Background The catastrophic antiphospholipid syndrome (CAPS) is an uncommon complication of the antiphospholipid syndrome (APS). It presents as acute multi-organ failure and more frequently involves the kidneys, lungs, gastrointestinal tract and the skin. CAPS is caused by widespread thrombosis of small and medium vessels.

Methods Case report: We describe the case of a young female admitted with severe and accelerated hypertension. No evidence of systemic disease was observed at that time and the physical examination revealed extensive livedo reticularis. In subsequent years she also suffered several transient cerebrovascular accidents with minor neurological sequelae. The anticardiolipin antibodies (ACL) titers were usually and repeatedly extremely high. Renal angiography found normal proximal renal arteries. Renal biopsy showed ischaemic glomeruli without proliferative lesions, but with focal intimal fibrosis, thrombosis of the intrarenal vessels in the absence of vasculitis. The diagnostic of primary antiphospholipid syndrome was formulated. She was treated with antihypertensive drugs, anticoagulant and antiaggregant therapy, escalating doses of steroids, plasmapheresis.

Results Clinical course: Despite of this treatment she developed progressive multiorgan failure with malignant hypertension, renal failure, central nervous system manifestation (grand mal seizures, hemiparesis, amaurosis fugax, steady deterioration of consciousness) and gastrointestinal involvement (abdominal pain due to infarction or ischaemic ulceration of the bowel). She died as a result of a combination of renal and respiratory failure. Postmortem examination revealed extensive multiorgan arterial microthrombi.

Conclusion Discussion: The case suggests that hypertension and renal failure should be recognised as a complication of APS, the aetiology being primary intrarenal vascular disease with nephroangiosclerosis. It illustrates also how primary APS presenting with renal manifestation can result in a fatal form of CAPS.

References

1. Asherson RA. The catastrophic antiphospholipid syndrome 1998. A review of the clinical features, possible pathogenesis and treatment. Lupus 1998;7(Suppl 2):S55–62

2. Durand JM, Lefevre P, Kaplanski G, Casanova P, Soubeyrand J. Accelerated hypertension and nephroangiosclerosis associated with antiphospholipid syndrome. Report of two cases and review of the literature. Clin Rheumatol. 1994;13(4):635–40