Successful treatment of catastrophic antiphospholipid syndrome with therapeutic plasma exchange: a case report

Velásquez-Rimachi, Victor; Palma-García, Luis; Pacheco‐Barrios, Kevin; Alva-Díaz, Carlos Alexander

doi:10.1016/j.htct.2019.07.003

Cited by 3 publications

(3 citation statements)

References 10 publications

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“…CAPS associated with SLE is more likely to have severe brain or cardiac involvement and a higher mortality rate (48%). The highest recovery rate was found in patients receiving a combination of anticoagulant, corticosteroid, and plasma exchange therapy (17,(20)(21)(22). The mortality rate in patients who received triple therapy (glucocorticosteroids, anticoagulants, TPE) was 29% (22).…”

Section: Discussionmentioning

confidence: 98%

Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review

BEREANU,

PISALTU,

BEREANU

et al. 2023

In Vivo

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Background/Aim: Catastrophic antiphospholipid syndrome (CAPS) may be the first manifestation ("de novo") of antiphospholipid syndrome (APS) or a complication in the clinical course of patients known to have this syndrome. Approximately 40% of patients had an associated autoimmune disease, mainly, systemic lupus erythematosus (SLE). The trigger can be one of the following: infections, surgical interventions, neoplasms, pregnancy, discontinuation of anticoagulant treatment, and others. CAPS is a medical emergency in which early identification and prompt initiation of aggressive therapy is extremely important. According to the Guidelines for the use of Therapeutic Apheresis in Clinical Practice developed by the American Society for Apheresis (ASFA), last updated in April 2023, in CAPS, the indication for therapeutic plasma exchange (TPE) is category I, grade 2C. Case Report: We present a case of probable CAPS secondary to systemic lupus erythematosus (SLE) in an elderly patient in whom clinical and biological improvement was achieved through a multidisciplinary approach and prompt initiation of TPE. Because TPE is considered first-line therapy in CAPS, we initiated the procedure as soon as the attending rheumatologist raised this suspicion. Four plasmapheresis sessions were performed in the Intensive Care Unit. We used TPE by membrane filtration. Following the therapeutic intervention with TPE, corticotherapy (Solumedrol in pulstherapy), cyclophosphamide and anticoagulant treatment, the evolution was favourable, with clinical and biological improvement. Conclusion: The prompt initiation of TPE, because of the suspicion of CAPS, increases the chances of a favourable evolution.

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Section: Discussionmentioning

confidence: 98%

Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review

BEREANU,

PISALTU,

BEREANU

et al. 2023

In Vivo

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“…Approximately 1% of APS patients develop CAPS, which has a mortality rate of up to 30%. Like many autoimmune conditions, women account for 70% of CAPS cases [ 2 ].…”

Section: Discussionmentioning

confidence: 99%

“…Catastrophic APS (CAPS), also known as Asherson’s syndrome, is a rare, life-threatening form of APS with a mortality rate of about 50% [ 2 ]. CAPS is defined by acute widespread thrombosis in several vascular beds leading to multiorgan ischemia and failure.…”

Section: Introductionmentioning

confidence: 99%

A Case of Catastrophic Antiphospholipid Syndrome with Acute Multiorgan Thrombosis and Concerns for Re-Emergence

Mittal,

Chaudhary,

Aung

et al. 2023

Am J Case Rep

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Patient: Female, 59-year-old Final Diagnosis: Catastrophic antiphospholipid syndrome (CAPS) Symptoms: Altered mental status • extremity weakness Clinical Procedure: Renal biopsy Specialty: Rheumatology Objective: Rare disease Background: Catastrophic antiphospholipid syndrome (CAPS) is a rare, life-threatening form of antiphospholipid syndrome characterized by widespread thrombotic complications leading to multiorgan ischemia and failure. Although there are no standard treatment guidelines for CAPS, it often involves triple therapy with anticoagulation, corticosteroids, and plasma exchange. Recently, biologics such as rituximab and eculizumab have also shown promise as potential new therapies for CAPS, as observed in our case. Case Report: We describe a 59-year-old female patient who presented with altered mental status and diffuse weakness. Imaging studies revealed multiorgan thrombosis along with thrombocytopenia that markedly improved with plasma exchange therapy, steroids, and a heparin drip. While the exact etiology of CAPS remained unknown, it was likely precipitated by her warfarin discontinuation and confirmed Haemophilus influenzae infection. The patient’s hospital course was complicated by hemorrhagic shock after a renal biopsy, followed by an acute drop in thrombocytopenia and new embolic infarcts in the brain that raised concern for CAPS re-emergence. To address the refractory nature of her condition, the patient underwent a trial of rituximab, which remarkably improved her clinical picture and platelet count by an 8-fold increase within 1 week. Conclusions: This case highlights the importance of early recognition and diagnosis of catastrophic antiphospholipid syndrome, a true rheumatological emergency that requires aggressive treatment to prevent irreversible complications. Our patient’s presentation and response to treatment also underscores the complexity of managing CAPS and the use of newer biological therapies in refractory cases.

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A case report on catastrophic antiphospholipid syndrome with cardiomyopathy

Indu¹,

Singh²,

Chawla³

2023

Ann Rheumatol Autoimmun

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Successful treatment of catastrophic antiphospholipid syndrome with therapeutic plasma exchange: a case report

Cited by 3 publications

References 10 publications

Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review

Therapeutic Plasma Exchange in Catastrophic Antiphospholipid Syndrome (CAPS): A Rare Case Report and Literature Review

A Case of Catastrophic Antiphospholipid Syndrome with Acute Multiorgan Thrombosis and Concerns for Re-Emergence

A case report on catastrophic antiphospholipid syndrome with cardiomyopathy

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