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The role of malignancies in patients with catastrophic anti-phospholipid (Asherson’s) syndrome

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Abstract

The catastrophic anti-phospholipid syndrome (CAPS) differs from the anti-phospholipid syndrome in its accelerated systemic involvement leading to multi-organic failure. In this study, the occurrence of malignancies in patients with CAPS was evaluated and the clinical findings of CAPS patients with and without malignancies were compared. We investigated the web site-based international registry of patients with CAPS for all cases in which both CAPS and underlying malignancies were present. The clinical characteristics of these cases were subsequently evaluated to establish common characteristics. The CAPS registry included information on a total of 262 cases. Twenty-three (9%) patients suffered from malignancies. In 78% of these patients, the malignancy itself or the treatment modalities instituted for the carcinoma was the precipitating factor of CAPS. Only 39% of CAPS patients with malignancies recovered in comparison to 58% of patients without malignancies (p = 0.07). Treatment modalities, however, did not differ significantly between these patients. Infections were not evident as precipitating factors for any of the malignancy patients. The mean age of patients with malignancies was 9 years older than the average age of other patients with CAPS and the prevalence of SLE was significantly less common than in patients without malignancy. Malignancy may play a pathogenic role in patients with CAPS, whereas infections are more important as triggering factors in patients without malignancies. CAPS patients with malignancies are generally older than CAPS patients without malignancies; they generally have the worst prognosis of the entire CAPS cohort.

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Authors and Affiliations

  1. Department of Internal Medicine III / Institute of Transfusion Medicine, Johann Wolfgang Goethe University Hospital, Theodor-Stern-Kai 7, 60590, Frankfurt, Germany

    W. Miesbach

  2. Division of Immunology, School of Pathology, University of the Witwatersrand and the Rosebank Clinic, Johannesburg, South Africa

    R. A. Asherson

  3. Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain

    R. Cervera, J. Gomez Puerta, G. Espinosa & S. Bucciarelli

  4. Department of Medicine ‘B’ and Center for Autoimmune Diseases, Sheba Medical Center (Affiliated to Tel-Aviv University), Tel Hashomer, Israel

    Y. Shoenfeld

Authors
  1. W. Miesbach
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  2. R. A. Asherson
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  3. R. Cervera
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  4. Y. Shoenfeld
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  5. J. Gomez Puerta
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  6. G. Espinosa
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  7. S. Bucciarelli
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Consortia

Members of the CAPS Registry Group

Corresponding author

Correspondence to W. Miesbach.

Additional information

*The members of the Catastrophic Antiphospholipid Syndrome Registry Project Group are listed in the Appendix.

Appendix

Appendix

The Catastrophic Antiphospholipid Syndrome Registry Project Group

The members of the Catastrophic APS Registry Project Group who contributed to this study are as follows: Christopher Davidson, Department of Cardiology, Royal Sussex Hospital, Brighton, UK; Alex E Denes, Division of Oncology, Department of Medicine, Washington University School of Medicine, St Louis, USA; Ronald H W M Derksen, Department of Rheumatology and Clinical Immunology, University Medical Centre, Utrecht, The Netherlands; J F Diaz Coto, Caja Costarricense del Seguro Social, San Jose, Costa Rica; Patrick Disdier, Service de Medecine Interne, Centre Hospitalier Universitaire Timone, Marseille, France; Rita M Egan, Department of Medicine, University of Kentucky Medical Center, Lexington, USA; R Enriquez, Nephrology Section, Hospital General de Elche, Spain; Fernanfa Falcini, Department of Paediatrics, University of Florence, Italy; Leslie S Fang, Renal Associates, Massachusetts General Hospital and Harvard Medical School, Boston, USA; John T Grandone, Neenah, Wisconsin, USA; Anagha Gurjal, Division of Hematology/Oncology, Barbara Ann Karmanos Cancer Institute, Detroit, Michigan, USA; Gilles Hayem, Department of Rheumatology, CHU Bichat-Claude-Bernard, Paris, France; Graham R V Hughes, Lupus Research Unit, The Rayne Institute, St Thomas’ Hospital, London, UK; Sohail Inam, Riyadh Armed Forces Hospital Riyadh, Saudi Arabia; K Shashi Kant, Department of Internal Medicine, University of Cincinnati College of Medicine, Ohio, USA; Craig S Kitchens, Department of Medicine, University of Florida, Gainesville, USA; Michael J Kupferminc, Department of Obstetrics and Gynaecology, Lis Maternity Hospital, Tel Aviv University, Tel Aviv, Israel; Roger A Levy, Department of Rheumatology, Faculdade de Ciencias Medicas, Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil; Siu Fai Lui, Department of Medicine, Prince of Wales Hospital and Chinese University of Hong Kong, Shatin, Hong Kong; Peter J Maddison, Gwynedd Rheumatology Service, Ysbyty Gwynedd, Bangor, UK; Yoseph A Mekori, Department of Medicine, Meir Hospital, Kfar Saba, Israel; Takako Miyamae, Department of Paediatrics, Yokohama City University School of Medicine, Yokohama, Japan; John Moore, Department of Haematology, St Vincent’s Hospital, Sydney, Australia; Francisco J Munoz-Rodriguez, Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Catalonia, Spain; Ayako Nakajima, Institute of Rheumatology, Tokyo Women’s Medical University, Tokyo, Japan; Michael C Neuwelt, Medical Service, VA Palo Alto Health Care System, USA; Ann Parke, Department of Internal Medicine, Division of Rheumatic Diseases, University of Connecticut Health Center, Connecticut, USA; Jorge Rojas-Rodriguez, Department of Rheumatology, Specialties Hospital, Manuel Avila Camacho National Medical Centre, Puebla, Mexico; Allen D Sawitzke, Division of Rheumatology, Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, USA; Cees G Schaar, Department of Haematology, Leiden University Medical Centre, The Netherlands; Yehuda Shoenfeld, Chaim-Sheba Medical Centre, Tel-Hashomer, Israel; Alex C Spyropoulos, Clinical Thrombosis Center, Albuquerque, New Mexico, USA; Carlos Vasconcelos, Hospital Geral de San Antonio, Poro, Portugal; and Margaret Wislowska, Outpatients Department of Rheumatology, Central Clinical Hospital,Warsaw, Poland.

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Miesbach, W., Asherson, R.A., Cervera, R. et al. The role of malignancies in patients with catastrophic anti-phospholipid (Asherson’s) syndrome. Clin Rheumatol 26, 2109–2114 (2007). https://doi.org/10.1007/s10067-007-0634-x

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  • DOI: https://doi.org/10.1007/s10067-007-0634-x

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