Chest
Diffuse Lung Disease: Original ResearchMechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis
Section snippets
Study Population
Patients with a diagnosis of PLCH assessed according to lung biopsy or the presence of a typical chest CT scan (evaluated by two radiologist experts) with a history of smoking13 were invited to participate in this cross-sectional study. The protocol was approved by the local research ethics committee, registered at www.clinicaltrials.gov,14 and had financial support from the São Paulo Research Foundation (FAPESP-2015/06604-0). All patients signed the informed consent form. Patients were
Results
Of the 38 patients with PCLH followed up at our center from February 2016 to August 2018, three patients were not included because of death (n = 1), lung transplantation (n = 1), and refusal to participate in the study (n = 1) (Fig 1). Thus, 35 patients were enrolled, and 68% were women. Clinical and functional data are described in Table 1. The mean age of the patients was 47 ± 11 years, 92% were current or ex-smokers, one patient was a marijuana user, and one patient had a history of cocaine
Discussion
To our knowledge, this study is the first that thoroughly evaluated the potential mechanisms of exercise limitation and the prevalence of DH and PH in patients with PLCH of various severities. The main findings of this study are as follows: (1) ventilatory and cardiocirculatory limitations, including those suggestive of PH, were the most common mechanisms of exercise limitation in PLCH; (2) multifactorial impairment occurred in 71% of those with reduced exercise capacity; (3) exercise
Interpretation
This study highlights that the majority of patients with PLCH present with reduced exercise capacity, secondary to multiple mechanisms of limitation, predominantly ventilatory and cardiocirculatory, and associated with functional impairment. Furthermore, DH and PH were common in PLCH and correlated with functional abnormalities. Future studies are necessary to define the role of different interventions over the potential mechanisms of impairment to improve exercise tolerance in PLCH.
Acknowledgments
Author contributions: B. G. B. is the guarantor of the paper and contributed to the study design, data collection, data analysis, writing, and manuscript review. G. I. H. contributed to the study design, data collection, data analysis, writing, and manuscript review; J. B. S. and C. S. G. F. contributed to the data collection and manuscript review; A. L. P. d. A., J. M. S., R. A. K., and C. R. R. C. contributed to the study design, writing, and manuscript review; C. J. C. d. S. F. contributed
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FUNDING/SUPPORT: The study protocol had financial support from the São Paulo Research Foundation [FAPESP-2015/06604-0].