Management Of Catastrophic Antiphospholipid Syndrome Research Articles

Diagnosis and Management of Catastrophic Antiphospholipid Syndrome and the Potential Impact of the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria.

Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality. The absence of prospective studies of CAPS limits the strength of evidence for guideline treatment protocols. This comprehensive review summarizes the current understanding of the disease, and discusses how the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria impact the definition and therapeutic management of CAPS, which is considered the most severe form of APS. The correct integration of 2023 ACR/EULAR APS classification criteria is poised to facilitate CAPS diagnosis, particularly in critical situations, offering a promising avenue for improved outcomes.

A Case Report on the Successful Management of Catastrophic Antiphospholipid Syndrome: Multidisciplinary Approach

ABSTRACT Catastrophic antiphospholipid syndrome (CAPS) is a multi-organ systemic thrombotic condition precipitated due to various secondary conditions such as infection, surgery, and estrogen use. The subsequent formation of various autoantibodies and various organ involvement such as the brain, heart, and kidneys cause rapid progression of the disease. The acute features of thrombosis and the predominant involvement of kidneys and the brain make the differentiation of this condition with other thrombotic microangiopathies difficult. However, early suspicion of CAPS with prompt management is key to favorable prognosis in such patients. Multidisciplinary approach to diagnosis and medical management plays an important role in the successful outcome of CAPS patients. The three pillars of management are therapeutic plasma exchange (TPE), steroids, and anticoagulants. TPE plays a major role in eliminating incriminating antibodies, cytokines, tumor necrosis factor, and complement. We present a case of 34-year-old female with definite CAPS, managed successfully with the triple regimen comprising a total of seven TPE sessions, pulse therapy with steroids, and anticoagulants.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: A VICIOUS CIRCLE OF CLOTTING AND BLEEDING

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME: A VICIOUS CIRCLE OF CLOTTING AND BLEEDING

O25 Catastrophic antiphospholipid crisis triggered by anticoagulant switch

O25 Catastrophic antiphospholipid crisis triggered by anticoagulant switch

Plasma Exchange in the Management of 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.

Current management of catastrophic antiphospholipid syndrome

The catastrophic variant of the antiphospholipid syndrome (APS) is the most severe form of APS with acute multiple organ involvement and small vessel thrombosis. At present, there are no studies on the pathophysiological mechanisms of catastrophic APS. The two theoretical explanations for the clinical manifestations of catastrophic APS are the development of thrombosis and the systemic inflammatory response syndrome (SIRS). From retrospective study data, first-line therapies should always include the combination of anticoagulation against thrombosis plus glucocorticoids against manifestations of SIRS plus plasma exchange and/or intravenous immunoglobulins to remove or block the antiphospholipid antibodies and cytokines involved in the SIRS. This review is focused on current management of catastrophic APS and some of the potential new therapeutic approaches.

Thromboprophylaxis and obstetric management of the antiphospholipid syndrome

There is consensus in treating patients with antiphospholipid syndrome (APS) and first venous thrombosis with oral anticoagulation to a target international normalized ratio of 2.0 – 3.0. A recent systematic review recommended a target international normalized ratio > 3.0 in the group of patients with APS and arterial thrombosis. The approach for women with obstetric manifestations of APS is based on the use of aspirin plus heparin. In addition, we summarize the evidence-based information about management of catastrophic APS and some difficult cases, such as ‘seronegative’ APS, patients who do not display formal classification criteria for APS, and recurrent thrombotic events despite optimal anticoagulation.

Therapeutic Plasma Exchange: Core Curriculum 2008

Therapeutic Plasma Exchange: Core Curriculum 2008