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, and Yehuda Shoenfeld Objective: The antiphospholipid syndrome is characterized by venous and arterial thrombotic events that are often recurrent, thrombocytopenia, recur- rent 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 gan- grenous 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 patho- genesis and amplification of the antiphospholipid syndrome. In certain pa- tients, this association probably is mediated via immune mechanisms, which also enhance the genesis of atherosclerosis. After the foci of infection (suppu- rative 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 auto- immune condition. Semin Arthritis Rheum 31:127-132. Copyright © 2001 by W.B. Saunders Company INDEX WORDS: Antiphospholipid syndrome; atherosclerosis; anti-2GP1 anti- bodies; anticardiolipin antibodies. From the Department of Medicine, Center of Autoimmune Diseases, and the Department of Medicine ‘B,’ Sheba Medical Center, Tel-Hashomer, Israel; the Department of Cardiology, Royal Sussex County Hospital, Brighton, England; the Depart- ment of Rheumatology, Karolinska Hospital, Stockholm, Swe- den; and the Rheumatic Diseases Unit, Groote Schuur Hospital, University of Cape Town School of Medicine, Cape Town, South Africa. Howard Amital, MD: Staff, Department of Medicine, Center of Autoimmune Diseases and the Department of Medicine ‘B,’ Sheba Medical Center, Tel-Hashomer, Israel; Yair Levy, MD: Staff, Department of Medicine, Center of Autoimmune Diseases and the Department of Medicine ‘B,’ Sheba Medical Center, Tel-Hashomer, Israel; Yelena Kosach, MD: Staff, Department of Medicine, Center of Autoimmune Diseases and the Depart- ment of Medicine ‘B,’ Sheba Medical Center, Tel-Hashomer, Israel; Yehuda Shoenfeld: MD: Professor, Department of Med- icine, Center of Autoimmune Diseases and the Department of Medicine ‘B,’ Sheba Medical Center, Tel-Hashomer, Israel; Chris Davidson, MA, MB, FRCP, FESC: Lead Consultant, Sussex Cardiac Center, Department of Cardiology, Royal Sus- sex County Hospital, Brighton, England; Ingrid Lundberg, MD, PhD: Associate Professor, Department of Rheumatology, Karo- linska Hospital, Stockholm, Sweden; Anders Harju, MD: Consul- tant, Department of Rheumatology, Karolinska Hospital, Stock- holm, Sweden; Ronald A. Asherson, MD, FRCP, FACP, FCP (SA): Professor, Honorary Consultant Physician Rheumatic Dis- eases Unit, The Rheumatic Diseases Unit, Groote Schuur Hospital, University of Cape Town School of Medicine, Cape Town, South Africa. Supported by the Freda and Leon Schaller Research Grant for Autoimmunity. Address reprint requests to Prof. Yehuda Shoenfeld, Head, Department of Medicine ‘B,’ Sheba Medical Center, Tel- Hashomer, 52621, Israel. E-mail: shoenfel@post.tau.ac.il Copyright © 2001 by W.B. Saunders Company 0049-0172/01/3102-1035$35.00/0 doi:10.1053/sarh.2001.27660 127 Seminars in Arthritis and Rheumatism, Vol 31, No 2 (October), 2001: pp 127-132