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Rituximab and antiphospholipid

Felisa Vázquez Gómez, María Prieto Arce, Luis Ignacio González-Granado, Eugenia Enríquez Merayo, Jaime de Inocencio Arocena
No abstract text is available yet for this article.
October 21, 2016: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
Nathalie Costedoat-Chalumeau, Laetitia Coutte, Véronique Le Guern, Nathalie Morel, Gaelle Leroux, Romain Paule, Luc Mouthon, Jean-Charles Piette
The catastrophic antiphospholipid syndrome (CAPS) develops in at least 1% of patients with antiphospholipid syndrome, either primary or associated with systemic lupus erythematosus. CAPS reveals the antiphospholipid syndrome in about 50% of cases. The CAPS is characterized by rapidly-progressive widespread thromboses mainly affecting the microvasculature in the presence of antiphospholipid antibodies. In a few days, the patients develop multiorgan failure with renal insufficiency with severe hypertension, pulmonary, cerebral, cardiac, digestive and/or cutaneous involvement...
September 9, 2016: La Presse Médicale
Ulf Müller-Ladner
BACKGROUND: Although the gastrointestinal (GI) tract including its related organs is not generally regarded as one of the primary organ systems of primary and secondary vasculitic disorders, there are numerous mechanisms of these diseases operative in or around the different structures and compartments of the GI tract. KEY MESSAGES: A majority of the respective clinical symptoms and problems are linked to an alteration of (peri)vascular homeostasis. Alteration of perivascular matrix metabolism can also affect the functional integrity and motility of the GI tract...
2016: Digestive Diseases
Mojca Bizjak, Or Bruck, Darja Kanduc, Sonja Praprotnik, Yehuda Shoenfeld
A 13-year-old girl developed immune thrombocytopenic purpura (ITP) and concomitant positive antiphospholipid antibodies (aPL) following vaccination with a quadrivalent human papillomavirus (HPV) vaccine. During the course of a disease, she developed clinical manifestation with bleeding and she was treated with intravenous immunoglobulins. Consequently, the number of her platelets remained critically low and she was put on corticosteroids and rituximab. Since then, her platelet count remain within the normal range, but her aPL are still present...
April 2016: Seminars in Hematology
M D Romero-Caballero, I Lozano-García, C Gómez-Molina, A I Gil-Liñán, I Arcas
CASE REPORT: We report a case of primary small-cell lymphocytic lacrimal gland lymphoma in a male diagnosed with primary antiphospholipid syndrome. These rare lymphomas are usually presented in the clinic as disseminations secondary to chronic lymphocytic leukaemia, and the primary site is rare in the orbit. DISCUSSION: Non-Hodgkin lymphomas are a heterogeneous group of tumours. Although treatment in the IE stage is usually radiotherapy, due to its association with antiphospholipid syndrome, systemic treatment with rituximab was administered...
June 3, 2016: Archivos de la Sociedad Española de Oftalmología
Shiber Shachaf, Molad Yair
BACKGROUND: Cryoglobulinemia is an immune-complex-mediated small vessel vasculitis that classically involves the skin, kidneys and peripheral nerves. Antiphospholipid syndrome (APS) is an autoimmune hypercoagulable disorder which causes blood vessel thrombosis. It can present as a multi-organ microthrombotic disorder which is called catastrophic APS. OBJECTIVE: In this case series we aim to describe the diagnostic and management challenges that arise when these two severe disorders simultaneously present in the same patient...
January 2016: Revista Brasileira de Reumatologia
Ahlam Al Marzooqi, Alessia Leone, Jamal Al Saleh, Munther Khamashta
Antiphospholipid syndrome (APS) is a prothrombotic disease characterized by thrombosis and pregnancy morbidity in the presence of antiphospholipid antibodies (apL). Management of thrombosis is based on long-term oral anticoagulation and patients with arterial events should be treated aggressively. Primary thrombo-prophylaxis is recommended in patients with systemic lupus erythromatosus (SLE) and obstetric APS. Obstetric APS care is based on high-risk management and treatment with aspirin and heparin. Possible future therapies include statins, hydroxychloroquine, rituximab, and new anticoagulant drugs...
September 2016: Expert Review of Clinical Immunology
Chrong-Reen Wang, Ming-Fei Liu
OBJECTIVES: Although the data from primary antiphospholipid syndrome (APS) suggests a beneficial effect of rituximab usage, its therapeutic role remains to be defined in systemic lupus erythematosus (SLE)-associated APS, a complex clinical situation with thrombotic events and lupus activity. METHODS: A single-center retrospective analysis of rituximab usages in APS was performed in 800 hospitalized SLE patients. RESULTS: There were 63 SLE-associated APS cases with 6 on rituximab therapy, all female aged 37...
August 2016: Seminars in Arthritis and Rheumatism
Nayef M Kazzaz, W Joseph McCune, Jason S Knight
PURPOSE OF REVIEW: Catastrophic antiphospholipid syndrome (CAPS) is a severe manifestation of antiphospholipid syndrome (APS). Although affecting only 1% of patients with APS, the condition is frequently fatal if not recognized and treated early. Here, we will review the current approach to diagnosis and treatment of CAPS. RECENT FINDINGS: Data from the international 'CAPS registry', spearheaded by the European Forum on Antiphospholipid Antibodies, have improved our understanding of at-risk patients, typical clinical features, and precipitating diagnoses...
May 2016: Current Opinion in Rheumatology
César Magro-Checa, Elisabeth J Zirkzee, Tom W Huizinga, Gerda M Steup-Beekman
Neuropsychiatric systemic lupus erythematosus (NPSLE) is a generic definition referring to a series of neurological and psychiatric symptoms directly related to systemic lupus erythematosus (SLE). NPSLE includes heterogeneous and rare neuropsychiatric (NP) manifestations involving both the central and peripheral nervous system. Due to the lack of a gold standard, the attribution of NP symptoms to SLE represents a clinical challenge that obligates the strict exclusion of any other potential cause. In the acute setting, management of these patients does not differ from other non-SLE subjects presenting with the same NP manifestation...
March 2016: Drugs
A Fanouriakis, C Pamfil, P Sidiropoulos, L Damian, A Flestea, G Gusetu, S Rednic, G Bertsias, D T Boumpas
Cyclophosphamide (CYC) is used in severe neuropsychiatric systemic lupus erythematosus (NPSLE), but long-term data regarding its efficacy and safety are lacking. We identified NPSLE cases who received CYC from two centres during the period 1999-2013 and had regular follow-up. General and neuropsychiatric outcome at last follow-up visit were determined, and major complications were documented. CYC was administered in 50 neuropsychiatric events. Median age was 45.0 years and 46% of patients were positive for antiphospholipid antibodies...
May 2016: Lupus
Masanobu Horikoshi, Shigeko Inokuma, Erika Matsubara, Yasunori Honda, Rika Okada, Mika Kobuna, Yoko Miura, Shoko Kobayashi, Shigeo Kawai, Toshio Kumasaka
Catastrophic antiphospholipid syndrome (CAPS) survivors rarely relapse. We herein report a case of a second CAPS episode with an unusual subacute course and no microangiopathic hemolytic anemia (MAHA), a common CAPS symptom. During the first episode, the 69-year-old woman responded well to high-dose glucocorticoids and plasma exchange. On relapse, these treatments plus rituximab were ineffective and she died of multi-organ failure and bacterial cholangitis. The absence of MAHA and a subacute course do not exclude a CAPS recurrence...
2015: Internal Medicine
Nayef M Kazzaz, Patrick Coit, Emily E Lewis, W Joseph McCune, Amr H Sawalha, Jason S Knight
OBJECTIVES: While diffuse alveolar haemorrhage (DAH) is recognised as a life-threatening complication of systemic lupus erythematosus (SLE), little is known about its risk factors and response to treatment. We describe 22 cases of DAH in a US lupus cohort of approximately 1000 patients, and compare them to 66 controls from the same outpatient cohort. METHODS: We captured variables pertaining to diagnoses of SLE and secondary antiphospholipid syndrome (APS), and analysed them by univariate testing...
2015: Lupus Science & Medicine
A N Rosenbaum, N S Anavekar, F C Ernste, S V Mankad, R J Le, K K Manocha, G W Barsness
This present case pertains to a 48-year-old woman with a history of antiphospholipid syndrome, who presented with progressive fatigue, generalized weakness, and orthopnea acutely. She had a prior diagnosis of antiphospholipid syndrome with recurrent deep vein thromboses (DVTs) and repeated demonstration of lupus anticoagulants. She presented in cardiogenic shock with markedly elevated troponin and global myocardial dysfunction on echocardiography, and cardiac catheterization revealed minimal disease. Cardiac magnetic resonance imaging was performed, which revealed findings of perfusion defects and microvascular obstruction, consistent with the pathophysiology of catastrophic antiphospholipid syndrome (CAPS)...
October 2015: Lupus
Monica Galli
The antiphospholipid syndrome is characterized by a combination of laboratory findings (i.e., the presence of at least one antiphospholipid antibody) and clinical manifestations (arterial and/or venous thrombosis, obstetrical complications). Long-term oral anticoagulant is recommended to prevent recurrence of both arterial and venous thrombosis, whereas (low molecular weight) heparin plus aspirin is the treatment of choice to prevent further obstetrical complications. In the rare case of catastrophic antiphospholipid syndrome, heparin plus high-dose corticosteroids plus plasma exchange is associated with the highest recovery rate...
June 2014: Auto- Immunity Highlights
Anum Fayyaz, Ann Igoe, Biji T Kurien, Debashish Danda, Judith A James, Haraldine A Stafford, R Hal Scofield
Our purpose was to compile information on the haematological manifestations of systemic lupus erythematosus (SLE), namely leucopenia, lymphopenia, thrombocytopenia, autoimmune haemolytic anaemia (AIHA), thrombotic thrombocytopenic purpura (TTP) and myelofibrosis. During our search of the English-language MEDLINE sources, we did not place a date-of-publication constraint. Hence, we have reviewed previous as well as most recent studies with the subject heading SLE in combination with each manifestation. Neutropenia can lead to morbidity and mortality from increased susceptibility to infection...
2015: Lupus Science & Medicine
Amy E Lin, Nadine Shehata, Heather Reich, David Barth, Carl Laskin, Stephen Lapinsky, Shital Gandhi
OBJECTIVES: We outline a case of a woman with lupus (SLE) and antiphospholipid syndrome (APLA) at 26 weeks' gestation who develops a complex multiorgan condition with a broad differential. METHODS: Case report. RESULTS: A 34 year old woman, G1P0, presented at 26 weeks gestation with a 5 day history of epigastric pain and emesis. She has a history of lupus anticoagulant (LAC), anticardiolipin (ACL) and anti-dsDNA (ADD) positive SLE and APLA syndrome diagnosed at age 13 with multiple small cerebral infarcts...
January 2015: Pregnancy Hypertension
G Sukara, M Baresic, M Sentic, L Brcic, B Anic
Catastrophic antiphospholipid syndrome (CAPS) is a rare, acute, life-threatening form of antiphospholipid syndrome. In the last several decades there has been a significant improvement in the treatment of patients with CAPS, but the overall mortality is still very significant. The use of rituximab has been reported in the treatment of refractory cases of CAPS but the data are still scarce and inconclusive. We report a case of 47-year old male patient with long standing SLE and secondary APS who presented with acute thromboembolic incident (partial thrombosis of superior mesenteric artery)...
April 2015: Acta Reumatológica Portuguesa
Chayawee Muangchan, Ronald F van Vollenhoven, Sasha R Bernatsky, C Douglas Smith, Marie Hudson, Murat Inanç, Naomi F Rothfield, Peter T Nash, Richard A Furie, Jean-Luc Senécal, Vinod Chandran, Ruben Burgos-Vargas, Rosalind Ramsey-Goldman, Janet E Pope
OBJECTIVE: To establish agreement on systemic lupus erythematosus (SLE) treatment. METHODS: SLE experts (n = 69) were e-mailed scenarios and indicated preferred treatments. Algorithms were constructed and agreement determined (≥50% respondents indicating ≥70% agreement). RESULTS: Initially, 54% (n = 37) responded suggesting treatment for scenarios; 13 experts rated agreement with scenarios. Fourteen of 16 scenarios had agreement as follows: discoid lupus: first-line therapy was topical agents and hydroxychloroquine and/or glucocorticoids then azathioprine and subsequently mycophenolate (mofetil); uncomplicated cutaneous vasculitis: initial treatment was glucocorticoids ± hydroxychloroquine ± methotrexate, followed by azathioprine or mycophenolate and then cyclophosphamide; arthritis: initial therapy was hydroxychloroquine and/or glucocorticoids, then methotrexate and subsequently rituximab; pericarditis: first-line therapy was nonsteroidal antiinflammatory drugs, then glucocorticoids with/without hydroxychloroquine, then azathioprine, mycophenolate, or methotrexate and finally belimumab or rituximab, and/or a pericardial window; interstitial lung disease/alveolitis: induction was glucocorticoids and mycophenolate or cyclophosphamide, then rituximab or intravenous gamma globulin (IVIG), and maintenance followed with azathioprine or mycophenolate; pulmonary hypertension: glucocorticoids and mycophenolate or cyclophosphamide and an endothelin receptor antagonist were initial therapies, subsequent treatments were phosphodiesterase-5 inhibitors and then prostanoids and rituximab; antiphospholipid antibody syndrome: standard anticoagulation with/without hydroxychloroquine, then a thrombin inhibitor for venous thrombosis, versus adding aspirin or platelet inhibition drugs for arterial events; mononeuritis multiplex and central nervous system vasculitis: first-line therapy was glucocorticoids and cyclophosphamide followed by maintenance with azathioprine or mycophenolate, and then rituximab, IVIG, or plasmapheresis; and serious lupus nephritis: first-line therapy was glucocorticoids and mycophenolate, then cyclophosphamide then rituximab...
September 2015: Arthritis Care & Research
Ignasi Rodríguez-Pintó, Ricard Cervera, Gerard Espinosa
The catastrophic antiphospholipid syndrome (CAPS) is characterized by thrombosis in more than three organs or systems developing over a short period of time. Despite conventional treatment with a combination of anticoagulation plus corticosteroids plus plasma exchange, and/or intravenous immunoglobulin, mortality remains high and some patients suffer from recurrent CAPS episodes. In selected patients, new therapies such as rituximab may be a treatment option. In this review, the rationale for using rituximab in CAPS is discussed...
February 2015: Therapeutic Advances in Musculoskeletal Disease
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