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Asherson's Syndrome of the Catastrophic Antiphospholipid Antibodies

Ronald Asherson died in May 2008. Born in South Africa, he studied medicine in Cape Town and then did one of his post-doctorates with Graham Hughes ("Hughes syndrome") in London. His main claim to fame is the description of the catastrophic antiphospholipid syndrome (cAPS)1-11 (Figure 1 and 2). He delineated the clinical characteristics of the condition, helped determine the diagnostic criteria, indicated the preferred therapy, and eventually reported on its prognosis.

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Figure 1. Prof. Asherson following the presentation of stamps featuring his portrait.

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Figure 2. The classical picture shown by many, including Prof. Asherson, alluding to the multiorgan involvement in the catastrophic antiphospholipid antibody syndrome; from Asherson RA. J Rheumatol 1992;19:508-12.

Long before his description of cAPS Ron showed a great desire for knowledge. He was born in Cape Town in 1934, and qualified in Medicine at the University of that city in 1957. After completing his internship, he moved to England in 1960 to become House Officer to Prof. Sir Christopher C. Booth at Hammersmith Hospital, London. In 1961, he accepted a fellowship at the Columbia Presbyterian and Francis Delafield Hospitals in New York, returning to South Africa to become Senior Registrar at the Groote Schuur Hospital in Cape Town from 1961 to 1964. After 10 years as Clinical Tutor in the Department of Medicine, he went back to the United States and was appointed Assistant Clinical Professor of Medicine at the New York Hospital–Cornell Medical Center under Prof. Henry O. Heineman, sharing his time with private practice. From 1981 to 1985, he was associated with the Rheumatology Department at the Royal Postgraduate Medical School of London (Hammersmith Hospital), working under Dr. Graham R.V. Hughes. It was at that time that he developed his interest in systemic autoimmune diseases and antiphospholipid antibodies. In 1985, he moved with Dr. Hughes to the Rayne Institute at St. Thomas' Hospital. In 1991 he took a sabbatical at St. Luke's Roosevelt Hospital Center in New York, working with Prof. Robert Lahita. In 1992, he returned to South Africa, where, in addition to a very active private practice, he took a position as a Principal Scientific Officer at the Rheumatic Disease Unit, Groote Schuur Hospital in Cape Town, and later as Associate Professor at the Division of Immunology, School of Pathology, University of the Witwatersrand, Johannesburg. He was also Visiting Professor at the Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Catalonia, Spain.

The research output of Ron Asherson has been prodigious, with a total of over 500 scientific articles published (more than 6000 citations12, plus chapters in more than 100 leading textbooks on Rheumatology and Internal Medicine). He edited several books, including Phospholipid Binding Antibodies, Vascular Manifestations of Systemic Autoimmune Diseases, and 2 editions of The Antiphospholipid Syndrome, and was series editor of the 10 volume Handbooks of Systemic Autoimmune Diseases, on which he was working when his terrible disease was declared.

In 1988, he was elected a Fellow of the American College of Physicians (FACP) as well as a Founding Fellow of the American College of Rheumatology (FACR). From 1988 to 1991 he was on the Council of the Royal Society of Medicine in London. In 1992, he was the co-winner of the European League Against Rheumatism (EULAR) Prize and in 1993 was part of Graham Hughes's team awarded the International League Against Rheumatism (ILAR) Prize, both for research on antiphospholipid antibodies. In 1994, he was elected Fellow of the Royal College of Physicians (FRCP) of London. In 2001, he was awarded Doctor Honoris Causa by the University of Pleven in Bulgaria.

Dr. Asherson was an invited speaker and visiting professor in most leading universities and hospitals in the world, served on the international organizing committee of many conferences, including the biannual International Conferences on Antiphospholipid Antibodies, in the international Congresses of Autoimmunity, and on editorial boards of several international journals, including The Journal of Rheumatology, as well as co-chairing the first Latin-American Congress on Autoimmunity in Galapagos, Ecuador.

In February 2008 he participated in the Mosaic of Autoimmunity meeting13-15 in Israel, only to learn that he had leukemia. He faced the disease with courage, writing continuously until the last, including the Editorial that appears in this issue of The Journal16. Following one case of cAPS that improved after leg amputation he embarked on an international cooperation (his "specialty") to review 23 similar cases17 — also recently published.

His mind was working additional hours to find out novel ramifications to the APS and cAPS, thus describing the microangiopathic APS1, etc.

We will remember his wide smile, bright mind, his many initiatives, and his illustrative lectures.

YEHUDA SHOENFELD, MD, FRCP, Department of Medicine B and Centre for Autoimmune Diseases, Sheba Medical Centre, Tel-Hashomer, Incumbent of the Laura Schwarz-Kipp Chair for Research of Autoimmune Diseases, Sackler Faculty of Medicine, Tel-Aviv University, Tel-Aviv, Israel;

RICARD CERVERA, MD, FRCP, Systemic Autoimmune Disease Unit, Hospital Clínic, Barcelona, Catalonia, Spain.

REFERENCES

Search PubMed for:

1. Asherson RA, Cervera R. Microvascular and microangiopathic antiphospholipid-associated syndromes ("MAPS"): semantic or antisemantic? Autoimmun Rev 2008;7:164-7. [MEDLINE]

2. Miesbach W, Asherson RA, Cervera R, et al. The catastrophic antiphospholipid (Asherson's) syndrome and malignancies. Autoimmun Rev 2006;6:94-7. [MEDLINE]

3. Makatsariya A, Asherson RA, Bitsadze V, Baimuradova S, Akinshina S. Catastrophic antiphospholipid (Asherson's) syndrome and genetic thrombophilic disorders in obstetrics. Autoimmun Rev 2006;6:89-93. [MEDLINE]

4. Asherson RA. Newsubsets of the antiphospholipid syndrome in 2006: "PRE-APS" (probable APS) and microangiopathic antiphospholipid syndromes ("MAPS"). Autoimmun Rev 2006;6:76-80.

5. Asherson RA. The catastrophic antiphospholipid (Asherson's) syndrome. Autoimmun Rev 2006;6:64-7. [MEDLINE]

6. Asherson RA. The catastrophic antiphospholipid (Asherson's) syndrome in 2004 — a review. Autoimmun Rev 2005;4:48-54. [MEDLINE]

7. Asherson RA, Cervera R. The antiphospholipid syndrome: multiple faces beyond the classical presentation. Autoimmun Rev 2003;2:140-51. [MEDLINE]

8. Asherson RA. Antiphospholipid antibodies, malignancies and paraproteinemias. J Autoimmun 2000;15:117-22. [MEDLINE]

9. Garic-Carrasco M, Escarcega RO, Mendoza-Pinto C, et al. Preventing death in the catastrophic antiphospholipid (Asherson) syndrome. Isr Med Assoc J 2007;9:628-9. [MEDLINE]

10. Asherson RA. Acute respiratory distress syndrome and other unusual manifestations of the catastrophic antiphospholipid (Asherson's) syndrome. Isr Med Assoc J 2004;6:360-3. [MEDLINE]

11. Asherson RA, Cervera R. The catastrophic antiphospholipid syndrome: a review of pathogenesis, clinical features and treatment. Isr Med Assoc J 2000;2:268-73. [MEDLINE]

12. Koike T, Bohgaki M, Amengual O, Atsumi T. Antiphospholipid antibodies: lessons from the bench. J Autoimmun 2007;28:129-22. [MEDLINE]

13. Shoenfeld Y, Gilburd B, Abu-Shakra M, et al. The mosaic of autoimmunity: genetic factors involved in autoimmune diseases – 2008. Isr Med Assoc J 2008;10:3-7.[MEDLINE]

14. Shoenfeld Y, Zandman-Goddard G, Stojanovich L, et al. The mosaic of autoimmunity: hormonal and environmental factors involved in autoimmune diseases — 2008. Isr Med Assoc J 2008;10:8-12.[MEDLINE]

15. Shoenfeld Y, Blank M, Abu-Shakra M, et al. The mosaic of autoimmunity: prediction, autoantibodies, and therapy in autoimmune diseases — 2008. Isr Med Assoc J 2008;10:13-9.[MEDLINE]

16. Asherson RA, Klumb EM. Antiphospholipid syndrome nephropathy in different scenarios. J Rheumatol 2008;35:1909-11.

17. Asherson RA, Cervera R, Klumb E, et al. Amputation of digits of limbs in patients with antiphospholipid syndrome. Semin Arthritis Rheum 2008 Jan 16. Epub ahead of print.[MEDLINE]



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