Catastrophic antiphospholipid syndrome: task force report summary

Cervera, Ricard; Rodríguez‐Pintó, Ignasi

doi:10.1177/0961203314540764

Cited by 45 publications

(16 citation statements)

References 13 publications

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“…Acute and extensive microvascular thrombosis, leading to multiorgan ischemia and failure, characterizes the rarely occurring and often lethal condition named CAPS. CAPS may present as idiopathic or secondary, the latter form mainly occurring in female lupus patients [14,97,98]. It commonly involves more than three organs (more frequently the kidneys, the lungs, the central nervous system, the heart, and the liver), always presents with antiphospholipid antibodies, occurs rapidly (within less than 1 week), and is histologically characterized by intravascular thrombosis (diagnostic criteria) [101,102].…”

Section: Pulmonary Thromboembolism and Capsmentioning

confidence: 99%

“…diffuse alveolar hemorrhage (DAH), acute capillaritis, or bland hemorrhage related to systemic lupus erythematosus (SLE) [13]]; (d) the lung vessels [e.g. acute pulmonary thromboembolism or the development of a catastrophic antiphospholipid syndrome (CAPS) in SLE [14]]; (e) the pleura [e.g. pneumothorax and/or pneumomediastinum mostly in dermatomyositis-polymyositis (DM-PM) [15]], and (f) the ventilatory muscles (e.g.…”

Section: Introductionmentioning

confidence: 99%

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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%

Section: Introductionmentioning

confidence: 99%

Acute Respiratory Events in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2016

Respiration

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show abstract

“…De los tratamientos individuales, los más empleados son anticoagulantes (82%), esteroides (76%), plasmaféresis (35%) e inmunoglobulina intravenosa (27%). Entre los tratamientos combinados (mayor efectividad, en teoría) destacan los anticoagulantes con esteroides (19%) y los anticoagulantes + esteroides + plasmaféresis/inmunoglobulina intravenosa (18%) [9][10][11] . Recientemente se han empleado anticuerpos monoclonales para el tratamiento del SAF catastrófico, pero la evidencia aún es escasa y solo hay reportes de casos anecdóticos.…”

Section: Figura 2 Datos De íLeo Líquido Libre Abdominal Y Neumatosiunclassified

Síndrome antifosfolípidos catastrófico. Reporte de caso y revisión bibliográfica

Carpio-Orantes

Martínez-Anaya

Bonilla-Casas

2017

GMM

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“…Lupus-related PAH is most commonly occurring in patients presenting antiphospholipid antibodies and requires treatment as idiopathic PAH [104] . Catastrophic antiphospholipid syndrome occurs in less than 1% of patients with antiphospholipid syndrome and relates to multiorgan failure due to diffuse thrombotic microvessel occlusions [105] . It is usually precipitated by an infection, and the lungs may present acute respiratory distress syndrome related to several mechanisms including sepsis and microthrombosis [105] .…”

Section: Pulmonary Thromboembolismmentioning

confidence: 99%

“…Catastrophic antiphospholipid syndrome occurs in less than 1% of patients with antiphospholipid syndrome and relates to multiorgan failure due to diffuse thrombotic microvessel occlusions [105] . It is usually precipitated by an infection, and the lungs may present acute respiratory distress syndrome related to several mechanisms including sepsis and microthrombosis [105] . Provision of excellent supportive care plus antibiotics, anticoagulants, plasma exchange and intravenous immunoglobulin administration for the most severe cases in addition to corticosteroids is the mainstay of treatment [94] .…”

Section: Pulmonary Thromboembolismmentioning

confidence: 99%

Investigation of Lung Involvement in Connective Tissue Disorders

Papiris¹,

Manali²,

Kolilekas³

et al. 2015

Respiration

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Lung involvement in connective tissue disorders (CTDs) may present as pleomorphic since any lung compartment may be involved such as airways, exocrine secretory and alveolar epithelia, interstitial lung structure, pulmonary vasculature and pleura as well as, in specific disorders, several tissues of the thoracoabdominal ventilator pump. Any combination of the above anatomic structures may be involved concomitantly although some specific combinations may include a determinant of rheumatic disorders. The diagnosis of a specific CTD requires the fulfilment of clearly defined clinical and laboratory criteria including in most cases positivity for autoantibodies, mostly specific serologic combinations. In this setting, serologic investigation targets mainly, although not exclusively, the detection of antinuclear antibodies. A specific serologic positivity or a combination of autoantibodies constitutes not only a diagnostic criterion for a specific CTD, but may also characterize the pattern of respiratory manifestation in a determinant rheumatic disorder. Therefore, the investigation of lung involvement in CTDs requires adequate skills in the ambit of a multidisciplinary approach and an extended spectrum of diagnostic tools and modalities able to detect both early clinical clues and serologic conversion as well as any pathophysiologic alteration that regards the complexity of respiratory functional status. Although many patients with CTDs suffer from a ‘vicious' combination of lung involvement, lung drug toxicity and infections related to the above two as well as to the ‘mater' disease, for space reasons this review will focus on the established lung manifestations that regard the 7 major CTDs.

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Catastrophic antiphospholipid syndrome: task force report summary

Abstract: The Task Force on Catastrophic Antiphospholipid Syndrome (CAPS) aimed to assess the current knowledge on pathogenesis, clinical and laboratory features, diagnosis and classification, precipitating factors and treatment of CAPS. This article summarizes the main aspects of its final report.

Cited by 45 publications

References 13 publications

Acute Respiratory Events in Connective Tissue Disorders

Acute Respiratory Events in Connective Tissue Disorders

Síndrome antifosfolípidos catastrófico. Reporte de caso y revisión bibliográfica

Investigation of Lung Involvement in Connective Tissue Disorders

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