Cryofibrinogenemia associated with Sjögren's syndrome: a case of successful treatment with high-dose corticosteroid.
Cryofibrinogenemia (CF) has not been often reported as a complication of various rheumatic diseases. We describe a 44-year-old woman with CF associated with Sjögren's syndrome (SS), who developed digital necrotic ulcerations and purpura of the lower legs. Cryoprecipitate was detected in her plasma, and immunoelectrophoresis showed that the cryoprecipitate was cryofibrinogen. Alprostadil was intravenously administered, but the ulceration was aggravated. Subsequently, administration of high-dose prednisolone (PSL) at 60 mg/day was started, and the ulceration remarkably improved. Cryofibrinogen, detected before the administration of high-dose PSL, was negative after PSL. This is the first case presentation of CF associated with SS successfully treated with high-dose corticosteroid.
Yoshida
Ken
K
Division of Rheumatology, Department of Internal Medicine, Jikei University School of Medicine, Tokyo. k.yoshida@jikei.ac.jp
Yokoyama
Toru
T
Toyokawa
Yasuhiko
Y
Yasuda
Jun
J
Kingetsu
Isamu
I
Kurosaka
Daitaro
D
Yamada
Akio
A
D000328:Adult; D003449:Cryoglobulinemia / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D003450:Cryoglobulins / Q000378:metabolism*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D015241:Fibrinogens, Abnormal / Q000378:metabolism*; D005500:Follow-Up Studies; D006801:Humans; D011239:Prednisolone / Q000008:administration & dosage*; D018570:Risk Assessment; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D016896:Treatment Outcome
D003450:Cryoglobulins; D015241:Fibrinogens, Abnormal; C041874:cryofibrinogen; D011239:Prednisolone