Unusual Acute Lupus Hemophagocytic Syndrome – A Test of Diagnostic Criteria: A Case Report

    July 2017 in “ Journal of Medical Case Reports ”
    Wijetunga Mudalige Udai Akalanka Wijetunga, Ravindra Satarasinghe, Balasuriya Mudiyanselage Dayananda, Ganhewage Kokila Darshani
    TLDR The 2012 criteria are better for diagnosing atypical lupus cases.
    A 57-year-old Sri Lankan woman presented with symptoms including fever, weight loss, confusion, and alopecia, and was diagnosed with hemophagocytic lymphohistiocytosis (HLH) due to an evolving autoimmune disorder, despite initially negative antinuclear antibodies (ANA). She improved with steroid treatment but was rehospitalized 6 months later with symptoms of systemic lupus erythematosus (SLE), confirmed by positive ANA and renal biopsy. This case highlighted the increased sensitivity of the 2012 Systemic Lupus International Collaborating Clinics criteria over the American College of Rheumatology criteria for diagnosing SLE, especially in atypical presentations.
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