Antiphospholipid anti-body syndrome presenting with diffuse alveolar haemorrhage and refractory autoimmune haemolytic anaemia.

Abstract

Antiphospholipid antibody syndrome (APS) is characterized by vascular thrombosis. Somewhat paradoxically, some patients with this disease develop diffuse alveolar haemorrhage. This bleeding is usually a delayed manifestation, occurring a few years after the onset of other manifestations and diagnosis of APS. We encountered a patient with an unusual presentation, i.e. onset with diffuse alveolar haemorrhage as well as vascular thromboses. He also had autoimmune haemolytic anaemia, which is infrequent in APS. The diagnosis of APS was based on elevated levels of IgM and IgG anti-cardiolipin antibody and IgG and IgM beta-2 GP1 antibodies. Treatment with high-dose glucocorticoids, anticoagulants and rituximab led to a decline in levels of these antibodies and was associated with a good and lasting clinical response.