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Review
. 2015 Sep-Oct;41(5):454-66.
doi: 10.1590/S1806-37132015000000152.

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

[Article in English, Portuguese]
José Baddini-Martinez  1 , Bruno Guedes Baldi  2 , Cláudia Henrique da Costa  3 , Sérgio Jezler  4 , Mariana Silva Lima  5 , Rogério Rufino  3
Affiliations
Review

Update on diagnosis and treatment of idiopathic pulmonary fibrosis

[Article in English, Portuguese]
José Baddini-Martinez et al. J Bras Pneumol. 2015 Sep-Oct.

Abstract
in English, Portuguese

Idiopathic pulmonary fibrosis is a type of chronic fibrosing interstitial pneumonia, of unknown etiology, which is associated with a progressive decrease in pulmonary function and with high mortality rates. Interest in and knowledge of this disorder have grown substantially in recent years. In this review article, we broadly discuss distinct aspects related to the diagnosis and treatment of idiopathic pulmonary fibrosis. We list the current diagnostic criteria and describe the therapeutic approaches currently available, symptomatic treatments, the action of new drugs that are effective in slowing the decline in pulmonary function, and indications for lung transplantation.

A fibrose pulmonar idiopática é um tipo de pneumonia intersticial crônica fibrosante, de causa desconhecida, associada à piora funcional respiratória progressiva e taxas elevadas de mortalidade. Em anos recentes, o interesse e os conhecimentos sobre essa moléstia têm aumentado substancialmente. O presente artigo de revisão aborda de maneira ampla aspectos relacionados ao diagnóstico e tratamento da doença. Nele são listados os critérios atuais para o diagnóstico e são discutidos as diferentes abordagens terapêuticas agora disponíveis, o tratamento sintomático, a ação de novas drogas eficazes em reduzir o ritmo de deterioração funcional pulmonar e as indicações para transplante pulmonar.

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Figures

Chart 1.
Chart 1.. HRCT criteria for the diagnosis of usual interstitial pneumonia.a
Chart 2
Chart 2. Histological criteria for the diagnosis of usual interstitial pneumonia.a
Chart 3.
Chart 3.. Combination of HRCT and lung biopsy findings for the diagnosis of idiopathic pulmonary fibrosis.
Chart 4.
Chart 4.. Combination of HRCT and lung biopsy findings for the diagnosis of idiopathic pulmonary fibrosis, in accordance with the 2012 Brazilian Thoracic Association Guidelines for Interstitial Lung Diseases.a
Chart 5.
Chart 5.. A few randomized, double-blind, placebo-controlled clinical trials whose primary outcomes were not met.
Chart 6.
Chart 6.. Indications for oxygen therapy in patients with idiopathic pulmonary fibrosis.
Chart 7.
Chart 7.. Indications and contraindications for lung transplantation in patients with idiopathic pulmonary fibrosis.
Chart 8.
Chart 8.. Contemporary treatment of idiopathic pulmonary fibrosis.
Quadro 1.
Quadro 1.. Critérios radiológicos de TCAR para o diagnóstico de pneumonia intersticial usual.a
Quadro 2.
Quadro 2.. Critérios histológicos para diagnóstico de pneumonia intersticial usual.a
Quadro 3.
Quadro 3.. Critérios diagnósticos de fibrose pulmonar idiopática em função da combinação de achados de TCAR e de biópsia pulmonar.a
Quadro 4.
Quadro 4.. Critérios diagnósticos de fibrose pulmonar idiopática em função da combinação de achados de TCAR e de biópsia pulmonar preconizados pelas Diretrizes Brasileiras de Doenças Intersticiais Pulmonares de 2012.a
Quadro 5.
Quadro 5.. Alguns ensaios clínicos controlados, duplo-cegos, randomizados, com desfechos primários não alcançados em fibrose pulmonar idiopática.
Quadro 6.
Quadro 6.. Indicações do uso de oxigenoterapia em pacientes com fibrose pulmonar idiopática.
Quadro 7.
Quadro 7.. Indicações e contraindicações para transplante pulmonar em pacientes com fibrose pulmonar idiopática.
Quadro 8.
Quadro 8.. Tratamento contemporâneo da fibrose pulmonar idiopática.
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References

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