Systemic mononuclear inflammatory vasculopathy associated with Sjögren's syndrome in a patient with primary biliary cirrhosis.
We report a 46-year-old woman with primary biliary cirrhosis (PBC) presenting with Sjögren's syndrome and systemic mononuclear inflammatory vasculopathy. Biopsy specimens of sural nerve showed findings consistent with vasculitic neuropathy. Perivascular inflammatory mononuclear cell infiltration was observed on muscle biopsy specimen. The findings of abdominal computed tomography and brain magnetic resonance imaging were suggestive of vasculitis. Clinical manifestations and radiologic findings were improved after high dose prednisolone therapy.
Min
J K
JK
Department of Internal Medicine, Catholic University Medical College, Holly Family Hospital, Wonmi-Gu, Pucheon-City, Kyunggi-Do, Korea.
Park
K S
KS
Yu
W J
WJ
Lee
Y S
YS
Park
S M
SM
Park
S H
SH
Cho
C S
CS
Kim
H Y
HY
D001707:Biopsy, Needle; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000150:complications* / Q000175:diagnosis*; D008875:Middle Aged; D011241:Prednisone / Q000008:administration & dosage; D012859:Sjogren's Syndrome / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology; D016896:Treatment Outcome; D014657:Vasculitis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology
D011241:Prednisone