Linfangioleiomiomatose pulmonar

Medeiros, Paulo José de; Carvalho, Carlos Roberto Ribeiro de

doi:10.1590/s1806-37132004000100013

Cited by 6 publications

(3 citation statements)

References 42 publications

(47 reference statements)

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“…Twenty‐five of them had been diagnosed as having idiopathic pulmonary fibrosis (IPF), 6 patients had chronic hypersensitivity pneumonitis (CHP), 5 patients had centrilobular fibrosis secondary to gastroesophageal reflux disease (GERD), and 15 patients received the diagnosis of lymphangioleiomyomatosis (LAM). 15 , 16 …”

Section: Methodsmentioning

confidence: 99%

Desaturation – distance ratio: a new concept for a functional assessment of interstitial lung diseases

Pimenta

Rocha

Baldi

et al. 2010

Clinics

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INTRODUCTION:The functional evaluation has become increasingly important in the understanding and management of patients with interstitial lung diseases. The cardiopulmonary exercise test and the six‐minute walk test (6MWT), through their isolated variables, have been used to do this evaluation, with some limitations.OBJECTIVES:We proposed a new composite index (desaturation distance ratio using continuous peripheral oxygen saturation (SpO2) and the distance walked as a more reliable tool for doing a functional evaluation of these patients.METHODS:6MWT was performed by interstitial lung diseases patients and controls. Analyzed parameters were walked distance and desaturation area (DAO2), obtained by taking the difference between maximal SpO2 possible (100%) and patient's SpO2 every 2 seconds. desaturation distance ratio was calculated using the ratio between DAO2 and distance walked.RESULTS:Forty‐nine interstitial lung diseases patients and 11 control subjects completed the protocol. The mean (SD) age was 60 (12) years and 65 (9) years, respectively (p:NS). Data obtained from 6MWT showed a significant statistical difference between interstitial lung diseases patients and controls: mean walked distance (430 and 602 meters, respectively); SpO2 minimal maintained at least 10 seconds ‐ SpO2 min (85% and 94%, respectively), and median desaturation distance ratio (10 and 2.5, respectively). A correlation analysis, considering interstitial lung diseases patients, revealed the best correlation between desaturation distance ratio and DLco (r = ‐ 0.72; p<0.001), being the correlation between SpO2 min and DLco of 0.61 (p<0.001) and among walked distance and DLco of 0.58 (p<0.05).CONCLUSION:Desaturation distance ratio is a promising concept and a more reliable physiologic tool to assess pulmonary diseases characterized by involvement of the alveolar‐capillary membrane, such as interstitial lung diseases.

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Section: Methodsmentioning

confidence: 99%

Desaturation – distance ratio: a new concept for a functional assessment of interstitial lung diseases

Pimenta

Rocha

Baldi

et al. 2010

Clinics

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“…The sporadic form is responsible for most cases, with an estimated prevalence of approximately 3.3 to 7.7 per 1,000,000 women [ 2 , 3 ]. Tuberous sclerosis is a dominant autosomal disease, associated with the suppressor oncogenes TSC-1 in chromosome 9, and TSC-2 in chromosome 16, and mutations in their alleles may lead to cellular proliferation [ 4 ]. It is estimated that about 80% of women with TSC will present pulmonary cysts in the follow-up [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Small airways morphological alterations associated with functional impairment in lymphangioleiomyomatosis

Mendonça,

Costa,

do Nascimento

et al. 2024

BMC Pulm Med

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Background Lymphangioleiomyomatosis (LAM) is a rare neoplastic and cystic pulmonary disease characterized by abnormal proliferation of the so-called LAM cells. Despite the functional obstructive pattern observed in most patients, few studies investigated the morphological changes in the small airways, most of them in patients with severe and advanced LAM undergoing lung transplantation. Understanding the morphological changes in the airways that may occur early in the disease can help us understand the pathophysiology of disease progression and understand the rationale for possible therapeutic approaches, such as the use of bronchodilators. Our study aimed to characterize the morphological alterations of the small airways in patients with LAM with different severities compared to controls, and their association with variables at the pulmonary function test and with LAM Histological Score (LHS). Methods Thirty-nine women with LAM who had undergone open lung biopsy or lung transplantation, and nine controls were evaluated. The histological severity of the disease was assessed as LHS, based on the percentage of tissue involvement by cysts and infiltration by LAM cells. The following morphometric parameters were obtained: airway thickness, airway closure index, collagen and airway smooth muscle content, airway epithelial TGF-β expression, and infiltration of LAM cells and inflammatory cells within the small airway walls. Results The age of patients with LAM was 39 ± 8 years, with FEV1 and DLCO of 62 ± 30% predicted and 62 ± 32% predicted, respectively. Patients with LAM had increased small airway closure index, collagen and smooth muscle content, and epithelial TGF-beta expression compared with controls. Patients with LAM with the more severe LHS and with greater functional severity (FEV1 ≤ 30%) presented higher thicknesses of the airways. Bronchiolar inflammation was mild; infiltration of the small airway walls by LAM cells was rare. LHS was associated with an obstructive pattern, air trapping, and reduced DLCO, whereas small airway wall thickness was associated with FEV1, FVC, and collagen content. Conclusion LAM is associated with small airway remodelling and partial airway closure, with structural alterations observed at different airway compartments. Functional impairment in LAM is associated with airway remodelling and, most importantly, with histological severity (LHS).

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“…É comum ocorrer a conclusão do diagnóstico da LAM em torno de 30 a 40 anos de idade da paciente, frequentemente anos depois do início dos sintomas 6 . Junior e Carvalho (2004) 4 40,41 .…”

Section: Quadro Clínico Na Lamunclassified

Alterações morfológicas das pequenas vias aéreas associadas ao comprometimento funcional na linfangioleiomiomatose

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Linfangioleiomiomatose pulmonar

Cited by 6 publications

References 42 publications

Desaturation – distance ratio: a new concept for a functional assessment of interstitial lung diseases

Desaturation – distance ratio: a new concept for a functional assessment of interstitial lung diseases

Small airways morphological alterations associated with functional impairment in lymphangioleiomyomatosis

Alterações morfológicas das pequenas vias aéreas associadas ao comprometimento funcional na linfangioleiomiomatose

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