Aust. Radiol. (1975). 19, 175
Pulmonary Alveolar Microlithiasis DR. S. THURAIRAJASINGAM, M.B.B.S. (CEY.);D.M.R.D.; F.F.R. (LoND.) DR.B. D. DHARMASENA, M.B.B.S. (CEY.);M.D. (CEY.);M.R.C.P. (LDND.) AND
DR. T. KASTHURIRATNA, M.B.B.S. (CEY.) Government General Hospital, Kandy, Sri Lanku INTRODUCTION Pulmonary alveolar microlithiasis is an extremely rare condition, evidenced by the fact that there are less than 100 cases reported in the world literature. The mean age of reported cases is about 35 years. Only about 14 patients have been diagnosed at 12 years or younger. The youngest documented cases were in premature twins (29 weeks' gestation) at 11 and 12 hours after birth (Caffrey and Altman 1965) and the oldest in a woman of 80 (Sears et al. 1971). The disease is characterized by myriads of dense, finely punctate opacities in the chest x-ray obscuring pulmonary and even mediastinal borders but with a paucity of clinical symptoms. The first account of pulmonary alveolar microlithiasis is attributed to Harbitz ( 1918), though Friedreich ( 1856) described several forms of concentrically laminated bodies found in the lung parenchyma. The name microlithiasis alveolaris pulmonarum was suggested by Puhr in 1933. CASEREPORTS Case 1: S.H., male, 12 years, was admitted to the General Hospital, Kandy, on 26/4/ 1971 for a urinary tract infection. He complained of fever, frequency of micturition and turbidity of urine. He had a slight cough of six days' duration. He had no finger clubbing. A chest x-ray was requested as a few crepitations were heard over the lungs. The x-rays (Figures I and 2) revealed bilateral diffuse punctate opacities compatible with pulmonary alveolar microlithiasis with pleural calcification. The heart was normal in appearance. Miliary tuberculosis and haemosiderosis (primary or secondary) were considered in the differential diagnosis but presumed to be less likely on the following grounds.
(a) The soft millet seed type of d i a r y mottling of the lungs seen in d i a r y tuberculosis did not fit into the patient's x-ray picture. Further, the general condition of the patient was good. b) The lack of the characteristic filigree pattern and the high density of the punctate opacities ruled out the possibility of ideopathic (primary) haemosiderosis. c) Secondary haemosiderosis caused by mitral stenosis, angiomatous maiformations of lungs and cardiac failure was eliminated by the absence of clinical and radiological evidence of any of these and the unusually dense character of the opacities seen on the x-ray. With a view to establishing the diagnosis and excluding all other possibilities, the child was investigated thoroughly and a detailed history obtained. It transpired that this patient had been visiting his uncle, who had tuberculosis and was being treated in another hospital. Being a tuberculosis contact, he was x-rayed (six months prior to his present admission to General Hospital, Kandy) and on the x-ray findings treated with anti-tuberculous drugs for six weeks. Thereafter treatment was stopped. The patient's sisters, brothers and parents were all well. Three other members of the family were x-rayed at Kandy Hospital and their chest x-rays were normal.
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FINDINGS LABORATORY 1. Urine showed profuse pus cells which
cleared later with treatment. 2. Urine for haemosidenn (three samples) : Nil. 3. Urinary calcium (24-hour sample of 1,170 ml): 108.8 mg %. 4. Serum calcium: 9.3 mg %. 5. Serum phosphorus: 2.8 mg % .
D. S . THURAIRAJASINGHAM, B. D. DHARMASENA A I D T. KASTHURIRATNA
6. Alkaline Phosphatase: 24 KA units. 7. WBC: 8,000 per cu mm N - 5 8 % , L -38%, E -4%. 8. ESR: 42 mm. 9. Platelets: 145,000 per cu mm. 10. Hb.: 8.3 gms %; P.C.V.: 30 %; M.C.H.C. 27 %. Red cells appear normocytic with mild degree of hypochromia. There is some increase of eosinophils. 11. Sputum for Tubercle bacilli: Negative (three specimens). Eye Surgeon’s Report Right eye: 6/6. Left eye: 6/6. No evidence of toxosatellites. Macula, disc and periphery normal. Lung Function Tests
(Body temperature and pressure; saturated
with water vapour.) Vital capacity ................................ 1,175 ml 500 ml Tidal volume ................................ Inspiratory reserve volume ............ 450 ml Expiratory reserve volume ............ 225 ml Forced expiratory volume ............ 1,175 d Lung function tests iUe within normal l h t S .
FIGUREZ-Oblique view (Case 1) showing a dense outlining of horizontal 6ssure and cardiac border.
A lung biopsy was refused by the patient’s father. The boy was treated for urinary tract infection and discharged. He was seen again in six months. He was still asymptomatic and a follow-up chest x-ray showed no change at all. Three years later, the. patient is still without symptoms. Although histological proof of the diagnosis was not obtained in this case, the contrast between the clinical and radiological manifestations, the laboratory findings and the three-year follow-up served to confirm the diagnosis. Case 2:
FIGUREI-Postereanterior View Of chest (cast 1). Numerous dense fine pin point opacities over both lungs with overlying liiear and reticular shadowing. The apices are comparatively spared.
M.G.K., female, 24-year-old housewife, married for seven years with no children. She was admitted to the Kandy Hospital in August, 1971, with cough of one year’s duration and dyspnoea for eight months. The cough was unproductive. She had no fever. There was no history of loss of weight or any family history of tubxculosis. She had taken ayurvedic treatment (traditional form of therapy with medicinal herbs and minerals) up to the time of admission to the hospital.. Nd history of exposure to any dust was obtained. There were Other children in the who were alive and well, as were the parents.
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FIGURE3 (Case 2)-Postero-anterior view of chest. Tracheal deviation to the right. Sand-like opacities in apices and upper zones which have run mto d u ence in the mid and lower zones. obscuring the car-diac borderdiagnostic of pulmonary alveolar microlithiasis. On examination, the patient was of small build and weighed 62 pounds. She had no clubbing of fingers or cyanosis. There was no lymphadenopathy. The blood pressure was 120/80 mm Hg.
Respiratory System The trachea was shifted to the right, vocal fremitus and vocal resonance were increased over both sides. Breath sounds were bronchial over the right apex and the mid-zones and diminished over the bases. Scattered crepitations were heard over both sides. Heart sounds were normal. No other abnormality was detected.
LABORATORY FINDINGS
.
1. Serum calcium: 10.0 mg % 2. Serum phosphorus: 1.6 mg 96. 3. ESR: 75 mm. 4. Hb: 9.8 gms %; P.C.V.: 34 %; M.C.H.C.: 29 % .
Red cells appeared normocytic with mild degree of hypochromia. There was mild neutrophil leucocytosis. Australasian Radiology, Vol. XIX, No. 2, June, I975
FIGURE4 (Case 2)-Latmal view showing liaa of calcific density behind the stmum in addition to the other features. 5. Prothrombin time: 19 seumds.
6. Sputum for tubercle bacilli (direct smear): Negative on three occasions. 7. A needle biopsy of lung was attempted, but this was not successful because the pleura and lung felt “hard and gritty” and a satisfactory specimen could not be obtained. Chest x-rays (Figures 3 and 4) showed dense fine mottling of upper zones overrun by linear reticular shadows. Similar shadows ran into confluence in the mid and lower zones rendering these regions totally opaque. The cardiac and mediastinal borders were completely obliterated. The left oblique fissure was thickened. The appearances were diagnostic of pulmonary alveolar microlithiasis. Lung Function Tests Tidal volume .............................. Inspiratory reserve volume .......... Expiratory reserve volume .......... Vital capacity .............................. Forced expiratory volume .......... (0.75 seconds)
212 mI 74 mI 52 mI 287 mI 255 ml 177
D. S. THURAIRAJASINGHAM, B. D. DHARMASENA AND T. KASTHURIRATNA Forced expiratory volume (percentage of V.C.) ..._....... . ...__. 89 (0.75 seconds) Indirect maximum breathing capacity .. . . . . .. .. . . .. _..._..... ... ..._. 10.18 L. All values expressed as STPD (standard temperature and pressure (dry) ). Respiratory rate .._ ... ... ... . ...... . 32 per min Comment: The results indicate a severe restrictive defect. Follow-up in February, 1972 (six months later), showed her condition to have deteriorated slightly. She developed dyspnoea even with the lightest of household duties and a tinge of cyanosis even at rest. A chest x-ray, however, showed no appreciable change. Further followup was not possible as the patient could not be contacted. The hard, gritty feel of the lung and pleura and the characteristic x-ray picture were considered sufficient evidence to make a firm diagnosis of pulmonary alveolar microlithiasis in this case despite absence of histological examination of the lung specimen. DISCUSSION The majority of cases of pulmonary alveolar microlithiasis are diagnosed on routine chest x-ray for an unrelated complaint like the young boy in our series. The radiological picture is characteristic and lung biopsy i s not always necessary for diagnosis. This opinion is also held by Lodge (1964). There is a variable latent period before the onset of symptoms. Our second patient sought medical advice for cough and dyspnoea from the age of 23 years. Cough is a common symptom. In the early stages there is very little expectoration. Haemoptysis is rare. Occasionally microliths may be coughed out. Progressive pulmonary insufficiency may lead to heart failure. The case described by Mansz (1954) had the disease at least for 25 years before she died of congestive cardiac failure at the age of 37 years. Nine out of the sixteen recorded deaths have been from cardiac failure secondary to lung disease. Symptoms are mild or absent before the cardiac complications set in. The contrast between minimal clinical signs and gross radiological changes is a characteristic feature of the disease. The progressive nature of the disease is also shown by the difference in radiological appearance in our two patientsdiscrete foci in the lungs in the 12-year-old and dense, blotchy shadows obscuring the heart and
mediastinum in the older patient. This fact has also been demonstrated by Sosman et a[. (1957). Finger clubbing has been described (Fuleihan et ai. 1969). Neither of our cases had finger clubbing. Rales were heard over their lungs even without heart failure. Spontaneous pneumothoraces and emphysema are known to occur (Sears et al. 1971). Mitral stenosis has been associated with this condition (Kent et al. 1955; Sharp and Danino 1953). This was distinct from haemosiderosis which is a complication of mitral stenosis. Panabokke (1963) states that an open lung biopsy in one of his cases of mitral stenosis subjected to valvotomy showed areas of intraalveolar calcification. This was clearly shown to be different from haemosiderosis by histochemical investigations and was reminiscent of the histological appearance of the condition described as pulmonary microlithiasis (personal communication). Clusters of microliths were demonstrated in the intact bronchial mucosa and thickened fibrosed septa1 walls (Sears et al. 1971). Coetzee ( 1970) noted calcospherites in the lumbar sympathetic chain and probably in the testes of a patient with pulmonary alveolar microlithiasis. The sexes are equally affected. The disease may be familial though this has not been our experience. Familial tendencies occurred in 15 families and in these females outnumbered the males. Aetiology is not known. Six of the reported cases were exposed to mineral or fibre dust. Chinachoti and Tangchai (1957) during routine chest radiography found abnormal pulmonary shadows in nine men who were addicted to inhalation of snuff containing 75 % of organic material, 15.4 % of silcates and 1.6 % of silica. Its calcium content was 9.47 % . This possible aetiological factor has not been noted by other authors including ourselves. Altogether 13 out of the reported cases had possible exposure to potentially irritating inhalants. Kent, Gilbst and Meyer (1955) suggest that it may bc a peculiar response to a variety of insults which include pneumonia and rheumatic fever. Caffrey and Altman (1965) state that this condition might well prove to be an enzyme defect, although the specific nature of the metabolic error has not been worked out. Their cases detected at the age of 1 1 and 12 hours after birth tend to support this theory, as do Coetzee’s findings. The disease should no longer be considered as being confined to the lungs but as a generalized one.
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PULMONARY ALVEOLAR MICROLITHIASIS Pathology In adults the pleura is hard, thickened and granular. The well-defined lines of calcific density corresponding to pleural reflections seen in the x-rays of our cases led us to believe that pleural calcification occurs in this condition. The predominance of the disease is in the basal regions of the lungs, which have a gritty sensation. The harder portions can only be cut with a saw; minute yellow brown granules fall out readily on sectioning the lung tissue. A foreign body giant cell reaction is a prominent feature. Interstitial fibrosis is usually present. Concentric laminated concretions are found in the affected alveoli and rarely in the irregularly thickened septal walls (Sears et aZ. 1971) or even sympathetic chain (Coetzee 1970). The concretions on analysis were shown to contain calcium, phosphorus (Greenberg 1957) with small amounts of magnesium and aluminium. Silica and iron may be found in trace amounts. The alveolar air spaces are obliterated. Occasionally small patches of osseous tissue may replace a few alveoli and enclose several of the calcific bodies (Abdel Hakim et aZ. 1959). Dense fibrous adhesions may be present. Vascular obliteration has also been recorded. In the premature twins autopsied by Caffrey and Altman the lungs did not show any gross macroscopic abnormality. Microliths seen on microscopic examination appeared to be arising from the alveolar septal cells and extruding into the lumen. There was no cellular infiltrate. Evidence of hyaline membrane disease was obtained but they felt this was not causally related to the microlithiasis.
Lung Function Tests Lung function tests are normal in the early stages as seen in our first patient. Later a restrictive type of pulmonary insufficiency develops-well exemplified by the lung function tests on our second case. This leads to reduced lung volume, reduced maximum breathing capacity and diminished compliance. A moderate degree of obstructive emphysema follows shown by an increase in the RV/TLC ratio and a decrease in the maximum rnid-expiratory volume. Gas diffusion across alveolar membrane becomes impaired leading to slight hypoxia at rest and serious hypoxia on exertion due to reduced arterial oxygen tension. Uneven distribution of inspired air is shown by the low nitrogen elimination rate. There is also an uneven Australasian Radiology, Vol.
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distribution of pulmonary blood flow and reduced total and vital capacities. The reduction in vital capacity may not be sufficient to produce dyspnoea with most ordinary activities (Thomson 1959), Viswanathan (1962), Finkbiner et al. (1957) and Sosman et al. (1957). Eventually, however, cor pulmonale, cardiac failure and death ensue. Management According to several workers, the disease is uninfluenced by drugs including the steroid group. Once the diagnosis was made our patients were not given any drugs. Their general conditions were improved during their stay in hospital and they were discharged with the instructions that they should report to the hospital in the event of a respiratory infection or an increase in shortness of breath. We have followed the boy for three years and there has been no deterioration in his condition or in the x-ray pictures. Though our second case showed some increase in dyspnoea and developed cyanosis after six months, her repeat chest films did not reveal any definite increase in the shadowing of the lungs even on very close study. Obviously the progress of the disease is a very slow one. Acknowledgments We are grateful to Dr. K. Dharmadasa for allowing us to study and report hi5 patient (Case 2 ) , Dr. E. S. C. Hettiarachchi for helping us with the lung function tests and Mr. Mahawatte of the Photographic department of the Faculty of Medicine, Peradeniya, for preparing the illustrations.
SUMMARY The clinical and radiological features of pulmonary alveolar microlithiasis have been reviewed and two of our cases, one in the early asymptomatic stage and the other in an incapacitated condition, have been added to cases reported. As far as we are aware, these are the first two cases of pulmonary alveolar microlithiasis reported from Sri Lanka where the diagnosis has been established on clinical and radiological grounds. REFERENCES ‘Abdel Hakim, M., Ei Malfah, S., Hasheem, M.. and Abdel Halim, S. (1959): “Pulmonary alveolar microlithiasis.” Thoruw, 14, 263. ‘Ballikian, J. P.. Fuleihan. F. J. D.. and Nucho, C. K. N. (1968): “Pulmonary alveolar microlithiasis.” Report of 5 cases with special reference to roentgen marifestations.” Amer. J . Roentgenol, 103, 509.
179
D. S . THURAIRAJASINGHAM, B. D. DHARMASENA A N D T. KASTHURIRATNA faffrey, P. R.. and Altman, R. S. (1965): “Pulmonary alveolar microlithiasis occuring in premature twins.” Journal of Paediatrics, 66.758. ‘Chinachoti, N., and Tangchai, P. (1957): “Pulmonary alveolar microlithiasis associated with inhalation of snuff in Thailand.” Dis. Chesr, 32, 687. ‘Coetzee, T. ( 1 970): “Pulmonary alveolar microlithiasis with invoivement of the sympathetic nervous system and gonads.” Thorax, 25, 637. ‘FFinLbiner, R. B., Decker, J. P., and Cooper, D. A. (1957): “Pulmonary alveolar microlithiasis.” Amer. Rev. Tuberc., 75, 122. ‘Friedreich, N. ( 1856a): “Corpora Amylacea in den Lungen.” Virchows Arch. Path. Anaf., 9, 613 and (1856b) 10, 201. ‘Fuleihan, F. J. D., Abboud, R. T., Ballikian, J. P., and Nucho, C. K. N. (1969): “Pulmonary alveolar microlithiasis: Lung function in 5 cases.” Thorax, 24, 84. ‘Greenberg, M. J. (1957): “Miliary shadows in the lungs due to microlithiasis alveolaris pulmonum.” Thorax, 12, 171. ‘“Harbitz, F. (1918): “Extensive calcification of the lungs as a distinct disease.” Arch. Intern. Med., 21, 139. “Kent, G., Gilbert, E. S., and Meyer, H. H. (1955): “Pulmonary microlithiasis.” A.M.A. Arch. Path., 60, 556.
=Lodge, T. (1964): “Recent Advances in Radiology,” 4th edition, pp291-298. London. J. and A. Churchill Ltd. “Manz, A. (1954): “Mikrolithiasis de Lungen.” Mil. Pilzbefall Beitr. Klin. Tuberk., 111, 598. ”Meyer, H. H., Gilbert, E. S., and Kent, G. (1956): “Clinical review of pulmonary microlithiasis.” I . Amer. med. Ass., 161, 1153. ’‘Panabokke, R. G. (1966): “Lung lesiocs seen in rheumatic mitral stenosis.” Ceylon nzed. I . , 2, 59. ’’Puhr, L. (1933): “Mikrolithiasis alveolaris pulmonum.” Virchows Arch. Path. Anat., 290, 156. ”Sambasiva Rao, C., Madhusudhana Rao, Y., Baskara Reddy, D., and Sarada, D. (1968): “Pulmonary alveolar microlithiasis.” I . Ind. med. Ass. 50, 427. ”Sharp, M. E., and Danino, E. A. (1953): “An unusual form of pulmonary calcification; microlithiasis alveolaris pulmonum.” I.Path. Bact., 65, 389. ’Isosman, M. C., Dodd, G. D., Jones, W. D., and Pillmore, G. U. (1957): “The familial occurrence of pulmonary alveolar microlithiasis.” Amer. I . Roentgenol., 77, 941. ‘Thomson, W. B. (1959): “Pulmonary alveolar microlithiasis.” Thorax, 14, 76. “Viswanathan, R. (1 962): “Pulmonary alveolar microlithiasis.” Thorax. 17, 251. nWaters, M. H. (1960): “Microlithiasis alveolaris.” Pulmonum Tubercle (Lond.). 41, 216.
NOTICE
The Royal Australasian College of Radiologists The following candidates were successful in the D.R.A.C.R. Examination March, 1975 RADIODIAGNOSIS, PART I RADIODIAGNOSIS, PARTI1 New South Wales A. Danernan P. K. Macintosh A. Dulimov New South Wales A. J. Scott 0. I. Morgan New South Wales P. L. Travers I. L. Macdonald I. W. Black A.C.T. M. G . Purcell Victoria R. E. Harbig R. K. Morcom South Australia A. D. Pryde E. W. Moriarty Queensland R. Sandaver M. A. Musgrave J. G. Stuckey Queensland T. J. Roberts New Zealand R. W. Lun D. G. Ross New Zealand B. L. G . Arneratunga E. Satguna-Seelan New Zealand D. Fong W. R. Harding RADIOTHERAPY PARTI K. W. Tiver D. A. Thomas
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held in February/
New South Wales New South Wales New South Wales Victoria Victoria Victoria Victoria Victoria Queensland New Zealand New Zealand New Zealand Victoria
Australasian Radiology, Vol. X I X , No. 2, June, 1975
Pulmonary Alveolar Microlithiasis DR. S. THURAIRAJASINGAM, M.B.B.S. (CEY.);D.M.R.D.; F.F.R. (LoND.) DR.B. D. DHARMASENA, M.B.B.S. (CEY.);M.D. (CEY.);M.R.C.P. (LDND.) AND
DR. T. KASTHURIRATNA, M.B.B.S. (CEY.) Government General Hospital, Kandy, Sri Lanku INTRODUCTION Pulmonary alveolar microlithiasis is an extremely rare condition, evidenced by the fact that there are less than 100 cases reported in the world literature. The mean age of reported cases is about 35 years. Only about 14 patients have been diagnosed at 12 years or younger. The youngest documented cases were in premature twins (29 weeks' gestation) at 11 and 12 hours after birth (Caffrey and Altman 1965) and the oldest in a woman of 80 (Sears et al. 1971). The disease is characterized by myriads of dense, finely punctate opacities in the chest x-ray obscuring pulmonary and even mediastinal borders but with a paucity of clinical symptoms. The first account of pulmonary alveolar microlithiasis is attributed to Harbitz ( 1918), though Friedreich ( 1856) described several forms of concentrically laminated bodies found in the lung parenchyma. The name microlithiasis alveolaris pulmonarum was suggested by Puhr in 1933. CASEREPORTS Case 1: S.H., male, 12 years, was admitted to the General Hospital, Kandy, on 26/4/ 1971 for a urinary tract infection. He complained of fever, frequency of micturition and turbidity of urine. He had a slight cough of six days' duration. He had no finger clubbing. A chest x-ray was requested as a few crepitations were heard over the lungs. The x-rays (Figures I and 2) revealed bilateral diffuse punctate opacities compatible with pulmonary alveolar microlithiasis with pleural calcification. The heart was normal in appearance. Miliary tuberculosis and haemosiderosis (primary or secondary) were considered in the differential diagnosis but presumed to be less likely on the following grounds.
(a) The soft millet seed type of d i a r y mottling of the lungs seen in d i a r y tuberculosis did not fit into the patient's x-ray picture. Further, the general condition of the patient was good. b) The lack of the characteristic filigree pattern and the high density of the punctate opacities ruled out the possibility of ideopathic (primary) haemosiderosis. c) Secondary haemosiderosis caused by mitral stenosis, angiomatous maiformations of lungs and cardiac failure was eliminated by the absence of clinical and radiological evidence of any of these and the unusually dense character of the opacities seen on the x-ray. With a view to establishing the diagnosis and excluding all other possibilities, the child was investigated thoroughly and a detailed history obtained. It transpired that this patient had been visiting his uncle, who had tuberculosis and was being treated in another hospital. Being a tuberculosis contact, he was x-rayed (six months prior to his present admission to General Hospital, Kandy) and on the x-ray findings treated with anti-tuberculous drugs for six weeks. Thereafter treatment was stopped. The patient's sisters, brothers and parents were all well. Three other members of the family were x-rayed at Kandy Hospital and their chest x-rays were normal.
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FINDINGS LABORATORY 1. Urine showed profuse pus cells which
cleared later with treatment. 2. Urine for haemosidenn (three samples) : Nil. 3. Urinary calcium (24-hour sample of 1,170 ml): 108.8 mg %. 4. Serum calcium: 9.3 mg %. 5. Serum phosphorus: 2.8 mg % .
D. S . THURAIRAJASINGHAM, B. D. DHARMASENA A I D T. KASTHURIRATNA
6. Alkaline Phosphatase: 24 KA units. 7. WBC: 8,000 per cu mm N - 5 8 % , L -38%, E -4%. 8. ESR: 42 mm. 9. Platelets: 145,000 per cu mm. 10. Hb.: 8.3 gms %; P.C.V.: 30 %; M.C.H.C. 27 %. Red cells appear normocytic with mild degree of hypochromia. There is some increase of eosinophils. 11. Sputum for Tubercle bacilli: Negative (three specimens). Eye Surgeon’s Report Right eye: 6/6. Left eye: 6/6. No evidence of toxosatellites. Macula, disc and periphery normal. Lung Function Tests
(Body temperature and pressure; saturated
with water vapour.) Vital capacity ................................ 1,175 ml 500 ml Tidal volume ................................ Inspiratory reserve volume ............ 450 ml Expiratory reserve volume ............ 225 ml Forced expiratory volume ............ 1,175 d Lung function tests iUe within normal l h t S .
FIGUREZ-Oblique view (Case 1) showing a dense outlining of horizontal 6ssure and cardiac border.
A lung biopsy was refused by the patient’s father. The boy was treated for urinary tract infection and discharged. He was seen again in six months. He was still asymptomatic and a follow-up chest x-ray showed no change at all. Three years later, the. patient is still without symptoms. Although histological proof of the diagnosis was not obtained in this case, the contrast between the clinical and radiological manifestations, the laboratory findings and the three-year follow-up served to confirm the diagnosis. Case 2:
FIGUREI-Postereanterior View Of chest (cast 1). Numerous dense fine pin point opacities over both lungs with overlying liiear and reticular shadowing. The apices are comparatively spared.
M.G.K., female, 24-year-old housewife, married for seven years with no children. She was admitted to the Kandy Hospital in August, 1971, with cough of one year’s duration and dyspnoea for eight months. The cough was unproductive. She had no fever. There was no history of loss of weight or any family history of tubxculosis. She had taken ayurvedic treatment (traditional form of therapy with medicinal herbs and minerals) up to the time of admission to the hospital.. Nd history of exposure to any dust was obtained. There were Other children in the who were alive and well, as were the parents.
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FIGURE3 (Case 2)-Postero-anterior view of chest. Tracheal deviation to the right. Sand-like opacities in apices and upper zones which have run mto d u ence in the mid and lower zones. obscuring the car-diac borderdiagnostic of pulmonary alveolar microlithiasis. On examination, the patient was of small build and weighed 62 pounds. She had no clubbing of fingers or cyanosis. There was no lymphadenopathy. The blood pressure was 120/80 mm Hg.
Respiratory System The trachea was shifted to the right, vocal fremitus and vocal resonance were increased over both sides. Breath sounds were bronchial over the right apex and the mid-zones and diminished over the bases. Scattered crepitations were heard over both sides. Heart sounds were normal. No other abnormality was detected.
LABORATORY FINDINGS
.
1. Serum calcium: 10.0 mg % 2. Serum phosphorus: 1.6 mg 96. 3. ESR: 75 mm. 4. Hb: 9.8 gms %; P.C.V.: 34 %; M.C.H.C.: 29 % .
Red cells appeared normocytic with mild degree of hypochromia. There was mild neutrophil leucocytosis. Australasian Radiology, Vol. XIX, No. 2, June, I975
FIGURE4 (Case 2)-Latmal view showing liaa of calcific density behind the stmum in addition to the other features. 5. Prothrombin time: 19 seumds.
6. Sputum for tubercle bacilli (direct smear): Negative on three occasions. 7. A needle biopsy of lung was attempted, but this was not successful because the pleura and lung felt “hard and gritty” and a satisfactory specimen could not be obtained. Chest x-rays (Figures 3 and 4) showed dense fine mottling of upper zones overrun by linear reticular shadows. Similar shadows ran into confluence in the mid and lower zones rendering these regions totally opaque. The cardiac and mediastinal borders were completely obliterated. The left oblique fissure was thickened. The appearances were diagnostic of pulmonary alveolar microlithiasis. Lung Function Tests Tidal volume .............................. Inspiratory reserve volume .......... Expiratory reserve volume .......... Vital capacity .............................. Forced expiratory volume .......... (0.75 seconds)
212 mI 74 mI 52 mI 287 mI 255 ml 177
D. S. THURAIRAJASINGHAM, B. D. DHARMASENA AND T. KASTHURIRATNA Forced expiratory volume (percentage of V.C.) ..._....... . ...__. 89 (0.75 seconds) Indirect maximum breathing capacity .. . . . . .. .. . . .. _..._..... ... ..._. 10.18 L. All values expressed as STPD (standard temperature and pressure (dry) ). Respiratory rate .._ ... ... ... . ...... . 32 per min Comment: The results indicate a severe restrictive defect. Follow-up in February, 1972 (six months later), showed her condition to have deteriorated slightly. She developed dyspnoea even with the lightest of household duties and a tinge of cyanosis even at rest. A chest x-ray, however, showed no appreciable change. Further followup was not possible as the patient could not be contacted. The hard, gritty feel of the lung and pleura and the characteristic x-ray picture were considered sufficient evidence to make a firm diagnosis of pulmonary alveolar microlithiasis in this case despite absence of histological examination of the lung specimen. DISCUSSION The majority of cases of pulmonary alveolar microlithiasis are diagnosed on routine chest x-ray for an unrelated complaint like the young boy in our series. The radiological picture is characteristic and lung biopsy i s not always necessary for diagnosis. This opinion is also held by Lodge (1964). There is a variable latent period before the onset of symptoms. Our second patient sought medical advice for cough and dyspnoea from the age of 23 years. Cough is a common symptom. In the early stages there is very little expectoration. Haemoptysis is rare. Occasionally microliths may be coughed out. Progressive pulmonary insufficiency may lead to heart failure. The case described by Mansz (1954) had the disease at least for 25 years before she died of congestive cardiac failure at the age of 37 years. Nine out of the sixteen recorded deaths have been from cardiac failure secondary to lung disease. Symptoms are mild or absent before the cardiac complications set in. The contrast between minimal clinical signs and gross radiological changes is a characteristic feature of the disease. The progressive nature of the disease is also shown by the difference in radiological appearance in our two patientsdiscrete foci in the lungs in the 12-year-old and dense, blotchy shadows obscuring the heart and
mediastinum in the older patient. This fact has also been demonstrated by Sosman et a[. (1957). Finger clubbing has been described (Fuleihan et ai. 1969). Neither of our cases had finger clubbing. Rales were heard over their lungs even without heart failure. Spontaneous pneumothoraces and emphysema are known to occur (Sears et al. 1971). Mitral stenosis has been associated with this condition (Kent et al. 1955; Sharp and Danino 1953). This was distinct from haemosiderosis which is a complication of mitral stenosis. Panabokke (1963) states that an open lung biopsy in one of his cases of mitral stenosis subjected to valvotomy showed areas of intraalveolar calcification. This was clearly shown to be different from haemosiderosis by histochemical investigations and was reminiscent of the histological appearance of the condition described as pulmonary microlithiasis (personal communication). Clusters of microliths were demonstrated in the intact bronchial mucosa and thickened fibrosed septa1 walls (Sears et al. 1971). Coetzee ( 1970) noted calcospherites in the lumbar sympathetic chain and probably in the testes of a patient with pulmonary alveolar microlithiasis. The sexes are equally affected. The disease may be familial though this has not been our experience. Familial tendencies occurred in 15 families and in these females outnumbered the males. Aetiology is not known. Six of the reported cases were exposed to mineral or fibre dust. Chinachoti and Tangchai (1957) during routine chest radiography found abnormal pulmonary shadows in nine men who were addicted to inhalation of snuff containing 75 % of organic material, 15.4 % of silcates and 1.6 % of silica. Its calcium content was 9.47 % . This possible aetiological factor has not been noted by other authors including ourselves. Altogether 13 out of the reported cases had possible exposure to potentially irritating inhalants. Kent, Gilbst and Meyer (1955) suggest that it may bc a peculiar response to a variety of insults which include pneumonia and rheumatic fever. Caffrey and Altman (1965) state that this condition might well prove to be an enzyme defect, although the specific nature of the metabolic error has not been worked out. Their cases detected at the age of 1 1 and 12 hours after birth tend to support this theory, as do Coetzee’s findings. The disease should no longer be considered as being confined to the lungs but as a generalized one.
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PULMONARY ALVEOLAR MICROLITHIASIS Pathology In adults the pleura is hard, thickened and granular. The well-defined lines of calcific density corresponding to pleural reflections seen in the x-rays of our cases led us to believe that pleural calcification occurs in this condition. The predominance of the disease is in the basal regions of the lungs, which have a gritty sensation. The harder portions can only be cut with a saw; minute yellow brown granules fall out readily on sectioning the lung tissue. A foreign body giant cell reaction is a prominent feature. Interstitial fibrosis is usually present. Concentric laminated concretions are found in the affected alveoli and rarely in the irregularly thickened septal walls (Sears et aZ. 1971) or even sympathetic chain (Coetzee 1970). The concretions on analysis were shown to contain calcium, phosphorus (Greenberg 1957) with small amounts of magnesium and aluminium. Silica and iron may be found in trace amounts. The alveolar air spaces are obliterated. Occasionally small patches of osseous tissue may replace a few alveoli and enclose several of the calcific bodies (Abdel Hakim et aZ. 1959). Dense fibrous adhesions may be present. Vascular obliteration has also been recorded. In the premature twins autopsied by Caffrey and Altman the lungs did not show any gross macroscopic abnormality. Microliths seen on microscopic examination appeared to be arising from the alveolar septal cells and extruding into the lumen. There was no cellular infiltrate. Evidence of hyaline membrane disease was obtained but they felt this was not causally related to the microlithiasis.
Lung Function Tests Lung function tests are normal in the early stages as seen in our first patient. Later a restrictive type of pulmonary insufficiency develops-well exemplified by the lung function tests on our second case. This leads to reduced lung volume, reduced maximum breathing capacity and diminished compliance. A moderate degree of obstructive emphysema follows shown by an increase in the RV/TLC ratio and a decrease in the maximum rnid-expiratory volume. Gas diffusion across alveolar membrane becomes impaired leading to slight hypoxia at rest and serious hypoxia on exertion due to reduced arterial oxygen tension. Uneven distribution of inspired air is shown by the low nitrogen elimination rate. There is also an uneven Australasian Radiology, Vol.
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distribution of pulmonary blood flow and reduced total and vital capacities. The reduction in vital capacity may not be sufficient to produce dyspnoea with most ordinary activities (Thomson 1959), Viswanathan (1962), Finkbiner et al. (1957) and Sosman et al. (1957). Eventually, however, cor pulmonale, cardiac failure and death ensue. Management According to several workers, the disease is uninfluenced by drugs including the steroid group. Once the diagnosis was made our patients were not given any drugs. Their general conditions were improved during their stay in hospital and they were discharged with the instructions that they should report to the hospital in the event of a respiratory infection or an increase in shortness of breath. We have followed the boy for three years and there has been no deterioration in his condition or in the x-ray pictures. Though our second case showed some increase in dyspnoea and developed cyanosis after six months, her repeat chest films did not reveal any definite increase in the shadowing of the lungs even on very close study. Obviously the progress of the disease is a very slow one. Acknowledgments We are grateful to Dr. K. Dharmadasa for allowing us to study and report hi5 patient (Case 2 ) , Dr. E. S. C. Hettiarachchi for helping us with the lung function tests and Mr. Mahawatte of the Photographic department of the Faculty of Medicine, Peradeniya, for preparing the illustrations.
SUMMARY The clinical and radiological features of pulmonary alveolar microlithiasis have been reviewed and two of our cases, one in the early asymptomatic stage and the other in an incapacitated condition, have been added to cases reported. As far as we are aware, these are the first two cases of pulmonary alveolar microlithiasis reported from Sri Lanka where the diagnosis has been established on clinical and radiological grounds. REFERENCES ‘Abdel Hakim, M., Ei Malfah, S., Hasheem, M.. and Abdel Halim, S. (1959): “Pulmonary alveolar microlithiasis.” Thoruw, 14, 263. ‘Ballikian, J. P.. Fuleihan. F. J. D.. and Nucho, C. K. N. (1968): “Pulmonary alveolar microlithiasis.” Report of 5 cases with special reference to roentgen marifestations.” Amer. J . Roentgenol, 103, 509.
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D. S . THURAIRAJASINGHAM, B. D. DHARMASENA A N D T. KASTHURIRATNA faffrey, P. R.. and Altman, R. S. (1965): “Pulmonary alveolar microlithiasis occuring in premature twins.” Journal of Paediatrics, 66.758. ‘Chinachoti, N., and Tangchai, P. (1957): “Pulmonary alveolar microlithiasis associated with inhalation of snuff in Thailand.” Dis. Chesr, 32, 687. ‘Coetzee, T. ( 1 970): “Pulmonary alveolar microlithiasis with invoivement of the sympathetic nervous system and gonads.” Thorax, 25, 637. ‘FFinLbiner, R. B., Decker, J. P., and Cooper, D. A. (1957): “Pulmonary alveolar microlithiasis.” Amer. Rev. Tuberc., 75, 122. ‘Friedreich, N. ( 1856a): “Corpora Amylacea in den Lungen.” Virchows Arch. Path. Anaf., 9, 613 and (1856b) 10, 201. ‘Fuleihan, F. J. D., Abboud, R. T., Ballikian, J. P., and Nucho, C. K. N. (1969): “Pulmonary alveolar microlithiasis: Lung function in 5 cases.” Thorax, 24, 84. ‘Greenberg, M. J. (1957): “Miliary shadows in the lungs due to microlithiasis alveolaris pulmonum.” Thorax, 12, 171. ‘“Harbitz, F. (1918): “Extensive calcification of the lungs as a distinct disease.” Arch. Intern. Med., 21, 139. “Kent, G., Gilbert, E. S., and Meyer, H. H. (1955): “Pulmonary microlithiasis.” A.M.A. Arch. Path., 60, 556.
=Lodge, T. (1964): “Recent Advances in Radiology,” 4th edition, pp291-298. London. J. and A. Churchill Ltd. “Manz, A. (1954): “Mikrolithiasis de Lungen.” Mil. Pilzbefall Beitr. Klin. Tuberk., 111, 598. ”Meyer, H. H., Gilbert, E. S., and Kent, G. (1956): “Clinical review of pulmonary microlithiasis.” I . Amer. med. Ass., 161, 1153. ’‘Panabokke, R. G. (1966): “Lung lesiocs seen in rheumatic mitral stenosis.” Ceylon nzed. I . , 2, 59. ’’Puhr, L. (1933): “Mikrolithiasis alveolaris pulmonum.” Virchows Arch. Path. Anat., 290, 156. ”Sambasiva Rao, C., Madhusudhana Rao, Y., Baskara Reddy, D., and Sarada, D. (1968): “Pulmonary alveolar microlithiasis.” I . Ind. med. Ass. 50, 427. ”Sharp, M. E., and Danino, E. A. (1953): “An unusual form of pulmonary calcification; microlithiasis alveolaris pulmonum.” I.Path. Bact., 65, 389. ’Isosman, M. C., Dodd, G. D., Jones, W. D., and Pillmore, G. U. (1957): “The familial occurrence of pulmonary alveolar microlithiasis.” Amer. I . Roentgenol., 77, 941. ‘Thomson, W. B. (1959): “Pulmonary alveolar microlithiasis.” Thorax, 14, 76. “Viswanathan, R. (1 962): “Pulmonary alveolar microlithiasis.” Thorax. 17, 251. nWaters, M. H. (1960): “Microlithiasis alveolaris.” Pulmonum Tubercle (Lond.). 41, 216.
NOTICE
The Royal Australasian College of Radiologists The following candidates were successful in the D.R.A.C.R. Examination March, 1975 RADIODIAGNOSIS, PART I RADIODIAGNOSIS, PARTI1 New South Wales A. Danernan P. K. Macintosh A. Dulimov New South Wales A. J. Scott 0. I. Morgan New South Wales P. L. Travers I. L. Macdonald I. W. Black A.C.T. M. G . Purcell Victoria R. E. Harbig R. K. Morcom South Australia A. D. Pryde E. W. Moriarty Queensland R. Sandaver M. A. Musgrave J. G. Stuckey Queensland T. J. Roberts New Zealand R. W. Lun D. G. Ross New Zealand B. L. G . Arneratunga E. Satguna-Seelan New Zealand D. Fong W. R. Harding RADIOTHERAPY PARTI K. W. Tiver D. A. Thomas
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held in February/
New South Wales New South Wales New South Wales Victoria Victoria Victoria Victoria Victoria Queensland New Zealand New Zealand New Zealand Victoria
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