ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (6)
Granulomatosis with polyangiitis - Pulmonary
See also in: Overview,External and Internal Eye,Oral Mucosal Lesion
Print
Other Resources UpToDate PubMed

Granulomatosis with polyangiitis - Pulmonary

See also in: Overview,External and Internal Eye,Oral Mucosal Lesion
Print Images (6)
Contributors: Philip I. Song MD, Susan Burgin MD, Lowell A. Goldsmith MD, MPH
Other Resources UpToDate PubMed

Synopsis

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.

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, long-term remissions are now possible in more than 90% of patients with the advent of cytotoxic therapy. 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

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

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.

Subscription Required

References

Subscription Required

Last Reviewed: 07/10/2017
Last Updated: 09/26/2017
Copyright © 2018 VisualDx®. All rights reserved.
Granulomatosis with polyangiitis - Pulmonary
See also in: Overview,External and Internal Eye,Oral Mucosal Lesion
Print 6 Images
View all Images (6)
(with subscription)
Granulomatosis with polyangiitis (General Manifestations) : Cough, Hematuria, Malaise, Palpable purpura, Proteinuria, Symmetric extremities, Weight loss, Arthralgia, Dyspnea, Pulmonary infiltrates, Bloody mucus
Clinical image of Granulomatosis with polyangiitis
Copyright © 2018 VisualDx®. All rights reserved.