Clinical summary: A 16-year-old, active, athletic youth was admitted to the hospital in 10/96 for dyspnea at rest and paroxysmal cough. He was healthy until 3 1/2 years before when he began to have intermittent episodes of abdominal discomfort, vomiting, and diarrhea with watery brown stools. Some episodes were followed by swelling of a foot and focal purpura, a biopsy of which showed a leukocytoclastic vasculitis. Laboratory values included WBC 7.4 k/µl, hb 11.3 g/dl, hct 36.3%, plt 542 k/µl, alkaline phosphatase 307 U/L, ALT 102 U/L, and ESR 20 mm/hr. When seen in clinic in late 1994, he appeared healthy (weight 50th centile; height 65th centile). He was afebrile with BP 115/60 mm Hg and HR 68/min. He had no hepatosplenomegaly or abdominal tenderness. There was no blood in the stool, and clubbing was absent. Blood values were unchanged. A gammaglutamyl transferase was 129 U/L; immune complexes were present. Endoscopic retrograde cholangiopancreatography suggested sclerosing cholangitis and an upper GI series was negative for Crohn's disease, but colonoscopic biopsies were consistent with that diagnosis. He had poor weight gain, and intermittent oral aphthous ulcers occurred, but he felt fine between attacks. He was treated with sulfasalazine. Three months later he was admitted for nausea, vomiting and a 9 pound weight loss over 10 days. Temperature was 37.1°, BP 98/60, hb 12 g/dl, plt 510 k/µl, and ESR 27 mm/hr. Liver function tests were normal. A p-ANCA (not anti-myeloperoxidase) was positive. Steroids were begun and then tapered. Other medications included 6-mercaptopurine and sulfasalazine. He was hospitalized two more times for his bowel disease, and responded each time to increased doses of prednisone.
In early August, 1996, he developed a non-productive cough followed by pleuritic pain and progressive dyspnea on exercise. There was no weight loss or hemoptysis. Intermittent fever to 102.7° and night sweats occurred. Purpura developed on the left ankle. His bowel disease was inactive. Medications included prednisone, 6-mercaptopurine, and sulfasalazine. He was treated with clarithromycin with no improvement. A chest film showed bilateral pleural effusions, and he was admitted on 8/30. Respiratory rate was 20/min. There was dullness to percussion bilaterally at the bases but no crackles above. WBC was 17.8 k/µl with a left shift, hb 9.8 g/dl, hct 29.1%, plt 785 k/µl. Total protein was 7.5 g/dl and albumin was 3.5 g/dl. ESR was >100 mm/hr. An exudative pleural effusion (protein 4.9 g/dl, glucose 51 mg/dl, LDH 1650 U/L, WBC 55.8 k/µl) was tapped. Five hundred milliliters of brown, sterile fluid was removed. He was discharged after 2 days on increased doses of prednisone but returned 10/5 following one week of dyspnea, paroxysmal cough, and fever to 104°. WBC was 22.5 k/µl, hb 11 g/dl, plt 669 k/µl, and ESR 93 mm/hr, but transaminases were normal. Oxygen saturation on room air was 96%. A chest film and CT scan showed bilateral lower lobe opacities. On bronchoscopy, friable, edematous mucosa was noted, but purulent secretions were absent. Culture of lavage fluid yielded no organisms. Two generous lower lobe biopsies were obtained by a left thoracotomy.
Histopathologic changes: Image 1 | Image 2 | Image 3 | Image 4Discussion
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