Publication: Afectación global respiratoria en pacientes con el complejo esclerosis tuberosa
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2021-06-20
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Universidad Complutense de Madrid
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Abstract
La afectación respiratoria en el complejo esclerosis tuberosa (CET) puede ser debida a dos patologías principalmente, la linfangioleiomiomatosis (LAM) y/o la hiperplasia micronodular neumocitaria multifocal (HMNM). La primera de ellas, con la presencia esencialmente de quistes parenquimatosos, se puede asociar al complejo en un 40% de los casos, con predominio en mujeres y diferenciándose de la forma esporádica en el carácter hereditario, un curso clínico menos agresivo y menor gravedad en cuanto a la afectación en las pruebas de función pulmonar. Es la causa más frecuente de fallecimiento en mujeres adultas con el CET con una reducción en 7 años con respecto a CET sin LAM. En cambio la segunda patología, definida por la presencia de nódulos pulmonares (NP), con una prevalencia 40-70%, posee una baja incidencia de afectación clínica o funcional significativa con un pronóstico favorable. En algunos estudios se han diferenciado y comparado variables respiratorias entre los grupos de pacientes con LAM con los que no, un estudio con pacientes con HMNM con los que no, o algunos donde ambas coexistían, sin embargo no existen artículos donde se analicen en todos los subgrupos (LAM, HMNM, CET sin afectación pulmonar y coexistiendo ambas patologías LAM-HMNM) la clínica respiratoria, las variables radiológicas, la función respiratoria y la afectación extrapulmonar. OBJETIVOS: Principales: I. Calcular las prevalencias de LAM e HMNM en los pacientes con CET. II. Calcular la prevalencia de la población CET con exclusivamente LAM (LAM sólo), HMNM (HMNM sólo) o CET (CET sólo) y de la población con ambas patologías (LAM-HMNM). III. Analizar las características clínicas respiratorias, variables radiológicas torácicas y las asociadas a las pruebas de función pulmonar en pacientes LAM, HMNM, LAM sólo, HMNM sólo, CET sólo y LAM-HMNM...
Respiratory involvement in the tuberous sclerosis complex (TSC) may be due to two main pathologies, lymphangioleiomyomatosis (LAM) and / or multifocal micronodular pneumocyte hyperplasia (MMPH). The first of these, with the presence of parenchymal cysts, can be associated in 40% of cases, predominantly in women and differentiating itself from the sporadic form in the hereditary character, a less aggressive clinical course and less severity in the pulmonary function tests. It is the most frequent cause of death in adult women with TSC with a 7-year reduction compared to TSC without LAM. On the other hand, MMPH, defined by the presence of pulmonary nodules (PN), with a prevalence of 40-70%, has a more equal affectation in gender, and has a low incidence of significant clinical or functional involvement in these patients with a favorable prognosis. In some studies, respiratory variables have been differentiated and compared between the groups of patients with LAM with those who did not, a study with patients with MMPH with those who did not (or somewhere both coexisted) however there are no articles comparing the clinical findings, extrapulmonary involvement and respiratory function among patients with exclusively LAM, MMPH, CET and both LAM-HMNM pathologies coexisting. OUTCOMES: Primary outcomes: I. Calculate the prevalences of LAM and HMNM in patients with TSC. II. Calculate the prevalence of the CET population with exclusively LAM (LAM only), exclusively HMNM (HMNM only), the population with both pathologies (LAM-HMNM) and without pulmonary involvemente (CET alone). III. Analyze the respiratory clinical characteristics, thoracic radiological variables and those associated with pulmonary function tests in LAM, HMNM, LAM only, HMNM only, LAM-HMNM and CET alone...
Respiratory involvement in the tuberous sclerosis complex (TSC) may be due to two main pathologies, lymphangioleiomyomatosis (LAM) and / or multifocal micronodular pneumocyte hyperplasia (MMPH). The first of these, with the presence of parenchymal cysts, can be associated in 40% of cases, predominantly in women and differentiating itself from the sporadic form in the hereditary character, a less aggressive clinical course and less severity in the pulmonary function tests. It is the most frequent cause of death in adult women with TSC with a 7-year reduction compared to TSC without LAM. On the other hand, MMPH, defined by the presence of pulmonary nodules (PN), with a prevalence of 40-70%, has a more equal affectation in gender, and has a low incidence of significant clinical or functional involvement in these patients with a favorable prognosis. In some studies, respiratory variables have been differentiated and compared between the groups of patients with LAM with those who did not, a study with patients with MMPH with those who did not (or somewhere both coexisted) however there are no articles comparing the clinical findings, extrapulmonary involvement and respiratory function among patients with exclusively LAM, MMPH, CET and both LAM-HMNM pathologies coexisting. OUTCOMES: Primary outcomes: I. Calculate the prevalences of LAM and HMNM in patients with TSC. II. Calculate the prevalence of the CET population with exclusively LAM (LAM only), exclusively HMNM (HMNM only), the population with both pathologies (LAM-HMNM) and without pulmonary involvemente (CET alone). III. Analyze the respiratory clinical characteristics, thoracic radiological variables and those associated with pulmonary function tests in LAM, HMNM, LAM only, HMNM only, LAM-HMNM and CET alone...
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Tesis inédita de la Universidad Complutense de Madrid, Facultad de Medicina, Departamento de Medicina, leída el 19/04/2021