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.

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 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 Early diagnosis and successful treatment of catastrophic antiphospholipid syndrome complicated by multiple organ failure

2010 ◽  
Vol 33 (1) ◽  
pp. 24-30 ◽  
Author(s):  
Daichi INOUE ◽  
Katsuhiro TOGAMI ◽  
Norihiro SHIMOIKE ◽  
Ryo TAMURA ◽  
Yukihiro IMAI ◽  
...  
Keyword(s):  
Early Diagnosis ◽  
Multiple Organ Failure ◽  
Organ Failure ◽  
Antiphospholipid Syndrome ◽  
Successful Treatment ◽  
Multiple Organ ◽  
Catastrophic Antiphospholipid Syndrome

scholarly journals A probable case of catastrophic antiphospholipid syndrome: Should high-dose steroids be given in the setting of polymicrobial sepsis?

2019 ◽  
Vol 7 ◽  
pp. 2050313X1983953 ◽  
Author(s):  
Shanna Ariane Tucker ◽  
Justin Choi ◽  
Dhruv Khullar
Keyword(s):  
Diagnostic Criteria ◽  
Antiphospholipid Syndrome ◽  
Clinical Manifestations ◽  
Intravenous Immunoglobulins ◽  
Polymicrobial Sepsis ◽  
High Dose ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Probable Case ◽  
Best Management ◽  
Uremic Syndrome

In this clinical vignette, we present a case of a 59-year-old woman with catastrophic antiphospholipid syndrome likely triggered by polymicrobial sepsis. The diagnostic criteria and clinical manifestations of catastrophic antiphospholipid syndrome are reviewed. We also compare diagnostic criteria and clinical manifestations with other clinical entities in the differential diagnosis, including thrombotic thrombocytopenic purpura–hemolytic-uremic syndrome, disseminated intravascular coagulation, sepsis, and inflammatory bowel disease. Catastrophic antiphospholipid syndrome is a rare, but lethal condition, and treatment recommendations are based on expert consensus and analyses of the international Catastrophic Antiphospholipid Syndrome Registry. Current management guidelines recommend triple therapy, with anticoagulation, glucocorticoids, and plasma exchange or intravenous immunoglobulins. This case brings this rare clinical entity to the attention of clinicians and emphasizes the need for more research to understand the best management. It also raises the question of whether high-dose steroids should be continued for treatment of catastrophic antiphospholipid syndrome in the setting of a severe sepsis.

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

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.

Catastrophic antiphospholipid syndrome with bilateral adrenal hemorrhage: a case report

2019 ◽  
Author(s):  
De Marchi Lucrezia ◽  
M K de Filette Jeroen ◽  
Sol Bastiaan ◽  
E Andreescu Corina ◽  
Kunda Rastislav ◽  
...  
Keyword(s):  
Case Report ◽  
Antiphospholipid Syndrome ◽  
Adrenal Hemorrhage ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Bilateral Adrenal Hemorrhage

scholarly journals AB0310 TOLERABILITY AND SAFETY OF 23-VALENT POLYSACCHARIDE PNEUMOCOCCAL VACCINE IN PATIENTS WITH ANTIPHOSPHOLIPID SYNDROME, PRELIMINARY RESULTS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1180.3-1181
Author(s):  
G. Tarasova ◽  
B. Belov ◽  
M. Cherkasova ◽  
E. Aseeva ◽  
T. Reshetnyak ◽  
...  
Keyword(s):  
Antiphospholipid Syndrome ◽  
Lupus Erythematosus ◽  
Pneumococcal Vaccine ◽  
Respiratory Infections ◽  
Vascular Complications ◽  
Initial Increase ◽  
Post Vaccination ◽  
Preliminary Results ◽  
One Year ◽  
Polysaccharide Pneumococcal Vaccine

Background:Antiphospholipid syndrome (APS) is an autoimmune disease often associated with severe, life-threatening vascular complications. The majority of patients (and in case of secondary APS, in 100% of cases) receive immunosuppressive therapy. Immunization with pneumococcal vaccines in patients with both primary APS (PAPS) and APS+SLE or secondary (sAPS) is necessary to prevent severe respiratory infections in these patients.Objectives:Purpose of the study - to study the tolerance and safety of 23-valent polysaccharide pneumococcal vaccine (PPV-23) in patients with PAPS and sAPS.Methods:At this stage, the study included 28 patients with APS, of which 10 with PAPS, 18 with sAPS proceeding against the background of systemic lupus erythematosus (SLE), of which 23 women (82%), 5 men (18%). The average age (Me) of patients was 43 (35.5; 53.0) g. 20 patients received glucocorticoids (GC) 5-30 mg/day equivalent to prednisone, 17- hydroxychloroquine, 6- cytostatics (3-cyclophosphamide, 2-azathioprine, 1- mycophenolate mofetil), 8- biologics: 5-rituximab (RTM), 3-belimumab (BLM); 20-received anticoagulants (direct-10, indirect-10).1 dose (0.5 ml) of PPV-23 was administered subcutaneously. The follow-up time was 1 year in 23 patients and 5-5.5 months in 5-5.5 months. During the visits, standard clinical and laboratory tests were performed, immunological blood test and the level of antibodies to S.pneumoniaeResults:Vaccination was well tolerated in all patients. In 29% of cases, vaccine reactions of mild severity were observed: in 7 (25%) - a local reaction (pain in the arm for 1-3 days-at 7, redness up to 2 cm at the injection site-at 1), in 1 (3,6%), the patient experienced general weakness (moderately pronounced) for 1 month. Vaccinal reactions were completely reversible and did not require additional prescriptions. Post-vaccination complications develop, as a rule, in the first 1-2 months after vaccination. During the observation period, none of the patients had an exacerbation of the disease, reliably associated with the vaccination. There was no recurrence of thrombosis, both in patients receiving anticoagulant therapy and without it. No new autoimmune phenomena, both clinical and laboratory, were identified. The dynamics of the production of anti-streptococcal antibodies during the year was followed in 16 patients. One year after vaccination, 31% of patients showed a significant (more than 2-fold compared to the initial) increase in the concentration of antibodies to polysaccharides of the cell wall of S. pneumoniae (“responders”), 69% of patients were “non-responders” to the vaccine. At the same time, all 5 patients with PAPS were “non-responders”, and 45.5% “respondents” with sAPS.Conclusion:Preliminary results show that patients with APS tolerate PPV-23 vaccination well. In the next post-vaccination period, exacerbations of the disease, thrombosis were not recorded. Attention is drawn to the large number of “non-responders” in PAPS, however, to obtain statistically reliable results, it is necessary to continue the study and recruit more patients.Disclosure of Interests:None declared

Catastrophic antiphospholipid syndrome: Updated diagnostic algorithms

2010 ◽  
Vol 10 (2) ◽  
pp. 74-79 ◽  
Author(s):  
Doruk Erkan ◽  
Gerard Espinosa ◽  
Ricard Cervera
Keyword(s):  
Antiphospholipid Syndrome ◽  
Catastrophic Antiphospholipid Syndrome ◽  
Diagnostic Algorithms
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