scholarly journals A case of probable catastrophic antiphospholipid syndrome with multi-organ failure presenting as a transient increase of antiphospholipid antibody levels

2014 ◽  
Vol 37 (3) ◽  
pp. 183-188
Author(s):  
Rubuna SATO ◽  
Hiroshi SATO ◽  
Atsuma NISHIWAKI ◽  
Isamu YOKOE ◽  
Shinji TSURUTA ◽  
...  
Keyword(s):  
Organ Failure ◽  
Antiphospholipid Syndrome ◽  
Transient Increase ◽  
Antiphospholipid Antibody ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Multi Organ Failure ◽  
Antibody Levels

The Catastrophic Antiphospholipid Syndrome 1996: Acute Multi-Organ Failure Associated with Antiphospholipid Antibodies: A Review of 31 Patients

Lupus ◽  
1996 ◽  
Vol 5 (5) ◽  
pp. 414-417 ◽  
Author(s):  
RA Asherson ◽  
J-C Piette
Keyword(s):  
Rheumatoid Arthritis ◽  
Organ Failure ◽  
Antiphospholipid Syndrome ◽  
Disseminated Intravascular Coagulation ◽  
Antiphospholipid Antibodies ◽  
Primary Antiphospholipid Syndrome ◽  
Precipitating Factors ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Myocardial Failure ◽  
Multi Organ Failure

Thirty one patients with antiphospholipid antibodies who developed multi-organ failure (“Catastrophic Antiphospholipid Syndrome”) are reviewed. Thirteen suffered from a ‘Primary’ antiphospholipid syndrome, 13 from defined SLE, 4 from ‘lupus-like’ disease and one from rheumatoid arthritis. In more than one third precipitating factors were evident (e.g. infections, major/minor surgical procedures, oral contraceptives). Death occurred in 60% of patients from a variety of causes (myocardial failure, ARDS, CNS causes or, often a combination). Disseminated Intravascular Coagulation was present in 8 of 31 patients. Plasmapheresis appeared to be useful in several who had not responded to conventional therapy (e.g. IV heparin, steroids, immunosuppression).

scholarly journals Elevated Anti–Annexin V Antibody Levels in Antiphospholipid Syndrome and Their Involvement in Antiphospholipid Antibody Specificities

2000 ◽  
Vol 114 (4) ◽  
pp. 619-628 ◽  
Author(s):  
Hirokazu Ogawa ◽  
Dandan Zhao ◽  
Jeffrey S. Dlott ◽  
Gregory S. Cameron ◽  
Masahide Yamazaki ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Annexin V ◽  
Antiphospholipid Antibody ◽  
Antibody Specificities ◽  
Antibody Levels

scholarly journals COVID-19 and Catastrophic Antiphospholipid Syndrome

2021 ◽  
Author(s):  
Hisyovi Cardenas Suri ◽  
David Jimomila Bening ◽  
Benjamín Demah Nuertey
Keyword(s):  
Organ Failure ◽  
Diagnostic Criteria ◽  
Antiphospholipid Syndrome ◽  
Antinuclear Antibody ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Partial Form ◽  
Critical Patients ◽  
One Year

One year after the beginning of the epidemic, mortality continues to be high despite several different protocols being tried. Critical patients with Covid 19 in some degree of organ failure and thrombotic events meet the diagnostic criteria of a complete or incomplete catastrophic antiphospholipid syndrome (CAPS) or at least we may need to consider a partial form of it. The findings of autopsies and the involvement of different organs and systems are similar to those of CAPS. Currently the only therapy that has been shown to reduce mortality include steroids, anticoagulation and an antinuclear antibody. The same therapy has been shown to be effective for CAPS.

scholarly journals Severe mesenteric ischemia with multiple organ failure in a patient previously treated with a humanized monoclonal antibody against programmed death receptor-1 (pembrolizumab), a case of pembrolizumab associated catastrophic antiphospholipid syndrome?

2020 ◽  
Vol 8 ◽  
pp. 2050313X2097222
Author(s):  
E M W Mintjens-Jager ◽  
M E Vos ◽  
G Kats-Ugurlu ◽  
G A P Hospers ◽  
A Rutgers ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Multiple Organ Failure ◽  
Organ Failure ◽  
Antiphospholipid Syndrome ◽  
Immune Checkpoint ◽  
Immune Checkpoint Inhibitors ◽  
Checkpoint Inhibitors ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome

Immune checkpoint inhibitors are used in the treatment of different types of tumors including melanoma and non-small cell lung carcinoma. The use of these inhibitors is associated with a broad spectrum of immune-related adverse effects. Here we report a case of a patient admitted to the intensive care unit with multiple organ failure due to catastrophic antiphospholipid syndrome following treatment with pembrolizumab, an immune checkpoint inhibitor, because of metastatic melanoma. The presented patient had multiple organ failure of lung, gastro-intestinal, renal, and the liver. Vascular thrombosis was confirmed by both imaging (pulmonary embolism on computed tomography–thorax) and histopathological examination of the intestines. In combination with the presence of IgA anti-cardiolipin antibodies and initially IgM anti-cardiolipin antibodies, catastrophic antiphospholipid syndrome was suspected. Despite treatment with plasmapheresis and corticosteroids, the patient died due to multiple organ failure. Catastrophic antiphospholipid syndrome is difficult to recognize and has high mortality rates despite supportive treatment. In this case report, discussion is provided regarding the possible immunological mechanism behind catastrophic antiphospholipid syndrome during or after treatment with immune checkpoint inhibitors. It is important to realize that in modern intensive care unit, more patients with immune-related adverse effects of the treatment with immune checkpoint inhibitors will be admitted, because of an increase in the number of patients treated with these checkpoint inhibitors. When these patients are admitted on the intensive care unit, multi-disciplinary consultation is important because of the difficulty of early recognition and optimal treatment of these possible lethal side effects.

scholarly journals A Rare Association of Antiphospholipid Antibody Syndrome, Systemic Lupus Erythematosus and Aortic Dissection: A Striking Presentation with Multi-Organ Failure?

2020 ◽  
Author(s):  
Firdevs Ulutaş ◽  
Veli Çobankara ◽  
Aslı Bozdemir ◽  
Uğur Karasu
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Aortic Dissection ◽  
Organ Failure ◽  
Lupus Erythematosus ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Systemic Lupus ◽  
Pregnancy Morbidity ◽  
Multi Organ Failure ◽  
Life Threatening

Systemic lupus erythematosus is a chronic autoimmune disease with a wide variety of clinical presentations induced by different immunocomplexes and autoantibodies. Antiphospholipid antibody syndrome (APLAS) is a life-threatening clinical condition characterized by venous and arterial thromboses or pregnancy morbidity in the presence of persistent moderate/high levels of antiphospholipid antibodies. Aortic dissection is rarely associated with APLAS and always requires prompt diagnosis and early treatment. We report a rare case with a striking presentation. The patient developed multi-organ failure due to lethal aortic dissection and the obstruction of abdominal and thoracic branch vessels.

scholarly journals Improvements in diagnosis and risk assessment of primary and secondary antiphospholipid syndrome

Hematology ◽  
2019 ◽  
Vol 2019 (1) ◽  
pp. 415-420 ◽  
Author(s):  
Michelle Petri
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Risk Identification ◽  
Classification Criteria ◽  
Antiphospholipid Antibody ◽  
Systemic Lupus Erythematosus Disease ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Glycoprotein I ◽  
Thrombosis Risk

Abstract Classification criteria for antiphospholipid syndrome have not been updated since the revised Sapporo classification criteria were published in 2006. These criteria have limitations in that they omit nonclassical manifestations (hematologic and neurologic), include anticardiolipin and anti–β2-glycoprotein I immunoglobulin (Ig)M isotypes, and do not separately consider primary (no autoimmune disease) or secondary (usually systemic lupus erythematosus) disease. Recent findings in antiphospholipid antibody include fluctuation of antiphospholipid antibodies, recognition that IgA isotypes do confer risk, identification of the role of complementopathy in catastrophic antiphospholipid syndrome, and elucidation of the role of thrombosis risk equations.

scholarly journals P0330RENAL INVOLVEMENT IN OBSTETRIC CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Yuia Korotchaeva ◽  
Natalia Kozlovskaya ◽  
Kseniya Demyanova
Keyword(s):  
Organ Failure ◽  
Kidney Function ◽  
Antiphospholipid Syndrome ◽  
Hellp Syndrome ◽  
Early Stage ◽  
Hematological Parameters ◽  
Renal Involvement ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Short Treatment ◽  
Kidney Involvement

Abstract Background and Aims Catastrophic antiphospholipid syndrome (CAPS) is an uncommon, often fatal, variant of the antiphospholipid syndrome (APS) that defined as multiorgan thrombosis affecting three and more organs confirmed 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 overlap with other obstetric TMA syndromes. Because of the high associated mortality rate, prompt recognition and treatment are paramount. Some studies have shown that complement activation contributes in antiphospholipid antibodies (aPL) mediated thrombosis in CAPS. Short treatment with eculizumab can result in a rapid improvement of pts with TMAs other than aHUS with persisted hematological and renal symptoms worsened despite treatment of the TMA-inducing condition. Aims: Analysis of renal involvement, course and outcomes of obstetric CAPS Method From 2016 to 2019 we observed 11 pts aged 19 - 38 years with CAPS, occurred during pregnancy (from 16 to 40 weeks) or postpartum period. Only 1 patient had a diagnosis of APS with SLE before pregnancy. 5 out of 11 pts had the first pregnancy, 6 pts already had a history of pregnancy, but all previous pregnancies were failed: miscarriage or antenatal death of the fetus. Results Preeclampsia (PE) and/or HELLP syndrome, surgical intervention in combination with obstetric complications (bleeding, manual separation of the placenta, Сaesarean delivery, etc.) preceded CAPS manifestation in all pts. Moreover, in 1 patient with SLE, secondary APS and triple aPL positivity, HELLP syndrome developed very early stage of 16 weeks. In all cases, aPL circulation was detected (anti-cardiolipin antibodies and/or anti-beta-2-glycoprotein-1, lupus anticoagulant). All pts (100%) had complete MAHA (microangiopathic hemolysis): hemoglobin level 73,0 ± 19,2 g/l, LDH level 1066,7 ± 1056,7 U/l, schizocytosis), thrombocytopenia (122,0±157,9х10µL) and multi-organ failure (MOF): kidney damage (81%, 9 of 11), liver (63%, 7 of 18), gastrointestinal tract, heart, lungs, central nervous system - 55% (6 of 11), skin (gangrene 1-9%). A feature of kidney involvement was the development of AKI (mean creatinine level 207,6 ±140,3 mg/dl, oliguria or anuria) not all pts (9 of 11), in 2 case was observed only minimnal urinary syndrome without increasing sCr. All pts underwent traditional treatment, including plasma exchange and/or plasma infusions+corticosteroids+anticoagulants, which was effective in 6 out of 11 pts. In the 2 most severe plasma-resistant cases complement-blocking treatment with eculizumab was prescribed, with rapid positive dynamics was observed: the relief of MOF, hematological parameters normalization after the first infusion and recovery of kidney function by the end of the induction course. Favorable outcome with complete normalization of lab parameters and clinical improvement with kidney function normalization was achieved in 8 of 11 women. In 1 patient chronic kidney disease (CKD) stages 3B has occurred as outcome of AKI. 2 pts died despite ongoing therapy with PT + ST+IG+AC from progressive multiple organ failure. Conclusion 1. Сomplicated obstetric history in patients with acute obstetric TMA requires mandatory exception of CAPS 2. Proven role of complement system involvement in the pathogenesis of CAPS gives a reason for the complement-blocking therapy appointment in severe postpartum TMA, which increases the patients' chance of recovery.

scholarly journals Vascular Endothelial Cell Function in Catastrophic Antiphospholipid Syndrome: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
B. Routy ◽  
T. Huynh ◽  
R. Fraser ◽  
C. Séguin
Keyword(s):  
Mortality Rate ◽  
Antiphospholipid Syndrome ◽  
Cell Function ◽  
Myocardial Infarct ◽  
Immunosuppressive Agents ◽  
Vascular Endothelial Cell ◽  
Antiphospholipid Antibody ◽  
Antiphospholipid Antibody Syndrome ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Endothelial Cell Function

Catastrophic antiphospholipid syndrome (CAPS) is a rare autoimmune condition, which has been associated with a high mortality rate. However, with current management that includes a combination of anticoagulation, glucocorticoid administration, and plasma exchange, mortality rate has declined. Despite survival improvement with new generation immunosuppressive agents, their mechanisms of action are poorly defined, and CAPS is still considered a high-risk complication in patients known with antiphospholipid antibody syndrome. Herein, we present a case of a 79-year-old male who presented with a myocardial infarct and renal failure secondary to CAPS following a splenectomy for immune thrombocytopenia. Regardless of rapid combination of first-line treatment and rituximab therapy, the patient developed lethal cardiogenic shock secondary to mitral valve papillary muscle necrosis. Discussion of the pathophysiology and avenues of future therapies in CAPS are reported.

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