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Granulomatosis With Polyangiitis, With Atypical Features

Granulomatosis With Polyangiitis, With Atypical Features

A 49-year-old uninsured man from Mexico who had hypertension, diabetes and chronic rhinitis and sinusitis presented for hospitalization with two weeks of cough and hemoptysis, culminating in two days of chills, night sweats, left-sided chest pain that radiated to the shoulders, as well as dyspnea on exertion and orthopnea. 

mediastinal mass in granulomatosis with polyangiitis

A chest CT with contrast showed an 8.5 cm left mediastinal mass extending to the left hilum (above, white arrow), abutting the aorta (above, black arrow head 1), main pulmonary artery, and left pulmonary artery (above, black arrow head 2), encasing the left subclavian artery (below, arrow head 2) and origin of the left common carotid artery (below, white arrow 1). 

mediastinal mass in granulomatosis with polyangiitis

Two years ago, polyarthritis developed in his shoulders, wrists, hands, knees, and ankles. A year later, microscopic hematuria and proteinuria were noted, with normal creatinine clearance. 

Six months previously, a pacemaker had been implanted for complete heart block, after he presented with erythema of the left eye, pain, photophobia, headaches, and the sensation that his eyes were “popping out.” Our review of a chest CT taken at that time showed a large pericardial effusion, pulmonary vascular congestion with patchy ground-glass opacities bilaterally, interstitial infiltrates, pneumonitis in the right lower lobe, and a small left mediastinal mass. 

Highest on the list for differential diagnosis were neoplastic, infectious, and inflammatory causes. We performed an infectious workup, a lung biopsy, and autoimmune serologies.  

Click the link at right to read the clinical findings.

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