowell A. Goldsmith MD, MPH
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Synopsis

Granulomatosis with polyangiitis (General manifestations) : Chest pain, Cough, Fever, Hematuria, Malaise, Proteinuria, Rhinorrhea, Arthralgia, Dyspnea, Hemoptysis, Myalgia, Pulmonary infiltrates
General manifestations
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.

Codes

ICD10CM:
M31.30 – Wegener's granulomatosis without renal involvement
M31.31 – Wegener's granulomatosis with renal involvement

SNOMEDCT:
195353004 – Wegener's granulomatosis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

  • Drug-induced gingival hyperplasia may have a micropapillary surface, but no petechiae are seen. The patient also has a history of taking phenytoin, cyclosporin, or one of the calcium channel-blocking agents.
  • Gingival peripheral ossifying fibromas
  • Leukemic oral infiltrate is not typically micropapular, and patients often will have systemic signs and symptoms (eg, fatigue, shortness of breath, pallor).
  • Lymphoma is usually localized and would not typically have pulmonary or kidney involvement.
  • The gumma of tertiary syphilis may appear similar to GPA.
  • Deep fungal and mycobacterial infections may have pulmonary symptoms that mimic GPA. Biopsy should demonstrate the causative organisms.
  • Cocaine mucosal ulcer (oral, nasal)
  • Giant cell arteritis
Gingival hyperplasia (although none of these has the micropapular surface with superimposed petechial hemorrhage):
  • Gingival fibromatosis syndromes
  • Inflammatory gingival hyperplasia usually has a smooth surface with no petechiae.

Best Tests

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Management Pearls

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Therapy

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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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References

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Last Reviewed:05/17/2020
Last Updated:09/27/2022
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Granulomatosis with polyangiitis - Oral Mucosal Lesion
See also in: Overview,External and Internal Eye
Captions Print   View all Images (8)
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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.

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