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Doctoral Thesis
DOI
https://doi.org/10.11606/T.5.2020.tde-29012020-115137
Document
Author
Full name
Daniel Antunes Silva Pereira
E-mail
Institute/School/College
Knowledge Area
Date of Defense
Published
São Paulo, 2019
Supervisor
Committee
Kairalla, Ronaldo Adib (President)
Shinjo, Samuel Katsuyuki
Tibana, Regina Celia Carlos
Lima, Mariana Silva
Title in Portuguese
Pneumonia intersticial com aspectos autoimunes: sobrevivência e evolução funcional em pacientes com autoimunidade sistêmica e doença pulmonar intersticial predominante
Keywords in Portuguese
Autoanticorpos
Autoimunidade
Doenças do tecido conjuntivo
Doenças pulmonares intersticiais
Fibrose pulmonar
Fibrose pulmonar idiopática
Abstract in Portuguese
INTRODUÇÃO: Pneumonia Intersticial com Aspectos Autoimunes (PIAA) é uma classificação preliminar para designar um grupo de pacientes com doença pulmonar intersticial (DPI) que combina características das doenças do tecido conjuntivo (DTC) e das Pneumonias Intersticiais Idiopáticas (PII). Porém, a relevância dessa terminologia e seus critérios para diferenciar esses três grupos ainda está em debate. OBJETIVOS: Avaliar sobrevida e evolução funcional de pacientes com PIAA em comparação aos grupos DTC e PII. MÉTODOS: Realizamos um estudo de coorte histórica iniciado em 2012, incluindo pacientes com DTC, PII e PIAA e comparamos suas características clínicas, funcionais, morfológicas e de sobrevivência. RESULTADOS PRINCIPAIS: Foram incluídos 131 pacientes (56 DTC, 37 PII e 38 PIAA). O grupo PIAA era majoritariamente feminino e jovem (mediana 56,5 anos), mediana %predCVF 72%, e com maior prevalência de padrão não-PIU. Havia entre os três grupos diferenças significativas em idade, gênero, positividade de autoanticorpos e padrão tomográfico. Na amostra geral, pacientes eram apenas levemente restritivos ao diagnóstico, mas com significativa piora da %predCVF após 5 anos (p < 0,001). Em termos de variação absoluta, deterioração funcional aconteceu em 86% das PII, em 74% das PIAA e em apenas 46% das DTC. Análise de sobrevivência demonstrou diferença estatística entre os grupos (p=0,004). Pacientes com DTC tinham melhor sobrevivência que as PII (p=0,002), mas não havia diferença entre PIAA e DTC ou PII. Na análise multivariada, o grupo DTC apresentava melhor sobrevivência que o grupo PIAA (HR=0,18), enquanto não houve distinção entre PII e PIAA ou PII e DTC. CONCLUSÃO: PIAA tem comportamento intermediário entre DTC e PII, com aspectos clínicos, sorológicos e morfológicos das DTC combinados a piores prognóstico e evolução funcional, características das PII
Title in English
Interstitial pneumonia with autoimmune features: survival and functional evolution in patients with systemic autoimmunity and predominant interstitial lung disease
Keywords in English
Autoantibodies
Autoimmunity
Connective tissue diseases
Idiopathic pulmonar fibrosis
Lung diseases interstitial
Pulmonary fibrosis
Abstract in English
RATIONALE: Interstitial pneumonia with autoimmune features (IPAF) is a provisional term used for interstitial lung disease (ILD) patients with characteristics of connective tissue disease (CTD) and idiopathic interstitial pneumonia (IIP); however, the power of the existing criteria to differentiate among the three diagnoses is debated. OBJECTIVES: To evaluate survival and functional evolution of IPAF patients in comparison with CTD and IIP groups. METHODS: A historical cohort of CTD, IIP and IPAF patients was enrolled in 2012. Clinical, functional, morphological and survival characteristics were compared. MAIN RESULTS: We included 131 patients (56 CTD, 37 IIP and 38 IPAF). The IPAF patients were predominantly female and younger (median 56.5 years), mildly restrictive with median %predFVC 72%, mostly non-usual interstitial pneumonia (UIP). There were significant differences in age, gender, autoantibody positivity, and CT patterns between groups. Overall, patients had mild functional impairment at the baseline and a significant change in the %predFVC after 5 years (p < 0.001); deterioration occurred in 86% of the IIP, 74% of the IPAF, and 46% of the CTD patients. The survival analysis showed a significant difference among the groups (p=0.004). CTD patients had better survival than the IIP patients (p=0.002), but there was no difference in survival between IPAF and CTD or IIP patients. The CTD group had a significantly better hazard ratio than the IPAF group (HR=0.18) in the multivariate analysis, but the HR of the IIP group did not differ from that of any other group. CONCLUSION: IPAF is intermediate between CTD and IIP, with the clinical, serological and morphological aspects of CTD and the worse prognosis and functional evolution of IIP
 
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Publishing Date
2020-01-29
 
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