Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia – A diagnostic and management challenge

Sadick, Victoria; Lane, Stuart; Fischer, Eddy; Seppelt, Ian; Shetty, Anita; McLean, Anthony S.

doi:10.1177/1751143718762343

Cited by 3 publications

(4 citation statements)

References 27 publications

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“…Despite the criteria, CAPS symptoms and laboratory findings often overlap with other obstetric complications such as HELLP syndrome, thrombotic thrombocytopenic purpura (TTP), or acute fatty liver of pregnancy (AFLP), leading to a delayed diagnosis and treatment. [6] , [7] …”

Section: Discussionmentioning

confidence: 99%

Catastrophic antiphospholipid syndrome during pregnancy

Carrasco

Triana

García

et al. 2021

European Journal of Obstetrics & Gynecology and Reproductiv

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Catastrophic antiphospholipid syndrome (CAPS) is an uncommon and the most severe form of antiphospholipid syndrome (APS). A 33-week pregnant patient with Klippel-Trenaunay syndrome, past SARS-CoV-2 infection and type I fetal growth restriction with shortening of the fetal long bone was diagnosed in our center with a probable CAPS. Cesarean section was performed four days after the diagnosis due to the torpid evolution of the patient. Clinical improvement was noted a few days later and the mother and baby were discharged within a week. We review the current literature on CAPS during pregnancy and provide an updated view.

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Section: Discussionmentioning

confidence: 99%

Catastrophic antiphospholipid syndrome during pregnancy

Carrasco

Triana

García

et al. 2021

European Journal of Obstetrics & Gynecology and Reproductiv

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“…Fetal morbidities, such as premature birth, growth restriction, or mortality, are associated with placental insufficiency [51,52]. CAPS symptoms and laboratory findings can overlap with other obstetric disorders including acute fatty liver of pregnancy (AFLP), thrombotic thrombocytopenic purpura (TTP), or HELLP syndrome, delaying diagnosis and treatment [53][54][55]. Catastrophic antiphospholipid syndrome remains a diagnostic challenge due to its low frequency and non-specific clinical presentation.…”

Section: Caps During Pregnancymentioning

confidence: 99%

Catastrophic Antiphospholipid Syndrome: A Review

Siniscalchi,

Basaglia,

Riva

et al. 2023

Immuno

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Antiphospholipid syndrome (APS) is a systemic autoimmune disease characterized by thrombotic or obstetric events occurring in individuals who have persistent antiphospholipid antibodies. Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially fatal form of APS characterized by severe thrombotic complications occurring in multiple organs over a short period of time or simultaneously. CAPS is associated with a high (50%) death rate. Infections, multi-organ failure, and cerebral and heart thrombosis represent the main complications of this syndrome. Generally, anticoagulants, glucocorticoids, therapeutic plasmapheresis (TPE), and intravenous immunoglobulin (IVIG) are used in combination for treatment. Multidisciplinary care involving different specialists from hematology, rheumatology, nephrology, infectious disease, critical care, and obstetrics is often required due to the complexity of the disease. Recent data emphasize the effectiveness of biologics such as anti-TNF-a monoclonal antibodies (adalimumab, certolizumab), anti-CD38 monoclonal antibody (daratumumab), BAFF/Blys inhibitor (belimumab), and BTK inhibitor (zanubrutinib) against CAPS. In order to understand the underlying causes of CAPS, one future possibility involves investigating and characterizing the hereditary and acquired risk factors associated with CAPS.

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“…The International CAPS Registry’s recent update reported skin involvement (n = 242) in 47% of the 517 CAPS episodes, including livedo reticularis (92%), cutaneous necrosis (55%), cutaneous ulcers (51%), and purpura (30%), but did not report detailed information about cutaneous manifestations or describe their course . A few case reports have described skin involvement in detail, mostly with extensive skin necrosis . Finally, no study has evaluated the diagnostic role or potential complications of skin biopsies in CAPS, although its classification criteria require histologic proof of small-vessel occlusion for definite diagnosis …”

Section: Introductionmentioning

confidence: 99%

“…6 A few case reports have described skin involvement in detail, mostly with extensive skin necrosis. [7][8][9][10][11] Finally, no study has evaluated the diagnostic role or potential complications of skin biopsies in CAPS, although its classification criteria require histologic proof of smallvessel occlusion for definite diagnosis. [12][13][14] Because, to our knowledge, no large series details the cutaneous manifestations associated with CAPS, and little is known about their clinical or histopathological features or prognosis, we aimed to describe these in this series of patients with CAPS and to compare these patients with those without cutaneous involvement.…”

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confidence: 99%

Cutaneous Involvement in Catastrophic Antiphospholipid Syndrome in a Multicenter Cohort of 65 Patients

et al. 2023

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ImportanceCatastrophic antiphospholipid syndrome (CAPS) is a severe, rare complication of antiphospholipid syndrome (APS), but cutaneous involvement has not yet been adequately described.ObjectiveTo describe cutaneous involvement during CAPS, its clinical and pathological features, and outcomes.Design, Setting, and ParticipantsThis cohort study was a retrospective analysis of patients included in the French multicenter APS/systemic lupus erythematosus register (ClinicalTrials.gov: NCT02782039) by December 2020. All patients meeting the revised international classification criteria for CAPS were included, and patients with cutaneous manifestations were analyzed more specifically.Main Outcomes and MeasuresClinical and pathological data as well as course and outcome in patients with cutaneous involvement during CAPS were collected and compared with those in the register without cutaneous involvement.ResultsAmong 120 patients with at least 1 CAPS episode, the 65 (54%) with skin involvement (43 [66%] women; median [range] age, 31 [12-69] years) were analyzed. Catastrophic antiphospholipid syndrome was the first APS manifestation for 21 of 60 (35%) patients with available data. The main lesions were recent-onset or newly worsened livedo racemosa (n = 29, 45%), necrotic and/or ulcerated lesions (n = 27, 42%), subungual splinter hemorrhages (n = 19, 29%), apparent distal inflammatory edema (reddened and warm hands, feet, or face) (n = 15, 23%), and/or vascular purpura (n = 9, 14%). Sixteen biopsies performed during CAPS episodes were reviewed and showed microthrombi of dermal capillaries in 15 patients (94%). These lesions healed without sequelae in slightly more than 90% (58 of 64) of patients. Patients with cutaneous involvement showed a trend toward more frequent histologically proven CAPS (37% vs 24%, P = .16) than those without such involvement, while mortality did not differ significantly between the groups (respectively, 5% vs 9%, P = .47).Conclusions and RelevanceIn this cohort study, half the patients with CAPS showed cutaneous involvement, with a wide spectrum of clinical presentations, including distal inflammatory edema. Skin biopsies confirmed the diagnosis in all but 1 biopsied patient.

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Post-partum catastrophic antiphospholipid syndrome presenting with shock and digital ischaemia – A diagnostic and management challenge

Cited by 3 publications

References 27 publications

Catastrophic antiphospholipid syndrome during pregnancy

Catastrophic antiphospholipid syndrome during pregnancy

Catastrophic Antiphospholipid Syndrome: A Review

Cutaneous Involvement in Catastrophic Antiphospholipid Syndrome in a Multicenter Cohort of 65 Patients

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