Antiphospholipid (aPL) antibodies are commonly found in patients with chronic thromboembolic pulmonary hypertension (CTEPH), and may be predictive biomarkers for the disease. This is according to a systematic review and meta-analysis published in Internal and Emergency Medicine.

Investigators independently reviewed online databases for studies reporting inherited acquired thrombophilia results in patients with CTEPH. A total of 8 studies that included patients with CTEPH and data on patients’ aPL antibody profiles were included in the final analysis.

Significant heterogeneity among included studies was observed (I2, 76.33%; 95% CI, 52.75%-88.14%; P =.0001). In the crude analysis, patients with CTEPH had a higher aPL prevalence rate (11.8%) compared with the general population, as well as patients with pulmonary embolism (95% CI, 10.09%-13.80%). The weighted mean proportion and 95% CIs resulted in an aPL antibody-positive profile rate of 12.06% (95% CI, 8.12%-16.65%) in patients with CTEPH. In addition, the researchers found that the association between aPL and CTEPH was still noticeable when the study with the largest weight was excluded from the meta-analysis (rate of aPL, 12.76%; 95% CI, 7.81%-18.70%).

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According to the investigators, the main limitation of the study was the incompleteness of some of the thrombophilia data.

With regard to the study’s potential clinical implications, the researchers wrote, “[a]ntiphospholipid antibodies should be sought in young patients with ‘idiopathic’ [pulmonary embolism] and ‘provoked’ [pulmonary embolism] caused by mild provoking risk factors (e.g., estroprogestinic drugs).”

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They added, “The presence of a combination of more than one aPL test (especially triple positivity) might be an important predictor of recurrence, and should raise awareness about anticoagulation prolongation.”


Cheng C-Y, Zhang Y-X, Denas G, Du Y, Jing Z-C, Pengo V. Prevalence of antiphospholipid (aPL) antibodies among patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis [published online January 2, 2018]. Intern Emerg Med. doi:10.1007/s11739-018-02021-z