Hippokratia 2004, 8(4):151-154
P Boura, S Papadopoulos, K Tselios, P Skendros, O Dioritou, G Malamis, P Makris, N Lefkos
Division of Clinical Immunology, 2nd Dpt of Medicine, Aristotle University of Thessaloniki, Greece
Intensive Care Unit, Hippokratio General Hospital, Thessaloniki, Greece
Coagulation and Haemostasis Unit, AHEPA Hospital, Thessaloniki, Greece
A 31-year-old woman was admitted to the hospital for the investigation of left lower limb thrombophlebitis. History revealed one 1st trimester abortion, arthralgias, photosensitivity and leucopenia during the last months. Laboratory investigation showed positive antinuclear antibodies (ANA) and high titers of anticardiolipin antibodies (ACA). The patient was diagnosed to suffer from SLE and secondary APS. Treatment included steroids, azathioprine, aspirin and low molecular weight heparin (LMWH). Sixty-three days later, she was admitted to the hospital again because of high fever, macroscopic hematuria and dyspnea. Laboratory testing showed anemia and impaired renal function. High-resolution chest CT revealed bilateral multiple peribronchial infiltrates with hemorrhage. MRI angiography of the kidneys revealed left renal vein thrombosis combined with ischemia of the left kidney. Cyclophosphamide and methylprednisolone pulse treatment as well as intravenous immunoglobulins were started immediately. On day 3, the patient developed acute respiratory failure and was transferred to the ICU. Despite intensive immunosuppressive and supportive treatment, she suffered three relapses of alveolar hemorrhage and died on day 40, due to severe intracerebral bleeding. Final diagnosis was catastrophic APS with diffuse alveolar hemorrhage and kidney involvement. The rarity and individuality of the patient, concerning disease aggressiveness and therapeutic interventions is discussed.