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A 39-year-old man was admitted to the hospital because of chest pain, arthralgias, and a mediastinal mass. Two months before admission, pain in the middle and right chest developed, which radiated intermittently to the right arm, increased in intensity with deep inspiration or changes in position, and was accompanied by fatigue and mild shortness of breath; He had had pericarditis 1 year earlier and optic neuritis 5 years earlier.

On examination, the first heart sound was absent, the second was loud with a prominent split, and there was a new systolic ejection murmur at the left upper sternal border. Imaging showed an infiltrative mediastinal mass surrounding the aorta and narrowing the lumen of the main and right pulmonary arteries.

The key elements of this case are the subacute appearance and progressive growth of a middle mediastinal mass affecting the pulmonary arteries and cardiac conduction system, the recent onset of arthritis, other extrathoracic organ system disease, previous and current glucocorticoid use, and a positive test for antineutrophil cytoplasmic autoantibodies (ANCA). The discussants review each of these as a way of arriving at a diagnosis.