Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Sep-Oct;43(5):393-398.
doi: 10.1590/S1806-37562016000000368.

Usual interstitial pneumonia: typical, possible, and "inconsistent" patterns

[Article in English, Portuguese]
Pedro Paulo Teixeira E Silva Torres  1 , Marcelo Fouad Rabahi  2 , Maria Auxiliadora Carmo Moreira  2 , Gustavo de Souza Portes Meirelles  3 , Edson Marchiori  4
Affiliations

Usual interstitial pneumonia: typical, possible, and "inconsistent" patterns

[Article in English, Portuguese]
Pedro Paulo Teixeira E Silva Torres et al. J Bras Pneumol. 2017 Sep-Oct.

Abstract
in English, Portuguese

Idiopathic pulmonary fibrosis is a severe and progressive chronic fibrosing interstitial lung disease, a definitive diagnosis being established by specific combinations of clinical, radiological, and pathological findings. According to current international guidelines, HRCT plays a key role in establishing a diagnosis of usual interstitial pneumonia (UIP). Current guidelines describe three UIP patterns based on HRCT findings: a typical UIP pattern; a pattern designated "possible UIP"; and a pattern designated "inconsistent with UIP", each pattern having important diagnostic implications. A typical UIP pattern on HRCT is highly accurate for the presence of histopathological UIP, being currently considered to be diagnostic of UIP. The remaining patterns require further diagnostic investigation. Other known causes of a UIP pattern include drug-induced interstitial lung disease, chronic hypersensitivity pneumonitis, occupational diseases (e.g., asbestosis), and connective tissue diseases, all of which should be included in the clinical differential diagnosis. Given the importance of CT studies in establishing a diagnosis and the possibility of interobserver variability, the objective of this pictorial essay was to illustrate all three UIP patterns on HRCT.

RESUMO: A fibrose pulmonar idiopática é uma pneumopatia intersticial fibrosante crônica de curso grave e progressivo, e seu diagnóstico se dá em combinações específicas de correlações clínicas e radiológicas, ou ainda patológicas. A TCAR tem papel chave no diagnóstico morfológico do padrão de pneumonia intersticial usual (PIU), segundo as recomendações internacionais vigentes. Os níveis de certeza para a leitura do padrão tomográfico foram inseridos em diretrizes atuais, descritos como padrão PIU típico, padrão PIU possível e padrão inconsistente com PIU, cada qual com importantes implicações na cadeia diagnóstica. A presença do padrão PIU típico tem alta concordância com o padrão PIU histopatológico, e, nessa situação, a TCAR é tida como suficiente para a determinação do padrão morfológico. Nos demais padrões, investigações diagnósticas complementares são indicadas. O diagnóstico diferencial com outras entidades, incluindo pneumopatias intersticiais por exposição medicamentosa, exposições extrínsecas (pneumonite de hipersensibilidade crônica), doenças ocupacionais (asbestose) e doenças do tecido conjuntivo, deve ser considerado clinicamente. Dada a importância da abordagem tomográfica, a qual pode apresentar relevantes discordâncias na avaliação interobservador, nosso objetivo foi ilustrar os padrões tomográficos de PIU neste ensaio pictórico.

PubMed Disclaimer

Figures

Figure 1
Figure 1. A 77-year-old female patient presenting with a typical usual interstitial pneumonia pattern. In A, axial CT scans of the chest with lung window settings, showing reticular opacities, traction bronchiectasis, and extensive honeycombing. In B, coronal reformatted CT images showing an apicobasal gradient of involvement.
Figure 2
Figure 2. A 75-year-old male patient presenting with a possible usual interstitial pneumonia pattern. Axial CT scan of the chest with lung window settings (in A) and coronal reformatted CT image (in B) showing peripheral reticular opacities and traction bronchiolectasis (in A) and an apicobasal gradient (in B), without honeycombing.
Figure 3
Figure 3. A 38-year-old female patient presenting with an inconsistent with usual interstitial pneumonia pattern and diagnosed with chronic hypersensitivity pneumonitis. Axial HRCT scan of the chest with lung window settings, showing diffuse reticular opacities with traction bronchiolectasis. Note severe peribronchovascular bundle involvement in the right upper lobe (arrow).
Figure 4
Figure 4. A 63-year-old female patient presenting with an inconsistent with usual interstitial pneumonia pattern and diagnosed with sarcoidosis. Axial HRCT scan of the chest with lung window settings, showing confluent, predominantly peribronchovascular reticular opacities with characteristics of micronodules with a perilymphatic distribution (arrows).
Figure 5
Figure 5. A 61-year-old female patient presenting with an inconsistent with usual interstitial pneumonia pattern and diagnosed with chronic hypersensitivity pneumonitis. Axial HRCT scans of the chest with lung window settings, showing diffuse reticular opacities, as well as areas of ground-glass attenuation associated with areas of decreased attenuation (arrows), characterizing a mosaic pattern.
Figure 6
Figure 6. Axial CT scans of the chest with lung window settings. In A, scan taken during an inadequately performed inspiratory maneuver, the resulting image resembling diffuse ground-glass opacity. In B, a new scan, taken during an adequately performed inspiratory maneuver.
Figure 7
Figure 7. In A, axial HRCT scan of the chest with lung window settings. In B, coronal reformatted CT image (minimum intensity projection). In A, images suggestive of a cluster of subpleural cysts (arrows), suspected of being honeycombing but found to be traction bronchiectasis (arrows) on oblique coronal reformatted CT images (in B).
Figure 8
Figure 8. Axial HRCT scans of the chest with lung window settings, showing the right lower lobe. In A, initial CT findings meeting the criteria for possible usual interstitial pneumonia (UIP), i.e., reticular opacities and ground-glass attenuation, without honeycombing. In B, follow-up CT findings six years later, meeting the criteria for a typical UIP pattern, with disease progression and honeycombing.
Figura 1
Figura 1. Paciente do sexo feminino, 77 anos; padrão típico de pneumonia intersticial usual. Imagens de TC do tórax em janela de pulmão no plano axial (em A) mostrando opacidades reticulares, bronquiectasias de tração e extenso faveolamento associado, e imagens em reformatação coronal (em B) evidenciando gradiente apicobasal do acometimento.
Figura 2
Figura 2. Paciente do sexo masculino, 75 anos; padrão possível de pneumonia intersticial usual. Imagens de TC em janela de pulmão no plano axial (em A) e reformatação coronal (em B) mostrando opacidades reticulares e bronquiolectasias de tração de distribuição periférica (em A), com gradiente apicobasal (em B), sem definição de faveolamento.
Figura 3
Figura 3. Paciente do sexo feminino, 38 anos; padrão inconsistente com pneumonia intersticial usual, com diagnóstico de pneumonite de hipersensibilidade crônica. Imagem de TCAR do tórax em janela de pulmão no plano axial evidenciando opacidades reticulares esparsas com bronquiolectasias de tração, destacando-se importante acometimento do feixe peribroncovascular no lobo superior direito (seta).
Figura 4
Figura 4. Paciente do sexo feminino, 63 anos; padrão inconsistente com pneumonia intersticial usual, com diagnóstico de sarcoidose. Imagem de TCAR do tórax em janela de pulmão no plano axial evidenciando opacidades reticulares confluentes, predominantemente peribroncovasculares, porém com caracterização de micronódulos de distribuição perilinfática (setas).
Figura 5
Figura 5. Paciente do sexo feminino, 61 anos; padrão inconsistente com pneumonia intersticial usual, com diagnóstico de pneumonite de hipersensibilidade crônica. Imagens de TCAR do tórax em janela de pulmão no plano axial evidenciando opacidades reticulares esparsas, além de áreas em vidro fosco com áreas de menor atenuação associadas (setas), caracterizando padrão em mosaico.
Figura 6
Figura 6. Imagens axiais de TC do tórax em janela de pulmão. Em A, primeira aquisição realizada com nível inspiratório subótimo simulando presença de vidro fosco difuso e, em B, avaliação após reconvocação e nova aquisição com parâmetros inspiratórios adequados.
Figura 7
Figura 7. Imagem axial de TCAR em janela pulmonar (em A) e reformatação em projeção de intensidade mínima (em B). Em A, caracterizam-se imagens císticas agrupadas em situação subpleural suspeitas para faveolamento (setas), que se confirmaram somente como bronquiectasias de tração na reformatação coronal oblíqua em B (setas).
Figura 8
Figura 8. Imagens axiais de TCAR do tórax em janela pulmonar evidenciando o lobo inferior direito. Em A, exame inicial preenchendo critérios padrão possível com pneumonia intersticial usual (PIU), com opacidades reticulares e vidro fosco, sem faveolamento. Em B, estudo de controle após seis anos evidenciando a progressão dos achados e o surgimento de faveolamento, preenchendo, então, os critérios para padrão PIU típico.
See this image and copyright information in PMC

References

    1. 1 Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK, et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788-824. https://doi.org/10.1164/rccm.2009-040GL - DOI - PMC - PubMed
    2. Raghu G, Collard HR, Egan JJ, Martinez FJ, Behr J, Brown KK. An official ATS/ERS/JRS/ALAT statement idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med. 2011;183(6):788–824. https://doi.org/10.1164/rccm.2009-040GL - DOI - PMC - PubMed
    1. 2 Baddini-Martinez J, Baldi BG, Costa CH, Jezler S, Lima MS, Rufino R. Update on diagnosis and treatment of idiopathic pulmonary fibrosis. J Bras Pneumol. 2015;41(5):454-66. https://doi.org/10.1590/S1806-37132015000000152 - DOI - PMC - PubMed
    2. Baddini-Martinez J, Baldi BG, Costa CH, Jezler S, Lima MS, Rufino R. Update on diagnosis and treatment of idiopathic pulmonary fibrosis. J Bras Pneumol. 2015;41(5):454–466. https://doi.org/10.1590/S1806-37132015000000152 - DOI - PMC - PubMed
    1. 3 Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J, et al. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline: Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3-19. https://doi.org/10.1164/rccm.201506-1063ST - DOI - PubMed
    2. Raghu G, Rochwerg B, Zhang Y, Garcia CA, Azuma A, Behr J. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline Treatment of Idiopathic Pulmonary Fibrosis. An Update of the 2011 Clinical Practice Guideline. Am J Respir Crit Care Med. 2015;192(2):e3–19. https://doi.org/10.1164/rccm.201506-1063ST - DOI - PubMed
    1. 4 Travis WD, Costabel U, Hansell DM, King Jr TE, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6),733-48. https://doi.org/10.1164/rccm.201308-1483ST - DOI - PMC - PubMed
    2. Travis WD, Costabel U, Hansell DM, King TE, Jr, Lynch DA, Nicholson AG, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188(6):733–748. https://doi.org/10.1164/rccm.201308-1483ST - DOI - PMC - PubMed
    1. 5 Hodnett PA, Naidich DP. Fibrosing interstitial lung disease. A practical high-resolution computed tomography-based approach to diagnosis and management and a review of the literature. Am J Resp Crit Care Med. 2013;188(2):141-9. https://doi.org/10.1164/rccm.201208-1544CI - DOI - PubMed
    2. Hodnett PA, Naidich DP. Fibrosing interstitial lung disease A practical high-resolution computed tomography-based approach to diagnosis and management and a review of the literature. Am J Resp Crit Care Med. 2013;188(2):141–149. https://doi.org/10.1164/rccm.201208-1544CI - DOI - PubMed