Intracerebral hemorrhage in a patient with SLE and catastrophic antiphospholipid syndrome (CAPS): report of a case

Abstract

A 31-year-old woman was admitted to the hospital for investigation of left lower limb thrombophlebitis. History, physical examination, and laboratory investigations led to the diagnosis of systemic lupus erythematosus (SLE), complicated by secondary antiphospholipid syndrome (APS). Treatment included steroids, azathioprine, aspirin, and low molecular weight heparin. 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 computed tomography (CT) revealed bilateral multiple peribronchial infiltrates with hemorrhage. Magnetic resonance imaging (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. Despite intensive immunosuppressive and supportive treatment, she suffered three relapses of alveolar hemorrhage and died on day 40, due to severe intracerebral bleeding. The final diagnosis was catastrophic APS with diffuse alveolar hemorrhage and kidney involvement. The unusual combination of recurrent alveolar hemorrhage and death from intracerebral hemorrhage rather than thrombosis in a CAPS patient is discussed.

Histopathological analysis of cerebral hemorrhage in systemic lupus erythematosus complicated with antiphospholipid syndrome

“…Although intracranial hemorrhage has been reported in APS [7][8][9][10], most of such cases were associated with antiplatelet or anti-coagulation therapy [7][8][9]. It should be noted, however, that intracranial hemorrhage has been shown to occur due to catastrophic APS [10]. Alternatively, other conditions such as vasculitis may result in cerebral hemorrhage.…”

“…Antiphospholipid syndrome (APS) is an important complication that causes infarction in multiple organs, including the brain, due to thrombosis [4][5][6]. Although intracranial hemorrhage has been reported in APS [7][8][9][10], most of such cases were associated with antiplatelet or anti-coagulation therapy [7][8][9]. It should be noted, however, that intracranial hemorrhage has been shown to occur due to catastrophic APS [10].…”

Reversible Posterior Leukoencephalopathy Syndrome in Catastrophic Antiphospholipid Syndrome

“…The causal role of the mask anesthesia administration, with a compressive effect of the fingers holding the anesthesia mask and anchoring it by extending over the mandible and encroaching on the carotid bifurcation, has been suggested [6,7] .…”

“…Ischaemic stroke (with haemorrhagic transformation) is unlikely in our patient, as the lesions encompass more than 1 vascular territory, and the MR signal is not consistent with cytotoxic oedema. Moreover, all reported cases of intracerebral haemorrhage in APS/CAPS had anticoagulation treatment [6] .…”

Catastrophic Antiphospholipid Syndrome

“…Duration depends on the clinical response. Intravenous immunoglobulin (IVIG) is proposed instead of plasma exchanges in case of hemodynamic instability [13,14], usually at a dose of 2 g/kg in 4 to 5 days [6,9]. The combination of anticoagulation with corticosteroids and IVIG provides 69% of success [15].…”