2355 Rare on Rare: A Simultaneous Presentation of SLE and PBC

Abstract

INTRODUCTION: Though autoimmune diseases commonly co-exist, systemic lupus erythematosus and concomitant primary biliary cirrhosis are uncommon. A recent literature review encompassing 34 cases noted that PBC was diagnosed in 69% of cases; in 5/34 cases, SLE and PBC were diagnosed simultaneously. CASE DESCRIPTION/METHODS: A 51 year old African American female with chronic kidney disease stage 3, hypertension, vitiligo presented with fatigue, anorexia, and >20 lbs weight loss over 3-4 months. Vitals were notable for blood pressure of 91/47, pulse 62, T 97.3F, and she was breathing room air. On exam, she appeared lethargic and fatigued. Scleral icterus and sublingual jaundice were noted. Discoid rash involving both ears was noted. Vitiligo of lower extremities was noted. Initial labs noted leukocytosis of 13.5 k/cumm, normocytic anemia, BUN 125 mg/dL, K 6.7 mmol/L, Cr 5.5 mg/dL, total bilirubin of 5.0, AST 86 U/L, ALT 218 U/L, ALP 1363 U/LDouble stranded DNA antibody was >13.0 IU/mL. F-actin and anti-smooth muscle antibody were negative. Anti-nuclear antibody was 1:2560. Anti-mitochondrial antibody (IgG) was 3.5. The patient’s hyperkalemia was treated, and after stabilization, patient underwent kidney biopsy and transjugular liver biopsy. These resulted, respectively, nephritis ISN/RPS Class III (C) and a cirrhotic liver severe bile ductular proliferation of the portal triads with absent bile ducts. Diagnoses of SLE with lupus nephritis and AMA negative PBC were made, and the patient was started on plaquenil and ursodiol. DISCUSSION: Liver involvement in patients with SLE can include primary liver disease as well as involvement due to SLE itself. Distinguishing between the true etiology of liver damage in these patients can be challenging, often due to confounding serological assays and use of immunosuppressive therapy in SLE. It is currently unknown whether SLE and PBC concurrence shares a common genetic or environmental factor, or if the diseases co-occur by chance. While liver dysfunction is not a diagnostic criteria for SLE, SLE related hepatitis (subclinical hepatopathy) has been described, particularly as a hypertransaminasemia. This is often noted during SLE flares, and improves or resolves with steroid therapy. Unfortunately, steroid therapy independent of other immunosuppressive therapies is associated with worsening outcomes in PBC, as well as development of fatty liver disease. Further exploration regarding co-occurrence and comanagement of SLE and PBC is needed, despite its rare incidence.