Background and Aims The first manifestation of systemic lupus erythrematosus (SLE) could vary, atypical, and often confusing. Hemolytic anaemia can be the only manifestation of SLE. With low index of clinical suspicion or inadequate follow up, the diagnosis of SLE could be delayed. We present a case of autoimmune hemolytic anaemia (AIHA) patient to describe the nature of AIHA which could evolve to SLE.

Methods Case report of a 12-year-old Indonesian female was admitted to Dr. Hasan Sadikin General Hospital with complaints of pallor, chest pain, prolonged fever and redness on her cheek. She was diagnosed with idiopathic AIHA but never routinely checked up after being discharged from the hospital 6 months ago. On admission she was alert, febrile, had muffled heart sound, and malar rash. Her laboratory investigation revealed anaemia (Hb: 6,8 g/dl), low C3 and C4, and increased titer of anti ds-DNA. Chest radiograph showed cardiomegaly and echocardiography showed pericardial effusion. A diagnosis of autoimmune hemolytic anaemia, systemic lupus erythrematosus, and pericardial effusion was confirmed. She was administered metilprednisolon 40 mg/day.

Results The patient had good response to metilprdnisolon. Fever, malar rash, chest pain start to resolve at day 4th of steroid administration. She was discharged at day 7th. One month after hospital discharge lupus activity disease remained stable and steroid was tappered off.

Conclusions We conclude that AIHA can be the only first symptoms of SLE. A careful observation of AIHA is important to prevent late diagnosis and treatment of SLE.