Home | About us | Editorial Board | Search | Ahead of print | Current Issue | Archives | Instructions | Online submissionContact Us   |  Subscribe   |  Advertise   |  Login  Page layout
Wide layoutNarrow layoutFull screen layout
Lung India Official publication of Indian Chest Society  
  Users Online: 16   Home Print this page  Email this page Small font size Default font size Increase font size


 
CASE REPORT
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).
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-2113.44430

Rights and Permissions

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 Sep 24];23:42-4. Available from: http://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

1.Moran CA, Hochholzer L, Hasleton PS, Johnson FB, Koss MN. Pulmonary alveolar microlithiasis: a clinicopathologic and chemical analysis of seven cases. Arch Pathol Lab Med 1997 ;121 :607-611.  Back to cited text no. 1  [PUBMED]  
2.Friedreick N.Corpora Amylacea in den Lungen. Virchows Arch Pathol Anat 1856; 9: 613- 618. (Medline)  Back to cited text no. 2    
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    
5.Vishwanathan R Pulmonary alveolar microlithiasis. Thorax 1%2 ;17: 251-256.  Back to cited text no. 5    
6.Castellanta G and LamorgeseV.Pulmonary alveolar microlithiasis.World cases and review of literature. Respiration 2003 ;70 :549-555.  Back to cited text no. 6    
7.Anthony Seaton. Some less common pulmonary diseases.ln Anthony Seaton, Douglas Seaton, A Gordon Leich, editors. Crofton and Douglas's Respiratory Diseases. 5th ed. Vol.2. London: Blackwell Science: 2000. p.1330-1345.  Back to cited text no. 7    
8.Ucan ES, Keyf AI, Aydilek, et al. Pulmonmy alveolar microlithiasis: review of Turkish reports. Thorax 1993; 48:171-173.  Back to cited text no. 8    
9.Sharma SK, Ray S, Kundu S,Kundu B, Ray S. Pulmonary alveolar microlithiasis: Report of two siblings. Lung lndia 2004; 21 :56-61.  Back to cited text no. 9    
10.Singh NK. Gupta A Pulmonary alveolar microlithiasis. J Assoc Physicians India 1995; 43:647- 648.   Back to cited text no. 10    
11.Sosman MC, Dodd D, Jones WD, et al. The familial occurrence of pulmonary alveolar microlithiasis. Am J Roentgenol1957; 77: 947-1012.  Back to cited text no. 11    
12.LautaVM. Pulmonary alveolar microlithiasis: an overview of clinical and pathological features together with possible therapies. Respir Med 2003; 97:1081-1085.  Back to cited text no. 12    
13.Castellana G, Gentile M, Castellana R, Fiorente P, Lamorgese V. Pulmonary alveolar microlithiasis: clinical features, evolution of the phenotype, and review of the literature. Am J Med Genetics 2002; 111:220-224.  Back to cited text no. 13    
14.Meyer, HH, Gilbert ES and Kent G. Clinical review of pulmonary microlithiasis. JAMA 1956; 161:1153-1157.  Back to cited text no. 14    
15.Prakash UBS. Basham SS. Rosenow EC. Brown ML and Payne WS.Pulmonary alveolar microlithiasis. A review including ultrastructural and pulmonary studies. Mayo Clin Proc 1983; 58:290-300.  Back to cited text no. 15    
16.Turktas I, Saribas S. Balkanci F. Pulmonary alveolar microlithiasis presenting with chronic cough. Postgrad Med J 1993; 69: 70-71.  Back to cited text no. 16    
17.Thind GS. Bhatia JL.Pulmonary alveolar microlithiasis. Br J Dis Chestl978; 72: 151-152.  Back to cited text no. 17    
18.Sears MR. Chang AR, Taylor AJ. Pulmonary alveolar microlithiasis. Thorax 1971; 26:704- 711.  Back to cited text no. 18    
19.Balkian JP. Fule:ihan FID. Nucho CKN. Pulmonary alveolar microlithiasis: report on 5 cases with special reference to roentgen manifestations.AJR 1968; 103:509-518.  Back to cited text no. 19    
20.Hoshino H. Koba H. Inomata S. et al. Pulmonary alveolar microlithiasis: high resolution CT and MR fmdings. J Comput Assist Tomogr 1998; 22: 245-248.  Back to cited text no. 20    
21.Ochs RH. Fishman AP. Depositional diseases of the lungs.In AP Fishman, Elias JA. Fishman JA. Crippi MA. Kaiser LR,Senior RM, editors.Fishman's Pulmonary Diseases and Disorders. 3d ed. Vol. I. New york: McGraw Hill: 1998.p. 1151-1161.  Back to cited text no. 21    
22.Sandhyamani S. Verma K, Sharma SK and Pande JN. Pulmonary alveolar microlithiasis. Indian J Chest Dis Allied Sci 1982; 38:33-35.  Back to cited text no. 22    
23.Hira HS. Singh T. Chowdhary V. Pulmonary alveolar microlithiasis:Role of transbronchial lung biopsy. J Assoc Phy India 2000; 48:832-833.  Back to cited text no. 23    
24.Chatterji R, Gaude GS and Patil P. V. Pulmonary alveolar microlithiasis:Diagnosis by sputum examination and transbronchial biopsy. Indian J Chest Dis Allied Sci 1997; 39:263-267.  Back to cited text no. 24    
25.Dial DH. Metabolic and other diseases. In Dial DH and Hammar SF. editors. Pulmonary pathology. Newyork: Spriger- Verlag: 1988, p 535-587.  Back to cited text no. 25    
26.Chalmers AG. Wyatt J and Robinson Pl. Computed tomographic and pathological finding. in Pulmon ary alveolar microlithiasis. Br J Radiol I986; 59:408-411.  Back to cited text no. 26    
27.Mittal MR, Gupta DM. Jindal SK. Radioisotope bone scanning in Pulmonary alveolar microlithiasis. Clin Nucl Med 2000; 25: 474-475.  Back to cited text no. 27    
28.Pant K, Shah A, Mathur RK, Chhabra SK, Jain SK. Pulmonary alveolar microlithiasis with pleural calcification and nephrolithiasis. Chest l990; 98:245-246.  Back to cited text no. 28    
29.Malhotra B and Kallan BM.Pulmonary alveolar microlithiasiS.Ind J Tuberc 1993; 40:31-33.  Back to cited text no. 29    
30.CoetzeeT. Pulmonary alveolar microlithiasis with involvement of sympathetic nervous system and gonads. Thorax 1970; 25:637-642.  Back to cited text no. 30    
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    
36.Edelman JD, Bavaria J. Kaiser LR. Lit:zky LA, Palevsky I. Kotloff RM. Bilateral sequential lung transplantation for pulmonary alveolar microlithiasis. Chest 1997; 112:1140­-1144.  Back to cited text no. 36    


    Figures

  [Figure 1], [Figure 2]



 

Top
 
  Search
 
  
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Introduction
    References
    Article Figures

 Article Access Statistics
    Viewed1523    
    Printed49    
    Emailed0    
    PDF Downloaded309    
    Comments [Add]    

Recommend this journal