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Clinical Presentation of GPAJessica MeikleE2-CBL10/13/2011

What’s in a Name?

http://www.nytimes.com/2008/01/22/health/22dise.html?_r=1&oref=slogin

Who?

•mostly older adults, but possible in all ages

•M=F•caucasian

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Constitutional

•fever, migratory arthralgias, malaise, anorexia and weight loss; weeks to months

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ENT

•Nasal crusting, sinusitis, otitis media, persistent rhinorrhea, purulent/bloody nasal discharge, oral and/or nasal ulcers

•Also earache, both conductive and sensorineural hearing loss, or otorrhea

•Saddle nose typical

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Pulmonary

•involvement of airways or pulmonary parenchyma: hoarseness, cough, dyspnea, stridor, wheezing, hemoptysis or pleuritic pain

•There may also be: tracheal or subglottic stenosis, pulmonary consolidation and/or pleural effusion.

•Lung nodules common, tumor-like masses (breast, kidney) are possible but rare.

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Renal

•acute renal failure with hematuria, red cell and other casts, and proteinuria

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Skin

•most common skin lesion is leukocytoclastic angiitis which causes purpura (lower extremities)

• focal necrosis and ulceration possible •Skin lesions may also include urticaria,

livida reticularis, and tender nodules.

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Livida Reticularis

Purpura

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Other• Eyes (conjunctivitis, corneal ulceration,

episcleritis/scleritis, optic neuropathy, nasolacrimal duct obstruction, proptosis, diplopia, retinal vasculitis, and uveitis)

• Nervous system (mononeuritis multiplex, cranial nerve abnormalities, central nervous system mass lesions, external ophthalmoplegia, hearing loss)

• Less common: GI tract, heart (pericarditis, myocarditis, conduction system abnormalities), lower GU tract (including the ureters and prostate), parotid glands, thyroid, liver, or breast

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Limited Form

•1/4 of cases•just upper resp tract symptoms•younger, women•more likely to have saddle nose•less likely to be ANCA pos•likely to develop renal disease (80%)

Sources•Up-to-Date:  Clinical manifestations and

diagnosis of granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis