Autopsy and Case Reports
https://autopsyandcasereports.org/article/doi/10.4322/acr.2019.133
Autopsy and Case Reports
Article / Autopsy Case Report

Pulmonary alveolar microlithiasis: Incidental finding - should we Ignore?

Manisha Agarwal; Gurpreet Singh Bhalla; Kavita Sahai

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Abstract

Pulmonary alveolar microlithiasis (PAM) is a rare entity, presenting mostly as an incidental finding. This disease has an autosomal recessive inheritance with inactivating mutations in the gene “solute carrier family 34 member 2”. The present study was conducted to bring attention to this rare though preventable disease. The study was a cross-sectional descriptive study, conducted at the Department of Pathology, of a tertiary care hospital in New Dehli-India. PAMs were incidentally seen in two patients diagnosed with micronodular hepatic cirrhosis leading to reanalysis of 212 autopsies, retrospectively. Statistical analysis was done using Stata 14.0. We observed three forms (Type A, B and C) of round hyaline bodies measuring in diameter with thin delicate, radiating fibrils. These bodies were PAS positive, showed black discolouration of the pigment with von Kossa stain and birefringence on polarized microscopy using Congo red stain, however the refringence was light green as compared to apple green birefringence seen with amyloid deposition. PAM has a slow progressive course leading to a high rate of incidental detection. Drugs known to inhibit the micro-crystal growth of hydroxyapatite may slow the disease progression. The family members of patients with PAM may also be kept on follow up with regular imaging. Key messages: It is important to bring out the incidental finding as, seemingly innocuous observations may provide valuable insight into incurable diseases, especially rare diseases.

Keywords

Incidental Findings, Autopsy, Calcification, Physiologic

References

Friedreich N. Corpora amylacea in den Lungen. Arch Path Anat. 1856;9:613-18.

Harbitz E. Extensive calcification of the lungs as a distinct disease. Arch Intern Med (Chic). 1918;21(1):139-46. [https://doi.org/10.1001/archinte.1918.00090070150012].

Puhr L. Mikrolithiasis alveolaris pulmonum. Arch Path Anat. 1933;290:156-160.

Michaels L, Levene C. Pulmonary corpora amylacea. J Pathol Bacteriol. 1957;74(1):49-56. [https://doi.org/10.1002/path.1700740108].

Dobashi M, Yuda F, Masuda A, Terashima K, Imai Y. Electron microscopic study on amyloid fibril formation in human lymph nodes. Histol Histopathol. 1986;1(3):277-89. [PMID:2980120]

Pracyk JB, Simonson SG, Young SL, Ghio AJ, Roggli VL, Piantadosi CA. Composition of lung lavage in pulmonary alveolar microlithiasis. Respiration. 1996;63(4):254-60. [https://doi.org/10.1159/000196556]. [PMID:8815975]

Ferreira Francisco FA, Pereira e Silva JL, Hochhegger B, Zanetti G, Marchiori E. Pulmonary alveolar microlithiasis. State-of-the-art review. Respir Med. 2013;107(1):1-9. [https://doi.org/10.1016/j.rmed.2012.10.014]. [PMID:23183116]

Kent G, Gilbert ES, Meyer HH. A clinical review of pulmonary microlithiasis. JAMA. 1956;161(12):1-2.

Corut A, Senyigit A, Ugur SA, et al. Mutations in SLC34A2 cause pulmonary alveolar microlithiasis and are possibly associated with testicular microlithiasis. Am J Hum Genet. 2006;79(4):650-6. [https://doi.org/10.1086/508263]. [PMID:16960801]

Huqun IS, Izumi S, Miyazawa H, et al. Mutations in the SLC34A2 gene are associated with pulmonary alveolar microlithiasis. Am J Respir Crit Care Med. 2007;175(3):263-8. [https://doi.org/10.1164/rccm.200609-1274OC]. [PMID:17095743]

Ferreira Francisco FA, Pereira e Silva JL, Hochhegger B, Zanetti G, Marchiori E. Pulmonary alveolar microlithiasis. State-of-the-art review. Respir Med. 2013;107(1):1-9. [https://doi.org/10.1016/j.rmed.2012.10.014]. [PMID:23183116]

Castellana G, Lamorgese V. Pulmonary alveolar microlithiasis. World cases and review of the literature. Respiration. 2003;70(5):549-55. [https://doi.org/10.1159/000074218]. [PMID:14665786]

Mariotta S, Ricci A, Papale M, et al. Pulmonary alveolar microlithiasis: report on 576 cases published in the literature. Sarcoidosis Vasc Diffuse Lung Dis. 2004;21(3):173-81. [PMID:15554073]

Viswanathan R. Pulmonary alveolar microlithiasis. Thorax. 1962;17(3):251-6. [https://doi.org/10.1136/thx.17.3.251]. [PMID:13926265]

Khurana A, Malik R, Sharma J, Khurana U, Goyal A. Pulmonary Alveolar Microlithiasis: A commonly misdiagnosed rare entity. Sultan Qaboos Univ Med J. 2018;18(2):236-8. [https://doi.org/10.18295/squmj.2018.18.02.021]. [PMID:30210859]

Gayathri Devi HJ, Mohan Rao KN, Prathima KM, Das JK. Pulmonary alveolar microlithiasis. Lung India. 2011;28(2):139-41. [https://doi.org/10.4103/0970-2113.80334]. [PMID:21712928]

Chandra S, Mohan A, Guleria R, Das P, Sarkar C. Bilateral stony lung: pulmonary alveolar microlithiasis. BMJ Case Rep. 2009;2009:1012. [PMID:21686505]

Hira HS, Singh T, Chowdhary V. Pulmonary alveolar microlithiasis: role of transbronchial lung biopsy. J Assoc Physicians India. 2000;48(8):832-3. [PMID:11273481]

Malhotra B, Sabharwal R, Singh M, Singh A. Pulmonary alveolar microlithiasis with calcified pleural plaques. Lung India. 2010;27(4):250-2. [https://doi.org/10.4103/0970-2113.71967]. [PMID:21139727]

Singh NK, Gupta A. Pulmonary alveolar microlithiasis. J Assoc Physicians India. 1995;43(9):647-8. [PMID:8773074]

Stamatis G, Zerkowski HR, Doetsch N, Greschuchna D, Konietzko N, Reidemeister JC. Sequential bilateral lung transplantation for pulmonary alveolar microlithiasis. Ann Thorac Surg. 1993;56(4):972-5. [https://doi.org/10.1016/0003-4975(93)90370-W]. [PMID:8215680]

Edelman JD, Bavaria J, Kaiser LR, Litzky LA, Palevsky HI, Kotloff RM. Bilateral sequential lung transplantation for pulmonary alveolar microlithiasis. Chest. 1997;112(4):1140-4. [https://doi.org/10.1378/chest.112.4.1140]. [PMID:9377936]

Jackson KB, Modry DL, Halenar J, L’abbe J, Winton TL, Lien DC. Single lung transplantation for pulmonary alveolar microlithiasis. J Heart Lung Transplant. 2001;20(2):226. [https://doi.org/10.1016/S1053-2498(00)00500-3]. [PMID:11250426]

 

 

 


Publication date:
12/13/2019

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