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Vol. 20. Issue 5.
Pages 286 (May 2024)
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Vol. 20. Issue 5.
Pages 286 (May 2024)
Letter to the Editor
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From clots to cuts in antiphospholipid syndrome
De los coágulos a los cortes en el síndrome antifosfolípido
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Abihai Lucas Hernándeza,
Corresponding author
abihai.lucas@comunidad.unam.mx

Corresponding author.
, Yehuda Shoenfeldb,c
a Departamento de estudios de Posgrado, Universidad Nacional Autónoma de México (UNAM), Mexico City, Mexico
b Zabludowicz Center for Autoimmune Diseases, Sheba Medical Center, Tel-Hashomer, Ramat Gan, Tel-Aviv, Israel
c Reichman University, Herzliya, Israel
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Dear Editor,

In June 2023, a report on antiphospholipid syndrome (APS) was published by Darwish et al.1 in which a previously healthy 44-year-old man with a smoking history presented with fever, polyarthritis, and acrocyanosis of fingers and toes, his condition worsened, eventually requiring the amputation of several toes due to gangrene. As the authors mentioned in this case report, this is not a common disease manifestation. Nevertheless, similar cases were reported previously by our group. Two cases of remission of catastrophic APS following leg amputations: the first case of a 47-year-old man with painful ulcers in both legs that worsened with neurologic manifestations, brain infarctions, and severe acute respiratory distress syndrome (ARDS); the second case of a 52-year-old man with ischemic cerebrovascular accident (CVA) and leg ulcerations that required below-knee amputation after both feet became gangrenous. In both clinical scenarios, the patients resolved symptoms after amputations.2 We also reported 21 cases of patients with APS who presented gangrene and ended up with amputation of the affected limb.3

As we can see, the mentioned case reports, contribute to the literature and enhance our understanding of the various clinical presentations in APS, encompassing not only thrombosis but also ulcers and gangrene of the limbs with the necessity in certain cases of amputation due to the severe presentation with repercussion to quality of life. These manifestations were previously categorized as “Non-criteria” APS by Pires de Rosa et al.4 in their nomenclature proposal and hold significant importance in clinical decision-making, treatment, and prognosis. Also, as Ferrari et al.5 reported, there are certain cases where vascular manifestations can be assessed by capillaroscopy to find microvascular damage in patients with APS even before symptoms are presented. Nevertheless, it is worth noting that during the 2022 EULAR conference, draft criteria for APS were proposed; however, ulcers and gangrene are not included in the microvascular domain. As we mentioned previously, skin manifestations with their complications must be considered when we suspect APS, even if they are not included in the classification criteria.

References
[1]
Darwish N, Manaa M, Reyes G, Freeman JG. Case report: a patient presents with digital ischemia & gangrene [Internet]. Rheumatologist. Available from: https://www.the-rheumatologist.org/article/case-report-a-patient-presents-with-digital-ischemia-gangrene/ [cited 6.9.23].
[2]
R.A. Asherson, R. Cervera, Y. Shoenfeld.
Peripheral vascular occlusions leading to gangrene and amputations in antiphospholipid antibody-positive patients.
Ann N Y Acad Sci, 1108 (2007), pp. 515-529
[3]
H. Amital, Y. Levy, C. Davidson, I. Lundberg, A. Harju, Y. Kosach, et al.
Catastrophic antiphospholipid syndrome: remission following leg amputation in 2 cases.
Semin Arthritis Rheum, 31 (2001), pp. 127-132
[4]
G. Pires da Rosa, P. Bettencourt, I. Rodríguez-Pintó, R. Cervera, G. Espinosa.
Non-criteria” antiphospholipid syndrome: a nomenclature proposal.
Autoimmun Rev, 19 (2020), pp. 102689
[5]
G. Ferrari, E. Gotelli, S. Paolino, G. Pesce, L. Nanni, B.M. Colombo, et al.
Antiphospholipid antibodies and anticoagulant therapy: capillaroscopic findings.
Arthritis Res Ther, 23 (2021), pp. 175
Copyright © 2023. Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología
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