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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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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Publicações mais relevantes
Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.
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.
DIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.
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.
Radiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.
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.
"Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".
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.
Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.
Publicações recentes
Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.
Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.
DIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.
Radiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.
"Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".
📚 EuropePMC206 artigos no totalmostrando 108
Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.
Internal medicine journalRadiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.
RadiologyDIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.
Clinical medicine insights. Case reportsRadiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.
Seminars in roentgenology"Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".
Seminars in roentgenologyUnderstanding cystic lung lesions in smokers with interstitial lung disease: radiologic-pathological correlation.
Insights into imagingThe Dual Threat Combined Pulmonary Fibrosis and Emphysema (CPFE): Two Cases.
International journal of chronic obstructive pulmonary diseaseUpdate of the international multidisciplinary classification of the interstitial pneumonias: an ERS/ATS statement.
The European respiratory journalReview of research on pulmonary cysts in sjögren's syndrome.
Journal of cardiothoracic surgeryKorean Guidelines for Diagnosis and Management of Interstitial Lung Diseases.
Tuberculosis and respiratory diseasesTreatment of Desquamative Interstitial Pneumonia and Systemic Sclerosis with Corticosteroids and Immunosuppressants.
Internal medicine (Tokyo, Japan)Anti-annexin A4 antibody as a biomarker for desquamative interstitial pneumonia.
Journal of autoimmunityKorean Guidelines for the Diagnosis and Management of Interstitial Lung Disease: Other Forms of Interstitial Lung Disease.
Tuberculosis and respiratory diseasesPrevalence of Gastroesophageal Reflux in Interstitial Lung Diseases Clinic.
CureusSmoking-related interstitial lung disease: A narrative review.
Chronic respiratory diseaseWhy making smoking cessation a priority for rare interstitial lung disease smokers?
Tobacco prevention & cessationSmoking-Related Interstitial Lung Disease and Emphysema.
Clinics in chest medicineSmoking-Related Interstitial Lung Disease: Historical Perspective and Advances in the Twenty-first Century.
Surgical pathology clinicsMultidisciplinary Approach to the Diagnosis of Idiopathic Interstitial Pneumonias: Focus on the Pathologist's Key Role.
International journal of molecular sciencesDesquamative interstitial pneumonia: A case report.
Experimental and therapeutic medicinePeribronchiolar Metaplasia: A Marker of Cigarette Smoke-Induced Small Airway Injury in a Rural Cohort.
Clinical pathology (Thousand Oaks, Ventura County, Calif.)Sequence of Rare Diagnoses in a Young Patient: Altitude Barotrauma Hemopneumothorax and Desquamative Interstitial Pneumonia.
Diagnostics (Basel, Switzerland)Imaging Features of Idiopathic Interstitial Lung Diseases.
Journal of thoracic imagingDesquamative Interstitial Pneumonia with Progressive Pulmonary Fibrosis.
Internal medicine (Tokyo, Japan)Inescapable Fibrosis: The Development of Desquamative Interstitial Pneumonia Post-Lung Transplantation Performed for a Patient with Idiopathic Pulmonary Fibrosis.
Case reports in transplantationDesquamative Interstitial Pneumonia.
The New England journal of medicineSmoking-Related Interstitial Lung Diseases.
Immunology and allergy clinics of North AmericaABCA3-related interstitial lung disease beyond infancy.
ThoraxSmoking-related interstitial lung disease.
RadiologiaDiffuse cystic lung diseases: Imaging spectrum and diagnostic approach using high-resolution computed tomography.
Lung India : official organ of Indian Chest SocietySmoking-Related Interstitial Fibrosis (SRIF) in Patients Presenting With Diffuse Parenchymal Lung Disease.
American journal of clinical pathologyImaging of Smoking and Vaping Related Diffuse Lung Injury.
Radiologic clinics of North AmericaSimultaneous pathological findings in biopsy specimens of patients with surgically resected lung carcinoids and their role in survival.
Oncology lettersIgG4-related lung disease with a desquamative interstitial pneumonia pattern radiologically and pathologically.
BMJ case reports[Smoking-related interstitial lung diseases].
Radiologie (Heidelberg, Germany)Desquamative interstitial pneumonia after tear gas exposure.
Respiratory medicine and researchSafety and accuracy of transbronchial lung cryobiopsy in diagnosing desquamative interstitial pneumonia.
The clinical respiratory journalUsual Interstitial Pneumonia is the Most Common Finding in Surgical Lung Biopsies from Patients with Persistent Interstitial Lung Disease Following Infection with SARS-CoV-2.
EClinicalMedicineIdiopathic desquamative interstitial pneumonia diagnosed using transbronchial lung cryobiopsy: A case report.
Respiratory medicine case reportsInterstitial Lung Disease in Systemic Sclerosis: A Single-center Retrospective Analysis.
Current rheumatology reviewsFibrotic Idiopathic Interstitial Lung Disease: The Molecular and Cellular Key Players.
International journal of molecular sciencesBiopsy in interstitial lung disease: specific diagnosis and the identification of the progressive fibrotic phenotype.
Current opinion in pulmonary medicineA Case of Pulmonary Adenocarcinoma Presenting with Diffuse Cystic Lesions.
Case reports in oncologyThe 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 biosciencesDiffuse interstitial pneumonia-like/macrophage activation syndrome-like changes in patients with COVID-19 correlate with length of illness.
Annals of diagnostic pathologyPathology, Radiology, and Genetics of Interstitial Lung Disease in Patients With Shortened Telomeres.
The American journal of surgical pathologyInterstitial lung abnormality (ILA) and nonspecific interstitial pneumonia (NSIP).
European journal of radiology openHigh-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 DiseaseTransbronchial lung cryobiopsy in smoking-related interstitial lung diseases.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGCOVID-19 mimics on chest CT: a pictorial review and radiologic guide.
The British journal of radiologyCOVID-19 Like Findings in a Fatal Case of Idiopathic Desquamative Interstitial Pneumonia Associated With IgA Glomerulonephritis in a 13-Month-Old Child.
Frontiers in pediatricsDesquamative interstitial pneumonia induced by metal exposure. A case report and literature review.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGFive simple reasons to discard DIP, or why we should stop calling dolphins big fish.
Journal of clinical pathologyThe interstitial lung disease spectrum under a uniform diagnostic algorithm: a retrospective study of 1,945 individuals.
Journal of thoracic diseaseInterstitial lung disease in dermatomyositis complicated by right ventricular thrombus secondary to macrophage activation syndrome- a case report.
AME case reportsLung Involvement in Primary Sjögren's Syndrome-An Under-Diagnosed Entity.
Frontiers in medicine"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 SocietyUnusual pneumoconiosis in two patients with heavy print toner, and paper dust exposure.
American journal of industrial medicineDesquamative interstitial pneumonia: still orphan and not always benign.
European respiratory review : an official journal of the European Respiratory SocietyDesquamative interstitial pneumonia: a systematic review of its features and outcomes.
European respiratory review : an official journal of the European Respiratory SocietyBiphasic malignant mesothelioma with epithelioid and sarcomatoid components (dedifferentiated mesothelioma) and intrapulmonary growth: a rare entity mimicking desquamative interstitial pneumonia.
Journal of clinical pathologyAluminium toxicosis: a review of toxic actions and effects.
Interdisciplinary toxicologyHigh 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[Diffuse Intraparenchymal Mesothelioma: An Atypical Presentation].
Acta medica portuguesaDesquamative interstitial pneumonia in a non-smoker with neurofibromatosis type 1 (Von Recklinghausen syndrome).
BMJ case reportsProbe-based confocal laser endomicroscopy in diagnosis of desquamative interstitial pneumonia in nonsmoker.
Monaldi archives for chest disease = Archivio Monaldi per le malattie del toraceA Stepwise Diagnostic Approach to Cystic Lung Diseases for Radiologists.
Korean journal of radiologyIncreased Pulmonary GM-CSF Causes Alveolar Macrophage Accumulation. Mechanistic Implications for Desquamative Interstitial Pneumonitis.
American journal of respiratory cell and molecular biologyKorean Guidelines for Diagnosis and Management of Interstitial Lung Diseases: Part 1. Introduction.
Tuberculosis and respiratory diseasesDesquamative interstitial pneumonia with clinical, radiological and histologic correlation.
Radiology case reportsDesquamative interstitial pneumonia: Risk factors, laboratory and bronchoalveolar lavage findings, radiological and histopathological examination, clinical features, treatment and prognosis.
Experimental and therapeutic medicineAnti-Ku antibody-positive desquamative interstitial pneumonia.
Respiratory medicine case reportsIdiopathic interstitial pneumonias: review of the latest American Thoracic Society/European Respiratory Society classification.
Radiologia brasileiraSuccessful lung transplantation in an HIV seropositive patient with desquamative interstitial pneumonia: a case report.
BMC pulmonary medicineIgG4-related disease presenting with combined pulmonary fibrosis and emphysema (CPFE).
Respiratory medicine case reportsA Review of Smoking-Related Interstitial Fibrosis, Respiratory Bronchiolitis, and Desquamative Interstitial Pneumonia: Overlapping Histology and Confusing Terminology.
Archives of pathology & laboratory medicineSmoking Relapse Causing an Acute Exacerbation of Desquamative Interstitial Pneumonia with Pleural Effusions and Mediastinal Adenopathies.
Case reports in pulmonologyPulmonary Adenocarcinoma Mimicking Desquamative Interstitial Pneumonia: Report of 2 Cases With Genetic Analysis.
International journal of surgical pathologyHermansky-Pudlak syndrome type 2 manifests with fibrosing lung disease early in childhood.
Orphanet journal of rare diseasesDesquamative interstitial pneumonia complicated by diffuse alveolar haemorrhage.
Respirology case reportsPulmonary Neuroendocrine Cell Hyperplasia Associated with Surfactant Protein C Gene Mutation.
Case reports in pulmonologySmoking-related lung abnormalities on computed tomography images: comparison with pathological findings.
Japanese journal of radiologyCurrent Concepts in Pathogenesis, Diagnosis, and Management of Smoking-Related Interstitial Lung Diseases.
ChestUnfavourable outcome of glucocorticoid treatment in suspected idiopathic pulmonary fibrosis.
Respirology (Carlton, Vic.)Overlaps and uncertainties of smoking-related idiopathic interstitial pneumonias.
International journal of chronic obstructive pulmonary disease[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 diseasesPulmonary Adenocarcinoma, Intra-alveolar Variant: A Rare Entity Mimicking Desquamative Interstitial Pneumonia.
International journal of surgical pathologyDesquamative Interstitial Pneumonia Complicated with IgG4-related Lung Disease.
Internal medicine (Tokyo, Japan)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 embryologieA case of mixed dust pneumoconiosis with desquamative interstitial pneumonia-like reaction in an aluminum welder.
Respiratory medicine case reportsHard metal lung disease: a case series.
Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e TisilogiaClinical-Radiologic-Pathologic Correlation of Smoking-Related Diffuse Parenchymal Lung Disease.
Radiologic clinics of North AmericaTransbronchial Cryobiopsy in Diffuse Parenchymal Lung Disease: Retrospective Analysis of 74 Cases.
Chest[Interstitial lung diseases associated with smoking].
Ceskoslovenska patologieCystic Lung Diseases: Algorithmic Approach.
ChestTransbronchial cryobiopsy in the diagnosis of desquamative interstitial pneumonia.
Revista portuguesa de pneumologiaMutually 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.
HistopathologySirolimus-Induced Diffuse Alveolar Hemorrhage: A Case Report.
American journal of therapeuticsAmerican 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, IncSmoking and interstitial lung diseases.
European respiratory review : an official journal of the European Respiratory SocietyNear fatal desquamative interstitial pneumonia with bilateral recurrent tension pneumothorax.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGUltrasound in Rheumatologic Interstitial Lung Disease: A Case Report of Nonspecific Interstitial Pneumonia in Rheumatoid Arthritis.
Case reports in rheumatologyPulmonary fibrosis and exposure to steel welding fume.
Occupational medicine (Oxford, England)Smoking-related idiopathic interstitial pneumonia: A review.
Respirology (Carlton, Vic.)Inflammatory Diseases of the Lung Induced by Conventional Cigarette Smoke: A Review.
ChestUsual interstitial pneumonia end-stage features from explants with radiologic and pathological correlations.
Annals of diagnostic pathologyInterstitial lung disease related to smoking: imaging considerations.
Current opinion in pulmonary medicine[The morphology and molecular bases of damage to the stem cell niche of respiratory acini in idiopathic interstitial pneumonias].
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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.
- Radiologic Pattern of Fibrosis in Combined Pulmonary Fibrosis and Emphysema: Impact on Disease Trajectories and Prognostic Outcomes.
- DIP-like Adenocarcinoma Presenting as a Part-Solid Nodule: A Case Report.
- Radiologic Approach to Cystic Lung Diseases: From Cyst Definition to Diagnosis.
- "Smoking Related Interstitial Lung Disease: Pattern Really Does Matter".
- Steroid refractory desquamative interstitial pneumonia responsive to intravenous cyclophosphamide.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:98852(Orphanet)
- OMIM OMIM:263000(OMIM)
- MONDO:0009887(MONDO)
- GARD:16864(GARD (NIH))
- Busca completa no PubMed(PubMed)
- 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.
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