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Catastrophic antiphospholipid syndrome

Victoria Sadick, Stuart Lane, Eddy Fischer, Ian Seppelt, Anita Shetty, Anthony McLean
Catastrophic antiphospholipid syndrome is a rare multisystem autoimmune condition characterised by rapid development of widespread thrombotic disease and subsequent multi-organ failure. It is the most severe complication of antiphospholipid syndrome, carrying significant morbidity and mortality. We report a patient with post-partum catastrophic antiphospholipid syndrome with cardiac, hepatic, renal and cutaneous manifestations. The diagnostic challenges in establishing a definitive diagnosis in catastrophic antiphospholipid syndrome is discussed, along with the difficulties in managing these patients in the intensive care unit...
November 2018: Journal of the Intensive Care Society
John M Gansner, Nancy Berliner
Catastrophic antiphospholipid antibody syndrome (CAPS) and macrophage activation syndrome (MAS) are both life-threatening hematologic disorders that infrequently afflict patients with rheumatologic disease. CAPS is characterized by fulminant multiorgan damage related to small vessel thrombosis in the setting of persistent antiphospholipid antibodies. It can occur in patients with rheumatologic diseases such as systemic lupus erythematosus but can also affect patients who do not have rheumatologic disease. By contrast, the term MAS is applied when patients with rheumatologic disease develop hemophagocytic lymphohistiocytosis (HLH); therefore, patients with MAS have an underlying rheumatologic disease by definition...
November 30, 2018: Hematology—the Education Program of the American Society of Hematology
R Shah, S V Brodsky, L Hebert, B H Rovin, T Nadasdy, A A Satoskar
Antiphospholipid antibody syndrome (APS) is an acquired prothrombotic autoimmune disease caused by the presence of antibodies against anionic phospholipids or plasma proteins bound to phospholipids on cell membranes. It can be a primary disease or secondary to other autoimmune diseases, most commonly systemic lupus erythematosus (SLE). Laboratory testing for antiphospholipid antibodies (aPL) may be only transiently positive, so APS could be missed until a catastrophic thrombotic episode or pregnancy morbidity occurs...
November 7, 2018: Lupus
Jose Maria Morales, Manuel Serrano, Jose Antonio Martinez-Flores, Dolores Perez, Antonio Serrano
Renal allograft thrombosis is the most frequent and devastating complication in the early postrenal transplantation period. Several risk factors to develop graft thrombosis depending on donors and recipients are well known. Antiphospholipid syndrome (APS) is well recognized as an important cause of kidney injury, with specific clinical and histological features that may lead to renal injury caused by thrombosis at any location within the renal vasculature. There are 3 forms of APS, primary (the most common form), associated to other systemic autoimmune diseases (SAD-APS) and catastrophic...
October 30, 2018: Transplantation
Rosanna Satta, Gabriele Biondi
Antiphospholipid syndrome (APS) is an autoimmune thrombophilic condition characterized by the onset of venous and/or arterial thrombosis, often multiple, and pregnancy morbidity in a background of antiphospholipid antibodies (aPL) positivity. Some patients can be carrier of aPL with no clinical symptoms, in other cases clinical manifestation can range from the classical presentation to an acute life-threatening condition named Catastrophic APS. APS can be considered as primary or associated to other disease, however pregnancy acts as a triggering factor on a susceptible background that lead to the clinical manifestations through immunological and non-immunological mechanism...
October 29, 2018: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
M Plüß, M Zeisberg, G A Müller, R Vasko, P Korsten
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...
November 2018: Lupus
Patrizia Rovere-Querini, Valentina Canti, Roberta Erra, Esperia Bianchi, Giorgio Slaviero, Armando D'Angelo, Susanna Rosa, Massimo Candiani, Maria Teresa Castiglioni
RATIONALE: Hypercoagulability and pregnancy morbidity are hallmarks of the antiphospholipid syndrome (APS). Catastrophic antiphospholipid syndrome (CAPS) is a potentially life-threatening omplication of APS, with widespread acute thrombotic microangiopathy (TMA) that can be precipitated by pregnancy and delivery and result in multiorgan damage. Unrestrained activation of the complement cascade is involved, favoring endothelial activation, tissue factor expression by leukocytes, and platelet aggregation...
October 2018: Medicine (Baltimore)
Amelia Ruffatti, Giustina De Silvestro, Piero Marson, Marta Tonello, Antonia Calligaro, Maria Favaro, Teresa Del Ross, Ariela Hoxha, Elena Mattia, Vittorio Pengo
The study aimed to evaluate the clinical significance of laboratory findings in patients with catastrophic antiphospholipid syndrome (CAPS) and to report the effects of a well-defined treatment protocol in 14 consecutive cases. Thirteen patients (12 presenting one and one presenting two episodes of CAPS) were consecutively treated and monitored between 1986 and 2017. Antiphospholipid antibody (aPL) characteristics of the patients were compared with those of 64 matched controls (45 antiphospholipid syndrome patients and 19 aPL carriers) who did not develop CAPS during the same mean follow-up period (12 years ± 9...
September 2018: Journal of Autoimmunity
Claudia Mendoza-Pinto, Mario García-Carrasco, Ricard Cervera
PURPOSE OF REVIEW: The antiphospholipid syndrome (APS) is characterized by the development of thrombotic events and pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). An infectious etiology for this syndrome has been postulated. The present review is aimed to summarize recent evidence about the role of infections and vaccines in the pathogenesis of the APS (including its catastrophic variant). RECENT FINDINGS: There is an increased risk of developing aPL in various infections, particularly in viral infections...
August 20, 2018: Current Rheumatology Reports
Myat Han Soe, Krishna Adit Agarwal, Alueshima Akough-Weir
Catastrophic antiphospholipid syndrome (CAPS) is a rare but highly fatal clinical syndrome that occurs in up to 1% of patients with antiphospholipid syndrome (APS). The diagnosis of CAPS is often delayed because its presentation with multiple organ thromboses can be confused with other thrombotic microangiopathies and severe sepsis. We report a case of CAPS in a patient with APS and systemic lupus erythematosus (SLE) presenting with thrombotic storm precipitated by trauma, cytomegalovirus (CMV) infection, and noncompliance with anticoagulation therapy...
2018: Case Reports in Medicine
Mathilde Guillot, Cedric Rafat, David Buob, Paul Coppo, Matthieu Jamme, Eric Rondeau, Olivier Fain, Arsène Mekinian
No abstract text is available yet for this article.
November 1, 2018: Rheumatology
Zakaria Hindi, Mosa Hussein, Abdallah Gad, Abdallah A Khaled, Talal Zahoor
Catastrophic antiphospholipid syndrome is a rare disorder that remains under-recognized causing a high mortality rate even with treatment. Factors such as infections and systemic lupus erythematosus flare play as an inciting event in the thrombotic crisis which underlies catastrophic antiphospholipid syndrome. The use of plasmapheresis has improved the outcome of such cases with a reduction in mortality rate from over 50% to less than 30%, according to some studies. However, the definitive treatment of this disabling and fatal condition remains an area warranting research...
2018: SAGE Open Medical Case Reports
Mohamed Momtaz, Ahmed Fayed, Khaled Marzouk, Amr Shaker
Therapeutic plasma exchange is used in treating different immunological and non-immunological diseases. We analyzed the outcome of 308 patients treated by 1783 membrane plasma exchange sessions from January 2011 until January 2017 at Cairo University Hospital. Thrombotic microangiopathies were the commonest indication [73 (23.7%) patients] with response in 63/73 patients (86.3%), followed by systemic vasculitis with pulmonary-renal involvement [40(13%) patients] with recovery in 32/40 patients (80.0%), Guillain-Barré syndrome [39(12...
July 15, 2018: Therapeutic Apheresis and Dialysis
Vittorio Pengo, Gentian Denas
Antiphospholipid Syndrome (APS) is a condition characterized by the occurrence of thromboembolic events and/or pregnancy loss combined with one laboratory criterion among Lupus Anticoagulant- LAC, anticardiolipin -aCL, and anti β2-Glycoprotein I -aβ2GPI antibodies. Several hypotheses were put forward to explain the causal role of antibodies in the clinical events but none is fully convincing. Current laboratory diagnosis is based on three tests (LAC, IgG/IgM aβ2GPI and IgG/IgM aCL antibodies). The triple-positive profile (all the three tests positive, same isotype) is associated with a higher risk for thrombosis...
September 2018: Thrombosis Research
Romain Paule, Nathalie Morel, Véronique Le Guern, Micaela Fredi, Laetitia Coutte, Meriem Belhocine, Luc Mouthon, Claire le Jeunne, Anthony Chauvin, Jean-Charles Piette, Nathalie Costedoat-Chalumeau
OBJECTIVES: To assess the limitations of the SLICC (Systemic Lupus International Collaborating Clinics) classification criteria for systemic lupus erythematosus (SLE), in patients with primary antiphospholipid syndrome (PAPS). METHODS: Retrospective study of a cohort of APS patients (Sydney criteria). We successively excluded patients with (1) at least one "SLE-specific" manifestation (biopsy-proven SLE nephropathy, arthritis, cutaneous, or neurologic SLE manifestations, pericarditis, autoimmune haemolytic anaemia, oral and nasal ulcers, non-scarring alopecia, anti-dsDNA, and anti-Sm antibodies), (2) any other autoimmune connective tissue disease, and/or (3) antinuclear antibodies >1/320...
September 2018: Autoimmunity Reviews
Kanon Jatuworapruk, Lantarima Bhoopat, Punchong Hanvivadhanakul
OBJECTIVES: To describe the characteristics of patients with antiphospholipid syndrome (APS) in an Asian clinical practice setting. METHODS: We conducted a single-center, retrospective study of APS patients attending the rheumatology or hematology clinics, between January 2012 and December 2016. RESULTS: There were 450 patients suspected of having APS referred to our clinics. Seventy-four (16.4%) were diagnosed of APS, 51% of which were definite...
July 8, 2018: Asian Pacific Journal of Allergy and Immunology
K Legault, H Schunemann, C Hillis, C Yeung, E A Akl, M Carrier, R Cervera, M Crowther, F Dentali, D Erkan, G Espinosa, M Khamashta, J J Meerpohl, K Moffat, S O'Brien, V Pengo, J H Rand, I Rodriguez Pinto, L Thom, A Iorio
Background The McMaster RARE-Bestpractices project group selected the catastrophic antiphospholipid syndrome (CAPS) for a pilot exercise in guideline development for a rare disease. Objectives The objectives of this exercise were to provide a proof of principle that guidelines can be developed for rare diseases and assist in clinical decision making for CAPS. Patients/Methods The GIN-McMaster Guideline Development checklist and GRADE methodology were followed throughout the guideline process. The CAPS guideline was coordinated by a steering committee, and the guideline panel was formed with representation from all relevant stakeholder groups...
June 7, 2018: Journal of Thrombosis and Haemostasis: JTH
Liza Mariam Thomas, Niaz Ahmed Shaikh, Ranjana Pradeep
A 43-year-old woman with no known cardiovascular risk factors was admitted with a second episode of ischaemic stroke. She was not a known case of connective tissue disease like systemic lupus erythematosus or antiphospholipid syndrome (APS). During the current episode, she was found to have markedly deranged coagulation parameters and laboratory evidence of microangiopathic haemolysis, but no evidence of sepsis or active bleeding. Further investigation revealed multiple organ infarcts. A diagnosis of probable catastrophic APS was made and she improved dramatically with a combination of plasmapheresis, corticosteroids and therapeutic anticoagulation...
June 4, 2018: BMJ Case Reports
Konstantinos Tselios, Dafna D Gladman, Zahi Touma, Jiandong Su, Nicole Anderson, Murray B Urowitz
OBJECTIVE: Disease course in systemic lupus erythematosus (SLE) is primarily relapsing-remitting. Long quiescent and chronically active patterns are less frequent. We recently described an atypical "monophasic" course in a small number of patients. The aim of the present study was to assess the prevalence and characteristics of such patients in a defined SLE cohort. METHODS: The inception patients of the University of Toronto Lupus Clinic (enrolled within 18 mos of diagnosis) were investigated...
August 2018: Journal of Rheumatology
Eryk Hakman, Sasha Mikhael
Background: Catastrophic APS (CAPS) is a rare but life-threatening form of APS defined as multiorgan thrombosis affecting a minimum of three organs with confirmation by histopathology of small vessel occlusions in at least one organ or tissue. The development of CAPS in pregnancy poses many diagnostic challenges as a result of its broad range of clinical presentations and its overlap with other obstetric complications and microangiopathic diseases. Because of the high associated mortality rate, prompt recognition and treatment are paramount...
2018: Case Reports in Obstetrics and Gynecology
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