Raras
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Pneumonia intersticial descamativa
ORPHA:98852CID-10 · J84.1CID-11 · CB03.3OMIM 263000DOENÇA RARA

Uma inflamação pulmonar rara e de causa desconhecida, caracterizada pelo acúmulo de um tipo de célula de defesa (macrófagos) nos pequenos sacos de ar dos pulmões (alvéolos), além de inflamação no tecido de suporte do órgão. Geralmente afeta fumantes. Alguns pacientes podem desenvolver uma cicatriz progressiva nos pulmões (fibrose pulmonar), que atinge o tecido de suporte.

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Introdução

O que você precisa saber de cara

📋

Uma inflamação pulmonar rara e de causa desconhecida, caracterizada pelo acúmulo de um tipo de célula de defesa (macrófagos) nos pequenos sacos de ar dos pulmões (alvéolos), além de inflamação no tecido de suporte do órgão. Geralmente afeta fumantes. Alguns pacientes podem desenvolver uma cicatriz progressiva nos pulmões (fibrose pulmonar), que atinge o tecido de suporte.

Publicações científicas
439 artigos
Último publicado: 2026 Feb 24
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: J84.1
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

🫁
Pulmão
6 sintomas
📏
Crescimento
1 sintomas
🫘
Rins
1 sintomas

+ 6 sintomas em outras categorias

Características mais comuns

Hipertrofia de pneumócitos tipo II
Taquipneia
Anormalidade do metabolismo/homeostase
Cianose
Déficit de crescimento
Tosse
14sintomas
Sem dados (14)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 14 características clínicas mais associadas, ordenadas por frequência.

Hipertrofia de pneumócitos tipo IIType II pneumocyte hypertrophy
TaquipneiaTachypnea
Anormalidade do metabolismo/homeostaseAbnormality of metabolism/homeostasis
CianoseCyanosis
Déficit de crescimentoFailure to thrive

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico439PubMed
Últimos 10 anos110publicações
Pico202016 papers
Linha do tempo
2026Hoje · 2026🧪 2013Primeiro ensaio clínico📈 2020Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

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Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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Tratamento e manejo

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Pipeline de tratamentos
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2Fase 21
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 2 ensaios
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Ensaios clínicos abertos e novidades científicas recentes

Pesquisa e ensaios clínicos

2 ensaios clínicos encontrados.

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Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

Timeline de publicações
112 papers (10 anos)
#1

Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.

Radiology2026 Feb

Background Combined pulmonary fibrosis and emphysema (CPFE) presents with heterogeneous radiologic patterns, but its impact on disease progression and clinical outcomes remains poorly understood. Purpose To evaluate the impact of different fibrosis patterns on disease trajectory and outcomes in patients with CPFE. Materials and Methods This retrospective study included patients who underwent high-resolution CT between January 2011 and December 2021, with CPFE defined as emphysema (any subtype) involving at least 5% of total lung volume and fibrosis at CT, and who had available follow-up data. Radiologic fibrosis patterns were classified as usual interstitial pneumonia (UIP) and/or probable UIP (radiologic UIP) or as other patterns, including nonspecific interstitial pneumonia (NSIP), smoking-related interstitial fibrosis (SRIF), and desquamative interstitial pneumonia (DIP). Deep learning-based quantification was used to assess the extent of emphysema and fibrosis. Longitudinal outcomes were analyzed using the Cox proportional hazards models. Results A total of 236 patients with CPFE were included (mean age, 65 years ± 7.8 [SD]; 232 men). Of the 236 patients, 99 (41.9%) were classified with UIP, 73 (30.9%) with NSIP, 48 (20.3%) with SRIF, 14 (5.93%) with DIP, and two (0.85%) with an unclassifiable pattern. Radiologic fibrosis patterns were associated with the rate of fibrotic progression, which was fastest in UIP (median, 1.2 years), followed by NSIP (median, 2.1 years), and slowest in SRIF or DIP (median, 3.0 years; P < .001). However, radiologic fibrosis patterns were not significantly associated with emphysema progression (univariable P = .27), lung transplant-free survival (multivariable P = .55), time-dependent lung cancer (multivariable P = .65), or acute exacerbation (multivariable P = .64). Greater baseline fibrosis extent (adjusted hazard ratio [HR]: 1.04; 95% CI: 1.02, 1.06), lung cancer (adjusted HR: 3.90; 95% CI: 2.55, 5.96), and acute exacerbation (adjusted HR: 4.31; 95% CI: 2.68, 6.94) were associated with worse survival. Conclusion In patients with CPFE, radiologic fibrosis patterns were associated with the rate of fibrosis progression, whereas survival was primarily determined by baseline fibrosis extent and adverse clinical events, including the development of lung cancer. © The Author(s) 2026. Published by the Radiological Society of North America under a CC BY 4.0 license. Supplemental material is available for this article.

#2

DIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.

Clinical medicine insights. Case reports2026

Pure ground-glass and part-solid lung nodules are becoming increasingly appreciated as harbingers of malignancy. However, occasionally diagnostic biopsies can demonstrate unexpected findings that may lead to a misleading diagnosis and clinical confusion. Here we present the case of a 70-year-old Taiwanese man with a light smoking history (10-20 pack years), emphysema, and childhood-treated tuberculosis who presented with a part-solid nodule that was diagnosed as a very rare case of adenocarcinoma of the lung with desquamative interstitial pneumonia (DIP)-like microscopic morphology. DIP is a non-neoplastic interstitial lung disease which presents as a diffuse and bilateral process and primarily occurs in smokers. When the morphologic pattern of DIP presents as a focal process, usually around bronchioles, it is labeled respiratory bronchiolitis. (RB)- a very common entity and almost universal in patients with significant smoking history. We describe this case to help educate and guide pathologists on the existence of this very rare entity and to consider performing lung cancer immunohistochemical markers whenever faced with this unexpected histological pattern in a pulmonary nodule.

#3

Radiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.

Seminars in roentgenology2026 Jan

Cystic lung diseases represent a heterogeneous group of conditions characterized by the presence of multiple pulmonary cysts. Accurate recognition and differentiation of true cysts from their mimics (such as cavities, emphysema, bullae, blebs, and honeycombing) are essential, as management strategies and prognoses vary significantly. This review provides a systematic approach to the radiologic evaluation of cystic lung diseases, beginning with the definition and pathogenesis of pulmonary cysts and progressing through a structured diagnostic algorithm. We detail the characteristic imaging findings and clinical associations for major cystic lung diseases, including lymphangioleiomyomatosis (LAM), Birt-Hogg-Dubé syndrome (BHD), light-chain deposition disease (LCDD), lymphocytic interstitial pneumonia (LIP), desquamative interstitial pneumonia (DIP), pulmonary Langerhans cell histiocytosis (PLCH), and amyloidosis. Additionally, we discuss uncommon etiologies, such as cystic metastases, hypersensitivity pneumonitis, and cystic changes associated with genetic syndromes like neurofibromatosis type 1 (NF-1) and trisomy 21. The review emphasizes key imaging clues, such as cyst distribution, wall characteristics, associated nodules, and parenchymal abnormalities, that aid in narrowing the differential diagnosis. With the growing use of CT imaging, cystic lung diseases are increasingly identified in both symptomatic and asymptomatic patients. Familiarity with their imaging patterns, clinical contexts, and distinguishing features is essential for radiologists and clinicians alike. By following a stepwise, pattern-based approach, early and accurate diagnosis can be achieved, potentially improving patient outcomes through timely surveillance and targeted management.

#4

"Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".

Seminars in roentgenology2026 Jan

Despite overwhelming data detailing the harm of cigarette use, nearly 1.25 billion people worldwide continue to smoke. Amongst the myriad afflictions associated with cigarette use, smoking related interstitial lung disease is a common and likely underrecognized entity given the overlap and lack of consensus in clinical, pathological, and radiological diagnosis. Many characteristic patterns of disease have been identified on diagnostic chest imaging, and using a pattern-based approach to radiologic diagnosis can improve diagnostic accuracy. Several discrete disease processes are categorized as smoking related interstitial lung disease and range from a potentially reversible desquamative interstitial pneumonia/alveolar macrophage pneumonia, to chronic and insidious onset idiopathic pulmonary fibrosis with high morbidity and mortality. This article reviews the common clinical findings and demographics associated with patient presentation, histopathological findings for each of the smoking related interstitial lung diseases upon tissue sampling or biopsy and suggests a pattern-based approach to radiologic diagnosis.

#5

Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.

Internal medicine journal2026 Feb 24

Publicações recentes

Ver todas no PubMed

📚 EuropePMC206 artigos no totalmostrando 108

2026

Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.

Internal medicine journal
2026

Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.

Radiology
2026

DIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.

Clinical medicine insights. Case reports
2026

Radiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.

Seminars in roentgenology
2026

"Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".

Seminars in roentgenology
2025

Understanding cystic lung lesions in smokers with interstitial lung disease: radiologic-pathological correlation.

Insights into imaging
2025

The Dual Threat Combined Pulmonary Fibrosis and Emphysema (CPFE): Two Cases.

International journal of chronic obstructive pulmonary disease
2025

Update of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement.

The European respiratory journal
2025

Review of research on pulmonary cysts in sjögren's syndrome.

Journal of cardiothoracic surgery
2025

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases.

Tuberculosis and respiratory diseases
2025

Treatment of Desquamative Interstitial Pneumonia and Systemic Sclerosis with Corticosteroids and Immunosuppressants.

Internal medicine (Tokyo, Japan)
2025

Anti-annexin A4 antibody as a biomarker for desquamative interstitial pneumonia.

Journal of autoimmunity
2025

Korean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.

Tuberculosis and respiratory diseases
2024

Prevalence of Gastroesophageal Reflux in Interstitial Lung Diseases Clinic.

Cureus
2024

Smoking-related interstitial lung disease: A narrative review.

Chronic respiratory disease
2024

Why making smoking cessation a priority for rare interstitial lung disease smokers?

Tobacco prevention &amp; cessation
2024

Smoking-Related Interstitial Lung Disease and Emphysema.

Clinics in chest medicine
2024

Smoking-Related Interstitial Lung Disease: Historical Perspective and Advances in the Twenty-first Century.

Surgical pathology clinics
2024

Multidisciplinary Approach to the Diagnosis of Idiopathic Interstitial Pneumonias: Focus on the Pathologist's Key Role.

International journal of molecular sciences
2024

Desquamative interstitial pneumonia: A case report.

Experimental and therapeutic medicine
2023

Peribronchiolar Metaplasia: A Marker of Cigarette Smoke-Induced Small Airway Injury in a Rural Cohort.

Clinical pathology (Thousand Oaks, Ventura County, Calif.)
2023

Sequence of Rare Diagnoses in a Young Patient: Altitude Barotrauma Hemopneumothorax and Desquamative Interstitial Pneumonia.

Diagnostics (Basel, Switzerland)
2023

Imaging Features of Idiopathic Interstitial Lung Diseases.

Journal of thoracic imaging
2024

Desquamative Interstitial Pneumonia with Progressive Pulmonary Fibrosis.

Internal medicine (Tokyo, Japan)
2023

Inescapable Fibrosis: The Development of Desquamative Interstitial Pneumonia Post-Lung Transplantation Performed for a Patient with Idiopathic Pulmonary Fibrosis.

Case reports in transplantation
2023

Desquamative Interstitial Pneumonia.

The New England journal of medicine
2023

Smoking-Related Interstitial Lung Diseases.

Immunology and allergy clinics of North America
2023

ABCA3-related interstitial lung disease beyond infancy.

Thorax
2022

Smoking-related interstitial lung disease.

Radiologia
2022

Diffuse cystic lung diseases: Imaging spectrum and diagnostic approach using high-resolution computed tomography.

Lung India : official organ of Indian Chest Society
2023

Smoking-Related Interstitial Fibrosis (SRIF) in Patients Presenting With Diffuse Parenchymal Lung Disease.

American journal of clinical pathology
2022

Imaging of Smoking and Vaping Related Diffuse Lung Injury.

Radiologic clinics of North America
2022

Simultaneous pathological findings in biopsy specimens of patients with surgically resected lung carcinoids and their role in survival.

Oncology letters
2022

IgG4-related lung disease with a desquamative interstitial pneumonia pattern radiologically and pathologically.

BMJ case reports
2022

[Smoking-related interstitial lung diseases].

Radiologie (Heidelberg, Germany)
2022

Desquamative interstitial pneumonia after tear gas exposure.

Respiratory medicine and research
2022

Safety and accuracy of transbronchial lung cryobiopsy in diagnosing desquamative interstitial pneumonia.

The clinical respiratory journal
2021

Usual Interstitial Pneumonia is the Most Common Finding in Surgical Lung Biopsies from Patients with Persistent Interstitial Lung Disease Following Infection with SARS-CoV-2.

EClinicalMedicine
2021

Idiopathic desquamative interstitial pneumonia diagnosed using transbronchial lung cryobiopsy: A case report.

Respiratory medicine case reports
2022

Interstitial Lung Disease in Systemic Sclerosis: A Single-center Retrospective Analysis.

Current rheumatology reviews
2021

Fibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players.

International journal of molecular sciences
2021

Biopsy in interstitial lung disease: specific diagnosis and the identification of the progressive fibrotic phenotype.

Current opinion in pulmonary medicine
2021

A Case of Pulmonary Adenocarcinoma Presenting with Diffuse Cystic Lesions.

Case reports in oncology
2021

The Application of Transbronchial Lung Cryobiopsy and Uniportal and Tubeless Video-Assisted Thoracic Surgery in the Multidisciplinary Diagnosis of Interstitial Lung disease-A Real-World Prospective Study.

Frontiers in molecular biosciences
2021

Diffuse interstitial pneumonia-like/macrophage activation syndrome-like changes in patients with COVID-19 correlate with length of illness.

Annals of diagnostic pathology
2021

Pathology, Radiology, and Genetics of Interstitial Lung Disease in Patients With Shortened Telomeres.

The American journal of surgical pathology
2021

Interstitial lung abnormality (ILA) and nonspecific interstitial pneumonia (NSIP).

European journal of radiology open
2021

High-resolution CT in smoking-related interstitial lung diseases.

The international journal of tuberculosis and lung disease : the official journal of the International Union against Tuberculosis and Lung Disease
2020

Transbronchial lung cryobiopsy in smoking-related interstitial lung diseases.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
2021

COVID-19 mimics on chest CT: a pictorial review and radiologic guide.

The British journal of radiology
2020

COVID-19 Like Findings in a Fatal Case of Idiopathic Desquamative Interstitial Pneumonia Associated With IgA Glomerulonephritis in a 13-Month-Old Child.

Frontiers in pediatrics
2020

Desquamative interstitial pneumonia induced by metal exposure. A case report and literature review.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
2020

Five simple reasons to discard DIP, or why we should stop calling dolphins big fish.

Journal of clinical pathology
2020

The interstitial lung disease spectrum under a uniform diagnostic algorithm: a retrospective study of 1,945 individuals.

Journal of thoracic disease
2020

Interstitial lung disease in dermatomyositis complicated by right ventricular thrombus secondary to macrophage activation syndrome- a case report.

AME case reports
2020

Lung Involvement in Primary Sjögren's Syndrome-An Under-Diagnosed Entity.

Frontiers in medicine
2020

"Desquamative interstitial pneumonia: a systematic review of its features and outcomes." Merel E. Hellemons, Catharina C. Moor, Jan von der Thüsen, Mariska Rossius, Arlette Odink, Laila Haugen Thorgersen, Johny Verschakelen, Wim Wuyts, Marlies S. Wijsenbeek and Elisabeth Bendstrup. Eur Respir Rev 2020; 29: 190181.

European respiratory review : an official journal of the European Respiratory Society
2020

Unusual pneumoconiosis in two patients with heavy print toner, and paper dust exposure.

American journal of industrial medicine
2020

Desquamative interstitial pneumonia: still orphan and not always benign.

European respiratory review : an official journal of the European Respiratory Society
2020

Desquamative interstitial pneumonia: a systematic review of its features and outcomes.

European respiratory review : an official journal of the European Respiratory Society
2020

Biphasic malignant mesothelioma with epithelioid and sarcomatoid components (dedifferentiated mesothelioma) and intrapulmonary growth: a rare entity mimicking desquamative interstitial pneumonia.

Journal of clinical pathology
2019

Aluminium toxicosis: a review of toxic actions and effects.

Interdisciplinary toxicology
2020

High resolution computed tomography patterns in interstitial lung disease (ILD): prevalence and prognosis.

Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
2020

[Diffuse Intraparenchymal Mesothelioma: An Atypical Presentation].

Acta medica portuguesa
2020

Desquamative interstitial pneumonia in a non-smoker with neurofibromatosis type 1 (Von Recklinghausen syndrome).

BMJ case reports
2019

Probe-based confocal laser endomicroscopy in diagnosis of desquamative interstitial pneumonia in nonsmoker.

Monaldi archives for chest disease = Archivio Monaldi per le malattie del torace
2019

A Stepwise Diagnostic Approach to Cystic Lung Diseases for Radiologists.

Korean journal of radiology
2020

Increased Pulmonary GM-CSF Causes Alveolar Macrophage Accumulation. Mechanistic Implications for Desquamative Interstitial Pneumonitis.

American journal of respiratory cell and molecular biology
2019

Korean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction.

Tuberculosis and respiratory diseases
2019

Desquamative interstitial pneumonia with clinical, radiological and histologic correlation.

Radiology case reports
2019

Desquamative interstitial pneumonia: Risk factors, laboratory and bronchoalveolar lavage findings, radiological and histopathological examination, clinical features, treatment and prognosis.

Experimental and therapeutic medicine
2019

Anti-Ku antibody-positive desquamative interstitial pneumonia.

Respiratory medicine case reports
2018

Idiopathic interstitial pneumonias: review of the latest American Thoracic Society/European Respiratory Society classification.

Radiologia brasileira
2018

Successful lung transplantation in an HIV seropositive patient with desquamative interstitial pneumonia: a case report.

BMC pulmonary medicine
2018

IgG4-related disease presenting with combined pulmonary fibrosis and emphysema (CPFE).

Respiratory medicine case reports
2018

A Review of Smoking-Related Interstitial Fibrosis, Respiratory Bronchiolitis, and Desquamative Interstitial Pneumonia: Overlapping Histology and Confusing Terminology.

Archives of pathology &amp; laboratory medicine
2018

Smoking Relapse Causing an Acute Exacerbation of Desquamative Interstitial Pneumonia with Pleural Effusions and Mediastinal Adenopathies.

Case reports in pulmonology
2018

Pulmonary Adenocarcinoma Mimicking Desquamative Interstitial Pneumonia: Report of 2 Cases With Genetic Analysis.

International journal of surgical pathology
2018

Hermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood.

Orphanet journal of rare diseases
2018

Desquamative interstitial pneumonia complicated by diffuse alveolar haemorrhage.

Respirology case reports
2017

Pulmonary Neuroendocrine Cell Hyperplasia Associated with Surfactant Protein C Gene Mutation.

Case reports in pulmonology
2018

Smoking-related lung abnormalities on computed tomography images: comparison with pathological findings.

Japanese journal of radiology
2018

Current Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases.

Chest
2018

Unfavourable outcome of glucocorticoid treatment in suspected idiopathic pulmonary fibrosis.

Respirology (Carlton, Vic.)
2017

Overlaps and uncertainties of smoking-related idiopathic interstitial pneumonias.

International journal of chronic obstructive pulmonary disease
2017

[Desquamative interstitial pneumonia: report of one case and review of literature].

Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases
2018

Pulmonary Adenocarcinoma, Intra-alveolar Variant: A Rare Entity Mimicking Desquamative Interstitial Pneumonia.

International journal of surgical pathology
2017

Desquamative Interstitial Pneumonia Complicated with IgG4-related Lung Disease.

Internal medicine (Tokyo, Japan)
2017

Respiratory bronchiolitis-associated interstitial lung disease - an unexpected form of idiopathic interstitial pneumonia in a young male.

Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
2017

A case of mixed dust pneumoconiosis with desquamative interstitial pneumonia-like reaction in an aluminum welder.

Respiratory medicine case reports
2016

Hard metal lung disease: a case series.

Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia
2016

Clinical-Radiologic-Pathologic Correlation of Smoking-Related Diffuse Parenchymal Lung Disease.

Radiologic clinics of North America
2017

Transbronchial Cryobiopsy in Diffuse Parenchymal Lung Disease: Retrospective Analysis of 74 Cases.

Chest
2016

[Interstitial lung diseases associated with smoking].

Ceskoslovenska patologie
2016

Cystic Lung Diseases: Algorithmic Approach.

Chest
2016

Transbronchial cryobiopsy in the diagnosis of desquamative interstitial pneumonia.

Revista portuguesa de pneumologia
2016

Mutually exclusive extracellular signal-regulated kinase pathway mutations are present in different stages of multi-focal pulmonary Langerhans cell histiocytosis supporting clonal nature of the disease.

Histopathology
2016

Sirolimus-Induced Diffuse Alveolar Hemorrhage: A Case Report.

American journal of therapeutics
2015

American Thoracic Society-European Respiratory Society Classification of the Idiopathic Interstitial Pneumonias: Advances in Knowledge since 2002.

Radiographics : a review publication of the Radiological Society of North America, Inc
2015

Smoking and interstitial lung diseases.

European respiratory review : an official journal of the European Respiratory Society
2015

Near fatal desquamative interstitial pneumonia with bilateral recurrent tension pneumothorax.

Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG
2015

Ultrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis.

Case reports in rheumatology
2015

Pulmonary fibrosis and exposure to steel welding fume.

Occupational medicine (Oxford, England)
2016

Smoking-related idiopathic interstitial pneumonia: A review.

Respirology (Carlton, Vic.)
2015

Inflammatory Diseases of the Lung Induced by Conventional Cigarette Smoke: A Review.

Chest
2015

Usual interstitial pneumonia end-stage features from explants with radiologic and pathological correlations.

Annals of diagnostic pathology
2015

Interstitial lung disease related to smoking: imaging considerations.

Current opinion in pulmonary medicine
2014

[The morphology and molecular bases of damage to the stem cell niche of respiratory acini in idiopathic interstitial pneumonias].

Arkhiv patologii
Ver todos os 206 no EuropePMC

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.
    Radiology· 2026· PMID 41733469mais citado
  2. DIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.
    Clinical medicine insights. Case reports· 2026· PMID 41728051mais citado
  3. Radiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.
    Seminars in roentgenology· 2026· PMID 41521085mais citado
  4. "Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".
    Seminars in roentgenology· 2026· PMID 41513511mais citado
  5. Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.
    Internal medicine journal· 2026· PMID 41735806mais citado

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:98852(Orphanet)
  2. OMIM OMIM:263000(OMIM)
  3. MONDO:0009887(MONDO)
  4. GARD:16864(GARD (NIH))
  5. Busca completa no PubMed(PubMed)
  6. Q5265029(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Pneumonia intersticial descamativa
Compêndio · Raras BR

Pneumonia intersticial descamativa

ORPHA:98852 · MONDO:0009887
CID-10
J84.1 · Outras doenças pulmonares intersticiais com fibrose
CID-11
MedGen
UMLS
C0238378
Repurposing
47 candidatos
atovaquonemitochondrial electron transport inhibitor
azithromycinbacterial 50S ribosomal subunit inhibitor
benzylpenicillinpenicillin binding protein inhibitor
+17 outros
EuropePMC
Wikidata
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