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Year : 2006  |  Volume : 16  |  Issue : 4  |  Page : 879-881
Pulmonary alveolar microlithiasis


Retd. DMET, Orissa, Consultant Radiologist, Patro Niwas, Friends' Colony, Burla - 768017, Orissa, India

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Date of Submission26-Dec-2005
Date of Acceptance10-Oct-2006
 

Keywords: Alveolar, Microlithiasis

How to cite this article:
Patro S S, Kar C R. Pulmonary alveolar microlithiasis. Indian J Radiol Imaging 2006;16:879-81

How to cite this URL:
Patro S S, Kar C R. Pulmonary alveolar microlithiasis. Indian J Radiol Imaging [serial online] 2006 [cited 2019 Aug 24];16:879-81. Available from: http://www.ijri.org/text.asp?2006/16/4/879/32373

   Introduction Top


Pulmonary alveolar microlithiasis is an uncommon pulmonary condition. We came across only four cases within a span of 20 years. No other pulmonary disease has a radiographic pattern as characteristic and diagnostic as that of pulmonary alveolar microlithiasis. Lack of association between radiologic and clinical findings is more striking in this disease than in any other condition [1].


   Case reports Top


Case 1:

A 32 year old male of Meher community presented with non productive cough and dyspnoea on exertion intermittently since 2 years. He had decreased breath sounds and inspiratory crepitations at both lung bases. Haemogram was normal.

Plain X-ray chest PA view (Fig. 1): Showed diffuse clouding of lung fields by numerous granular calcific opacities of less than 1mm size, mostly overlapping each other. The overall density was greater over the lower than upper zones. Mediastinal and diaphragmatic contours were obliterated and pulmonary vascular marking were indistinct. Minor fissure was thickened. A zone of increased lucency was present between costal margin and lung parenchyma (Black pleural line). Just inner and parallel to that black pleural line a dense linear shadow was seen at the upper zones.

Case 2:

A 26 year old female from Meher community presented with cough with scanty expectoration and chest discomfort for last 3 years intermittently. Breath sounds were decreased at both lung bases. Blood D.C. showed eisinophil 21%.

Plane X-ray chest P.A. view (Fig. 2): Showed both costophrenic angles obliterated. Obliteration of mediastinal and diaphragmatic outline. Both lung fields including apices were studded with numerous calcific granules overlapping each other. The lower lung fields were more dense than upper.

Case 3:

A 11 year old female child presented with non productive cough for 1 month. No abnormal physical signs were present in the chest. Haemogram was normal.

Plain X-ray of chest P.A. view (Fig. 3) : Showed numerous calcific granules not more than 1mm diameter present diffusely over both lungs including apices. The accumulation of granules were more at the bases than apices. The mediastinal and diaphragmatic contours were obliterated and pulmonary vascular marking were indistinct "Black pleural line" was seen from base to apex at both costal margins and medial to that a dense white line.

Case 4:

A 27 year old female of Meher community presented with cough and scanty expectoration, sneezing and chest discomfort for 1 years which was episodic in nature. There was no such family history and no known substance of allergy. She was having anaemia, decreased breath sound and scattered crepitations and rhonchi at both bases.

Plane X-ray chest PA view & magnifying view of Rt. Base (fig. 4): Showed multiple, calcific granular opacities present extensively in both lungs including apices. Accumulation of granules more at the lower zones than upper and so the density. There was obliteration of mediastinal and diaphragmatic contours and pulmonary vascular marking.
"Black pleural line" was present along both costal margins with dense white line inner and parallel to that. A dense thickened inter lobular septa of about 3 cm length seen at right lower zone laterally.


   Discussion Top


The cause of the disease is quite unknown. It may occur in any age between infancy and 80 years but the majority are diagnosed between the age of 30 - 50 years [2]. Some investigators have documented a predilection for men or women, however most have found no sex predominance. The disease exhibits a strong familial tendency. A familial occurrence has been noted in approximately half the reported cases. [3],[4]. The age of our 4 cases are between 2nd to 4th decade and 3 are females and 1 is male. Peculiarly 3 of them are from Meher community who are engaged in cloth weaving. All of them are from different parts of western Orissa and non of them are related to each other. None of them have familial history.

Most patients are asymptomatic when the disease is discovered by routine radiography[2]. When the patient is symptomatic, most common symptom is dyspnoea on exertion[4]. Cough develops in some patients although it is typically non productive. Chest pain is uncommon[3]. Haemoptysis occasionally occurs[5]. There are often no physical signs in the chest even when the radiography is grossly abnormal, later there may be inspiratory crepitation and ultimately the signs of cor-pulmonale [2], values of chemical analysis of blood are invariably within normal limits[2]. Biopsy shows calcified spherules filling alveolar spaces. The spherules have a concentric lamelleted appearance suggesting that they grow by the addition of successive layers. The spherules contain both calcium and phosphorus[6]. Our all four patients had symptoms like non productive cough, dyspnoea on exertion, cough with scanty expectoration and chest discomfort. The youngest patient had no physical signs but others had decreased breath sounds and scattered crepitations at lung bases.

The patients of pulmonary alveolar microlithiasis produce a characteristic radiographic appearance. The lung fields are diffusely occupied by discrete high density opacities resembling grains of sands and when profuse, produce 'white out' of the lung[7]. Regardless of the effect of superimposition or summation of shadows, individual deposits are usually identifiable measuring less than 1 mm diameter. The over all density is greater over the lower than the upper zone. The opacity may be so numerous as to appear confluent showing the lungs as almost uniformly white often with total obliteration of the mediastinal and diaphragmatic contours and pulmonary vascular marking becoming indistinct. Other findings that may be seen, include bullae in the lung apices, a zone of increased lucency between lung parenchyma and the ribs (Known as a black pleural line) and pleural calcification[1]. In some patients concentration of the nodules in subpleural, para septal and peribronchiolar alveoli can produce linear strands of calcification parallel to or perpendicular to the pleural surface.[6].

In addition to classical picture in all our 4 cases, except case no 2, other 3 cases had black pleural line along costal margin and dense white line inner and parallel to that, due to accumulation of microliths in subpleural alveoli. The minor fissure was thickened in one case and the inter lobular septa was thick and dense in other.

 
   References Top

1.Fraser & Pare's. Diagnosis of diseases of the chest. 4th Edn. Vol. IV. 1999; 2719-2722.  Back to cited text no. 1    
2.Crofton & Dugla's. Respiratory diseases 5th edn, Vol.II, 2000; 1339 -40.  Back to cited text no. 2    
3.Ucan E.S, Keyf AI, Aydilex R, et al: Pulmonary Alveolar microlithiasis - Review of Turkish reports. Thorax. 1993; 48:171.  Back to cited text no. 3    
4.Sosman MC, Dodd GD, Jones WD et al. The familial occurance of pulmonary alveolar microlithiasis. Am. J. Roentgenol 1957; 77: 947.  Back to cited text no. 4    
5.Thind G.S, Bhatia JI: Pulmonary Alveolar microlithiasis. Brit J. Dis. Chest. 1978; 72: 151.  Back to cited text no. 5    
6.Fishmans Pulmonary disease & disorders. 3rd edn, Vol I, 1998; 1155.  Back to cited text no. 6    
7.Grainger & Allisons, Diagnostic Radiology 3rd edn, Vol I, 1997; 415.  Back to cited text no. 7    

Top
Correspondence Address:
S S Patro
Retd. DMET, Orissa, Consultant Radiologist, Patro Niwas, Friends' Colony, Burla - 768017, Orissa
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.32373

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    Figures

  [Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]

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1 Two cases of pulmonary alveolar microlithiasis in a family
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[Pubmed]



 

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