Abstract
Pulmonary alveolar proteinosis (PAP) is a rare diffuse lung disease characterized by the abnormal accumulation of surfactant in the alveoli. It is classified as primary, secondary, and congenital PAP. Autoimmune PAP falls under the primary group and accounts for nearly 90% of all cases of PAP. These patients are at increased risk for secondary infections with opportunistic organisms. The initial presentation of autoimmune PAP complicated with pulmonary tuberculosis is very rare. A 34-year-old male presented with progressive dyspnea, dry cough, and constitutional symptoms of 4 weeks. On evaluation, his CT chest showed patchy areas of ground-glass opacities with interlobular septal thickening resembling crazy-paving pattern. His bronchoscopy revealed milky BAL, which formed a thick sediment on standing and was positive for Mycobacterium tuberculosis on the CBNAAT test and culture. His laboratory investigations showed raised levels of anti-GM-CSF antibodies. He was diagnosed as case of autoimmune PAP complicated with pulmonary tuberculosis and was started on antitubercular therapy. His symptoms improved with the therapy, and he is currently under regular follow-up at the respiratory outpatient department. This case report highlights the significance of screening for secondary infections in PAP patients both at the time of initial presentation and during follow-up, as treatment of these infections may improve the symptom burden.
Similar content being viewed by others
Data Availability
The data regarding the case will be available from the corresponding author upon request.
Code Availability
Not applicable.
References
Carey B, Trapnell BC. The molecular basis of pulmonary alveolar proteinosis. Clin Immunol. 2010;135(2):223–35.
Rosen SG, Castleman B, Liebow AA. Pulmonary alveolar proteinosis. N Engl J Med. 1958;258:1123–42.
Shah PL, Hansell D, Lawson PR, et al. Pulmonary alveolar proteinosis: clinical aspects and concepts on pathogenesis. Thorax. 2000;55:67–77.
Nogee LM. Genetic basis of children’s interstitial lung disease. Pediatr Allergy Immunol Pulmonol. 2010;23(1):15–24.
Inoue Y, Trapnell BC, Tazawa R, et al. Characteristics of a large cohort of patients with autoimmune pulmonary alveolar proteinosis in Japan. Am J Respir Crit Care Med. 2008;177(7):752–62.
Johkoh T, Itoh H, Muller NL, et al. Crazy-paving appearance at thin-section CT: spectrum of disease and pathologic findings. Radiology. 1999;211:155–60.
Suzuki T, Trapnell BC. Pulmonary alveolar proteinosis syndrome. Clin Chest Med. 2016;37(3):431–40. https://doi.org/10.1016/j.ccm.2016.04.006. Epub 2016 Jun 17. PMID: 27514590; PMCID: PMC5902187
Tekgül S, Bilaceroglu S, Ozkaya S, Coskun A, Komurcuoglu B, Cirak AK. Pulmonary alveolar proteinosis and superinfection with pulmonary tuberculosis in a case. Respir Med Case Rep. 2012;5:25–8. https://doi.org/10.1016/j.rmedc.2011.11.003. Epub 2011 Dec 22. PMID: 26057858; PMCID: PMC392042
Pereira-Silva JL, Marinho MM, Veloso TV, Coelho JCJC. Pulmonary alveolar proteinosis and tuberculosis in a diabetic patient: a rare or a seldom diagnosed association? Braz J Infect Dis. 2002;6(4):188–95. https://doi.org/10.1590/s1413-86702002000400006.
Yin WH, Chen XR, Zhu H, Su XY, Zhao LJ. Pulmonary alveolar proteinosis with pulmonary tuberculosis: a case report and literature review. Zhongguo Fanglao Zazhi. 2008;30:439–42.
Rekha C, Pralhad P, Pradeep V, Mahashur AA. Pulmonary alveolar proteinosis with pulmonary tuberculosis. Ind J Tub. 1996;43:27–9.
Shiohira S, Sakayori M, Yoshioka K, Kasai H, Hirama R, Abe M, Nishimura H, Suzuki T. Exacerbation of autoimmune pulmonary alveolar proteinosis that improved with lone treatment of complicating nontuberculous mycobacterial infection: a case report. Respir Med Case Rep. 2021;27(34):101521. https://doi.org/10.1016/j.rmcr.2021.101521. PMID: 34722142; PMCID: PMC8531841
Witty LA, Tapson VF, Piantadosi CA. Isolation of mycobacteria in patients with pulmonary alveolar proteinosis. Medicine (Baltimore). 1994;73:103–9.
Ramirez J, Savard EV, Hawkins JE. Biological effect of pulmonary washings from cases of alveolar proteinosis. Am Rev Respir Dis. 1966;94:244–6.
Borie R, Danel C, Debray MP, Taille C, Dombret MC, Aubier M, Epaud R, Crestani B. Pulmonary alveolar proteinosis. Eur Respir Rev. 2011;20(120):98–107.
Kamboj A, Lause M, Duggirala V. Severe pulmonary alveolar proteinosis in a young adult. Am J Med. 2018;131(5):e199–200.
Author information
Authors and Affiliations
Contributions
NS1 was responsible for concept, designing, writing of the draft, and final approval, KK and NS2 helped in editing the draft, RC collected relevant data, and AY helped in writing and critically editing the draft.
Corresponding author
Ethics declarations
Ethics Approval
Not applicable.
Consent to Participate
Not applicable.
Consent for Publication
Written consent to publish was obtained from the patient.
Conflict of Interest
The authors declare no competing interests.
Additional information
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
This article is part of the Topical Collection on Medicine
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Sharma, N., Kumar, K., Singh, N. et al. A Rare Case of Autoimmune Pulmonary Alveolar Proteinosis Complicated with Pulmonary Tuberculosis — a Case Report. SN Compr. Clin. Med. 5, 130 (2023). https://doi.org/10.1007/s42399-023-01469-1
Accepted:
Published:
DOI: https://doi.org/10.1007/s42399-023-01469-1