Charles M. Wiener, M.D., Victorine V. Muse, M.D., and Eugene J. Mark, M.D.
Presentation of Case
The patient had been in her usual state of health until approximately 3 weeks before admission, when dyspnea developed. Approximately 1 week later, she began to have tightness in the chest on exertion, as well as burning pain in the leftsubscapular region that radiated down the left arm and was relieved by massage therapy, acetaminophen, and ibuprofen. The dyspnea gradually increased in severity, until she became short of breath while walking up one flight of stairs or moving a lawn chair a few feet. She had a mild dry cough but no fever, chills, hemoptysis, nausea, vomiting, diaphoresis, night sweats, weight loss, anorexia, jointpain, or rashes.
On the afternoon before admission, the patient went to the emergency department of another hospital because of progressive dyspnea. On examination, she appeared comfortable. She rated the subscapular pain as 3 on a scale of 0 to 10, in which 10 is the most severe. The blood pressure was 104/72 mm Hg, the pulse 105 beats per minute, the temperature 36.4°C, the respiratory rate 20breaths per minute, and the oxygen saturation 93% while the patient was breathing ambient air. There were rales in both lungs, extending halfway up from the bases. The remainder of the examination was normal. An analysis of arterial blood while the patient was breathing ambient air revealed a pH of 7.40, a partial pressure of carbon dioxide of 38 mm Hg (reference range, 35 to 45), a partial pressureof oxygen of 69 mm Hg (reference range, 75 to 100), and a serum total carbon dioxide content of 22.5 mmol per liter (reference range, 20 to 26). Other laboratory-test results are shown in Table 1. An electrocardiogram revealed sinus tachycardia but was otherwise normal. A chest radiograph revealed mild, diffuse air-space disease in both lungs and no pleural effusions; the heart was normal insize. Computed tomography (CT) of the chest showed multiple patchy infiltrates peripherally in both lung bases. The central airways were clear; the pulmonary artery was 2.2 cm in diameter, and there was no evidence of pulmonary embolus or lymphadenopathy. She was transferred to this hospital
The patient had had ulcerative colitis for more than 10 years, with recurrent episodes of cramps, diarrhea,tenesmus, urgency of defecation, and mucus. She had been treated intermittently with balsalazide, hydrocortisone enemas, azathioprine, and tapering courses of oral prednisone. Nine months earlier, persistent bloody diarrhea developed that did not respond to prednisone; 6 months before admission, prednisone was discontinued. A skin test for tuberculosis was negative; a 90-day course of azathioprineand monthly infusions of infliximab were begun. The most recent dose of infliximab had been administered 15 days before admission. Regular screening colonoscopies showed pancolitis with no evidence of dysplasia. There was a history of osteoporosis, sinusitis, and bronchitis. At the time of the onset of symptoms, she lived with her husband in the southern United States and had been exposed to smokefrom fires in the area. During the preceding 6 months, she had traveled to the southwestern United States, and had been in contact with a person who was taking antibiotics for bronchiectasis. Ten days before admission, in late spring, she had traveled by car and boat to an island off the coast of Massachusetts, arriving 6 days before admission. She drank less than one glass of wine daily, hadstopped smoking cigarettes 30 years earlier, and owned a dog. There was no exposure to tuberculosis or to birds, and she did not recall recent insect or tick bites. Other medications included balsalazide disodium, ibandronate sodium, and conjugated estrogen. She was not allergic to any medications.
On examination, the blood pressure was 130/74 mm Hg, the pulse 88 beats per minute, the respiratory...