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Year : 2006  |  Volume : 23  |  Issue : 1  |  Page : 42-44 Table of Contents   

Pulmonary alveolar microlithiasis

1 Department of Tuberculosis and Respiratory Disease, Kasturba Medical College, Manipal., India
2 Department of General Medicine, Kasturba Medical College, Manipal., India

Correspondence Address:
K Gowrinath
Department of Tuberculosis and Respiratory Diseases, Kasturba Medical College, Manipal-576 104 (Karnataka).
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-2113.44430

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How to cite this article:
Gowrinath K, Warrier AR. Pulmonary alveolar microlithiasis. Lung India 2006;23:42-4

How to cite this URL:
Gowrinath K, Warrier AR. Pulmonary alveolar microlithiasis. Lung India [serial online] 2006 [cited 2020 Nov 25];23:42-4. Available from: https://www.lungindia.com/text.asp?2006/23/1/42/44430

   Introduction Top

Pulmonary alveolar microlithiasis (PAM) is a rare disease of unknown cause in which there is diffuse accumulation of numerous laminated calcispherites (salts of calcium and phosphate) in the alveolar spaces with a normal or thickened interstitium [1] . Friedreick [2] described this condition first in 1856 and later by Harbitz [3] in 1918. In 1933, Puhr [4] named this condition as microlithiasis alveolaris pulmonum. The first Indian case of PAM was reported by Viswanathan [5] in 1962. Until now, the highest number of cases of PAM have been reported from Europe followed by Asia and among the countries, Turkey has the highest number of reported cases of PAM followed by Italy [6] . PAM is usually diagnosed in the fourth decade and both sexes are affected equally [7] . PAM is often familial and detected among siblings [8] . In Indian literature, reports of PAM from early childhood [9] to old age [10] are available. The mechanism of formation of microliths (calcispherites) is not known. Since calcium metabolism is normal in PAM, it was postulated that the local accumulation of calcium salts may be due to an inborn defect in metabolism at the alveolar interface leading in increased alkalinity or that of mucopolysaccharide deposition [11] . Another hypothesis is that in patients with PAM, impaired mucociliary function may lead to excess mucus accumulation within alveoli and condensation of mucus may induce formation of microliths [12] . Review of literature showed that PAM is inherited as autosomal recessive condition and other non-genetic factors does not seem to play any role in its pathogenesis [13] .

The most striking feature of PAM is lack of significant symptoms despite extensive radiographic and pathological changes [14] . Most patients with PAM are asymptomatic at the time of diagnosis and the condition may progress slowly leading to progressive dyspnoea with or without cough and ultimately end up with respiratory insufficiency and corpulmonale [15] .Occasionally, PAM may present with chronic cough [16] or with haemoptysis [17] . Digital clubbing may be found in some cases of PAM. Chest examination may be normal or there may be few end inspiratory crackles at both the lung bases. Rupture of subpleural bullae in PAM can lead to recurrent spontaneous pneumothoraces [18] . The differential diagnosis of PAM includes sarcoidosis, miliary tuberculosis, metastatic lung carcinoma, primary or secondary haemosiderosis, healed histoplasmosis, pneumoconioses like silicosis, beryllosis, stannosis etc. Chest skiagram [Figure 1] may show bilateral diffuse micronodular calcific shadows more towards the mid and lower zones (sand stom appearance). The disease free pleura in the chest skiagram may appear as a black line (Black pleura sign) around the lungs' and under the ribs due to dense pulmonary opacities on one side and the ribs on the other side [19] . But high resolution computed tomography (CT) of thorax revealed that the black pleural line on chest skiagram is caused by a dense fat layer between ribs and the parenchymal calcifications [20] . CT of thorax [Figure 2] may show bilateral diffuse micronodular opacities within lung parenchyma distributed widely available, diagnosis of PAM has become simple and safer through bronchoalveolar lavage [22] or transbronchial lung biopsy [23] . Occasionally microliths may be detected in sputum specimens [24] . In PAM, histological examination of lung tissue shows calcified spherules filling alveolar spaces. Microscopically, these calcified spherules have a lamellated appearance and their size may increase by addition of more layers. The size of microliths vary from 0.01 to 0.5 mm and tend to fall out of lung tissue easily as they are not embedded in the fibrous tissue [25] . At postmortem examination, the lungs in PAM are hard, heavy, incollapsible with their cut surfaces feel like a sand paper [26] . The pathologic changes in PAM are mostly confined to lungs but there were isolated reports of associated pleural calcification [27] , diaphragmatic calcification [28] and calcification within sympathetic nervous system and gonads [29] . Bone scintigraphy shows diffuse uptake of technetium 99m in PAM and may be useful as a diagnostic adjunct [30] . In PAM, pulmonary function and exercise tests may be normal [31] or may show restrictive defect [32] . Since the pathologic changes are within alveoli, absence of interstitial involvement can be demonstrated through lung static pressure-volume analysis [33] .

At present no effective medical therapy is available either to treat or even to halt the progression of P AM. Previous attempts of treating this condition with systemic corticosteroids, calcium chelating agents and bronchoalveolar lavage have failed [7] . However there was an isolated report [34] wherer radiologic lesions due to PAM were found to be reduced after one year of treatment with disodium etidronate. Treatment with nasal continuous positive airway pressure (nCPAP) in a case of PAM with respiratory failure and corpulmonale [35] had reduced intra pulmonary shunt and helped in correcting hypoxaemia with smaller flow rate of oxygen. End stage lung disease due to PAM may require lung transplantation and bilateral sequential lung transplantation was reported to be successful in selected cases [36] .

   References Top

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3.Harbitz F. Extensive calcification of lungs as a distinct disease. Arch Intern Med 1918 ;21 : 139-146.  Back to cited text no. 3    
4.Puhr L. Microlithiasis Alveolaris Pulmonum.Virchows Arch Pathol Anat1933; 290:156-160.  Back to cited text no. 4    
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31.Jancovic S. Pavlov N. Ivkosic A, et al.Pulmonary alveolar microlithiasis in childhood: Clinical and radiological follow­up. Pediatric PulmonoI2002; 34:384-387 (Med line)  Back to cited text no. 31    
32.Sharma SK. Sharma S, Mukhopadhyaya S, Padhye AK. Vijayaraghavan M. Pulmonary alveolar microlithiasis - report of three cases with pulmonary function and exercise studies. Indian J Chest Dis Allied Sci 1992; 34:205-215.  Back to cited text no. 32    
33.Gupta D. Aggarwal AN. Jindal SK. Lung static pressure­volume analysis can demonstrate alveolar involvement in Pulmonary alveolar microlithiasis. Lung India 2000; 18: 64­-65.  Back to cited text no. 33    
34.Ozcelik U,Gulsun M. Ariyurek M. et al. Treatment and follow-up of IXllmonary alveolar microlithiasis with disodium editronate: radiological demonstration . Pediatric Radiology 2002; 32: 380-383.(Med line).  Back to cited text no. 34    
35.Freiberg DB. Young IH. Laks L. et al. Improvement in gas exchange in nasal continuous positive airway pressure in pulmonary alveolar microlithiasis. Am Rev Respir Dis 1992; 145:1215-1216.  Back to cited text no. 35    
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  [Figure 1], [Figure 2]


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