Catastrophic antiphospholipid syndrome and pregnancy: an experience of 13 cases

Hanouna, Guillaume; Morel, Nathalie; Huong, D. Lê Thi; Josselin, L.; Vauthier-Brouzes, D.; Saadoun, David; Kettaneh, A.; Lévesque, Kateri; Guern, Véronique Le; Goffinet, François; Carbonne, Bruno; Amoura, Zahir; Piette, Jean‐Charles; Nizard, J.; Costédoat-Chalumeau, Nathalie

doi:10.1093/rheumatology/ket167

Cited by 56 publications

(54 citation statements)

References 24 publications

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“…A systemic inflammatory syndrome characterized by fever, tachycardia, tachypnea, and leukocytosis commonly coexists and is associated with the extensive ischemic tissue damage [105,106]. Triggering factors identified in most patients include infections, surgery, obstetric complications, drugs or discontinuation of anticoagulants, and others [107,108]. The clinical manifestations are related to the specifics of organ involvement and include severe hypertension, acute renal failure, proteinuria, and hematuria (kidneys); ARDS, pulmonary embolism, and DAH (lungs); hypertensive and/or ischemic encephalopathy and stroke (central nervous system); coronary artery thrombosis and valve lesions (heart), and livedo reticularis, acrocyanosis, purpura, and others (skin) [109,110].…”

Section: Pulmonary Thromboembolism and Capsmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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Subacute-acute, hyperacute, or even catastrophic and fulminant respiratory events occur in almost all classic connective tissue disorders (CTDs); they may share systemic life-threatening manifestations, may precipitously lead to respiratory failure requiring ventilatory support as well as a combination of specific therapeutic measures, and in most affected patients constitute the devastating end-of-life event. In CTDs, acute respiratory events may be related to any respiratory compartment including the airways, lung parenchyma, alveolar capillaries, lung vessels, pleura, and ventilatory muscles. Acute respiratory events may also precipitate disease-specific extrapulmonary organ involvement such as aspiration pneumonia and lead to digestive tract involvement and heart-related respiratory events. Finally, antirheumatic drug-related acute respiratory toxicity as well as lung infections related to the rheumatic disease and/or to immunosuppression complete the spectrum of acute respiratory events. Overall, in CTDs the lungs significantly contribute to morbidity and mortality, since they constitute a common site of disease involvement; a major site of infections related to the ‘mater' disease; a major site of drug-related toxicity, and a common site of treatment-related infectious complications. The extreme spectrum of the abovementioned events, as well as the ‘vicious' coexistence of most of the aforementioned manifestations, requires skills, specific diagnostic and therapeutic means, and most of all a multidisciplinary approach of adequately prepared and expert scientists. Avoiding lung disease might represent a major concern for future advancements in the treatment of autoimmune disorders.

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Section: Pulmonary Thromboembolism and Capsmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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“…81 Catastrophic thrombotic syndromes during pregnancy Catastrophic APS has been described in patients who were pregnant or in the peripartum period, including patients with known APS during transition from warfarin to LMWH initiated because of pregnancy. 82,83 Patients without APS or other disorders can also develop a severe thrombotic syndrome during pregnancy or in the early postpartum setting. 2,84 Presentation may overlap with other pregnancy-related syndromes, including HELLP (hemolysis, elevated liver enzymes, low platelet count), preeclampsia, and eclampsia.…”

Section: Surgical Proceduresmentioning

confidence: 99%

“…2,84 Presentation may overlap with other pregnancy-related syndromes, including HELLP (hemolysis, elevated liver enzymes, low platelet count), preeclampsia, and eclampsia. 83 Anticoagulant therapy is essential, using LMWH early in the pregnancy, and unfractionated heparin around the time of delivery. Steroids, plasma exchange, and/or IV immunoglobulins can be used in pregnant patients presenting with catastrophic APS.…”

Section: Surgical Proceduresmentioning

confidence: 99%

How I treat catastrophic thrombotic syndromes

Ortel

Erkan

Kitchens

2015

Blood

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Catastrophic thrombotic syndromes are characterized by rapid onset of multiple thromboembolic occlusions affecting diverse vascular beds. Patients may have multiple events on presentation, or develop them rapidly over days to weeks. Several disorders can present with this extreme clinical phenotype, including catastrophic antiphospholipid syndrome (APS), atypical presentations of thrombotic thrombocytopenic purpura (TTP) or heparin-induced thrombocytopenia (HIT), and Trousseau syndrome, but some patients present with multiple thrombotic events in the absence of associated prothrombotic disorders. Diagnostic workup must rapidly determine which, if any, of these syndromes are present because therapeutic management is driven by the underlying disorder. With the exception of atypical presentations of TTP, which are treated with plasma exchange, anticoagulation is the most important therapeutic intervention in these patients. Effective anticoagulation may require laboratory confirmation with anti–factor Xa levels in patients treated with heparin, especially if the baseline (pretreatment) activated partial thromboplastin time is prolonged. Patients with catastrophic APS also benefit from immunosuppressive therapy and/or plasma exchange, whereas patients with HIT need an alternative anticoagulant to replace heparin. Progressive thrombotic events despite therapeutic anticoagulation may necessitate an alternative therapeutic strategy. If the thrombotic process can be controlled, these patients can recover, but indefinite anticoagulant therapy may be appropriate to prevent recurrent events.

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“…24 In a small retrospective series of 13 patients with pregnancy-related CAPS, CAPS usually followed HELLP syndrome (n = 12). 25 Therefore, clinicians should be very cautious about this potentially life-threatening condition especially when dealing with HELLP syndrome as CAPS can be lethal in about 50% of the cases. obstetrical complications Recurrent fetal loss and stillbirth A retrospective cohort study conducted from 1988 to 2006 on 1719 patients with unexplained recurrent miscarriages showed that there was no clear correlation between the number of preceding miscarriages and the age of the mother with APS diagnosis.…”

Section: Insights Of Some New Clinical Criteria Of Obstetricalmentioning

confidence: 99%

“…special considerations: preventing catastrophic antiphospholipid syndrome The best admitted treatment for CAPS is based on its prevention, therefore, postpartum period should be covered with 6 weeks of anticoagulation. 25 Once CAPS has been diagnosed, despite the lack of good studies on the treatment and the management of this entity, combined aggressive therapies, for example, with anticoagulants, corticosteroids, plasmapheresis, and rituximab are usually admitted as beneficial. Despite this, CAPS is still lethal in 50% of the cases.…”

Section: Management Of Obstetrical Antiphospholipid Syndromementioning

confidence: 99%