Autoimmune cholangiopathy associated with systemic lupus erythematosus.
We report a 39-year-old female who presented over 11 years with autoimmune cholangiopathy associated with kaleidoscopic manifestations of systemic lupus erythematosus (SLE), including, arthritis, skin changes, pleuritis, diffuse proliferative glomerulonephritis, lymphadenopathy, splenomegaly, hyperglobulinemia, and major depression. While antimitochondrial antibodies (AMA) were absent, antinuclear (ANA) and anti-DNA antibodies were detected in high titres associated with hypocomplementemia. The patient also had vitamin B12 deficiency and antiphospholipid antibodies. The patient required steroids and repeated courses of cyclophosphamide for the management of lupus nephritis, and ursodeoxycholic acid (ursolite) administration resulted in amelioration of cholestatic laboratory abnormalities. This unusual case report and review of literature illustrate that immune liver disease may be an important clinical manifestation of SLE, especially autoimmune cholangiopathy.
Heyman
Samuel N
SN
Department of Medicine, The Hebrew University Medical School, H6adassah University Hospital, Mt. Scopus, Israel. Heyman@cc.huji.ac.il
Spectre
Galia
G
Aamar
Suhail
S
Rubinger
Dvora
D
Pappo
Orit
O
Ackerman
Zvi
Z
D000328:Adult; D000752:Anemia, Pernicious / Q000209:etiology; D000974:Antibodies, Antinuclear / Q000032:analysis; D017152:Antibodies, Antiphospholipid / Q000032:analysis; D018501:Antirheumatic Agents / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D015432:Glomerulonephritis, Membranoproliferative / Q000209:etiology; D006801:Humans; D008105:Liver Cirrhosis, Biliary / Q000188:drug therapy / Q000209:etiology* / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D016896:Treatment Outcome; D014580:Ursodeoxycholic Acid / Q000627:therapeutic use
D000974:Antibodies, Antinuclear; D017152:Antibodies, Antiphospholipid; D018501:Antirheumatic Agents; D014580:Ursodeoxycholic Acid; D003520:Cyclophosphamide