Abstract: PUB568
Systemic Lupus Erythematosus During Treatment of Autoimmune Hepatitis and Primary Biliary Cirrhosis
Session Information
Category: Trainee Case Report
- 1202 Glomerular Diseases: Immunology and Inflammation
Authors
- Kawanishi, Miharu, Shimane University Faculty of Medicine, Izumo, Shimane, Japan
- Sonoda, Hirotaka, Shimane University Faculty of Medicine, Izumo, Shimane, Japan
- Hoshino, Yuki, Shimane University Faculty of Medicine, Izumo, Shimane, Japan
- Yamauchi, Asuka, Shimane University Faculty of Medicine, Izumo, Shimane, Japan
Introduction
The complication of systemic lupus erythematosus(SLE) with autoimmune hepatitis(AIH) and primary biliary cirrhosis(PBC) is very rare. We report a case developed SLE during treatment of AIH PBC.
Case Description
A 50-year-old woman had been receiving treatment for type 2 diabetes mellitus since 201X-10. There was no decline in her renal function, and her urinary protein(Up) tested negative. She was admitted with liver dysfunction in January 201X-7. Liver biopsy was performed, resulting in a diagnosis of AIH and PBC. Up gradually progressed from January 201X. She was admitted to our department in April 201X. Renal biopsy was performed. Twenty-six glomeruli were collected, and 18 of them had lesions. There were many chronic lesions, and more than 50% of the glomeruli were noted. In renal pathology studies, IgG, IgA, IgM, C3, C4, and C1q tested positive by the fluorescent antibody technique. During a serological examination, antinuclear antibody and ds-DNA antibody tested positive, and the patient was diagnosed as having lupus nephritis type IV(G)(C) based on the Systemic Lupus International Collaborating Clinics(2012) and International Society of Nephrology/Renal Pathology Society(2003). She began 60 mg of oral prednisolone(PSL) treatment. Proteinuria improved from 2.03 g/day at the start of treatment to 0.42 g/day on the 22nd hospital day. PSL dose was reduced to 50 mg/day. Then, Up tended to gradually increase, and pancytopenia was also observed during the same time. We considered the possibility that disease activity was high; therefore, mycophenolate mofetil 1 g/day was started and increased to 2 g/day. The Up level was slightly exacerbated with PSL dose reduction but leveled off at 0.7-0.9 g/day and then did not change with PSL dose reduction.
Discussion
The complication of SLE with AIH and PBC is very rare. A previous study demonstrated that autoimmune disease accounts for only 4.7% of cases of liver enzyme elevation in SLE patients. On the other hand, liver enzyme elevations are often accompanied by SLE; 50% of SLE patients have liver enzyme elevations. Therefore, it is very important to differentiate causes of liver enzyme elevations in SLE patients. The complications of SLE and AIH or SLE and PBC are relatively rare; however, AIH, PBC, or complications of AIH and PBC should be considered if liver disease is found in SLE patients.