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Catastrophic antiphospholipid syndrome

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...
October 5, 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.
August 7, 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
Karthik Nath, Andrew McCann
Catastrophic antiphospholipid syndrome is a rare condition with high morbidity and mortality. We present a refractory case of catastrophic antiphospholipid syndrome with a view to highlight the importance of early identification and aggressive treatment of this condition. A 36-year-old female presented with clinical manifestations of multiorgan vascular occlusion with a known history of primary antiphospholipid syndrome. The presentation was on a background of a recent change of her long-term anticoagulation from warfarin to therapeutic low-molecular-weight heparin...
2018: Case Reports in Hematology
Ericka Vargas-Quevedo, Eduardo Ordoñez-Gutiérrez, Jorge Enrique Trejo-Gómora, Lénica Anahí Chávez-Aguilar, Rubén Peña-Vélez
INTRODUCTION: The antiphospholipid syndrome is an acquired autoimmune thrombophilia, characterized by arterial and/or venous thrombosis. Rarely, this condition can have a catastrophic presenta tion, with high mortality, and presence of microangiopathy and involvement of three or more organs. OBJECTIVE: To describe the clinical presentation and evolution of a pediatric patient with catastrophic antiphospholipid syndrome, with a seronegative onset form, whose response to aggressive therapy was favorable...
April 2018: Revista Chilena de Pediatría
Ricard Cervera, Ignasi Rodríguez-Pintó, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is a life-threating variant of the antiphospholipid syndrome characterized by the development of multiple thrombosis in a short period of time, usually ending up in the failure of function of several vital organs. Most CAPS episodes are related to a prothrombotic situation or precipitating factor such as infections, surgical procedures or malignant diseases. In patients with CAPS, the development of multiple thrombosis leads to an important cytokine release that worsens the already critical patient's situation...
August 2018: Journal of Autoimmunity
Aynur Soyuöz, Ömer Karadağ, Tülay Karaağaç, Levent Kılıç, Şule Apraş Bilgen, Osman İlhami Özcebe
Objective: Therapeutic plasma exchange (TPE) offers an alternative therapeutic modality for patients with systemic lupus erythematosus (SLE) and primary antiphospholipid syndrome (APS). However, there is conflicting evidence regarding its efficacy in different sub-phenotypes. This study aimed to investigate the main clinical characteristics and outcomes of patients with different phenotypes of SLE and APS treated with TPE at a tertiary care center. Methods: The database of the Blood and Apheresis Unit between 2001 and 2013 was screened for patients with SLE and primary APS...
March 2018: European Journal of Rheumatology
Gökhan Özgür, Cengiz Beyan
Catastrophic thrombotic syndrome is characterized by occurrence of several thromboembolic occlusions which affect a variety of vascular beds over a short period of time. Catastrophic Antiphospholipid syndrome (CAPS) (1%) is regarded as a rare but severe variant of Antiphospholipid Syndrome. Treatment with anticoagulants, steroids and therapeutic plasma exchange has been shown to give the best results. However, to treat CAPS, the best substitute for plasma is still a debatable issue because there are no standard methods...
February 2018: Transfusion and Apheresis Science
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