Tuberc Respir Dis.  2019 Jan;82(1):53-61. 10.4046/trd.2018.0045.

Clinical Phenotype of a First Unprovoked Acute Pulmonary Embolism Associated with Antiphospholipid Antibody Syndrome

• Na YS
• Jang S
• Hong S
• Oh YM
• Lee SD
• Lee JS

• KMID: 2441730
• DOI: http://doi.org/10.4046/trd.2018.0045

Abstract

BACKGROUND
Antiphospholipid antibody syndrome (APS), an important cause of acquired thrombophilia, is diagnosed when vascular thrombosis or pregnancy morbidity occurs with persistently positive antiphospholipid antibodies (aPL). APS is a risk factor for unprovoked recurrence of pulmonary embolism (PE). Performing laboratory testing for aPL after a first unprovoked acute PE is controversial. We investigated if a specific phenotype existed in patients with unprovoked with acute PE, suggesting the need to evaluate them for APS.
METHODS
We retrospectively reviewed patients with PE and APS (n=24) and those with unprovoked PE with aPL negative (n=44), evaluated 2006-2016 at the Asan Medical Center. We compared patient demographics, clinical manifestations, laboratory findings, and radiological findings between the groups.
RESULTS
On multivariate logistic regression analysis, two models of independent risk factors for APS-PE were suggested. Model I included hemoptysis (odds ratio [OR], 12.897; 95% confidence interval [CI], 1.025-162.343), low PE severity index (OR, 0.948; 95% CI, 0.917-0.979), and activated partial thromboplastin time (aPTT; OR, 1.166; 95% CI, 1.040-1.307). Model II included age (OR, 0.930; 95% CI, 0.893-0.969) and aPTT (OR, 1.104; 95% CI, 1.000-1.217).
CONCLUSION
We conclude that patients with first unprovoked PE with hemoptysis and are age <40; have a low pulmonary embolism severity index, especially in risk class I-II; and/or prolonged aPTT (above 75th percentile of the reference interval), should be suspected of having APS, and undergo laboratory testing for aPL.

Keyword

Antiphospholipid Syndrome; Antibodies, Antiphospholipid; Pulmonary Embolism; Phenotype; Risk Factors

MeSH Terms

Antibodies, Antiphospholipid*
Antiphospholipid Syndrome*
Chungcheongnam-do
Demography
Hemoptysis
Humans
Logistic Models
Partial Thromboplastin Time
Phenotype*
Pregnancy
Pulmonary Embolism*
Recurrence
Retrospective Studies
Risk Factors
Thrombophilia
Thrombosis
Antibodies, Antiphospholipid

Figure

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  Figure 1 Flowchart of patient selection. One hundred seventy patients with predisposing factors were excluded. An additional 23 patients with unprovoked PE were excluded as they were not tested for APS. PE: pulmonary embolism; VTE: venous thromboembolism; APS: antiphospholipid antibody syndrome; SLE: systemic lupus erythematous; aPL: antiphospholipid antibody.

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