Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides are systemic autoimmune diseases that often present as non-specific prodromal symptoms such as fever, fatigue, headache, and weight loss.1 With modern treatment, the disease has changed from being universally fatal to being treatable. It can have a chronic relapsing and remitting course. Therefore, early diagnosis is necessary.2,3


A 53-year-old male with a past medical history of chronic sinusitis and some epistaxis needing multiple courses of antibiotics and steroids, was admitted after being diagnosed with high-grade heart block at another institution. He was found to have a heart rate of 26 and was given a temporary pacemaker prior to the transfer. Subsequently, a dual-chamber pacemaker was placed. An echocardiogram showed mild aortic stenosis and regurgitation with right ventricular enlargement and an ejection fraction of 65%. No etiology was found for the heart block and work up was negative for medications, electrolytes, ischemia, significant structural heart disease or lyme disease. Pacer placement was without any complications. The patient was discharged home and did well for 10 days and then returned again to the emergency room with complaints of dry cough, chest pain, progressive shortness of breath, fatigue, and fever to 101.2 degrees Fahrenheit. He reported a fifteen pound weight loss over the past month. His family history was noncontributory. He worked as an auto mechanic and reported former cocaine use over 20 years ago. He had no history of tuberculosis exposure and was up to date on his screening colonoscopy.

Physical exam findings included mildly congested bilateral nares with some postnasal thick secretions. On careful examination, he had “saddle nose” anatomy that he claimed to have noticed just months prior to presentation. Auscultation of his lungs revealed crackles diffusely and posterior left lung base egophony but no wheezes. The lung findings were new as compared to the recent admission for heart block. There was nothing significant on the basic laboratory tests. Chest x-ray and CT scan on admission revealed diffuse bilateral airspace opacities and nodule consolidation with mediastinal and right hilar adenopathy. These findings were suspicious for multifocal pneumonia with a separate infectious inflammatory process.