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.
    This case report described a 57-year-old Sri Lankan woman with symptoms including fever, weight loss, confusion, and alopecia, initially diagnosed with hemophagocytic lymphohistiocytosis (HLH) due to an evolving autoimmune disorder. Despite initial negative antinuclear antibodies (ANA) and double-stranded DNA (dsDNA) antibodies, she responded well to steroid treatment. Six months later, she was diagnosed with systemic lupus erythematosus (SLE) confirmed by positive ANA and dsDNA antibodies, reduced complement levels, and a renal biopsy showing class IV-G lupus nephritis. The case highlighted the increased sensitivity of the 2012 Systemic Lupus International Collaborating Clinics criteria over the American College of Rheumatology criteria for diagnosing SLE, even when initial antibody tests are negative. This was the first reported case of its kind in Sri Lanka and South Asia.
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