Goodpasture's Syndrome Initially Presenting with Alveolar Hemorrhage
Ichirota Nakajima1) Masahiro Sasaki1) Takefumi Ito1) Akiko Watanabe1) Masaaki Sano1) Masayuki Kasima1) Manabu Kagaya1) Atusi Komatuda2) Hideki Wakui2) Yuichi Imai2) Takanobu Shioya3) Mamoru Miura1)
1)Second Department of Internal Medicine, and 2)Third Department of Internal Medicine, Akita University School of Medicine 3)Department of Occupational Therapy, College of Allied Medical Science, Akita University
A 17-year-old man was admitted to our hospital because of exertional dyspnea, fever, and hemoptysis. Chest X-ray films disclosed diffuse alveolar infiltrates and patchy shadows in both lungs. Laboratory data included a hemoglobin level of 6.7 g/dl and white blood cell count of 8,100/μ l. Urinalysis revealed microscopic hematuria with RBC cast. Bronchoalveolar lavage fluid from the right middle lobe bronchus was bloody. Anti-GBM antibodies were detected at low levels in serum (10 EU/ml) by ELISA procedures. Renal and lung biopsies were performed. Immunofluorescent studies revealed linear deposits of IgG along glomerular basement membrane of the kidney, but not in alveolar walls of the lung. This case fulfilled the criteria for Goodpasture's syndrome. The patient was treated with methylprednisolone (1,000 mg/day, for 3 days) and plasma exchange (for 2 days), and demonstrated a dramatic improvement in his clinical condition and chest X-ray findings. We were unable to identify autoantibodies to the NC domain of the alpha 3 chain of type IV collagen. Another conformational epitope may play a role in the disease.
Goodpasture's Syndrome Alveolar hemorrhage Anti-glomerular basementmembrane antibody
Received 平成10年12月21日
JJRS, 37(8): 652-657, 1999