scholarly journals Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Dimitri Titeca-Beauport ◽  
Valery Salle ◽  
Loay Kontar ◽  
Julien Maizel ◽  
Gabriel Choukroun
Keyword(s):  
Plasma Exchange ◽  
Antiphospholipid Syndrome ◽  
General Condition ◽  
Oxygen Therapy ◽  
Single Case ◽  
Heparin Therapy ◽  
University Teaching ◽  
Optimal Timing ◽  
Rapid Improvement ◽  
Catastrophic Antiphospholipid Syndrome

Objective. Report of a case of catastrophic antiphospholipid syndrome (CAPS) with multiple organ involvement leading to a life-threatening condition despite early combination corticosteroid and heparin therapy. Initiation of plasma exchange led to rapid improvement of the patient’s general condition. Design. Case report. Setting. University teaching hospital medical intensive care unit. Patient. Single case: 52-year-old man hospitalized for catastrophic antiphospholipid syndrome (CAPS) with cardiac, renal, and cutaneous involvement. Despite early methylprednisolone and heparin therapy, the patient’s condition progressively deteriorated, resulting in acute renal failure, right adrenal hemorrhage, and pulmonary involvement, leading to acute respiratory distress on day 6, requiring high-flow nasal cannula oxygen therapy with FiO2 of 1.0. Interventions. Plasma exchange was started on day 6. Endpoints and Main Results. A marked improvement of the patient’s general condition was observed after initiation of plasma exchange, with successful weaning of oxygen therapy and normalization of platelet count, troponin, and serum creatinine within four days. Conclusions. This case illustrates the efficacy of plasma exchange in CAPS and the difficulty for physicians to determine the optimal timing of plasma exchange.

Recovery from catastrophic antiphospholipid syndrome by a plasma exchange procedure: report of four cases and review of the literature

2009 ◽  
Vol 8 (4) ◽  
pp. 297-301 ◽  
Author(s):  
Maria Bortolati ◽  
Piero Marson ◽  
Fabrizio Fabris ◽  
Vittorio Pengo ◽  
Myriam Facchinetti ◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Antiphospholipid Syndrome ◽  
Review Of The Literature ◽  
Catastrophic Antiphospholipid Syndrome

Thrombotic microangiopathy in the course of catastrophic antiphospholipid syndrome successfully treated with eculizumab: case report and systematic review of the literature

Lupus ◽  
2020 ◽  
Vol 29 (6) ◽  
pp. 631-639 ◽  
Author(s):  
Marta Skoczynska ◽  
Mark A Crowther ◽  
Malgorzata Chowaniec ◽  
Monika Ponikowska ◽  
Shruti Chaturvedi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Single Case ◽  
Case Reports ◽  
Review Of The Literature ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Total Remission

Objective The purpose of this study was to characterize the role of eculizumab, a monoclonal antibody against the terminal complement component C5, in patients with catastrophic antiphospholipid syndrome (CAPS). Methods We present a case report of a patient with systemic lupus erythematosus (SLE) and CAPS treated with eculizumab, as well as results of a systematic review of the literature. Results Including our patient, we identified 11 case reports of patients with CAPS treated with eculizumab. All of them had partial or total remission of symptoms. Conclusion Data on eculizumab efficacy in CAPS are promising but are limited to single case reports. More studies are needed to develop evidence-based recommendations for eculizumab use in CAPS.

Plasma exchange in catastrophic antiphospholipid syndrome

2019 ◽  
Vol 48 (11) ◽  
pp. 347-353 ◽  
Author(s):  
Ignasi Rodríguez-Pintó ◽  
Miquel Lozano ◽  
Joan Cid ◽  
Gerard Espinosa ◽  
Ricard Cervera
Keyword(s):  
Plasma Exchange ◽  
Antiphospholipid Syndrome ◽  
Catastrophic Antiphospholipid Syndrome

Therapeutic response to glucocorticoids, anticoagulation and plasma exchange in a patient with primary antiphospholipid syndrome presenting with purpura fulminans

Lupus ◽  
2018 ◽  
Vol 27 (13) ◽  
pp. 2170-2173
Author(s):  
M Plüß ◽  
M Zeisberg ◽  
G A Müller ◽  
R Vasko ◽  
P Korsten
Keyword(s):  
Plasma Exchange ◽  
Antiphospholipid Syndrome ◽  
Therapeutic Response ◽  
Therapeutic Option ◽  
Purpura Fulminans ◽  
High Dose ◽  
Primary Antiphospholipid Syndrome ◽  
Internal Organs ◽  
Catastrophic Antiphospholipid Syndrome

We report the case of a 25-year-old female patient who presented with purpura fulminans as a manifestation of primary antiphospholipid syndrome. Purpura fulminans is considered a rare cutaneous manifestation of antiphospholipid syndrome. Most frequently, it occurs in the context of catastrophic antiphospholipid syndrome and is associated with significant morbidity and mortality, either due to loss of affected extremities or thromboembolic damage to internal organs. After insufficient efficacy of parenteral anticoagulation and oral glucocorticosteroid treatment, we escalated treatment to high-dose intravenous glucocorticosteroid and five consecutive sessions of plasma exchange with good and sustained clinical response. At follow-up six months after admission, skin manifestations had healed with scarring, and no additional thrombotic events had occurred. Plasma exchange may hold promise as a therapeutic option in refractory or severe cases of antiphospholipid syndrome-related purpura fulminans with extensive cutaneous necrosis, although evidence is limited.

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