Plasma Exchange in the Management of Catastrophic Antiphospholipid Syndrome.

Dimitri Titeca-Beauport,Valery Salle,Julien Maizel,Gabriel Choukroun,Loay Kontar

doi:10.1155/2016/5375080

Dimitri Titeca-Beauport, Valery Salle + Show 3 more

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https://doi.org/10.1155/2016/5375080

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Abstract

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.

Highlights

Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by a combination of arterial and/or venous thrombosis, obstetric complications, and antiphospholipid antibodies
We report the case of a 52-year-old man with primary antiphospholipid syndrome (APS) for twelve years, with one episode of deep vein thrombosis, migraine, and persistent high titers of IgG anti-cardiolipin and aβ2GPI antibodies
Patients with catastrophic antiphospholipid syndrome (CAPS) represent less than 1% of all patients with APS, but CAPS constitutes the first manifestation of APS in approximately one-half of cases

Summary

Objective

Report of a case of catastrophic antiphospholipid syndrome (CAPS) with multiple organ involvement leading to a lifethreatening condition despite early combination corticosteroid and heparin therapy. Initiation of plasma exchange led to rapid improvement of the patient’s general condition. 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. 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. This case illustrates the efficacy of plasma exchange in CAPS and the difficulty for physicians to determine the optimal timing of plasma exchange

Introduction

Case Report

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Conclusion