Pub Date : 2021-08-05DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2051
A. Nieves-Ortiz, K. Hernandez-Moya, Juan Garcia-Puebla, Kimberly Padilla-Rodriguez, N. Roman-Velez, Giovanni Veloz-Irizarry, K. Mendez-Ramirez, H. Collazo-Santiago, José Adorno-Fontánez, Ricardo Fernández-González
Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease characterized by excessive accumulation of surfactant lipids and proteins in alveoli and terminal airways. It is caused by impaired GM-CSF signaling [1]. Surfactant is synthesized and secreted by alveolar type II epithelial cells, and removed by uptake and catabolism by these cells, and the alveolar macrophages. Patients with PAP usually describe gradual onset of progressive exertional dyspnea and non-productive cough. However, an asymptomatic presentation is observed in up to 25% of cases, even in the presence of diffuse radiographic changes. Three recognized subtypes exist. Autoimmune PAP is associated with neutralizing GM-CSF autoantibodies and accounts about 90% of cases. Secondary PAP may occur in the context of any disease that reduces the abundance or functionality of alveolar macrophages, resulting in impaired surfactant clearance. Congenital PAP is the result of genetic mutations that disrupt GM-CSF signaling, including mutations in the αor β-chains of the GM-CSF receptor [1-3].
{"title":"\"Fatty Lungs\": A Uncommon Case of Autoimmune Pulmonary Alveolar Proteinosis","authors":"A. Nieves-Ortiz, K. Hernandez-Moya, Juan Garcia-Puebla, Kimberly Padilla-Rodriguez, N. Roman-Velez, Giovanni Veloz-Irizarry, K. Mendez-Ramirez, H. Collazo-Santiago, José Adorno-Fontánez, Ricardo Fernández-González","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2051","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2051","url":null,"abstract":"Pulmonary Alveolar Proteinosis (PAP) is a rare lung disease characterized by excessive accumulation of surfactant lipids and proteins in alveoli and terminal airways. It is caused by impaired GM-CSF signaling [1]. Surfactant is synthesized and secreted by alveolar type II epithelial cells, and removed by uptake and catabolism by these cells, and the alveolar macrophages. Patients with PAP usually describe gradual onset of progressive exertional dyspnea and non-productive cough. However, an asymptomatic presentation is observed in up to 25% of cases, even in the presence of diffuse radiographic changes. Three recognized subtypes exist. Autoimmune PAP is associated with neutralizing GM-CSF autoantibodies and accounts about 90% of cases. Secondary PAP may occur in the context of any disease that reduces the abundance or functionality of alveolar macrophages, resulting in impaired surfactant clearance. Congenital PAP is the result of genetic mutations that disrupt GM-CSF signaling, including mutations in the αor β-chains of the GM-CSF receptor [1-3].","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"20 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2021-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"124628699","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2043
N. Haris, A. Ladzinski, J. Brooks, P. Patel
{"title":"Granulomatosis with Polyangiitis with Peripheral Eosinophilia and Diffuse Alveolar Hemorrhage","authors":"N. Haris, A. Ladzinski, J. Brooks, P. Patel","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2043","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2043","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"31 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"114457871","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2052
D. Kazmierski, N. Sharma, B. Noumi, M. Amjad, P. Ochieng
{"title":"White-Out Lungs: A Case of Autoimmune Pulmonary Alveolar Proteinosis","authors":"D. Kazmierski, N. Sharma, B. Noumi, M. Amjad, P. Ochieng","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2052","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2052","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130197283","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2067
S. San Martín, G.D.P. Bridi, G. Almeida, P. Colares, R. Kairalla
{"title":"Association of Interstitial Lung Disease and Pneumomediastine in Idiopathic inflammatory Myopathy","authors":"S. San Martín, G.D.P. Bridi, G. Almeida, P. Colares, R. Kairalla","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2067","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2067","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"243 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"128880867","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2063
E. Kistler, L. Hariri, K. Berg
{"title":"A Case of Progressive Interstitial Lung Disease in a 25-Year-Old Woman, 34 Weeks Pregnant, Who Presents with Cough and Fever","authors":"E. Kistler, L. Hariri, K. Berg","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2063","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2063","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"73 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"125761056","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2047
N. Zimmermann, P. Colares, I. Holz, A. F. Amaral, A. M. Kawassaki, B. Baldi, R. Kairalla
{"title":"Adult Patient with Pulmonary Hemosiderosis and Celiac Disease (Lane Hamilton Syndrome): A Case Report","authors":"N. Zimmermann, P. Colares, I. Holz, A. F. Amaral, A. M. Kawassaki, B. Baldi, R. Kairalla","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2047","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2047","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"129885423","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2056
R. Kunadharaju, D. Crossland, B. Ahmed, A. Saradna, M. Mador
{"title":"Immunoglobulin G4-Related Disease (IgG4-RD) - An Unusual Presentation Mimicking Metastatic Lung Cancer.","authors":"R. Kunadharaju, D. Crossland, B. Ahmed, A. Saradna, M. Mador","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2056","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2056","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"29 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"130507869","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2041
S. Shrestha, P. Mckillion, A. Abdullah, S. Penupolu
{"title":"A Case of Anti-Glomerular Basement Membrane Disease Presented with Pulmonary Renal Syndrome Treated with Rituximab","authors":"S. Shrestha, P. Mckillion, A. Abdullah, S. Penupolu","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2041","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2041","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"7 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"131657708","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2034
J. Bae, K. Hsieh, K. Bortfeld, S. D’Annunzio
{"title":"An Unusual Presentation of New Systemic Lupus Erythematous: Concomitant Methicillin-Susceptible Staphylococcus Aureus Bacteremia and Severe Hyponatremia","authors":"J. Bae, K. Hsieh, K. Bortfeld, S. D’Annunzio","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2034","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2034","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"1 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"126795186","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 1900-01-01DOI: 10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2031
M. Riego, J. Poon, R. Naik
{"title":"Misdiagnosis of Asthma in a Patient with Hypereosinophilic Syndrome with Cardiac Involvement","authors":"M. Riego, J. Poon, R. Naik","doi":"10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2031","DOIUrl":"https://doi.org/10.1164/ajrccm-conference.2021.203.1_meetingabstracts.a2031","url":null,"abstract":"","PeriodicalId":114116,"journal":{"name":"TP33. TP033 CASE REPORTS IN AUTOIMMUNE LUNG DISEASE","volume":"101 1","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"1900-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"122963059","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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