Multisystemic disease with intracranial hypertension and autoimmune cytopenia in chronic active hepatitis.
A 14-year-old girl with postinfectious chronic hepatitis developed a life threatening autoimmune multisystemic illness with arthralgias, intracranial hypertension, Coomb's positive hemolytic anemia and hemorrhagic diathesis (due to antiplatelet and antifactor X antibodies). Mannitol and immunosuppressive therapy abolished acute symptoms. During the first year of follow-up, two attempts of decreasing prednisone below 15 mg/day caused recurrence of the hemolytic anemia and appearance of low titer antinuclear antibodies, while doses above 15 mg/day caused an increase in transaminase levels. Daily prednisone dose was therefore fixed at 15 mg. The patient was well and, although signs of mild hemolysis persisted, there was no anemia. Transaminases were found greatly elevated on two occasions. An autoimmune multisystemic steroid sensitive disease was seemingly initiated by a postinfectious steroid irresponsive chronic hepatitis.
D000293:Adolescent; D000743:Anemia, Hemolytic / Q000188:drug therapy / Q000209:etiology / Q000628:therapy; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy; D002908:Chronic Disease; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006505:Hepatitis / Q000150:complications* / Q000503:physiopathology; D006801:Humans; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D011559:Pseudotumor Cerebri / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy; D012008:Recurrence; D013921:Thrombocytopenia / Q000188:drug therapy / Q000209:etiology* / Q000628:therapy
D003520:Cyclophosphamide; D011241:Prednisone