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| Year : 1982 | Volume
: 28
| Issue : 4 | Page : 235-7 |
Pulmonary alveolar microlithiasis. (A case report).
Saigal RK, Gupta KK, Sandhu SK, Sidhu PK
How to cite this article:
Saigal R K, Gupta K K, Sandhu S K, Sidhu P K. Pulmonary alveolar microlithiasis. (A case report). J Postgrad Med 1982;28:235 |
Pulmonary alveolar microlithiasis is a rare condition characterised by a diffuse bilateral filling of the majority of alveoli by calcific concretions called "Calcospherites". This condition was first described in 1856 by Friederich[7] as "Corpora-Amylacea in den lungen". Puhr in 1933,[12] used the term as Micralithasis alveolaris pulmonum. The aetiology and pathogenesis are obscure; a familial incidence has been reported in several instances. We report here a case of pulmonary alveolar microlithiasis because of its rarity.
A 45 year old female was admitted in November, 1981 in the T.B. Hospital attached to the Government Medical College, Patiala, with the chief complaint of dyspnoea for the last 2 years. Radiological examination of the chest revealed diffuse bilateral nodular opacities and it was clinically dagnosed as a case of pulmonary alveolar microlithiasis. Serum calcium was normal. Open lung biopsy was done and examined histopathologically.
Microscopic examination
The paraffin sections of the tissue stained by haematoxylin and eosin showed spherical calcified laminated bodies in the lumina of the alveoli; alveolar septa appeared normal except for mild congestion. The interstitial tissue was infiltrated by mononuclear cells. Some of the alveoli were distended [ Figs. 1] and [Fig. 2].
Pulmonary alveolar microlithiasis is rare and less than 100, cases have been reported in the world literature. The radiological picture which is very characteristic shows bilateral sand-like micro nodules of calcific density, usually most marked in the middle and lower zones with relative sparing of the apices. Although the radiological picture is diagnostic, many cases are mistaken for miliary tuberculosis, silicosis, berylliosis, sarcoidosis, haemosiderosis, fungal infections and carcinomatosis. Most patients remain symptom-free for many years despite extensive radiological changes.[6], [8], [15]
Mikhaylov[10] was the first to report the familial occurrence of pulmonary alveolar microlithiasis and was supported by Sosman et al.[15] In over half the reported cases, a familial incidence has been demonstrated almost invariably among Siblings[17] and only in two instances in a parent and child;[5] cases in infants have also been reported.[3] A genetic factor has been postulated because of familial occurrence. Sosman et a1[15] and Caffrey and Altman[3] have suggested the possibility of congenital error of metabolism at the level of alveolar surface membrane, possibly an enzymatic fault, resulting in the precipitation of calcium in the presence of undue alkalinity. If the condition is genetically determined and due to an inborn error of metabolism, older patients would be expected to show more severe pathologic changes. This has not been so. The occurrence of similar laminated calcified bodies within the bronchial wall is also an evidence against this theory.[13]
Sharp and Danino[14] believe that microliths develop in both lungs following single inflammatory or vascular episode, causing a generalised alveolar exudate based on the general uniformity of size and appearance of microliths. However, this hypothesis is difficult to accept as the alveolar exudate is a prominant feature of many cardio-pulmonary diseases and yet microlithiasis is very rare.
Baar and Ferguson[1] and Meyer et al[9] believe that the condition is the result of an inflamatory process, probably hyper immune to one or more likely a variety of irritants.
Badger et al[2] and Coetzee[4] attribute the condition to dysfunction of the parathyroid or other endocrine organs or to an abnormality of calcium metabolism. Careful study of the calcium metabolism in many reported cases, however, reveals no derangement, though elevated serum calcium and renal stones have been recorded in some cases.[2],[11] It may be assumed that hypercalcaemia in those cases may speed up the pathologic process of this condition in the presence of some additional factors.
Tao[16] has postulated that the microliths are originally formed over the Curshmann's spirals in the smaller bronchi or bronchioles rather than in the alveoli as generally speculated as the nuclei of microliths appear to be the loops (Coiled parts) of Curshmann's spirals. The Curshmann's spiral which is considered to be a cast of inspissated mucus are commonly seen in patients with chronic obstructive pulmonary disease. On the loops of the spirals, numerous microliths may form due to deposition of the calcium salts or other chemical substances which seems to be a continuous process. The free microliths find their way into the alveoli on inspiration and gradual accumulation of microliths into alveoli after a long period leads to clinically detectable pulmonary alveolar microlithiasis.
We thank Sh. O. P. Khosla, Photo-tine officer of this institution for preparing the photomicrographs.
| 1. | Baar, H. S. and Ferguson, F. F.: Microlithlasis alveolaris pulmonum. Association with diffuse interstitial pulmonary fibrosis. Arch,. Pathol., 76: 659-666, 1953.  |
| 2. | Badger, T. L., Gottlieb. L. and Gaensler, E. A.: Pulmonary alveolar microlithiasis or calcinosis of lungs. New Engl. J. Med., 253: 709-715, 1955. |
| 3. | Caffrey, P. R. and Altman, R. S.: Pulmonary alveolar microlithiasis occurring in premature twins. T. Pediat.. 66: 758763, 1965. |
| 4. | Coetzee, T.: Pulmonary alveolar microlithiasis with involvement of the sympathetic nervous system and gonads. Thorax, 25: 637-642. 1970. |
| 5. | Drinkovic, I., Strohal, K. and Sabljica, B.: Pulmonary alveolar microlithiasis. Fortsch. Rontgenstr., 97: 180-185, 1962. |
| 6. | Fraser, R. G. and Pare, P. J. A.: "Diagnosis of Diseases of The Chest." Vol. II, W. B. Saunders Company, Philadelphia and London, 1970, pp. 1131-1134. |
| 7. | Friederich, N.: Zur Entwickelungsgeschichte der corpora amylacea in den Lungen. Virchows Arch. Path. Anat., 10: 507, 1856. Quoted by Coetzee (1970).[4] |
| 8. | Mandi, L., Simay, A., Kelemen, J. T., Szabo, A. and Dayka, A.: Alveolar microlithiasis. Pray. Pneumol., 22: 230-237, 1968. |
| 9. | Meyer, H. H., Gilbert, E. S., and Kent, G.: Clinical review of microlithiasis. J. Amer. Med. Assoc., 161: 1153-1157, 1956. |
| 10. | Mikhaylov, V.: Pulmolithiasis endalveolaris et interstitialis diffusa (Diffuse interstitial and endalveolar pulmonary lirhiasis). Klin. Med. (Moskow), 32: 3136, 1954. |
| 11. | Portnoy, L. M., Amadeo, B. and Hennigar, G. R.: Pulmonary alveolar microlithiasis. An unusual case associated with milk alkali syndrome. Amer. J. Clin. Pathol., 41: 194-201, 1964. |
| 12. | Puhr, L.: Microlithiasis alveolaries pulmonum. Virchows Arch. Path. Anat., 290: 156-160, 1933. |
| 13. | Sears, M. R., Chang, A. R. and Taylor, A. J. : Pulmonary alveolar microlithiasis. Thorax, 26: 704-711, 1971. |
| 14. | Sharp, M. E. and Danino, E. A.: Unusual form of pulmonary calcification-"Microlithiasis alveolaries pulmonum." J. Pathol. Bacteriol., 65: 389-399, 1953. |
| 15. | Sosman, M. C., Dodd, G. D., Jones, W. D. and Pillmore, G. U.: The familial occurrence of pulmonary alveolar microlithiasis. Amer. J. Roentgenol., 77: 9471012, 1957. |
| 16. | Tao, Liang-Che: Microliths in Sputum specimens and their relationship to pulmonary alveolar microlithiasis. Amer. J. Clin. Path., 69: 482-485, 1978. |
| 17. | Thind, G. S. and Bhatia, J. L.: Pulmonary alveolar microlithiasis. Brit. J. Dis. Chest, 72: 151-154, 1978. |
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