A fibrose pulmonar idiopática (FPI) é uma pneumonia intersticial fibrosante crônica e progressiva de causa desconhecida, que ocorre principalmente em adultos mais velhos. Caracteriza-se pela piora progressiva da dispneia e da função pulmonar. A doença é limitada aos pulmões e está associada ao padrão histopatológico e/ou radiológico de pneumonia intersticial usual (PIU). A FPI tem prognóstico ruim, com sobrevida mediana de 3 a 5 anos a partir do diagnóstico. Os tratamentos aprovados incluem pirfenidona e nintedanib, que retardam a progressão da doença. O transplante pulmonar continua sendo a única intervenção que melhora a sobrevida.
Introdução
O que você precisa saber de cara
A fibrose pulmonar idiopática é uma doença pulmonar crônica e progressiva. Essa condição faz com que tecido cicatricial (fibrose) se acumule nos pulmões, o que impede que os pulmões transportem oxigênio para a corrente sanguínea de forma eficaz. A doença geralmente afeta pessoas entre 50 e 70 anos de idade. A fibrose pulmonar idiopática pertence a um grupo de condições chamadas doenças pulmonares intersticiais (também conhecidas como ILD), que descrevem doenças pulmonares que envolvem inflamação ou cicatrização no pulmão.
Os sinais e sintomas mais comuns da fibrose pulmonar idiopática são falta de ar e uma tosse seca e persistente, em forma de carranca. Muitas pessoas com a condição também apresentam perda de apetite e perda de peso gradual. Algumas pessoas com fibrose pulmonar idiopática desenvolvem pontas dos dedos das mãos e dos pés alargadas e arredondadas (clubbing) resultantes de deficiência de oxigênio. Essas características são relativamente inespecíficas; nem todas as pessoas com esses problemas de saúde têm fibrose pulmonar idiopática. Outras doenças respiratórias, algumas das quais são menos graves, podem causar sinais e sintomas semelhantes.
Nas pessoas com fibrose pulmonar idiopática, a cicatrização dos pulmões aumenta ao longo do tempo até que os pulmões não conseguem mais fornecer oxigênio suficiente aos órgãos e tecidos do corpo. Algumas pessoas com fibrose pulmonar idiopática desenvolvem outras condições pulmonares graves, incluindo câncer de pulmão, coágulos de sangue nos pulmões (êmbolos pulmonares), pneumonia ou pressão alta nos vasos sanguíneos que abastecem os pulmões (hipertensão pulmonar). A maioria das pessoas com a condição sobrevive de 3 a 5 anos após o diagnóstico. No entanto, o curso da doença é altamente variável; algumas pessoas com a condição ficam gravemente enfermas em poucos meses, enquanto outras podem viver com a doença por uma década ou mais.
Na maioria dos casos, a fibrose pulmonar idiopática ocorre em apenas uma pessoa em uma família. Esses casos são descritos como esporádicos. No entanto, uma pequena porcentagem de pessoas com essa doença tem pelo menos um outro membro da família afetado. Quando a fibrose pulmonar idiopática ocorre em vários membros da mesma família, é conhecida como fibrose pulmonar familiar.
Fonte: MedlinePlus Genetics (NLM/NIH), traduzidoA fibrose pulmonar idiopática (FPI) é uma pneumonia intersticial fibrosante crônica e progressiva de causa desconhecida, que ocorre principalmente em adultos mais velhos. Caracteriza-se pela piora progressiva da dispneia e da função pulmonar. A doença é limitada aos pulmões e está associada ao padrão histopatológico e/ou radiológico de pneumonia intersticial usual (PIU). A FPI tem prognóstico ruim, com sobrevida mediana de 3 a 5 anos a partir do diagnóstico. Os tratamentos aprovados incluem pirfenidona e nintedanib, que retardam a progressão da doença. O transplante pulmonar continua sendo a única intervenção que melhora a sobrevida.
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<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 20 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
14 genes identificados com associação a esta condição. Padrão de herança: Multigenic/multifactorial.
Cerebroretinal microangiopathy with calcifications and cysts 2
An autosomal recessive, multisystemic disorder characterized by intrauterine growth retardation and, later in life, premature aging symptoms, including poor growth, graying hair, liver fibrosis, portal hypertension, esophageal varices, osteopenia, pancytopenia, hypocellular bone marrow, and vascular telangiectasia resulting in gastrointestinal bleeding. Brain calcifications and white matter changes are responsible for signs including spasticity, ataxia, or dystonia observed in some patients.
Interstitial lung disease 2
A form of interstitial lung disease, a heterogeneous group of diseases affecting the distal part of the lung and characterized by a progressive remodeling of the alveolar interstitium. The disease spectrum ranges from idiopathic interstitial pneumonia or pneumonitis to idiopathic pulmonary fibrosis, that is associated with an increased risk of developing lung cancer. Clinical features of interstitial lung disease include dyspnea, clubbing of the fingers, and restrictive lung capacity. ILD2 inheritance is autosomal dominant.
Keratoderma, palmoplantar, striate 2
A dermatological disorder characterized by thickening of the skin on the palms (linear pattern) and the soles (island-like pattern) and flexor aspect of the fingers. Abnormalities of the nails, the teeth and the hair are rarely present.
Pulmonary surfactant metabolism dysfunction 2
A rare disease associated with progressive respiratory insufficiency and lung disease with a variable clinical course, due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.
Interstitial lung disease 1
A form of interstitial lung disease, a heterogeneous group of diseases affecting the distal part of the lung and characterized by a progressive remodeling of the alveolar interstitium. The disease spectrum ranges from idiopathic interstitial pneumonia or pneumonitis to idiopathic pulmonary fibrosis, that is associated with an increased risk of developing lung cancer. Clinical features of interstitial lung disease include dyspnea, clubbing of the fingers, and restrictive lung capacity. ILD1 inheritance can be autosomal dominant with incomplete penetrance, and autosomal recessive.
Interstitial lung disease 2
A form of interstitial lung disease, a heterogeneous group of diseases affecting the distal part of the lung and characterized by a progressive remodeling of the alveolar interstitium. The disease spectrum ranges from idiopathic interstitial pneumonia or pneumonitis to idiopathic pulmonary fibrosis, that is associated with an increased risk of developing lung cancer. Clinical features of interstitial lung disease include dyspnea, clubbing of the fingers, and restrictive lung capacity. ILD2 inheritance is autosomal dominant.
Dyskeratosis congenita, autosomal recessive, 5
A form of dyskeratosis congenita, a rare multisystem disorder caused by defective telomere maintenance. It is characterized by progressive bone marrow failure, and the clinical triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Common but variable features include premature graying, aplastic anemia, low platelets, osteoporosis, pulmonary fibrosis, and liver fibrosis among others. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy. DKCB5 is characterized by onset of bone marrow failure and immunodeficiency in early childhood. Most patients also have growth and developmental delay and cerebellar hypoplasia, consistent with a clinical diagnosis of Hoyeraal-Hreidarsson syndrome.
Deafness, autosomal dominant, 84
A form of non-syndromic, sensorineural hearing loss. Sensorineural hearing loss results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information. DFNA84 is characterized by slowly progressive, postlingual hearing loss.
Dyskeratosis congenita, autosomal recessive, 6
A form of dyskeratosis congenita, a rare multisystem disorder caused by defective telomere maintenance. It is characterized by progressive bone marrow failure, and the clinical triad of reticulated skin hyperpigmentation, nail dystrophy, and mucosal leukoplakia. Common but variable features include premature graying, aplastic anemia, low platelets, osteoporosis, pulmonary fibrosis, and liver fibrosis among others. Early mortality is often associated with bone marrow failure, infections, fatal pulmonary complications, or malignancy.
Pulmonary surfactant metabolism dysfunction 3
A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.
Hatipoglu immunodeficiency syndrome
An autosomal recessive immunologic disorder manifesting in infancy or early childhood, and characterized by failure to thrive, short stature, skin pigmentation abnormalities, pancytopenia, and susceptibility to recurrent infections.
Variantes genéticas (ClinVar)
302 variantes patogênicas registradas no ClinVar.
Classificação de variantes (ClinVar)
Distribuição de 2.730 variantes classificadas pelo ClinVar.
Vias biológicas (Reactome)
38 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
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Publicações mais relevantes
Mostrando amostra de 200 publicações de um total de 11.079
Correction: Integrated bioinformatics-based identification of proliferative diabetic retinopathy and idiopathic pulmonary fibrosis: Focus on fibrosis and immune infiltration.
Este documento é uma correção a um artigo científico anterior que investigou, por meio de bioinformática, características da Fibrose Pulmonar Idiopática (FPI) e Retinopatia Diabética Proliferativa, com foco nos mecanismos de fibrose e infiltração imune. Para pacientes e médicos, esta correção significa que houve uma atualização de informações importantes sobre os achados relacionados a essas doenças, sendo essencial consultar a versão corrigida para ter acesso aos dados mais precisos.
🇧🇷 traduzidoGEN-KnowRD: Reframing AI for Rare Disease Recognition.
Doenças raras, como a fibrose pulmonar idiopática (FPI), sofrem com longos atrasos diagnósticos que impedem intervenções precoces. O GEN-KnowRD é um novo sistema de inteligência artificial que utiliza modelos de linguagem avançados para gerar e organizar de forma escalável conhecimento sobre essas doenças, em vez de usá-los para diagnóstico direto. Essa abordagem demonstrou uma melhora robusta na identificação precoce da FPI e de outras doenças raras, prometendo diagnósticos mais rápidos e precisos para pacientes e permitindo aos médicos iniciar tratamentos de forma mais oportuna.
🇧🇷 traduzidoGene expression and metadata based identification of key genes for lung cancer, COPD, and IPF using machine learning and statistical models.
Este estudo usou abordagens de machine learning e bioinformática para identificar genes-chave em doenças pulmonares, incluindo fibrose pulmonar idiopática (FPI), DPOC e câncer de pulmão. A pesquisa destacou quatro genes candidatos principais (ETS1, MSH2, RORA e PMAIP1) que podem ser alvos terapêuticos. Estes achados oferecem uma base sólida para o desenvolvimento de novas estratégias de tratamento e potenciais medicamentos direcionados a esses genes, prometendo novas avenidas para a prática médica e para os pacientes.
🇧🇷 traduzidoDeep-learning-based de novo discovery and design of therapeutics that reverse disease-associated transcriptional phenotypes.
Este estudo utiliza inteligência artificial (deep learning) para descobrir e otimizar novos medicamentos, focando em reverter as características genéticas associadas a doenças. Na Fibrose Pulmonar Idiopática (FPI), a plataforma identificou um novo composto antifibrótico e um candidato a fármaco para reposicionamento. Ambos atuam corrigindo a expressão genética anormal em diferentes tipos de células pulmonares, oferecendo uma abordagem promissora e precisa para o desenvolvimento de tratamentos.
🇧🇷 traduzidoExtent of alveolar collapse in expiratory CT as a prognostic marker in idiopathic pulmonary fibrosis.
Este estudo investigou se a análise de tomografias computadorizadas (TCs) realizadas na inspiração e expiração, avaliando o colapso alveolar, pode prever a evolução de pacientes com fibrose pulmonar idiopática (FPI). Os resultados mostraram que uma medida específica da TC (chamada 'skewness'), obtida na expiração, teve um desempenho preditivo superior para identificar o risco de morte ou transplante pulmonar em 3 anos, comparado à capacidade vital forçada (CVF%) isolada. Isso sugere que a análise pareada da TC em inspiração/expiração oferece uma abordagem promissora para melhorar a previsão do prognóstico e avaliar o colapso alveolar na FPI, potencialmente auxiliando médicos e pacientes na tomada de decisões mais informadas.
🇧🇷 traduzidoPublicações recentes
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Psychometric properties of health-related quality of life assessment instruments in idiopathic pulmonary fibrosis: a systematic review applying COSMIN methodology.
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American journal of clinical and experimental immunologyIdentification of Glycosylation-Related Biomarkers in COPD and IPF Through Integrated Machine Learning and WGCNA Analysis.
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medRxiv : the preprint server for health sciencesTranscriptomic analysis reveals lipid metabolism and macrophage involvement associated with nintedanib treatment in a rat bleomycin model.
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Annals of the American Thoracic Society[Analysis of external respiratory function disturbances in combined pulmonary fibrosis and emphysema].
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The European respiratory journalIdentifying prioritization of potential targets for idiopathic pulmonary fibrosis: Proteome-wide Mendelian randomization and colocalization analyses.
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ERJ open researchThe COCOS-IPF core outcome set for idiopathic pulmonary fibrosis/progressive pulmonary fibrosis care: a mixed-method multi-perspective approach.
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American journal of industrial medicineExtent of alveolar collapse in expiratory CT as a prognostic marker in idiopathic pulmonary fibrosis.
PloS oneCAR T cell therapy for fighting IPF: perspectives on a living drug.
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The Indian journal of tuberculosisTGF-β Signaling as a Pathological Continuum Linking Idiopathic Pulmonary Fibrosis and Lung Cancer.
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F1000ResearchFrailty and functional outcomes in patients with progressive fibrosing interstitial lung diseases receiving antifibrotic therapy: a real-life observational study.
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Health science reportsEstrogen upregulates NR4A1 to counter TGF beta induced pulmonary fibrosis therapeutic insights for IPF.
iScienceIntegrated network toxicology and multi-omics analysis reveals the role of tobacco-specific carcinogen NNK in idiopathic pulmonary fibrosis pathogenesis.
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Frontiers in immunologyViral Pathogens and Pulmonary Fibrosis: EMT-Driven Mechanisms and Insights From Traditional Chinese Medicine.
Reviews in medical virologyInhalation-Based Nanoparticle Drug Delivery Targeting the Diseased Lower Airways in Idiopathic Pulmonary Fibrosis.
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Environmental pollution (Barking, Essex : 1987)Nerandomilast: First Approval.
DrugsVisualization analysis of the use of traditional Chinese medicine in the diagnosis and treatment of rare diseases in mainland China based on CiteSpace.
Intractable & rare diseases researchTargeting fused in sarcoma (FUS): a novel antisense strategy for treating idiopathic pulmonary fibrosis.
Signal transduction and targeted therapyMonoallelic PARN mutation presenting as pancytopenia, hepatic fibrosis and idiopathic pulmonary fibrosis.
BMJ case reportsLysophosphatidylcholine promotes pulmonary fibrosis following lung injury by facilitating alveolar type 2 cell senescence via Mfsd2a-dependent, Drp1-mediated mitochondrial fission.
Free radical biology & medicineA FGFR3 decoy receptor attenuates lung fibroblast-to-myofibroblast transition and pulmonary fibrosis.
American journal of respiratory cell and molecular biologyMCT4 mediates hypoxia-induced extracellular lactate release from IPF fibroblasts.
American journal of respiratory cell and molecular biologyIdentification of a conserved sequence of disease progression in Idiopathic Pulmonary Fibrosis.
American journal of respiratory and critical care medicineNerandomilast in idiopathic pulmonary fibrosis: data from the whole follow-up period of the FIBRONEER-IPF trial.
American journal of respiratory and critical care medicineSex-related transcriptomic differences in idiopathic pulmonary fibrosis.
American journal of respiratory and critical care medicineTransient receptor potential vanilloid 4 (TRPV4) channels mediate pulmonary surfactant protein A and D secretion.
American journal of respiratory cell and molecular biologyMolecular endotypes predict differential response to immunosuppressant therapy in non-IPF interstitial lung disease.
American journal of respiratory and critical care medicineSpatial Transcriptomics Uncovers Hybrid, Pro-Inflammatory and Pro-Fibrotic Cellular Niches in Pulmonary Granuloma of Patients with Chronic Sarcoidosis.
American journal of respiratory and critical care medicineHospitalisations in patients with idiopathic pulmonary fibrosis: insights from the IPF-PRO Registry and EMPIRE Registry.
ERJ open researchHealthcare professionals' perspectives on implementation of home monitoring in individuals with pulmonary fibrosis.
ERJ open researchHospitalisations and idiopathic pulmonary fibrosis: lessons from two continents.
ERJ open researchEmerging Therapies in Pulmonary Fibrosis.
Pulmonary therapyBreaking barriers in lung research with engineered airway epithelial cell culture models.
Current opinion in pulmonary medicineDecoding tamoxifen on idiopathic pulmonary fibrosis: integrating network toxicology and multi-omics.
International journal of surgery (London, England)Correlation between red cell distribution width to total calcium ratio and in-hospital mortality in patients with non-idiopathic pulmonary fibrosis interstitial lung diseases: A retrospective cohort study from the MIMIC-IV database.
MedicineIntegrated bioinformatics-based identification of proliferative diabetic retinopathy and idiopathic pulmonary fibrosis: Focus on fibrosis and immune infiltration.
PloS oneESRP1 drives epithelial-mesenchymal transition by activating EPAC-RAP1A signaling axis.
Frontiers in medicineShortened Telomere Length as a Risk Factor for Idiopathic Pulmonary Fibrosis: A Meta-Analysis.
The open respiratory medicine journalAntifibrotic Strategies Targeting Phosphodiesterase-4 in Idiopathic Pulmonary Fibrosis: Molecular Mechanisms and Clinical Translation.
Clinical pharmacology : advances and applicationsParathyroid hormone-related protein is a therapeutic target in idiopathic pulmonary fibrosis.
Signal transduction and targeted therapyImmune Dysregulation: Navigating Autoimmune Disorders in Exotic Companion Animals.
The veterinary clinics of North America. Exotic animal practiceUnraveling miRNA-mediated TGF-β/hedgehog Signaling in bleomycin-induced pulmonary fibrosis: targeting inflammation-fibrosis crosstalk with candesartan and chlorogenic acid.
Bioorganic chemistryMacrophage erythropoietin receptor signaling drives macrophage-myofibroblast transition via JAK2/STAT3 and TGF-β1/Smad3 pathways to exacerbate pulmonary fibrosis in mice.
Biochemical and biophysical research communicationsSafety and Tolerability of Nintedanib in Japanese Patients with Progressive Fibrosing Interstitial Lung Diseases: Final Results of 2-Year Post-Marketing Surveillance.
Advances in therapyA Journey through Scaffolds: Indolines, Pyrrolidines, and Azetidines in the Quest for Inhaled DDR Inhibitors for IPF.
Journal of medicinal chemistryRationale and design of uLtrasOund applicability in the assessment of patients with fibRosing interstitial lung Disease (LORD): a research protocol of a prospective cohort study.
BMJ openTranscriptomic signatures of IPF in ALI-cultured airway cells and their therapeutic implications.
ThoraxOptimization and aerodynamic performance of nebulizable pirfenidone-loaded human serum albumin nanoparticles for targeted pulmonary delivery.
European journal of pharmaceutical sciences : official journal of the European Federation for Pharmaceutical SciencesRadiologic Features of Airways Disease Are Associated with Interstitial Lung Abnormality Progression.
Respiratory careLung Transplantation: Highlights From the World Transplant Congress.
TransplantationRIPK3 Orchestrates Scar-Associated Macrophage Dysfunction to Drive Pulmonary Fibrosis.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)Mechanistic basis for the antifibrotic actions of cAMP-based therapies.
European respiratory review : an official journal of the European Respiratory SocietyChaperone-mediated autophagy supports organ regeneration and fibroblast quiescence in mouse models of fibrosis.
Science translational medicinePrognostic biomarkers for idiopathic pulmonary fibrosis: findings from ISABELA clinical trials.
ERJ open researchHigh density lipoproteins, disease severity and clinical outcomes in patients with idiopathic pulmonary fibrosis.
Respiratory researchPredicting Postoperative Complications in Patients With Lung Cancer and Idiopathic Pulmonary Fibrosis.
Respirology (Carlton, Vic.)Mechanism of macrophage mitochondrial transfer in CBNPs induced EndMT of pulmonary microvascular endothelial cells.
Journal of hazardous materialsGeospatial and temporal trends of interstitial lung disease subtypes and KL-6 biomarker in China: a nationwide study (2016-2024).
Respiratory researchSex Differences in IPF: Data from the German INSIGHTS Registry.
Respiration; international review of thoracic diseasesProphylactic quercetin administration attenuates pulmonary fibrosis via ferroptosis-resistant priming of alveolar epithelial cells.
Redox report : communications in free radical researchIntegrated Bioinformatics Analysis and Machine Learning to Identify Biomarkers and Fibroblast-related Features of Idiopathic Pulmonary Fibrosis.
Current medicinal chemistryChronic IL-21 exposure reshapes pulmonary environment, elevating risk of respiratory diseases.
Cellular and molecular life sciences : CMLSRisk factors, clinical features, and impact on survival of lung cancer in patients with idiopathic pulmonary fibrosis: A retrospective study.
MedicineThe impact of physical activity on people with idiopathic pulmonary fibrosis and the associated experience - a mixed methods structured review.
Irish journal of medical sciencePirfenidone-Induced Lichen Planus Pigmentosus in a Patient with Idiopathic Pulmonary Fibrosis.
Indian dermatology online journalOrcinol glucoside ameliorates pulmonary fibrosis by suppressing hyaluronic acid synthesis and macrophage M2 polarization via targeting hyaluronic acid synthase 2.
International journal of molecular medicineFactor XII-A New Therapeutic Target? A Systematic Review.
International journal of molecular sciencesCirculating Activin A and Follistatin-like Proteins in Rheumatoid Arthritis with Interstitial Lung Disease: A Cross-Sectional Comparative Study.
Diagnostics (Basel, Switzerland)SPECT as a translational non-invasive method to assess occupancy of the tissue transglutaminase-2 by zampilimab in the mouse lung.
Respiratory researchHuman iPSC-based Modeling of Pulmonary Fibrosis Reveals p300/CBP Inhibition Suppresses Alveolar Transitional Cell State.
Nature communicationsAbrogation of PDE7A ameliorates alveolar epithelial EMT-Driven pulmonary fibrosis.
Biochemical pharmacologyModeling cell-cell interactions to advance drug discovery in Idiopathic Pulmonary Fibrosis.
bioRxiv : the preprint server for biologyAssociations between leisure sedentary behaviors, physical activity, and chronic respiratory diseases: a Mendelian randomization study.
Archives of medical science : AMSClinical profiles and risk predictors of drug-induced liver injury from pirfenidone and nintedanib used to treat idiopathic pulmonary fibrosis: a real-world analysis of a tertiary Chinese hospital cohort combined with FAERS data mining.
Translational gastroenterology and hepatologyMolecular Mechanisms of Pulmonary Fibrosis: The Interaction of Epithelial-mesenchymal Transition and AMPK Pathways in a Bleomycin-induced Model.
Iranian journal of allergy, asthma, and immunologyAngiotensin-Converting Enzyme Inhibitor Use in Idiopathic Pulmonary Fibrosis: The Overlooked Roles of Contraindications, Transplantation, and Angiotensin Receptor Blockers.
ChestAngiotensin-Converting Enzyme Inhibitors in Idiopathic Pulmonary Fibrosis: Bias, Confounding, and the Path Forward.
ChestAssessment of mosaic loss of chromosome Y in pulmonary fibrosis reveals limited association with susceptibility or disease severity.
BMJ open respiratory researchUnraveling the matrix stiffness landscape in idiopathic pulmonary fibrosis: GSN and ARG1 as novel diagnostic biomarkers and potential therapeutic targets.
International immunopharmacologyInactivation Rap2a in Endothelial Cell Prevents Pulmonary Fibrosis by Regulating Immune Microenvironment Through MAP4K4-VCAM1 Signaling.
Advanced science (Weinheim, Baden-Wurttemberg, Germany)A lung-targeted self-assembled supramolecular nanofiber for synergistic therapy of idiopathic pulmonary fibrosis.
NanoscaleCold inducible RNA binding protein promotes fibroblast activation and its inhibition represents a potential therapeutic target in pulmonary fibrosis.
Scientific reportsIdentification and validation of the protective gene BCAT2 related to amino acid metabolism in idiopathic pulmonary fibrosis.
Clinical and experimental medicineIsoforskolin inhibits LUBAC/GSDMD/IL-1β cascades in pulmonary fibrosis.
Journal of thoracic diseaseA seven-gene panel with diagnostic and prognostic value for idiopathic pulmonary fibrosis and candidate herbal drugs identified based on these genes.
International journal of clinical and experimental pathologyThe impact of vertebral fractures on pulmonary function tests in patients with interstitial lung disease: a cross-sectional study.
Respiratory researchEngineered levothyroxine dry powder for inhalation to treat idiopathic pulmonary fibrosis.
Drug delivery and translational researchA multidisciplinary diagnostic approach to pulmonary fibrosis in dogs and cats.
Journal of veterinary diagnostic investigation : official publication of the American Association of Veterinary Laboratory Diagnosticians, IncDo megakaryocytes contribute to organ fibrosis?
QJM : monthly journal of the Association of PhysiciansAge-dependent Genetic Risk in Pulmonary Fibrosis Patients and Relatives.
medRxiv : the preprint server for health sciencesRespiratory oscillometry: the new kid on the lung function block.
BMJ open respiratory researchAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
A ABRAF é uma organização sem fins lucrativos dedicada ao suporte de pacientes que vivem com doenças respiratórias e cardíacas. Oferece grupos de apoio, linha de atendimento, conferências educativas e advocacia por políticas públicas de saúde.
Organização internacional sem fins lucrativos (sede nos EUA) dedicada às síndromes de telômero curto — Disqueratose Congênita, Hoyeraal-Hreidarsson e telomeropatias relacionadas. Mantém um registro mundial de famílias afetadas, conecta pacientes a estudos clínicos e financia pesquisa. Atende famílias em qualquer país, inclusive Brasil.
Centro oncológico de referência nacional com equipe de oncogenética que investiga e acompanha famílias com síndromes hereditárias de predisposição ao câncer, incluindo telomeropatias (TERT, TERC, DKC1, RTEL1, etc.). Realiza diagnóstico molecular e aconselhamento genético familiar.
Centro de referência em doenças raras e terapia gênica no interior de SP.
Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Fibrose pulmonar idiopática e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
É uma pneumonia intersticial crônica, progressiva e de causa desconhecida que provoca cicatrização no tecido pulmonar em adultos. Ela restringe a capacidade dos pulmões de transferir oxigênio adequadamente para a corrente sanguínea.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
10 publicações do grafo RarasNet (PubMed) · 6 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
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.
Citar este verbete
Raras. (s.d.). Fibrose pulmonar idiopática. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/fibrose-pulmonar-idiopatica
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
Fibrose pulmonar idiopática
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Alvos terapêuticos
- fonte: OpenTargets
- Reposicionamento
- fonte: Drug Repurposing Hub
- Ensaios clínicos
- fonte: ClinicalTrials.gov
