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VOLUME 23 , ISSUE 6 ( June, 2019 ) > List of Articles

CASE REPORT

Catastrophic Antiphospholid Syndrome – An Unusual Case Report

Sneha Madkaiker

Keywords : Catastrophic antiphospholipid syndrome, Cerebral infarcts, Lupus anticoagulant, Purpura, Renal failure

Citation Information : Madkaiker S. Catastrophic Antiphospholid Syndrome – An Unusual Case Report. Indian J Crit Care Med 2019; 23 (6):276-280.

DOI: 10.5005/jp-journals-10071-23180

License: CC BY-NC 4.0

Published Online: 01-12-2009

Copyright Statement:  Copyright © 2019; The Author(s).


Abstract

Antiphospholipid syndrome (APLS) is characterised by venous or arterial thrombosis and/or adverse pregnancy outcome in the presence of persistent laboratory evidence of antiphospholipid antibodies. Catastrophic Antiphospholipid Syndrome (CAPS) is a severe and rare form of antiphospholipid syndrome characterised by multiple site thrombosis involving small, medium and large blood vessels occurring over a short period of time (usually 1 week) causing multiorgan failure. We present an unusual case of left upper limb acute arterial thrombosis with purpura fulminans like skin lesions precipitated by swine flu (H1N1) infection with adult respiratory distress syndrome subsequently developing acute renal failure, retinal infarcts, multiple acute cerebral infarcts, cardiac valvular vegetations and hemolytic anemia with recurrent bleeding episodes. A positive lupus anticoagulant confirmed the diagnosis of CAPS. In spite of early initiation of triple therapy (anticoagulation, high dose steroids, plasmapheresis) our patient did not survive. This rare case of probable CAPS is presented with an aim to study the clinical manifestations, laboratory findings, efficacy of therapy and prognosis in the medical ICU.


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  1. Zahid H, Hassan S, Gul S, Rizwan K, Khan SU, Maghazi MA. Co-existing bilateral pulmonary embolism and intra-cardiac mass: A case of catastrophic antiphospholipid syndrome-like disease. Cureus. 2018; 10(10): e3438. doi: 10.7759/cureus.3438.
  2. Uthman I, Noureldine MHA, Ruiz-Irastorza G, Khamashta M. Management of antiphospholipid syndrome. Ann Rheum Dis. 2019;78:155– 161.
  3. Gu JY, Lu C, Shi H, Yang CD. Case series and clinical analysis of 14 cases of catastrophic antiphospholipid syndrome. Beijing Da Xue Xue Bao Yi Xue Ban. 2018;50(6):1033–1038.
  4. Saraf SS, Patel YP, Desai A, Desai UR. Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions. Case Rep Ophthalmol Med. 2015; 2015: 206906. doi: 10.1155/2015/206906.
  5. Prasad N, Bhadauria D, Agarwal N Gupta A, Gupta P, Jain M et al. Catastrophic antiphospholipid antibody syndrome in a child with thrombotic microangiopathy. Indian J Nephrol. 2012;22: 310–313. doi: 10.4103/0971-4065.101266
  6. Strakhan M, Hurtado-Sbordoni M, Galeas N, Bakirhan K, Alexis K, Elrafei T. 36year old female with catastrophic antiphospholipid syndrome treated with eculizumab: acase report and review of literature. Case Rep Hematol. 2014; 2014: 704371. doi: 10.1155/2014/704371
  7. Nayer A, Ortega LM. Catastrophic antiphospholipid syndrome: a clinical review. J Nephropathol. 2014; 3(1):9–17. doi: 10.12860/jnp.2014.03
  8. Mendoza-Pinto C, García-Carrasco M, Cervera R. Role of infectious diseases in the antiphospholipid syndrome (including its catastrophic variant). Curr Rheumatol Rep. 2018; 20:62.
  9. Cruz-Tapias P, Blank M, Anaya JM Shoenfeld Y. Infections and vaccines in the etiology of antiphospholipid syndrome. Curr Opin Rheumatol. 2012;24(4): 389–393. doi: 10.1097/BOR.0b013e32835448b8
  10. Asherson R, Cervera R, de Groot., Erkan D, Boffa M, Piette J, et al Catastrophic Antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines Lupus 2003;12(7):530–534. doi: 10.1191/0961203303lu394oa
  11. Aguiar CL, Erkan D. Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease. Ther Adv Musculoskeletal Dis, 2013;5(6):305–314. doi: 10.1177/1759720X13502919
  12. Cervera R, Rodríguez-Pintó I, Espinosa G. The diagnosis and clinical management of the catastrophic antiphospholipid syndrome: A comprehensive review. J Autoimmun. 2018;92: 1–11. doi: 10.1016/j. jaut.2018.05.007
  13. PluB M, Zeisberg M, Muller GA, Vasko R, Korsten P. Therapeutic response to glucocorticoids, anticoagulation and plasma exchange in a patient with primary antiphospholipid syndrome presenting with purpura fulminans. Lupus. 2018;27(13):2170–2073. doi: 10.1177/0961203318804884.
  14. Vieregge GB, Harrington TJ, Andrews DM, Carpintero MF, Green DF, Nayer A. Catastrophic antiphospholipid syndrome with severe acute thrombotic microangiopathy and hemorrhagic complications. Case Rep Med. 2013;2013:915309. doi: 10.1155/2013/915309
  15. Costedoat-Chalumeau N, Coutte L, Le Guern V, Morel N, Leroux G, Paule R et al. 2016 review on catastrophic antiphospholipid syndrome. Presse Med. 2016; 45(12 Pt 1):1084–1092. doi: 10.1016/j. lpm.2016.07.023
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