Authors :
Usha Topalkatti; Dr. Khachig K. Ishkhan; Ameer M Shazley
Volume/Issue :
Volume 10 - 2025, Issue 7 - July
Google Scholar :
https://tinyurl.com/usscd3sx
Scribd :
https://tinyurl.com/586ywkun
DOI :
https://doi.org/10.38124/ijisrt/25jul1733
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Abstract :
Catastrophic antiphospholipid syndrome (CAPS) is an exceedingly rare (< 1 % of APS cases) but life-threatening
variant of antiphospholipid syndrome, marked by rapid, widespread thrombosis that can precipitate myocardial infarction,
stroke, and multiorgan failure. We report a 72-year-old south Asian woman with established cardiovascular risk factors—rest
angina, hypertension, type 2 diabetes, hyperlipidaemia, and arthritis—who presented with acute right lower-limb ischemia
manifesting as pain, pallor, and coolness. CT angiography revealed complete occlusion from the distal right common femoral
artery into the crural vessels and severe stenosis of the left tibial and peroneal arteries. Despite emergent thrombectomy,
thrombolysis, full anticoagulation, high-dose corticosteroids, and intravenous immunoglobulin (IVIG), clinical deterioration
ensued, culminating in fatal multiorgan thrombosis. This case underscores the aggressive and often refractory nature of CAPS,
where even prompt triple-therapy intervention may prove insufficient. Clinicians must balance the urgent need for
anticoagulation against haemorrhagic risk, particularly in advanced ischemia or infarction. Mortality in CAPS was historically
around 50%, though recent series report reductions to ~30–35% with early multimodal therapy Heightened awareness and
rapid institution of anticoagulation combined with immunosuppression and vascular intervention remain critical to alter the
otherwise dismal prognosis.
References :
- Bernardi M, Spadafora L, Andaloro S, Piscitelli A, Fornaci G, Intonti C, Fratta AE, Hsu CE, Kaziròd-Wolski K, Metsovitis T, Biondi-Zoccai G, Sabouret P, Marzetti E, Cacciatore S. Management of Cardiovascular Complications in Antiphospholipid Syndrome: A Narrative Review with a Focus on Older Adults. J Clin Med. 2024 May 23;13(11):3064. doi: 10.3390/jcm13113064.
- Cohen H, Cuadrado MJ, Erkan D, Duarte-Garcia A, Isenberg DA, Knight JS, Ortel TL, Rahman A, Salmon JE, Tektonidou MG, Williams DJ, Willis R, Woller SC, Andrade D. 16th International Congress on Antiphospholipid Antibodies Task Force Report on Antiphospholipid Syndrome Treatment Trends. Lupus. 2020 Oct;29(12):1571-1593. doi: 10.1177/0961203320950461.
- Zhang J, Li C, Han X, Chen Z, Adhikari BK, Wang Y, Wang Y, Sun J. The digestive system involvement of antiphospholipid syndrome: pathophysiology, clinical characteristics, and treatment strategies. Ann Med. 2021 Dec;53(1):1328-1339. doi: 10.1080/07853890.2021.1962964.
- Yang L, Guo R, Liu H, Chen B, Li C, Liu R, Liao S, Xie Q, Yin G. Mechanism of antiphospholipid antibody-mediated thrombosis in antiphospholipid syndrome. Front Immunol. 2025 Mar 13;16:1527554. doi: 10.3389/fimmu.2025.1527554.
Catastrophic antiphospholipid syndrome (CAPS) is an exceedingly rare (< 1 % of APS cases) but life-threatening
variant of antiphospholipid syndrome, marked by rapid, widespread thrombosis that can precipitate myocardial infarction,
stroke, and multiorgan failure. We report a 72-year-old south Asian woman with established cardiovascular risk factors—rest
angina, hypertension, type 2 diabetes, hyperlipidaemia, and arthritis—who presented with acute right lower-limb ischemia
manifesting as pain, pallor, and coolness. CT angiography revealed complete occlusion from the distal right common femoral
artery into the crural vessels and severe stenosis of the left tibial and peroneal arteries. Despite emergent thrombectomy,
thrombolysis, full anticoagulation, high-dose corticosteroids, and intravenous immunoglobulin (IVIG), clinical deterioration
ensued, culminating in fatal multiorgan thrombosis. This case underscores the aggressive and often refractory nature of CAPS,
where even prompt triple-therapy intervention may prove insufficient. Clinicians must balance the urgent need for
anticoagulation against haemorrhagic risk, particularly in advanced ischemia or infarction. Mortality in CAPS was historically
around 50%, though recent series report reductions to ~30–35% with early multimodal therapy Heightened awareness and
rapid institution of anticoagulation combined with immunosuppression and vascular intervention remain critical to alter the
otherwise dismal prognosis.