CAPS criteria fail to identify most severely-ill thrombotic antiphospholipid syndrome patients requiring intensive care unit admission

Abstract

HAL is a multidisciplinary open access archive for the deposit and dissemination of scientific research documents, whether they are published or not. The documents may come from teaching and research institutions in France or abroad, or from public or private research centers. L'archive ouverte pluridisciplinaire HAL, est destinée au dépôt et à la diffusion de documents scientifiques de niveau recherche, publiés ou non, émanant des établissements d'enseignement et de recherche français ou étrangers, des laboratoires publics ou privés.

Immunomodulating Therapies in Acute Myocarditis and Recurrent/Acute Pericarditis

“…The C-APLAS is defined as the occurrence of ( 1 ) at least the involvement of 3 organs, tissues, or systems in <7 days; ( 2 ) with biopsy-proven small vessel occlusion; ( 3 ) in patients with persistent high title of antiphospholipid antibodies ( 65 ). These classification criteria should be considered with great caution as they do not encompass the full spectrum of severe APLAS microvascular thrombotic episodes and some patients may require treatment escalation even though they do not fulfill the criteria for C-APLAS ( 66 ). When available, EMB can reveal myocyte necrosis with small vessels occlusions ( 67 ).…”

Complement activity and complement regulatory gene mutations are associated with thrombosis in APS and CAPS

“…38 We included patients with both definite and probable CAPS, because biopsies to confirm small-vessel thrombosis are commonly omitted in critically ill patients who otherwise meet criteria for CAPS, and outcomes of patients with probable CAPS are comparable to those of patients with definite CAPS. 39,40 SLE was diagnosed according to the Systemic Lupus International Collaborating Clinics Criteria. 41 For genetic analysis, we used DNA samples from healthy pregnant women recruited from the obstetrics clinics at Johns Hopkins Hospital as a control cohort, as well as patients with atypical hemolytic uremic syndrome (aHUS) as a reference cohort for validation of our custom sequencing panel.…”

Response

“…Finally, our results are in agreement with previously published literature on APS and CAPS. 1,4 In conclusion, we acknowledge that the methodology of our study is inevitably associated with an inherent degree of chestjournal.org potential bias; however, we think the issue of immortal time bias does not substantially impede the internal validation of our study and therefore allows to underscore our principal findings on the relevance of anticoagulation in critically ill patients with APS. We read with interest the Contemporary Review in Critical Care Medicine entitled "Managing Massive Hemoptysis" previously published in CHEST (January 2020).…”

“…2,3 It clarified the role of bronchoscopy in both mild and moderate-severe hemoptysis 3 and improved the quality of the scientific evidence on this key issue, which was based on retrospective studies with low sample size and on studies that excluded patients with cancer-related bleeding. 4,5 Our study included patients with mild bleeding, who were compared with those with moderate-severe hemoptysis (< and > 20 mL estimated blood volume collected daily, respectively) 2,3 ; it enrolled patients with life-threatening bleedings, that is, with gas exchange impairment or hemodynamic instability, and with potentially serious hemorrhage (ie, >100-150 mL, the volume of the dead space). 2,3 As underscored by the authors, adequate management should not be deferred in patients with a good cardiorespiratory reserve, who can remain clinically stable during the initial stage of potentially severe bleeding.…”