The Great Imitator Masquerading as Autoimmune Hepatitis and Demystifying as Fulminant Syphilitic Retinitis

Syphilis is a "Great Masquerader" because of its versatile clinical manifestations. We aim to report a patient whose first presentation was with presumed autoimmune hepatitis. Fulminant retinitis subsequently ensued, thus enabling correct diagnosis and treatment.

Case description.

A 62-year-old male presented with bilateral drop in vision. One month earlier, right eye (RE) arteritic ischemic optic neuropathy was suspected because of severe headache, sudden drop in vision, relative afferent pupillary defect, and elevated inflammatory markers. Systemic steroids were instituted. Brain imaging and temporal artery biopsy were unyielding. Four months earlier, liver biopsy performed because of elevated cholestatic liver enzymes, revealed granulomatous hepatitis. After ruling out viral hepatitis, autoimmune etiology was presumed, and prednisone was started. On presentation, visual acuity (VA) was counting fingers in RE and 6/20 in the left eye (LE). Bilateral panuveitis with punctate inner retinitis, placoid chorioretinitis was diagnosed. Serological tests were strongly positive for syphilis. Lumbar puncture confirmed the existence of neurosyphilis. Systemic penicillin was initiated. One month later, VA improved to RE 6/10, LE 6/7.5, with marked resolution of posterior uveitis. No recurrence was observed over 27-month-period.

Acquired syphilitic hepatitis is rarely reported. Administering steroids potentially aggravated the infection. The characteristic features of placoid chorioretinitis and punctate inner retinitis connected the pieces of the puzzle together to the diagnosis of ocular and neurosyphilis. Intrahepatic cholestasis with negative serological panel of hepatotropic pathogens should raise the suspicion of non-hepatotropic pathogens especially syphilis.