• Keishi Sugino, Kyoko Gocho, Fumiaki Ishida, Naoshi Kikuchi, Nao Hirota, Keita Sato, Go Sano, Kazutoshi Isobe, Susumu Sakamoto, Yujiro Takai, Yoshinobu Hata, Kazutoshi Shibuya, Toshimasa Uekusa, Atsuko Kurosaki, and Sakae Homma.
• Department of Respiratory Medicine, Toho University Omori Medical Center, Japan. ks142129_ikusou@ybb.ne.jp
• Intern. Med. 2012 Jan 1;51(22):3151-4.

AbstractImmunoglobulin G4 (IgG4)-related lung diseases can occur in patients with autoimmune pancreatitis (AIP). However, the causal relationship between AIP and acquired hemophilia A (AH) is unknown. We herein report the first case of AH associated with IgG4-related lung disease that developed in a patient with AIP. A 65-year-old asymptomatic man with a history of AIP and sclerosing cholangitis diagnosed at the age of 57 was admitted to our hospital due to an abnormal reticulonodular shadow on chest X-ray. An examination of lung biopsy specimens revealed IgG4-positive plasma cell infiltration in the interstitium. The serum IgG4 level was elevated. One year later, the patient developed a progressive severe hematoma in the left femoral muscle. On admission, laboratory examinations revealed severe anemia with a markedly prolonged activated partial prothrombin time, a decreased level of factor VIII (FVIII) activity, and the existence of anti-FVIII antibodies. These findings were consistent with a diagnosis of AH. No relapse has been observed over the past 25 months, during which time, corticosteroid therapy has been continuously administered.

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