Aplastic Anemia Complicating Systemic Lupus Erythematosus

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disorder with a myriad of hematological manifestations. Pancytopenia occurs in 10%–50% of cases in these patients. Rare cases of aplastic anemia have been reported with SLE but mostly in adults. Clinical Description: A 6-year-old child presented with fever and painful multiple joint swelling for 6 months. Examination revealed pallor and polyarthritis involving small and large joints. Hemogram, bone marrow aspiration, and biopsy established the diagnosis of aplastic anemia. Since the clinical phenotype encompassed a childhood-onset multisystemic (musculoskeletal and hematological) chronic illness with constitutional symptoms, we considered included connective tissue disorders (CTDs), systemic-onset juvenile idiopathic arthritis with macrophage activation syndrome, hematological malignancy, and disseminated tuberculosis as differentials. Investigations confirmed the diagnosis of SLE. Management: The patient was treated with pulse doses of intravenous methylprednisolone for SLE and daily oral cyclosporine and supportive transfusions for aplastic anemia. She showed improvement in the constitutional symptoms and hematological parameters within 6 weeks. The arthritis resolved within 6 months of therapy, after which she was continued on low-dose steroids, hydroxychloroquine, and methotrexate. Conclusion: In cases of aplastic anemia with atypical features of CTDs such as arthritis, uveitis, oral ulcers, rash, and photosensitivity should be worked up for underlying autoimmune disorders.