Catastrophic antiphospholipid syndrome: Remission following leg amputation in 2 cases

Howard Amital, Yair Levy, Chris Davidson, Ingrid Lundberg, Anders Harju, Yelena Kosach, Ronald A. Asherson, Yehuda Shoenfeld*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review


Objective: The antiphospholipid syndrome is characterized by venous and arterial thrombotic events that are often recurrent, thrombocytopenia, recurrent fetal loss, and elevated titers of antiphospholipid antibodies. A subtype of patients with a particularly overwhelming clinical picture has been termed catastrophic antiphospholipid syndrome (CAPS). In this report, we present 2 patients who exhibited a similar multisystem disorder associated with gangrenous changes in the lower extremities. Methods: Two patients with CAPS are presented, highlighting the impact of this disorder on the patients and the response to various therapeutic modalities. Results: Both patients had pulmonary, cardiac, cutaneous, and neurologic findings consistent with CAPS. In addition, they had large purulent leg ulcers associated with livedo reticularis. Amputation of the legs in each case induced remission of the systemic illness. Conclusions: We believe that infection plays a significant role in the pathogenesis and amplification of the antiphospholipid syndrome. In certain patients, this association probably is mediated via immune mechanisms, which also enhance the genesis of atherosclerosis. After the foci of infection (suppurative leg ulcers) were removed, the underlying illness improved. These case studies provide an opportunity to study the interrelationship between several confounding factors that converge and lead to the development of this autoimmune condition.

Original languageEnglish
Pages (from-to)127-132
Number of pages6
JournalSeminars in Arthritis and Rheumatism
Issue number2
StatePublished - 2001
Externally publishedYes


  • Anti-β2GP1 antibodies
  • Anticardiolipin antibodies
  • Antiphospholipid syndrome
  • Atherosclerosis


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