Abstract

Case Report

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

Arnaldo A Nieves-Ortiz*, Kyomara Hernandez-Moya, Juan Garcia-Puebla, Kimberly Padilla-Rodriguez, Neshma Roman-Velez, Giovanni Veloz-Irizarry, Keren Mendez-Ramirez, Hector Collazo-Santiago, Jose Adorno-Fontanez and Ricardo Fernandez-Gonzale

Published: 05 August, 2021 | Volume 5 - Issue 1 | Pages: 076-077

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 α- or β-chains of the GM-CSF receptor [1-3].

Read Full Article HTML DOI: 10.29328/journal.jprr.1001029 Cite this Article Read Full Article PDF

Keywords:

Pulmonary alveolar proteinosis; GM-CSF autoantibodies; Autoimmune

References

  1. Trapnell BC, Whitsett JA, Nakata K. Pulmonary alveolar proteinosis. N Engl J Med. 2003; 349: 2527-2539. PubMed: https://pubmed.ncbi.nlm.nih.gov/14695413/
  2. Suzuki T, Trapnell BC. Pulmonary alveolar proteinosis syndrome. Clin Chest Med. 2016; 37: 431-440. PubMed: https://pubmed.ncbi.nlm.nih.gov/27514590/
  3. Pulmonary Alveolar Proteinosis (PAP). EBSCO Information Services. 2021. https://www.dynamed.com/condition/pulmonary-alveolar-proteinosis-pap

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  • “Fatty Lungs”: An uncommon case of Autoimmune Pulmonary Alveolar Proteinosis
    Arnaldo A Nieves-Ortiz*, Kyomara Hernandez-Moya, Juan Garcia-Puebla, Kimberly Padilla-Rodriguez, Neshma Roman-Velez, Giovanni Veloz-Irizarry, Keren Mendez-Ramirez, Hector Collazo-Santiago, Jose Adorno-Fontanez and Ricardo Fernandez-Gonzale Arnaldo A Nieves-Ortiz*,Kyomara Hernandez-Moya,Juan Garcia-Puebla,Kimberly Padilla-Rodriguez,Neshma Roman-Velez,Giovanni Veloz-Irizarry,Keren Mendez-Ramirez,Hector Collazo-Santiago,Jose Adorno-Fontanez,Ricardo Fernandez-Gonzale. “Fatty Lungs”: An uncommon case of Autoimmune Pulmonary Alveolar Proteinosis. . 2021 doi: 10.29328/journal.jprr.1001029; 5: 076-077

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