Life threatening macrophage activation syndrome as the initial presentation of systemic lupus erythematosus: A case report and review of the literature

• Published in: Egyptian rheumatologist 2023-01, Vol.45 (1), p.45-49
• Main Authors: Tharwat, Samar, Hamdy, Fatma, Abdelzaher, Mohamed Gamal, Bakr, Leila, El Hassany, Aya, Abdelsattar, Mostafa, Eldesoky, Rehab T., Ibrahim, Eman M.
• Format: Article
• Language: English
• Subjects: Fever; Macrophage activation syndrome; Systemic lupus erythematosus
• ISSN: 1110-1164; 2090-2433
• DOI: 10.1016/j.ejr.2022.10.001

Macrophage activation syndrome (MAS) is characterized by activation of hemophagocytic cells and is a very rare initial manifestation of systemic lupus erythematosus (SLE). To describe the features of a challenging case of SLE initially presented with MAS. A previously healthy 20-year-old female was referred to the Rheumatology clinic with a 25-days’ history of persistent fever, general fatigue, and significant weight loss. Clinical examination revealed a high-grade fever (41 °C), significant hair loss and general malaise. Laboratory investigations revealed leucopenia (1.5 × 109/L), neutropenia (0.7 × 109/L), lymphopenia (0.6 × 109/L), normocytic normochromic anemia (9 g/dL), thrombocytopenia (95 × 109/L), elevated liver enzymes, hyperbilirubinemia (2.6 mg/dl); hypertriglyceridemia (306 mg/dL), hyperferritinemia (16000 ng/mL), elevated D-Dimer (4.7 mg/L), elevated lactate dehydrogenase (1379 U/L) and direct and indirect Coombs' tests were positive. Erythrocyte sedimentation rate was 28 mm/1st h. Immunological screening was positive for antinuclear antibody (ANA) and anti-double stranded deoxyribonucleic acid (anti-dsDNA). Serum C3 and C4 complement factors were low and C reactive protein was negative. Soluble CD25 was elevated (9800 pg/ml). Bone marrow biopsy showed hypocellular bone marrow with normal megakaryocytes and erythroid series with evidence of hemophagocytosis. A diagnosis of SLE initially presented with MAS was made. The patient received intravenous methylprednisolone for 5 consecutive days followed by prednisolone and cyclosporine A and intravenous immunoglobulin. The condition deteriorated with progressive coagulopathy, thrombocytopenia and hyperferritinemia. Subsequently, the conscious level of the patient deteriorated, multiorgan failure developed and the patient died. MAS is a serious condition that may be the first presentation of SLE.