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Granulomatosis with polyangiitis - External and Internal Eye
See also in: Overview,Oral Mucosal Lesion,Pulmonary
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Granulomatosis with polyangiitis - External and Internal Eye

See also in: Overview,Oral Mucosal Lesion,Pulmonary
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Contributors: Philip I. Song MD, Susan Burgin MD, Harvey A. Brown MD
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.

Ocular involvement occurs in 30%-50% of patients with GPA and can occur without systemic manifestations. There can be orbital, retinal, nasolacrimal, and anterior segment involvement. The most common findings are scleritis and orbital disease. Ocular manifestations of GPA result from granulomatous sinusitis infiltrating the orbit, causing nasolacrimal duct obstruction and proptosis, putting increased pressure on the intraocular muscles and optic nerve. Many patients complain of decreased vision. Vision may also be lost, as proptosis can lead to exposure keratopathy and corneal ulceration. There may also be a focal vasculitis, such as conjunctivitis, episcleritis, scleritis, uveitis, or granulomatous vasculitis of the retina and optic nerve.

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.

This summary will focus on ocular manifestations of the disease.

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

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

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: 07/10/2017
Last Updated: 07/26/2017
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Granulomatosis with polyangiitis - External and Internal Eye
See also in: Overview,Oral Mucosal Lesion,Pulmonary
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View all Images (14)
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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.