Autoimmune hepatitis associated with Kikuchi-Fujimoto's disease.
We describe a 20-year-old woman with autoimmune hepatitis (AIH) with cirrhosis who developed Kikuchi-Fujimoto's disease (KFD) and de novo minor features of systemic lupus erythematosus (SLE). This is the first report of a patient with histologically confirmed AIH developing KFD (histiocytic necrotizing lymphadenitis). One previous case described KFD after AIH (diagnosed clinically but without biopsy). KFD is a rare condition of unknown aetiology, first described in 1972, characterized by fever and cervical adenopathy and has a self-limiting course. KFD is associated with SLE, and SLE in turn can be associated with abnormal liver function tests, which in a minority of cases may be due to AIH. The association of AIH, KFD, and SLE in our patient suggests an autoimmune pathogenesis of KFD.
Shusang
Vibhakorn
V
Liver Transplantation Hepatobiliary Unit, Royal Free Hospital, Pond Street, Hampstead, London, UK. boo.md@hotmail.com
Marelli
Laura
L
Beynon
Huw
H
Davies
Neil
N
Patch
David
D
Dhillon
Amar Paul
AP
Burroughs
Andrew K
AK
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D005260:Female; D019693:Hepatitis, Autoimmune / Q000150:complications*; D020042:Histiocytic Necrotizing Lymphadenitis / Q000188:drug therapy / Q000209:etiology*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000209:etiology*; D008198:Lymph Nodes; D008775:Methylprednisolone / Q000627:therapeutic use; D012008:Recurrence; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D008775:Methylprednisolone