Evaluation of the exercise capacity and mechanisms responsible for exercise limitation in patients with Pulmonary Langerhans Cell Histiocytosis

• Grant number: 15/06604-0
• Support Opportunities: Regular Research Grants
• Start date: September 01, 2015
• End date: August 31, 2017
• Field of knowledge: Health Sciences - Medicine - Medical Clinics
• Principal Investigator: Bruno Guedes Baldi
• Grantee: Bruno Guedes Baldi
• Host Institution: Instituto do Coração Professor Euryclides de Jesus Zerbini (INCOR). Hospital das Clínicas da Faculdade de Medicina da USP (HCFMUSP). Secretaria da Saúde (São Paulo - Estado). São Paulo , SP, Brazil
• City of the host institution: São Paulo
• Associated researchers: Glaucia Itamaro Heiden ; Rogério de Souza

Abstract

Pulmonary Langerhans Histiocytosis Cells (PLCH) is characterized by infiltration of Langerhans cells and formation of loose granulomas with lymphocytic infiltrate and formation of nodular and cystic lesions on chest CT, and is often associated with smoking. Functionally, there may be obstructive and / or restrictive defect, with reduced carbon monoxide diffusing capacity. Dyspnea and lower exercise tolerance are common in PLCH, but exercise capacity in this disease is poorly understood and has not been compared to controls. Besides, the mechanisms involved in limiting exercise are poorly understood and cover multiple factors such as change in gas exchange, pulmonary hypertension (PH), dynamic hyperinflation, physical deconditioning and left heart failure. The involvement of pulmonary circulation in PLCH has unknown prevalence, but contributes to the symptoms. In the PH classification, PLCH belongs to the group 5, of multifactorial etiology. The definition of the presence and contribution of dyspnea mechanisms in different severities of PLCH is important to understanding the disease and individualization of treatment. The objective of the study is to evaluate the exercise capacity of patients with HCLP, and determinate mechanisms of dyspnea and lower exercise tolerance beyond its impact on quality of life. (AU)

Scientific publications

(The scientific publications listed on this page originate from the Web of Science or SciELO databases. Their authors have cited FAPESP grant or fellowship project numbers awarded to Principal Investigators or Fellowship Recipients, whether or not they are among the authors. This information is collected automatically and retrieved directly from those bibliometric databases.)

HEIDEN, GLAUCIA ITAMARO; SOBRAL, JULIANA BARBOSA; GONCALVES FREITAS, CAROLINA SALIM; PEREIRA DE ALBUQUERQUE, ANDRE LUIS; SALGE, JOAO MARCOS; KAIRALLA, RONALDO ADIB; CESAR DOS SANTOS FERNANDES, CAIO JULIO; RIBEIRO CARVALHO, CARLOS ROBERTO; SOUZA, ROGERIO; BALDI, BRUNO GUEDES. Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis. CHEST, v. 158, n. 6, p. 2440-2448, DEC 2020. (15/06604-0)

HEIDEN, GLAUCIA ITAMARO; FREITAS, CAROLINA; SOBRAL, JULIANA BARBOSA; KAIRALLA, RONALDO; CARVALHO, CARLOS ROBERTO; SALGE, JOAO MARCOS; ALBUQUERQUE, ANDRE LUIS; SOUZA, ROGERIO; BALDI, BRUNO. Exercise in patients with pulmonary Langerhans cell histiocytosis: performance and mechanisms of limitation. European Respiratory Journal, v. 50, p. 1-pg., 2017-09-01. (15/06604-0)