Abstract
Growing evidence suggests that inflammation may pose an atypical risk factor for pulmonary embolism (PE), as it drives venous thrombosis via several pathways. The increased risk of PE in several autoimmune diseases has lent weight to this concept. However, the relative risk of PE among patients with pemphigus has not yet been established. We aimed to examine the risk of PE in patients with pemphigus. A large-scale population-based longitudinal cohort study was conducted to evaluate the relative risk (RR) of PE among 1,985 patients with pemphigus relative to 9,874 age-, sex-, and ethnicity-matched control subjects. A multivariate Cox regression model was utilized. The incidence of PE was 3.0 (95% CI, 2.2–4.0) and 1.2 (95% CI, 1.0–1.5) per 1,000 person-years among patients with pemphigus and controls, respectively. The period prevalence of PE corresponding to the study period was 2.2% (95% CI, 1.6–2.9%) among cases and 0.9% (95% CI, 0.7–1.1%) among controls. Patients with pemphigus were twice as likely to develop PE as compared to control subjects (adjusted RR, 1.98; 95% confidence interval [CI], 1.29–3.04). The highest PE risk was observed during the 1st year following the diagnosis of pemphigus (adjusted RR, 3.55; 95% CI, 1.78–7.09) and decreased over time. The increased risk was robust to a sensitivity analysis that included only cases managed by pemphigus-related systemic medications (adjusted RR, 1.82; 95% CI, 1.11–2.98). In conclusion, pemphigus is associated with an increased risk of PE, particularly during the 1st year of the disease. An awareness of this risk should be increased, additional precipitating factors for PE should be avoided, and thromboprophylaxis may be evaluated in high-risk patients. Further research is required to establish this risk.
Highlights
Pulmonary embolism (PE) is a life-threatening cardiovascular and cardiopulmonary condition associated with substantial burden [1]
This large-scale population-based study provides evidence that patients with pemphigus are twice as likely to develop pulmonary embolism (PE) than matched control subjects. This increased risk was most pronounced within the 1st year following the diagnosis of pemphigus and thereafter declined over time to lose its statistical significance
There is evidence that systemic inflammation, which exists in pemphigus as well as in other autoimmune diseases, potentiates venous thromboembolism by up-regulating procoagulants, downregulating anticoagulants, and suppressing fibrinolysis [4]
Summary
Pulmonary embolism (PE) is a life-threatening cardiovascular and cardiopulmonary condition associated with substantial burden [1]. The estimated annual incidence rate of PE ranges from 0.15 to 1.0 cases per 1,000 populations [1]. It is characterized by high mortality rates that may exceed 15% within the first 3 months following diagnosis [2], accounts for 5–10% of deaths among hospitalized patients, and is the most common preventable cause of inpatient death [3]. Growing evidence suggests that inflammation may pose an atypical risk factor for PE, as it drives venous thrombosis via several pathways [4]. Additional prominent risk factors are institutionalization, malignancies, trauma, congestive heart failure, central venous catheter, or pacemaker placement [6]
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