New Autoimmune Liver Disease or Immunologic Epiphenomenon? A Rare Case of Syphilitic Hepatitis Mimicking Autoimmune Liver Disease

Introduction: Syphilitic hepatitis (SH) is a rare manifestation of secondary syphilis presenting with cholestatic liver injury that can be confused with other cholestatic liver disease. We present a case of ocular syphilis and SH with positive anti-mitochondrial antibody (AMA) and anti-smooth muscle antibody (ASMA) which proved to be immunologic epiphenomena. Case Description/Methods: A 64-year-old male with hypertension, chronic kidney disease and Hepatitis C infection treated with ledipasvir and sofosbuvir in 2013 achieving SVR12 was admitted for orthostatic hypotension and acute right eye vision loss. Prior to this admission, he was evaluated by the hepatology outpatienservice for elevated alkaline phosphatase (ALP) and transaminases, and ultrasound finding of cirrhosis. His liver synthetic functions were all normal. Testing for chronic liver diseases was positive for AMA, ASMA and elevated immunoglobulins. The rest of the tests were negative. The patient previously used alcohol, but had been abstinent since 2013. He was scheduled for liver biopsy to rule out AIH/PBC overlap, but was postponed due to the admission. During the admission, ophthalmology was consulted, and he was found to have right eye panuveitis. Extensive testing for autoimmune conditions, vasculitides and malignancy was negative. Infectious testing came back positive for syphilis antibodies in the serum and CSF; HIV testing was negative. The patient was diagnosed with SH and ocular syphilis, and treated with penicillin-G 24 million units/day for 14 days. Liver enzymes eventually normalized. Following discharge, liver biopsy was performed showing no significant inflammatory infiltrates in the portal area, but found focal minimal lobular lymphohistiocytic inflammation. There was no evidence of interface hepatitis, bile duct injury, granuloma, or fibrosis. Discussion: Syphilis is known to be capable of compromising almost any organ. SH is a rare manifestation that has been described most often in HIV-infected patients. It usually presents in secondary syphilis with a predominantly cholestatic pattern of liver injury that resolves with appropriate treatment. In this case, the positivity of autoimmune markers with elevated alkaline phosphatase complicates the clinical picture and thus a liver biopsy was done to properly diagnose the disease. The positive antibody levels were felt to be secondary to SH, an immunologic epiphenomenon that has not previously been reported. This case again highlights syphilis as the great masquerader.Table 1.: Pertinent laboratory findings.