Left anterior descending artery aneurysm in a young patient with familial retinal arterial macroaneurysm: A case report.

Purpose: Familial retinal arterial macroaneurysm (FRAM) is a rare genetic disorder caused by a gene mutation in the insulin-like growth factor binding protein 7 (IGFBP7). Observations: We report a 30-year-old male with FRAM and IGFBP7 gene mutation who presented with an acute coronary syndrome (ACS). Invasive coronary angiography revealed a large aneurysm at the proximal part of the left anterior descending (LAD) artery. Conclusions and Importance: Few cases with systemic vascular involvement in patients with FRAM have been described before; however, our case represents the first documentation of a LAD artery aneurysm in a patient with FRAM and IGFBP7 gene mutation.

Costs of Workplace Productivity Loss in Patients with Connective Tissue Disease-associated Interstitial Lung Disease.

Rationale: Interstitial lung disease (ILD) develops in a large percentage of patients with connective tissue disease (CTD) and is associated with increased morbidity and mortality. Patients with CTD-associated ILD (CTD-ILD) often present at a young age, suggesting that ILD likely impacts workplace productivity.Objectives: We aimed to determine the employment rate and workplace productivity loss, along with its associated factors and estimated costs, in patients with fibrotic CTD-ILD.Methods: Patients with fibrotic CTD-ILD from the six centers of the Canadian Registry for Pulmonary Fibrosis were eligible. Health-related productivity loss was assessed using the Work Productivity and Activity Impairment questionnaire. Proposed factors associated with low workplace productivity were forced into a multivariable regression model. Average productivity loss in hours/week was used to calculate the costs of productivity loss based on hourly wages obtained from national census data matched for age and sex. Workplace productivity loss outcomes were compared between patients with CTD-ILD and patients with a non-CTD fibrotic ILD.Results: Of 375 eligible patients with fibrotic CTD-ILD, 113 (30%) were employed. Productivity loss was reported by 59% of employed patients, with a mean loss of 9.4 ± 1.2 hours/week, including 3.9 ± 0.9 hours/week from absenteeism and 5.5 ± 0.7 hours/week from presenteeism. Employment among patients 25-54 years of age with fibrotic CTD-ILD was 27% lower than that in the matched general Canadian population (56% vs. 83%; P < 0.001). Employment among patients ≥55 years of age with CTD-ILD was 17% lower than that in the matched population (19% vs. 36%; P < 0.001). Workplace productivity loss was not associated with respiratory symptoms or lung physiology. Annual costs of productivity loss were calculated at 13,593 Canadian dollars per employee with fibrotic CTD-ILD. Workplace productivity loss was similar in patients with fibrotic CTD-ILD and those with non-CTD fibrotic ILD.Conclusions: Patients with fibrotic CTD-ILD frequently report workplace productivity loss, which is unexplained by respiratory symptoms or lung physiology and is associated with significant costs.

Costs of Workplace Productivity Loss in Patients With Fibrotic Interstitial Lung Disease.

BACKGROUND: Fibrotic interstitial lung diseases (ILDs) are highly morbid chronic disorders that frequently occur in working age individuals. The goal of this study was to determine workplace productivity loss, its determinants, and its estimated costs in patients with fibrotic ILD. METHODS: Patients with idiopathic pulmonary fibrosis, chronic hypersensitivity pneumonitis, idiopathic nonspecific interstitial pneumonia, or unclassifiable ILD were identified from the six-center Canadian Registry for Pulmonary Fibrosis (CARE-PF). The Work Productivity and Activity Impairment questionnaire was used to determine health-related productivity loss. Independent predictors of low workplace productivity were identified by using multivariate regression. Patient data were compared with Canadian population census data. The average productivity loss (hours per week) and the individual's hourly wage were used to estimate the costs of productivity loss. RESULTS: Of 650 eligible patients, 148 (23%) were employed. Productivity loss was reported by 55% of employed patients with an average productivity loss of 7.8 ± 0.9 h per week (2.3 ± 0.6 h per week related to absenteeism and 5.5 ± 0.6 h per week related to presenteeism). Employment among patients with ILD aged 25 to 54 years was 23% lower than the age- and sex-matched general Canadian population (60% vs 83%; P < .001). Employment among patients with ILD aged ≥ 55 years was 18% lower than in the age- and sex-matched population (20% vs 38%; P < .001). Dyspnea and cough were independent predictors of workplace productivity loss. Estimated annual costs of productivity loss were 11,610 Canadian dollars per employee with ILD. CONCLUSIONS: Workplace productivity loss is common in fibrotic ILD, strongly correlated with symptom severity, and associated with significant cost.

Heterogeneity in Unclassifiable Interstitial Lung Disease. A Systematic Review and Meta-Analysis.

RATIONALE: Accurate diagnosis of interstitial lung disease is necessary to identify the most appropriate management strategy and to inform prognosis. Many patients cannot be provided a confident diagnosis, despite an exhaustive search for potential etiologies and review in a multidisciplinary conference, and are consequently labeled with unclassifiable interstitial lung disease. OBJECTIVES: To systematically review and meta-analyze previous studies reporting on the diagnostic criteria, prevalence, clinical features, and outcome of unclassifiable interstitial lung disease. METHODS: MEDLINE, Embase, and the Cochrane Central Register of Controlled Trials databases were systematically searched for all studies related to unclassifiable interstitial lung disease published before September 1, 2017. Two authors independently screened each citation for eligibility criteria, serially reviewing the title, abstract, and full-text manuscript, and then abstracted data pertaining to the study objectives from eligible studies. Articles were stratified by risk of selection bias, whether the publication stated that patients were reviewed in a multidisciplinary discussion, and by the frequency of surgical lung biopsy. Meta-analyses and meta-regression were performed to calculate the pooled prevalence of unclassifiable interstitial lung disease within an interstitial lung disease population and within specific subgroups to identify reasons for across-study heterogeneity. RESULTS: The search identified 10,130 unique citations, 313 articles underwent full-text review, and eligibility criteria were met in 88 articles. Twenty-two studies were deemed low risk of selection bias, including 1,060 patients with unclassifiable interstitial lung disease from a total of 10,174 patients with interstitial lung disease. The terminology and definition of unclassifiable interstitial lung disease varied substantially across publications, with inconsistent diagnostic criteria and evaluation processes. The prevalence of unclassifiable interstitial lung disease was 11.9% (95% confidence interval, 8.5-15.6%), with lower prevalence in centers that reported use of a formal multidisciplinary discussion of cases (9.5% vs. 14.5%). Four articles reported survival of unclassifiable interstitial lung disease, with 1-year, 2-year, and 5-year survival of 84% to 89%, 70% to 76%, and 46% to 70%, respectively. CONCLUSIONS: This systematic review and meta-analysis shows that unclassifiable interstitial lung disease is common but has substantial heterogeneity and inconsistent definitions across interstitial lung disease cohorts. These findings highlight important limitations in multicenter studies of fibrotic interstitial lung disease and the need for a standardized approach to interstitial lung disease diagnostic classification.