scholarly journals “Fatty Lungs”: An uncommon case of Autoimmune Pulmonary Alveolar Proteinosis

2021 ◽  
Vol 5 (1) ◽  
pp. 075-076
Author(s):  
Nieves-Ortiz Arnaldo A ◽  
Hernandez-Moya Kyomara ◽  
Garcia-Puebla Juan ◽  
Padilla-Rodriguez Kimberly ◽  
Roman-Velez Neshma ◽  
...  
Keyword(s):  
Lung Disease ◽  
Pulmonary Alveolar Proteinosis ◽  
Uncommon Case ◽  
Alveolar Proteinosis ◽  
Excessive Accumulation

Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease characterized by excessive accumulation of surfactant lipids and proteins in alveoli and terminal airways.

Autoimmune pulmonary alveolar proteinosis and idiopathic pulmonary haemosiderosis: a dual pathology

2021 ◽  
Vol 14 (4) ◽  
pp. e241048
Author(s):  
Laura Walsh ◽  
Cormac McCarthy ◽  
Michael Henry
Keyword(s):  
Pulmonary Alveolar Proteinosis ◽  
Thoracoscopic Surgery ◽  
Alveolar Haemorrhage ◽  
Idiopathic Pulmonary Haemosiderosis ◽  
History Of ◽  
Transbronchial Lung Cryobiopsy ◽  
Alveolar Proteinosis ◽  
Video Assisted Thoracoscopic Surgery ◽  
Excessive Accumulation

Pulmonary alveolar proteinosis (PAP) is a rare pulmonary condition which leads to excessive accumulation of proteinaceous material within the alveoli. Idiopathic pulmonary haemosiderosis (IPH) is another orphan lung disease and results in recurrent alveolar haemorrhage. This case study describes a case of these two rare pathologies occurring together. A man in his 50s presented with a 6-week history of haemoptysis and worsening dyspnoea. A CT scan of the thorax showed multifocal, bilateral ground glass opacification with a wide differential diagnosis. Full autoantibody screen including myositis panel and coeliac screen were negative. Bronchoscopy with bronchoalveolar lavage and tissue from a transbronchial lung cryobiopsy were non-diagnostic. Tissue from a video-assisted thoracoscopic surgery biopsy confirmed a diagnosis of PAP with IPH as a second separate pathology. The association of IPH and PAP has not previously been described. We discuss these conditions and postulate how and if they may be related.

Mutation ofSFTPCin infantile pulmonary alveolar proteinosis with or without fibrosing lung disease

2004 ◽  
Vol 126A (1) ◽  
pp. 18-26 ◽  
Author(s):  
Mohammed Tredano ◽  
Matthias Griese ◽  
Frank Brasch ◽  
Silja Schumacher ◽  
Jacques de Blic ◽  
...  
Keyword(s):  
Lung Disease ◽  
Pulmonary Alveolar Proteinosis ◽  
Alveolar Proteinosis

Update in Nonneoplastic Lung Diseases

2009 ◽  
Vol 133 (7) ◽  
pp. 1096-1105
Author(s):  
Ilyssa O. Gordon ◽  
Nicole Cipriani ◽  
Qudsia Arif ◽  
A. Craig Mackinnon ◽  
Aliya N. Husain
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Diseases ◽  
Pulmonary Alveolar Proteinosis ◽  
Molecular Techniques ◽  
Classification Systems ◽  
Pulmonary Hemosiderosis ◽  
Wide Range ◽  
Alveolar Proteinosis

Abstract Context.—Nonneoplastic lung diseases include a wide range of pathologic disorders from asthma to interstitial lung disease to pulmonary hypertension. Recent advances in our understanding of the pathophysiology of many of these disorders may ultimately impact diagnosis, therapy, and prognosis. It is important for the practicing pathologist to be aware of this new information and to understand how it impacts the diagnosis, treatment, and outcome of these diseases. Objective.—To update current progress toward elucidating the pathophysiology of pulmonary alveolar proteinosis, idiopathic pulmonary hemosiderosis, and pulmonary arterial hypertension, as well as to present classification systems for pulmonary hypertension, asthma, and interstitial lung disease and describe how these advances relate to the current practice of pulmonary pathology. Data Sources.—Published literature from PubMed (National Library of Medicine) and primary material from the authors' institution. Conclusions.—Improved understanding of the pathophysiology of pulmonary alveolar proteinosis, pulmonary hypertension, and idiopathic hemosiderosis may impact the role of the surgical pathologist. New markers of disease may need to be assessed by immunohistochemistry or molecular techniques. The classification systems for interstitial lung disease, asthma, and pulmonary hypertension are evolving, and surgical pathologists should consider the clinicopathologic context of their diagnoses of these entities.

scholarly journals Serum proteome analysis of systemic JIA and related pulmonary alveolar proteinosis identifies distinct inflammatory programs

2021 ◽  
Author(s):  
Guangbo Chen ◽  
Gail Deutsch ◽  
Grant Schulert ◽  
Hong Zheng ◽  
SoRi Jang ◽  
...  
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Lung Disease ◽  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Pulmonary Alveolar Proteinosis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
List Type ◽  
New Findings ◽  
Alveolar Proteinosis ◽  
Activation Syndrome

AbstractObjectivesRecent observations in systemic Juvenile Idiopathic Arthritis (sJIA) suggest an increasing incidence of high-mortality interstitial lung disease (ILD), characterized pathologically by a variant of pulmonary alveolar proteinosis (PAP). The co-occurrence of macrophage activation syndrome (MAS) and PAP in sJIA suggested a shared pathology, but features of drug reaction such as anaphylaxis, rashes, and eosinophilia are also common in these patients. We sought to investigate immunopathology and identify biomarkers of this lung disease.MethodsWe used SOMAscan to measure >1300 analytes in serum samples from healthy controls and patients with sJIA-PAP, sJIA, sJIA-MAS, and other associated diseases, and verified selected findings by ELISA and lung immunostaining. Because a sample’s proteome may reflect multiple states (SJIA, MAS, SJIA-PAP), we used linear regression modeling to identify subsets of altered proteins associated with each state.ResultsMarkers identified for sJIA, including SAA, S100A9, and S100A12, were consistent with previous reports. Proteome alterations in sJIA and MAS overlapped substantially, including new findings of heat shock proteins and glycolytic enzymes. sJIA, MAS, and sJIA-PAP shared elevation of IL-18. Importantly, we identified a unique sJIA-PAP signature whose expression was independent of sJIA-MAS activity. Key proteins were ICAM5 and MMP7, previously observed markers of fibrotic ILD. Immunohistochemistry showed expression of these proteins in sJIA-PAP lung, supporting a pulmonary source. The eosinophil chemoattractant CCL11 was elevated in sJIA-PAP, but not sJIA/MAS or other PAP.ConclusionsWe found novel circulating proteins associated specifically with sJIA, sJIA/MAS and sJIA-PAP. Select biomarkers, such as ICAM5, could aid in early detection and management of sJIA-PAP.Key MessagesPulmonary Alveolar Proteinosis (PAP) occurring in the setting of Systemic Juvenile Idiopathic Arthritis (sJIA) is an increasingly-noted, dangerous condition that has been associated with Macrophage Activation Syndrome (MAS).We evaluated >1300 serum proteins by aptamer array, verifying and localizing key proteins, and identified novel pathways associated with MAS (HSPs, glycolysis), candidate pathways/proteins associated with PAP in sJIA (e.g., ICAM5, MMP7, and type 2 chemokines), and divergence of the sJIA/MAS and sJIA-PAP proteomes.This analysis supports the evaluation of novel pathways in MAS, the validation of screening/monitoring biomarkers in sJIA-PAP, and the management of PAP as a disease process enmeshed with, but distinct from, sJIA and MAS.

scholarly journals Bilateral lung disease, extensive and diffuse. Diagnosis of pulmonary alveolar proteinosis by bronchoscopic cryobiopsy

2017 ◽  
Vol 22 ◽  
pp. 260-262
Author(s):  
Sebastián Gando ◽  
Roberto Duré ◽  
Damián Violi ◽  
Bibiana Vazquez ◽  
Gonzalo Labarca ◽  
...  
Keyword(s):  
Lung Disease ◽  
Pulmonary Alveolar Proteinosis ◽  
Alveolar Proteinosis ◽  
Bilateral Lung
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