A Proteinose Alveolar Pulmonar (PAP) é uma doença rara do pulmão, caracterizada pelo acúmulo de uma substância gordurosa e proteica nas pequenas bolsas de ar mais profundas dos pulmões, que reage positivamente a um teste de coloração chamado ácido periódico de Schiff (PAS).
Introdução
O que você precisa saber de cara
A Proteinose Alveolar Pulmonar (PAP) é uma doença rara do pulmão, caracterizada pelo acúmulo de uma substância gordurosa e proteica nas pequenas bolsas de ar mais profundas dos pulmões, que reage positivamente a um teste de coloração chamado ácido periódico de Schiff (PAS).
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Entender a doença
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 16 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 28 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
1 gene identificado com associação a esta condição. Padrão de herança: Multigenic/multifactorial, Not applicable.
A beta chain of antigen-presenting major histocompatibility complex class II (MHCII) molecule. In complex with the alpha chain HLA-DRA, displays antigenic peptides on professional antigen presenting cells (APCs) for recognition by alpha-beta T cell receptor (TCR) on HLA-DRB1-restricted CD4-positive T cells. This guides antigen-specific T-helper effector functions, both antibody-mediated immune response and macrophage activation, to ultimately eliminate the infectious agents and transformed cells
Cell membraneEndoplasmic reticulum membraneLysosome membraneLate endosome membraneAutolysosome membrane
Variantes genéticas (ClinVar)
28 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
7 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
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
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Ensaios em destaque
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1 pesquisa recrutando participantes. Converse com seu médico sobre a possibilidade de participar.
Outros ensaios clínicos
16 ensaios clínicos encontrados, 4 ativos.
Publicações mais relevantes
Lipid nanoparticle GM-CSF replacement for autoimmune pulmonary alveolar proteinosis.
Granulocyte-macrophage colony-stimulating factor (GM-CSF) deficiency drives autoimmune pulmonary alveolar proteinosis (aPAP), a disease characterized by impaired macrophage-mediated clearance of pulmonary surfactants. Clinical data suggest that inhaled recombinant GM-CSF reduces symptoms in aPAP patients, providing a rationale for mRNA-based GM-CSF replacement therapies. However, these require effective mRNA delivery after nebulization. Here, we report the iterative in vivo design of a lipid nanoparticle, named nebulized lung delivery 2 (NLD2), that efficiently delivers mRNA after nebulization. NLD2 carrying GM-CSF mRNA transfected alveolar macrophages in vivo, leading to interleukin-10 pathway activation and subsequent surfactant lipoprotein clearance. In a preclinical disease model of aPAP, GM-CSF mRNA delivery reduced surfactant protein thickness more than recombinant GM-CSF. These data support continued exploration of nebulized lipid nanoparticle therapies for aPAP.
A long-term observational study on autoimmune pulmonary alveolar proteinosis revealed a sustained and generalized decrease in serum autoantibody levels.
Timing of Whole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis with Concurrent Opportunistic Infection: A Case Report and Systematic Review.
Autoimmune pulmonary alveolar proteinosis (aPAP) is a rare lung disorder characterized by abnormal accumulation of surfactant in the alveoli, often leading to progressive respiratory failure. While whole lung lavage (WLL) remains the standard therapeutic intervention, its timing and safety in the context of active infection remain a critical clinical dilemma. We present a fatal case of a 42-year-old Tibetan female with aPAP and progressive respiratory failure requiring V-V ECMO. Critically, serological tests were negative, but targeted next-generation sequencing (tNGS) of bronchoalveolar lavage fluid revealed a dominant mixed opportunistic infection (Cryptococcus neoformans: 3,877 reads; Enterococcus faecium: 118,062 reads). Despite this finding, the patient underwent emergent WLL and subsequently succumbed to the uncontrolled infection. To contextualize our findings, we conducted a systematic literature search in PubMed and Embase from their inception to November 21, 2025, which identified 27 relevant case reports and series. This review demonstrated that the majority of WLL procedures were performed after the active infection was controlled or had resolved following the initiation of antimicrobial therapy. The outcome was generally favorable, with clinical improvement reported in 23 cases (85.2%). This fatal aPAP case suggests a staged management approach whereby WLL should be deferred until infection control is achieved. Early tNGS-guided pathogen identification appears crucial for directing antimicrobial therapy. This target-then-lavage strategy merits further validation but offers a prudent framework for managing these high-risk patients.
Clinical characterization of 39 patients with autoimmune pulmonary alveolar proteinosis.
ObjectivesWe aimed to explore the clinical manifestations, examination findings, diagnostic methods, and treatment efficacy of patients with pulmonary alveolar proteinosis and improve clinicians' understanding of this disease.MethodsWe retrospectively analyzed the clinical characteristics of 39 patients diagnosed with autoimmune pulmonary alveolar proteinosis admitted to Fuzhou Pulmonary Hospital, Fujian Province, from August 2013 to December 2024. We also analyzed the correlations between lactate dehydrogenase, arterial partial oxygen pressure, and pulmonary alveolar arterial oxygen differential pressure, lung function, tumor markers, blood lipids, and disease severity. Furthermore, the relevant literature was reviewed.ResultsOf the 39 patients, 23 were male, 16 were female, 18 were smokers, and 11 had pneumoconiosis. The main clinical symptoms were cough and shortness of breath. The typical manifestations of chest computed tomography were "map-like" changes or "crazy-paving pattern". The main manifestations of pulmonary function were diffusion dysfunction and restricted ventilation dysfunction. pulmonary alveolar proteinosis disease severity score was positively correlated with lactate dehydrogenase, pulmonary alveolar arterial oxygen differential pressure and the tumor markers carcinoembryonic antigen, cytokeratin 19 fragment antigen 21-1, neuron specific enolase, and carbohydrate antigen 153 (r = 0.689, P < 0.01; r = 0.801, P < 0.01; r = 0.414, P < 0.05; r = 0.570, P < 0.01; r = 0.488, P < 0.01; and r = 0.415, P < 0.05, respectively). The disease severity score was negatively correlated with the percentage of arterial partial oxygen pressure and carbon monoxide dispersion to the predicted value (r = -0.952, r = -0.554, P < 0.01). Lactate dehydrogenase was negatively correlated with carbon monoxide dispersion to the predicted value (r = -0.522, P < 0.01). Arterial partial oxygen pressure was negatively correlated with carcinoembryonic antigen, cytokeratin 19 fragment antigen 21-1, neuron specific enolase, and carbohydrate antigen 153 (r = -0.479, r = -0.607, r = -0.598, and r = -0.425, respectively, P < 0.01). In contrast, arterial partial oxygen pressure was positively correlated with carbon monoxide dispersion to the predicted value (r = 0.616, P < 0.01). pulmonary alveolar arterial oxygen differential pressure was positively correlated with carcinoembryonic antigen, cytokeratin 19 fragment antigen 21-1, neuron specific enolase, and carbohydrate antigen 153 (r = 0.529, P < 0.01; r = 0.546, P < 0.01; r = 0.554, P < 0.01; and r = 0.348, P < 0.05, respectively). Pulmonary alveolar arterial oxygen differential pressure was negatively correlated with carbon monoxide dispersion to the predicted value (r = -0.641, P < 0.01).ConclusionPulmonary alveolar proteinosis is a rare diffuse lung disease without specific clinical manifestations. Whole lung high-volume alveolar lavage is the most effective treatment for pulmonary alveolar proteinosis. Arterial partial oxygen pressure, pulmonary alveolar arterial oxygen differential pressure, lactate dehydrogenase, tumor markers, and carbon monoxide dispersion to the predicted value were correlated with the severity of pulmonary alveolar proteinosis.
A Case of Autoimmune Pulmonary Alveolar Proteinosis.
Chemotherapeutic agents or regular doses of Rituximab may represent a potential therapeutic option for refractory cases of autoimmune pulmonary alveolar proteinosis.
Publicações recentes
The burden of autoimmune pulmonary alveolar proteinosis: a systematic review.
Delayed Microbiological Diagnosis and Fluoroquinolone-Associated Tendon Rupture in Pulmonary Nocardiosis Complicating Autoimmune Pulmonary Alveolar Proteinosis: A Case Report.
Timing of Whole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis with Concurrent Opportunistic Infection: A Case Report and Systematic Review.
A long-term observational study on autoimmune pulmonary alveolar proteinosis revealed a sustained and generalized decrease in serum autoantibody levels.
📖 RevisãoClinical characterization of 39 patients with autoimmune pulmonary alveolar proteinosis.
📚 EuropePMC144 artigos no totalmostrando 154
Timing of Whole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis with Concurrent Opportunistic Infection: A Case Report and Systematic Review.
Infection and drug resistanceA long-term observational study on autoimmune pulmonary alveolar proteinosis revealed a sustained and generalized decrease in serum autoantibody levels.
Orphanet journal of rare diseasesClinical characterization of 39 patients with autoimmune pulmonary alveolar proteinosis.
The Journal of international medical researchEfficacy of recombinant human GM-CSF compared to placebo for autoimmune pulmonary alveolar proteinosis: a systematic review and meta-analysis.
BMC pulmonary medicineRare case of autoimmune pulmonary alveolar proteinosis with ANCA positivity.
BMC pulmonary medicineA Case of Autoimmune Pulmonary Alveolar Proteinosis.
Clinical case reportsSignificant but Temporary Efficacy of Statin for a Patient With Severe Autoimmune Pulmonary Alveolar Proteinosis: A Case Report.
Respirology case reportsLipid nanoparticle GM-CSF replacement for autoimmune pulmonary alveolar proteinosis.
Proceedings of the National Academy of Sciences of the United States of AmericaExtracellular DNA in bronchoalveolar lavage fluid as a candidate biomarker of disease severity in autoimmune pulmonary alveolar proteinosis.
Respiratory investigationAutoimmune Pulmonary Alveolar Proteinosis Triggered by Salazosulfapyridine in a Patient With Rheumatoid Arthritis: A Case Report.
CureusA New, Rapid Way to Test for Autoimmune Pulmonary Alveolar Proteinosis.
Respirology (Carlton, Vic.)Autoimmune Pulmonary Alveolar Proteinosis.
Seminars in respiratory and critical care medicineRapid Serological Detection of Anti-GM-CSF Autoantibodies in Autoimmune Pulmonary Alveolar Proteinosis Using a Novel Immunochromatographic Test.
Respirology (Carlton, Vic.)Recombinant GM-CSF drug evaluation review.
ImmunotherapyA Rare Case of Pulmonary Alveolar Proteinosis.
CureusSerial Anti-GM-CSF Autoantibody Levels Reflect Disease Activity in Hypersensitivity Pneumonitis with Autoimmune Pulmonary Alveolar Proteinosis: Case Report.
Pathophysiology : the official journal of the International Society for PathophysiologyPharmacokinetics and pharmacodynamics of inhaled molgramostim in healthy people.
BMJ open respiratory researchTurbidity of whole lung lavage fluid in patients with autoimmune pulmonary alveolar proteinosis: Association between turbidity and pulmonary function.
Respiratory investigationHyaluronan in the pathogenesis of lung fibrosis associated to autoimmune pulmonary alveolar proteinosis (aPAP).
Frontiers in medicinePharmacotherapy for Autoimmune Pulmonary Alveolar Proteinosis.
DrugsGM-CSF in Autoimmune Pulmonary Alveolar Proteinosis.
The New England journal of medicinePhase 3 Trial of Inhaled Molgramostim in Autoimmune Pulmonary Alveolar Proteinosis.
The New England journal of medicineDyspnea and Deception: Overcoming Diagnostic Hurdles in Pulmonary Alveolar Proteinosis.
CureusThe Novel Use of Daratumumab in the Treatment of Refractory Autoimmune Pulmonary Alveolar Proteinosis.
Respirology case reportsCorrigendum: Exogenous GM-CSF therapy for autoimmune pulmonary alveolar proteinosis: a systematic literature review.
Frontiers in medicineSpontaneous Resolution in Autoimmune Pulmonary Alveolar Proteinosis: A Case Series.
ChestExogenous GM-CSF therapy for autoimmune pulmonary alveolar proteinosis: a systematic literature review.
Frontiers in medicineA Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Developing During the Course of Eosinophilic Granulomatosis With Polyangiitis.
Respirology case reportsA New Era in the Treatment of Autoimmune Pulmonary Alveolar Proteinosis.
Respirology (Carlton, Vic.)Anti-GM-CSF autoantibodies in myeloid leukemias.
Best practice & research. Clinical haematologyTherapeutic Whole Lung Lavage for Pulmonary Alveolar Proteinosis: Technique With Continuous Video-Enabled Double Lumen Endotracheal Tube.
Respirology case reportsAutoimmune Pulmonary Alveolar Proteinosis: A Rare Diagnosis in Pediatric Age.
Journal of investigative medicine high impact case reportsPosition-Dependent Lung Shadow Movement.
Respirology case reportsA patient journey map for people living with autoimmune pulmonary alveolar proteinosis.
Respiratory medicineLong-term outcomes in five patients with autoimmune pulmonary alveolar proteinosis treated with molgramostim inhalation solution.
ERJ open researchAutoimmune pulmonary alveolar proteinosis developed during treatment for systemic sclerosis: a case report.
BMC pulmonary medicineSuccessful granulocyte-macrophage colony-stimulating factor inhalation therapy for recurrent autoimmune pulmonary alveolar proteinosis after lung transplantation: A case report.
Respiratory investigationRituximab Therapy in Pulmonary Alveolar Proteinosis: A Case Report.
CureusPulmonary fibrosis in patients with autoimmune pulmonary alveolar proteinosis: a retrospective nationwide cohort study.
ERJ open researchSilicosis complicated with autoimmune pulmonary alveolar proteinosis caused by long-term dust inhalation during construction of bridge pier columns: A case report.
HeliyonSeverity and prognosis of COVID-19 complicated by autoimmune pulmonary alveolar proteinosis.
Respiratory investigationPulmonary hypertension during high-dose GM-CSF therapy of autoimmune pulmonary alveolar proteinosis.
Pulmonary circulationWhole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis: Unique Challenges in a Resource-Limited Setting.
Clinical case reportsRepeated inhalation of GM-CSF by nonhuman primates induces bronchus-associated lymphoid tissue along the lower respiratory tract.
Respiratory researchAutoimmune pulmonary alveolar proteinosis presenting as localized multifocal GGOs: A case report.
Radiology case reportsInhaled molgramostim therapy for the treatment of autoimmune pulmonary alveolar proteinosis (aPAP): a plain language summary of the IMPALA trial.
Hospital practice (1995)Nocardia Infection in Patients With Anti-Granulocyte-Macrophage Colony-Stimulating Factor Autoantibodies: A Prospective Multicenter French Study.
Open forum infectious diseasesAutoimmune pulmonary alveolar proteinosis during the treatment of nonspecific interstitial pneumonia complicated by clinically amyopathic dermatomyositis: A case report.
Respirology case reportsA case of autoimmune pulmonary alveolar proteinosis responding to oral statin therapy.
Respiratory medicine case reportsCytokine profiles associated with disease severity and prognosis of autoimmune pulmonary alveolar proteinosis.
Respiratory investigationA case of autoimmune pulmonary alveolar proteinosis during the course of treatment of rapidly progressive interstitial pneumonia associated with anti-MDA5 antibody-positive dermatomyositis.
BMC pulmonary medicineA Case of Autoimmune Pulmonary Alveolar Proteinosis With Predominantly Peripheral Opacities Diagnosed by Transbronchial Lung Biopsy.
CureusOptical Biopsies Using Probe-Based Confocal Laser Endomicroscopy for Autoimmune Pulmonary Alveolar Proteinosis.
Respiration; international review of thoracic diseasesDisseminated nocardiosis and anti-GM-CSF antibodies.
European journal of clinical microbiology & infectious diseases : official publication of the European Society of Clinical MicrobiologyWill inhalational GM-CSF replace whole lung lavage as a treatment for autoimmune pulmonary alveolar proteinosis? Many pole positions, not yet the final winner.
The European respiratory journalCoexistence of Autoimmune Pulmonary Alveolar Proteinosis With Small Cell Lung Cancer in a Man Presenting With Acute Onset Respiratory Failure.
CureusNebulised granulocyte-macrophage colony-stimulating factor (GM-CSF) in autoimmune pulmonary alveolar proteinosis: a systematic review and meta-analysis.
European respiratory review : an official journal of the European Respiratory SocietyInhaled recombinant GM-CSF reduces the need for whole lung lavage and improves gas exchange in autoimmune pulmonary alveolar proteinosis patients.
The European respiratory journalEffects of COVID-19 infection in patients with autoimmune pulmonary alveolar proteinosis: a single-center study.
Orphanet journal of rare diseasesAltered macrophage phenotypes in a case of autoimmune pulmonary alveolar proteinosis.
ERJ open researchAutoimmune Pulmonary Alveolar Proteinosis Complicated by Myelodysplastic Syndrome.
Internal medicine (Tokyo, Japan)Infections in autoimmune pulmonary alveolar proteinosis: a large retrospective cohort.
ThoraxOpportunistic infections in autoimmune pulmonary alveolar proteinosis: opportunity to better understand the role of GM-CSF in the innate immune response.
ThoraxFunctional autoantibodies: Definition, mechanisms, origin and contributions to autoimmune and non-autoimmune disorders.
Autoimmunity reviewsA case of development of autoimmune pulmonary alveolar proteinosis during the treatment of hypersensitivity pneumonitis.
Respiratory medicine case reportsAutoimmune Pulmonary Alveolar Proteinosis That Improved after a COVID-19 Episode.
Internal medicine (Tokyo, Japan)Case Report: Autoimmune Pulmonary Alveolar Proteinosis after COVID-19: A Report of Two Cases.
The American journal of tropical medicine and hygieneNationwide survey of adult patients with pulmonary alveolar proteinosis using the National Database of designated intractable diseases of Japan.
Respiratory investigationA case report of Covid-19 in an autoimmune pulmonary alveolar proteinosis: An association in tune with the times!
Respiratory medicine case reportsUpdated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China.
Frontiers in medicineRecombinant GM-CSF for diseases of GM-CSF insufficiency: Correcting dysfunctional mononuclear phagocyte disorders.
Frontiers in immunologyAutoimmune pulmonary alveolar proteinosis and sarcoidosis in the same patient: Case report and systematic review.
Lung India : official organ of Indian Chest SocietyQuantitative Evaluation of Changes in Three-Dimensional CT Density Distributions in Pulmonary Alveolar Proteinosis after GM-CSF Inhalation.
Respiration; international review of thoracic diseasesAspergillus Nodule in a Patient With Autoimmune Pulmonary Alveolar Proteinosis.
CureusUpper Lobe-predominant Autoimmune Pulmonary Alveolar Proteinosis.
Internal medicine (Tokyo, Japan)A dried blood spot test for diagnosis of autoimmune pulmonary alveolar proteinosis.
Journal of immunological methods[Consensus statement on inhaled recombinant human granulocyte-macrophage colony-stimulating factor for autoimmune pulmonary alveolar proteinosis (2022)].
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseasesAutoimmune Pulmonary Alveolar Proteinosis with Suspected Exacerbation after Osimertinib Administration for Lung Cancer.
Internal medicine (Tokyo, Japan)Autoimmune Pulmonary Alveolar Proteinosis during the Treatment of Idiopathic Inflammatory Myopathy.
Internal medicine (Tokyo, Japan)A case of autoimmune pulmonary alveolar proteinosis with severe respiratory failure treated with segmental lung lavage and oral statin therapy.
Respiratory medicine case reportsAn exploratory study investigating biomarkers associated with autoimmune pulmonary alveolar proteinosis (aPAP).
Scientific reportsAutoimmune pulmonary alveolar proteinosis with features similar to nonspecific interstitial pneumonia.
Respiratory medicine case reportsOpportunistic Infection Associated With Elevated GM-CSF Autoantibodies: A Case Series and Review of the Literature.
Open forum infectious diseasesA Bibliometric Analysis of Pulmonary Alveolar Proteinosis From 2001 to 2021.
Frontiers in medicineUnilateral Autoimmune Pulmonary Alveolar Proteinosis with Polymyositis-related Interstitial Lung Disease.
Internal medicine (Tokyo, Japan)Autoimmune pulmonary alveolar proteinosis exacerbated by steroid therapy due to misdiagnosis as anti-aminoacyl-tRNA synthetase (ARS) antibody positive- interstitial pneumonia: a case report.
BMC pulmonary medicineAutoimmune pulmonary alveolar proteinosis in children.
ERJ open researchRituximab rescue therapy for autoimmune pulmonary alveolar proteinosis.
Respiratory medicine case reportsA mini-whole lung lavage to treat autoimmune pulmonary alveolar proteinosis (PAP).
Respiratory research[Segmental Bronchoalveolar Lavage Provided a Favorable Clinical Course in a Case of Autoimmune Pulmonary Alveolar Proteinosis].
Journal of UOEHCYFRA21-1 is a more sensitive biomarker to assess the severity of pulmonary alveolar proteinosis.
BMC pulmonary medicineSirolimus-induced secondary pulmonary alveolar proteinosis.
Respiratory medicine case reportsThe Role of GM-CSF Autoantibodies in Infection and Autoimmune Pulmonary Alveolar Proteinosis: A Concise Review.
Frontiers in immunologyExacerbation of autoimmune pulmonary alveolar proteinosis that improved with lone treatment of complicating nontuberculous mycobacterial infection: A case report.
Respiratory medicine case reportsProteinaceous Lung With COVID-19: The Mimicker.
CureusAutoimmune pulmonary alveolar proteinosis with a history of vaping and vitamin E-positive bronchoalveolar lavage.
Respirology case reportsSargramostim (rhu GM-CSF) as Cancer Therapy (Systematic Review) and An Immunomodulator. A Drug Before Its Time?
Frontiers in immunologyAutoimmune pulmonary alveolar proteinosis: a discrepancy between symptoms and CT findings.
Lancet (London, England)Autoimmune pulmonary alveolar proteinosis successfully treated with lung lavage in an adolescent patient: a case report.
Journal of medical case reportsIncreased Efficacy of Whole Lung Lavage Treatment in Alveolar Proteinosis Using a New Modified Lavage Technique.
Journal of bronchology & interventional pulmonologyAutoimmune Pulmonary Alveolar Proteinosis: Evidence of the Pathogenicity of GM-CSF Antibodies.
American journal of respiratory and critical care medicineExpression profiles and potential functions of long noncoding RNAs and mRNAs in autoimmune pulmonary alveolar proteinosis patients.
AgingAutoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology.
BMJ case reportsB cell-activating factors in autoimmune pulmonary alveolar proteinosis.
Orphanet journal of rare diseasesGenetic determinants of risk in autoimmune pulmonary alveolar proteinosis.
Nature communicationsClinical significance of serum anti-granulocyte-macrophage colony-stimulating factor autoantibodies in patients with sarcoidosis and hypersensitivity pneumonitis.
Orphanet journal of rare diseasesInhaled Molgramostim Therapy in Autoimmune Pulmonary Alveolar Proteinosis.
The New England journal of medicineAlveolar proteinosis due to toxic inhalation at workplace.
Respiratory medicine case reportsInhaled granulocyte-macrophage colony stimulating factor for mild-to-moderate autoimmune pulmonary alveolar proteinosis - a six month phase II randomized study with 24 months of follow-up.
Orphanet journal of rare diseasesAutoimmune Pulmonary Alveolar Proteinosis Complicated with Sarcoidosis: the Clinical Course and Serum Levels of Anti-granulocyte-macrophage colony-stimulating Factor Autoantibody.
Internal medicine (Tokyo, Japan)Reduction in Alveolar Macrophage Size in Refractory Autoimmune Pulmonary Alveolar Proteinosis After Treatment With Pioglitazone.
Journal of bronchology & interventional pulmonologyAutoimmune pulmonary alveolar proteinosis mimicking Mycoplasma pneumonia in an adolescent.
Respiratory medicine case reportsAutoimmune pulmonary alveolar proteinosis prior to myelodysplastic syndrome.
Respirology case reportsA Case of Autoimmune Pulmonary Alveolar Proteinosis with Pulmonary Fibrosis and Asbestosis-Like Features.
The Kurume medical journalAutoimmune pulmonary alveolar proteinosis developed during immunosuppressive treatment in polymyositis with interstitial lung disease: a case report.
BMC pulmonary medicineProteogenomic analysis of granulocyte macrophage colony- stimulating factor autoantibodies in the blood of a patient with autoimmune pulmonary alveolar proteinosis.
Scientific reportsNatural Autoantibodies in Chronic Pulmonary Diseases.
International journal of molecular sciencesValidation of a new serum granulocyte-macrophage colony-stimulating factor autoantibody testing kit.
ERJ open researchPulmonary alveolar proteinosis (PAP) in idiopathic hypoparathyroidism.
BMJ case reportsRemoval of lung lavage fluid during whole-lung lavage using biphasic cuirass ventilation chest percussion in a patient with autoimmune pulmonary alveolar proteinosis.
Lung India : official organ of Indian Chest SocietyDoes maternal autoantibody that transfer to newborn cause disease?
Respirology case reportsInhaled GM-CSF for Pulmonary Alveolar Proteinosis.
The New England journal of medicineMemory B cell pool of autoimmune pulmonary alveolar proteinosis patients contains higher frequency of GM-CSF autoreactive B cells than healthy subjects.
Immunology lettersAutoimmune pulmonary alveolar proteinosis in a patient with sarcoidosis.
Clinical case reportsAutoimmune Pulmonary Alveolar Proteinosis Diagnosed after Exposure to a Fire Extinguisher Containing Silica Powder.
Internal medicine (Tokyo, Japan)Incidence of autoimmune pulmonary alveolar proteinosis estimated using Poisson distribution.
ERJ open research[Crazy Paving Pattern of the Lung].
Pneumologie (Stuttgart, Germany)Autoimmune pulmonary alveolar proteinosis presenting peripheral ground-glass opacities.
Respirology case reportsBetter approach for autoimmune pulmonary alveolar proteinosis treatment: inhaled or subcutaneous granulocyte-macrophage colony-stimulating factor: a meta-analyses.
Respiratory researchAssay system development to measure the concentration of sargramostim with high specificity in patients with autoimmune pulmonary alveolar proteinosis after single-dose inhalation.
Journal of immunological methodsAutoimmune pulmonary alveolar proteinosis in an adolescent successfully treated with inhaled rhGM-CSF (molgramostim).
Respiratory medicine case reportsPulmonary Alveolar Proteinosis with Ulcerative Colitis.
Internal medicine (Tokyo, Japan)Pulmonary alveolar proteinosis following cryptococcal meningitis: a possible cause?
BMJ case reportsPeripheral alveolar nitric oxide concentration reflects alveolar inflammation in autoimmune pulmonary alveolar proteinosis.
ERJ open researchClinical significance of serum anti-GM-CSF autoantibody levels in autoimmune pulmonary alveolar proteinosis.
Biomarkers in medicineElevated Serum Anti-GM-CSF Antibodies before the Onset of Autoimmune Pulmonary Alveolar Proteinosis in a Patient with Sarcoidosis and Systemic Sclerosis.
The Tohoku journal of experimental medicinePathological features of explant lungs with fibrosis in autoimmune pulmonary alveolar proteinosis.
Respirology case reportsSequential Granulocyte-Macrophage Colony-Stimulating Factor Inhalation after Whole-Lung Lavage for Pulmonary Alveolar Proteinosis. A Report of Five Intractable Cases.
Annals of the American Thoracic SocietyTwo cases of autoimmune pulmonary alveolar proteinosis with rheumatoid arthritis.
Allergology international : official journal of the Japanese Society of AllergologyClassification of idiopathic interstitial pneumonias using anti-myxovirus resistance-protein 1 autoantibody.
Scientific reportsA case of autoimmune pulmonary alveolar proteinosis with fluctuating lung shadows in parallel with cigarette smoke burden.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGTemporary remission of autoimmune pulmonary alveolar proteinosis after infectious episodes.
Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOGRecurrence of pulmonary alveolar proteinosis after bilateral lung transplantation in a patient with a nonsense mutation in CSF2RB.
Respiratory medicine case reportsSuccessful Treatment of Autoimmune Pulmonary Alveolar Proteinosis in a Pediatric Patient.
The American journal of case reportsIncrease in autoimmune pulmonary alveolar proteinosis after the 2011 Fukushima disaster.
Allergology international : official journal of the Japanese Society of AllergologyAutoimmune Pulmonary Alveolar Proteinosis Following Pulmonary Aspergillosis.
Internal medicine (Tokyo, Japan)Cardiovascular risk in pulmonary alveolar proteinosis.
Expert review of respiratory medicineWhole Lung Lavage Treatment of Chinese Patients with Autoimmune Pulmonary Alveolar Proteinosis: A Retrospective Long-term Follow-up Study.
Chinese medical journalOutcome of corticosteroid administration in autoimmune pulmonary alveolar proteinosis: a retrospective cohort study.
BMC pulmonary medicineLocalized Airspace Consolidation of Pulmonary Alveolar Proteinosis Mimicking Malignant Lesions in 18F-FDG PET/CT Imaging: One Case Report.
Clinical nuclear medicineMarked improvement in autoimmune pulmonary alveolar proteinosis with severe hypoxemia in a patient treated with ambroxol: a case report.
Journal of medical case reportsAutoimmune pulmonary alveolar proteinosis with primary lung cancer in a patient of very advanced years.
Geriatrics & gerontology internationalPulmonary alveolar proteinosis: time to shift?
Expert review of respiratory medicineCharacteristics of serum lipid metabolism in patients with autoimmune pulmonary alveolar proteinosis.
Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae SinicaeAssociações
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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.
- Lipid nanoparticle GM-CSF replacement for autoimmune pulmonary alveolar proteinosis.Proceedings of the National Academy of Sciences of the United States of America· 2026· PMID 41671176mais citado
- A long-term observational study on autoimmune pulmonary alveolar proteinosis revealed a sustained and generalized decrease in serum autoantibody levels.
- Timing of Whole Lung Lavage in Autoimmune Pulmonary Alveolar Proteinosis with Concurrent Opportunistic Infection: A Case Report and Systematic Review.
- Clinical characterization of 39 patients with autoimmune pulmonary alveolar proteinosis.
- A Case of Autoimmune Pulmonary Alveolar Proteinosis.
- The burden of autoimmune pulmonary alveolar proteinosis: a systematic review.
- Delayed Microbiological Diagnosis and Fluoroquinolone-Associated Tendon Rupture in Pulmonary Nocardiosis Complicating Autoimmune Pulmonary Alveolar Proteinosis: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:747(Orphanet)
- OMIM OMIM:610910(OMIM)
- MONDO:0012579(MONDO)
- GARD:7499(GARD (NIH))
- Variantes catalogadas(ClinVar)
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
- Q448698(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
