1 Clinical Presentation of GPA Jessica Meikle E2-CBL 10/13/2011

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Who? mostly older adults, but possible in all ages M=F caucasian

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

5 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

6

7 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.

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

9 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.

10 Livida Reticularis Purpura

11 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

12 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