Dissociated cholestasis at onset of systemic lupus erythematosus in a male patient: an atypical case

Introduction: Biliary involvement in systemic lupus erythematosus is uncommon, and dissociated cholestasis, defined as elevated alkaline phosphatase and gamma-glutamyl transferase with normal bilirubin, has rarely been described. Case description: A 41-year-old diabetic man without cutaneous manifestations presented with myalgia and progressive, disabling muscle weakness without creatine kinase elevation. On admission, he had dissociated cholestasis with normal transaminases and concurrent glomerular involvement (proteinuria and haematuria); magnetic resonance cholangiopancreatography was normal. Several days later, he developed rapidly progressive polyserositis requiring urgent pericardiotomy. Strongly positive antinuclear and anti-double-stranded DNA antibodies supported the diagnosis of systemic lupus erythematosus. He improved clinically and biochemically with methylprednisolone and hydroxychloroquine, followed by azathioprine at discharge; alkaline phosphatase and proteinuria normalised at six months, while gamma-glutamyl transferase remained elevated. Discussion: A normal cholangiogram, the acute course and improvement with immunosuppression made primary sclerosing cholangitis unlikely, although its small-duct variant cannot be excluded without liver biopsy, which was not performed. In the literature reviewed, the dissociated pattern in systemic lupus erythematosus has rarely been described. Conclusion: In this patient, dissociated cholestasis was a presenting manifestation of systemic lupus erythematosus that coincided with renal involvement and preceded severe serositis. It may be an early marker of lupus activity, a hypothesis that requires validation in a larger series.