Download PDF
Semin Respir Crit Care Med 2002; 23(2): 103-114
DOI: 10.1055/s-2002-25300
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Pulmonary Alveolar Microlithiasis

Udaya B.S. Prakash
  • Pulmonary, Critical Care, and Internal Medicine, Mayo Medical School and Mayo Medical Center, Rochester, Minnesota
Further Information

Publication History

Publication Date:
24 April 2002 (online)

    Permissions and Reprints

ABSTRACT

Pulmonary alveolar microlithiasis is a rare pulmonary disorder of unknown etiology characterized by intra-alveolar development and deposition of microliths or calcispherytes consisting of calcium phosphate. Approximately 400 cases of this rare disorder have been described in the literature. The age range of patients is from newborn to 80 years, with a mean age at diagnosis of about 35 years. No sexual predominance has been noted. In about half of the reported cases, a familial pattern has been found, with the disease usually affecting the siblings. The progression of the disease is generally very slow, some patients having been followed up for more than 30 years without evidence of change. Pulmonary function studies demonstrate a tendency toward a restrictive pattern. Chest roentgenography, high-resolution computed tomographic chest scan, and technetium-99m lung scan are useful in the diagnosis. A definitive diagnosis can be established by bronchoalveolar lavage or lung biopsy. There is no known therapy for this unusual disorder. In patients who develop progressive respiratory insufficiency and cor pulmonale, lung transplantations have been performed.

KEYWORD

Pulmonary calcification - micronodules - microlithiasis - microliths - diffuse calcispherytes - technetium-99m diphosphonate lung scan

REFERENCES

  • 1 Friedreich N. Corpora amylacea in den lungen.  Virchows Arch [Path Anat] . 1856;  9 613-618
    PubMedGoogle Scholar
  • 2 Harbitz F. Extensive calcification of the lungs as a distinct disease.  Arch Intern Med . 1918;  21 139-146
    PubMedGoogle Scholar
  • 3 Puhr L. Mikrolithiasis alveolaris pulmonum.  Virchows Arch [Path Anat] . 1933;  290 156-160
    PubMedGoogle Scholar
  • 4 Sosman M C, Dodd G D, Jones W D, Pillmore G U. The familial occurrence of pulmonary alveolar microlithiasis.  Am J Roentgenol . 1957;  77 947-1012
    PubMedGoogle Scholar
  • 5 Prakash U BS, Barham S S, Rosenow III C E, Brown M L, Payne W S. Pulmonary alveolar microlithiasis: a review including ultrastructural and pulmonary function studies.  Mayo Clin Proc . 1983;  58 290-300
    PubMedGoogle Scholar
  • 6 Mariotta S, Guidi L, Papale M, Ricci A, Bisetti A. Pulmonary alveolar microlithiasis: review of Italian reports.  Eur J Epidemiol . 1997;  13 587-590
    CrossrefPubMedGoogle Scholar
  • 7 Miro J M, Moreno A, Coca A, Segura F, Soriano E. Pulmonary alveolar microlithiasis with an unusual radiological pattern.  Br J Dis Chest . 1982;  76 91-96
    PubMedGoogle Scholar
  • 8 Gubbawy H. Pulmonary alveolar microlithiasis.  Pneumologie . 2001;  55 149-151
    Article in Thieme ConnectPubMedGoogle Scholar
  • 9 Ucan E S, Keyf A I, Aydilek R. Pulmonary alveolar microlithiasis: review of Turkish reports.  Thorax . 1993;  48 171-173
    PubMedGoogle Scholar
  • 10 Senyigit A, Yaramis A, Gurkan F. Pulmonary alveolar microlithiasis: a rare familial inheritance with report of six cases in a family: contribution of six new cases to the number of case reports in Turkey.  Respiration . 2001;  68 204-209
    CrossrefPubMedGoogle Scholar
  • 11 Erelel M, Kiyan E, Cuhadaroglu C, Yilmazbayhan D, Kilicaslan Z. Pulmonary alveolar lithiasis in two siblings.  Respiration . 2001;  68 327-330
    CrossrefPubMedGoogle Scholar
  • 12 Jakob S M, Kraft R, Im Hof V. Three siblings with identical, rare pneumopathy.  Schweiz Med Wochenschr . 1997;  127 1528-1530
    PubMedGoogle Scholar
  • 13 Drinkovic I, Strohal K, Sabljica B. Mikrolithiasis alveolaris pulmonum.  Fortschr Geb Rontgenostr . 1962;  97 180-185
    PubMedGoogle Scholar
  • 14 Mittal B R, Gupta D, Jindal S K. Radioisotope bone scanning in pulmonary alveolar microlithiasis.  Clin Nucl Med . 2000;  25 474-475
    CrossrefPubMedGoogle Scholar
  • 15 Chatterji R, Gaude G S, Patil P V. Pulmonary alveolar microlithiasis: diagnosed by sputum examination and transbronchial biopsy.  Indian J Chest Dis Allied Sci . 1997;  39 263-267
    PubMedGoogle Scholar
  • 16 Arslan A, Yalin T, Akan H, Belet U. Pulmonary alveolar microlithiasis associated with calcifications in the seminal vesicles.  J Belge Radiol . 1996;  79 118-119
    PubMedGoogle Scholar
  • 17 Ritchie D A, O'Connor S A, McGivern D. An unusual presentation of pulmonary alveolar microlithiasis and diaphyseal aclasia.  Br J Radiol . 1992;  65 178-181
    PubMedGoogle Scholar
  • 18 Emri S, Coplu L, Selcuk Z T, Sahin A A, Baris Y I. Hypertrophic pulmonary osteoarthropathy in a patient with pulmonary alveolar microlithiasis.  Thorax . 1991;  46 145-146
    PubMedGoogle Scholar
  • 19 Ratjen F A, Schoenfeld B, Wiesemann H G. Pulmonary alveolar microlithiasis and lymphocytic interstitial pneumonitis in a ten year old girl.  Eur Respir J . 1992;  5 1283-1285
    PubMedGoogle Scholar
  • 20 Pant K, Shah A, Mathur R K, Chhabra S K, Jain S K. Pulmonary alveolar microlithiasis with pleural calcification and nephrolithiasis.  Chest . 1990;  98 245-246
    CrossrefPubMedGoogle Scholar
  • 21 Badger T L, Gottlieb L, Gaensler E A. Pulmonary alveolar microlithiasis, or calcinosis of the lungs.  N Eng J Med . 1955;  253 709-715
    PubMedGoogle Scholar
  • 22 Hasan S, Cevat O, Sami C. Pulmonary alveolar microlithiasis with pectus excavatum: case report.  Indian J Med Sci . 1994;  48 199-200
    PubMedGoogle Scholar
  • 23 Sharp M E, Danino E A. An unusual form of pulmonary calcification: ``microlithiasis alveolaris pulmonum''.  J Pathol Bacteriol . 1953;  65 389-399
    CrossrefPubMedGoogle Scholar
  • 24 Portnoy L M, Amadeo B, Henningar G R. Pulmonary alveolar microlithiasis: an unusual case (associated with milk-alkali syndrome).  Am J Clin Pathol . 1964;  41 194-201
    PubMedGoogle Scholar
  • 25 Varma B N. Pulmonary alveolar microlithiasis in a child of thirteen years.  Br J Dis Chest . 1963;  57 213-215
    PubMedGoogle Scholar
  • 26 Bunger P, Fassbender C W, Schutze G. Zur microlithiasis alveolaris pulmonum.  Fortschr Geb Rontgenostr . 1962;  97 775-779
    PubMedGoogle Scholar
  • 27 Richardson J, Slovis B, Miller G, Dummer S. Development of pulmonary alveolar microlithiasis in a renal transplant recipient.  Transplantation . 1995;  59 1056-1057
    PubMedGoogle Scholar
  • 28 Gomez G E, Lichtemberger E, Santamaria A. Familial pulmonary alveolar microlithiasis: four cases from Columbia, S.A. Is microlithiasis also an environmental disease?.  Radiology . 1959;  72 550-561
    PubMedGoogle Scholar
  • 29 Nouh M S. Is the desert lung syndrome (nonoccupational dust pneumoconiosis) a variant of pulmonary alveolar microlithiasis?.  <~>Report of four cases with review of the literature. Respiration . 1989;  55 122-126
    PubMedGoogle Scholar
  • 30 Chinachoti N, Tangchai P. Pulmonary alveolar microlithiasis associated with the inhalation of snuff in Thailand.  . 1957;  32 687-689
    PubMedGoogle Scholar
  • 31 Tao L C. Microliths in sputum specimens and their relationship to pulmonary alveolar microlithiasis.  Am J Clin Pathol . 1978;  69 482-485
    PubMedGoogle Scholar
  • 32 Brix A E, Latimer K S, Moore G E, Roberts R E. Pulmonary alveolar microlithiasis and ossification in a dog.  Vet Pathol . 1994;  31 382-385
    PubMedGoogle Scholar
  • 33 Liu S K, Suter P F, Ettinger S. Pulmonary alveolar microlithiasis with ruptured chordae tendineae in mitral and tricuspid valves in a dog.  J Am Vet Med Assoc . 1969;  155 1692-1703
    PubMedGoogle Scholar
  • 34 Kelly D F. Pulmonary alveolar microlithiasis in the orang-utan (Pongo pygmaeus).  Acta Zool Pathol Antverp . 1976;  66 53-57
    PubMedGoogle Scholar
  • 35 Bush M, James Jr E A, Montali R J, Stitik F P. Pulmonary alveolar microlithiasis in a binturong (Arctictis binturong): a case report.  Am Vet Radiol Soc . 1976;  17 157-160
    PubMedGoogle Scholar
  • 36 Madarame H, Kumagai M, Suzuki J, Watanabe A, Konno S. Pulmonary alveolar microlithiasis in Afghan pika (Ochotona rufescens rufescens).  Vet Pathol . 1989;  26 333-337
    PubMedGoogle Scholar
  • 37 Caffrey P R, Altman R S. Pulmonary alveolar microlithiasis occurring in premature twins.  J Pediatr . 1965;  66 758-763
    CrossrefPubMedGoogle Scholar
  • 38 Kawakami M, Sato S, Takishima T. Electron microscopic studies on pulmonary alveolar microlithiasis.  Tohoku J Exp Med . 1978;  126 343-361
    CrossrefPubMedGoogle Scholar
  • 39 Moran C A, Hochholzer L, Hasleton P S, Johnson F B, Koss M N. Pulmonary alveolar microlithiasis: a clinicopathologic and chemical analysis of seven cases.  Arch Pathol Lab Med . 1997;  121 607-611
    PubMedGoogle Scholar
  • 40 Sears M R, Chang A R, Taylor A J. Pulmonary alveolar microlithiasis.  Thorax . 1971;  26 704-711
    PubMedGoogle Scholar
  • 41 Coetzee T. Pulmonary alveolar microlithiasis with involvement of the sympathetic nervous system and gonads.  Thorax . 1970;  25 637-642
    CrossrefPubMedGoogle Scholar
  • 42 Barnard N J, Crocker P R, Blainey A D, Davies R J, Ell S R, Levison D A. Pulmonary alveolar microlithiasis: a new analytical approach.  Histopathology . 1987;  11 639-645
    PubMedGoogle Scholar
  • 43 Hawass N D, Noah M S. Pulmonary alveolar microlithiasis.  Eur J Respir Dis . 1986;  69 199-203
    PubMedGoogle Scholar
  • 44 Bab I, Rosenmann E, Ne'eman Z, Sela J. The occurrence of extracellular matrix vesicles in pulmonary alveolar microlithiasis.  Virchows Arch A Pathol Anat Histol . 1981;  391 357-361
    CrossrefPubMedGoogle Scholar
  • 45 Kino T, Kohara Y, Tsuji S. Pulmonary alveolar microlithiasis: a report of two young sisters.  An Rev Resp Dis . 1972;  105 105-110
    PubMedGoogle Scholar
  • 46 Pracyk J B, Simonson S G, Young S L, Ghio A J, Roggli V L, Piantadosi C A. Composition of lung lavage in pulmonary alveolar microlithiasis.  Respiration . 1996;  63 254-260
    PubMedGoogle Scholar
  • 47 Barnard N J, Crocker P R, Blainey A D, Davies R J, Ell S R, Levison D A. Pulmonary alveolar microlithiasis: a new analytical approach.  Histopathology . 1987;  11 639-645
    PubMedGoogle Scholar
  • 48 Cale W F, Petsonk E L, Boyd C B. Transbronchial biopsy of pulmonary alveolar microlithiasis.  Arch Intern Med . 1983;  143 358-359
    CrossrefPubMedGoogle Scholar
  • 49 Thurairajasingam S, Dharmasena B D, Kasthuriratna T. Pulmonary alveolar microlithiasis.  Australas Radiol . 1975;  19 175-180
    PubMedGoogle Scholar
  • 50 Volle E, Kaufmann H J. Pulmonary alveolar microlithiasis in pediatric patients: review of the world literature and two new observations.  Pediatr Radiol . 1987;  17 439-442
    PubMedGoogle Scholar
  • 51 Turktas I, Saribas S, Balkanci F. Pulmonary alveolar microlithiasis presenting with chronic cough.  Postgrad Med J . 1993;  69 70-71
    PubMedGoogle Scholar
  • 52 Brown M L, Swee R G, Olson R J, Bender C E. Pulmonary uptake of 99mTc-diphosphonate in alveolar microlithiasis.  Am J Roentgenol . 1978;  131 703-704
    PubMedGoogle Scholar
  • 53 Korn M A, Schurawitzki H, Klepetko W, Burghuber O C. Pulmonary alveolar microlithiasis: findings on high-resolution CT.  Am J Roentgenol . 1992;  158 981-982
    PubMedGoogle Scholar
  • 54 Waters M H. Microlithiasis alveolaris pulmonum.  Tubercle . 1960;  41 276-280
    PubMedGoogle Scholar
  • 55 Winzelberg G G, Boller M, Sachs M, Weinberg J. CT evaluation of pulmonary alveolar microlithiasis.  J Comput Assist Tomogr . 1984;  8 1029-1031
    PubMedGoogle Scholar
  • 56 Stamatis G, Zerkowski H R, Doetsch N, Greschuchna D, Konietzko N, Reidemeister J C. Sequential bilateral lung transplantation for pulmonary alveolar microlithiasis.  Ann Thorac Surg . 1993;  56 972-975
    PubMedGoogle Scholar
  • 57 Freiberg D B, Young I H, Laks L, Regnis J A, Lehrhaft B, Sullivan C E. Improvement in gas exchange with nasal continuous positive airway pressure in pulmonary alveolar microlithiasis.  Am Rev Respir Dis . 1992;  145 1215-1216
    PubMedGoogle Scholar
  • 58 Brown J, Leon W, Felton C. Hemodynamic and pulmonary studies in pulmonary alveolar microlithiasis.  Am J Med . 1984;  77 176-178
    CrossrefPubMedGoogle Scholar
  • 59 Fuleihan F JD, Abboud R T, Balikain J P, Nucho C KN. Pulmonary alveolar microlithiasis: lung function in five cases.  Thorax . 1969;  24 84-90
    PubMedGoogle Scholar
  • 60 Saputo V, Zocchi M, Mancosu M, Bonaldi U, Croce P. Pulmonary alveolar microlithiasis: a case report with a discussion of differential diagnosis.  Helv Paediatr Acta . 1979;  34 245-255
    PubMedGoogle Scholar
  • 61 Weinstein D S. Pulmonary sarcoidosis: calcified micronodular pattern simulating pulmonary alveolar microlithiasis.  J Thor Imag . 199;  14 218-220
    PubMedGoogle Scholar
  • 62 Lee Y C, Milne D, Rea H H. Pulmonary alveolar microlithiasis: an alternative diagnosis to miliary tuberculosis.  Aust N Z J Med . 1997;  27 336-237
    PubMedGoogle Scholar
  • 63 Turktas H, Ozturk C, Guven M, Ugur P, Erzen C. Pulmonary alveolar microlithiasis with the absence of technetium-99m MDP uptake of lungs.  Clin Nucl Med . 1988;  13 883-885
    PubMedGoogle Scholar
  • 64 Helbich T H, Wojnarovsky C, Wunderbaldinger P, Heinz-Peer G, Eichler I, Herold C J. Pulmonary alveolar microlithiasis in children: radiographic and high-resolution CT findings.  Am J Roentgenol . 1997;  168 63-65
    PubMedGoogle Scholar
  • 65 Balikian J P, Fuleihan F JD, Nucho C N. Pulmonary alveolar microlithiasis: report on five cases with special reference to roentgen manifestations.  Am J Roentgenol . 1968;  103 509-518
    PubMedGoogle Scholar
  • 66 Melamed J W, Sostman H D, Ravin C E. Interstitial thickening in pulmonary alveolar microlithiasis: an underappreciated finding.  J Thorac Imaging . 1994;  9 126-128
    PubMedGoogle Scholar
  • 67 Hoshino H, Koba H, Inomata S. Pulmonary alveolar microlithiasis: high-resolution CT and MR findings.  Comput Assist Tomogr . 1998;  22 245-248
    PubMedGoogle Scholar
  • 68 Cheong W Y, Wang Y T, Tan L K, Cheah E S, Poh S C. Pulmonary alveolar microlithiasis.  Australas Radiol . 1988;  32 401-404
    PubMedGoogle Scholar
  • 69 Felson B. The roentgen diagnosis of disseminated pulmonary alveolar diseases.  Semin Roentgenol . 1967;  2 3-9
    PubMedGoogle Scholar
  • 70 Cluzel P, Grenier P, Bernadac P, Laurent F, Picard J D. Pulmonary alveolar microlithiasis: CT findings.  J Comput Assist Tomogr . 1991;  15 938-942
    PubMedGoogle Scholar
  • 71 Kang E Y, Grenier P, Laurent F, Muller N L. Interlobular septal thickening: patterns at high-resolution computed tomography.  J Thorac Imaging . 1996;  11 260-264
    CrossrefPubMedGoogle Scholar
  • 72 Garty I, Giladi N, Flatau E. Bone scintigraphy in two siblings with pulmonary alveolar microlithiasis.  Br J Radiol . 1985;  58 763-766
    PubMedGoogle Scholar
  • 73 Coolens J L, Devos P, De Roo M. Diffuse pulmonary uptake of 99mTc bone-imaging agents: case report and survey.  Eur J Nucl Med . 1985;  11 36-42
    PubMedGoogle Scholar
  • 74 Shigeno C, Fukunaga M, Morita R, Maeda H, Hino M, Torizuka K. Bone scintigraphy in pulmonary alveolar microlithiasis: a comparative study of radioactivity and density distribution.  Clin Nucl Med . 1982;  7 103-107
    PubMedGoogle Scholar
  • 75 Sharma S K, Sharma S, Mukhopadhyaya S, Padhy A K, Vijayraghavan M. Pulmonary alveolar microlithiasis: report of three cases with pulmonary function and exercise studies.  Indian J Chest Dis Allied Sci . 1992;  34 205-215
    PubMedGoogle Scholar
  • 76 Viswanathan R. Pulmonary alveolar microlithiasis.  Thorax . 1962;  17 251-256
    PubMedGoogle Scholar
  • 77 O'Neill R P, Cohn J E, Pellegrino E D. Pulmonary alveolar microlithiasis: a family study.  Ann Int Med . 1967;  67 957-967
    PubMedGoogle Scholar
  • 78 Wallis C, Whitehead B, Malone M, Dinwiddie R. Pulmonary alveolar microlithiasis in childhood: diagnosis by transbronchial biopsy.  Pediatr Pulmonol . 1996;  21 62-64
    CrossrefPubMedGoogle Scholar
  • 79 Schmidt H, Lorcher U, Kitz R, Zielen S, Ahrens P, Konig R. Pulmonary alveolar microlithiasis in children.  Pediatr Radiol . 1996;  26 33-36
    CrossrefPubMedGoogle Scholar
  • 80 Gocmen A, Toppare M F, Kiper N, Buyukpamukcu N. Treatment of pulmonary alveolar microlithiasis with a diphosphonate: preliminary results of a case.  Respiration . 1992;  59 250-252
    PubMedGoogle Scholar
  • 81 Mariotta S, Guidi L, Mattia P. Pulmonary microlithiasis: report of two cases.  Respiration . 1997;  64 165-169
    CrossrefPubMedGoogle Scholar
  • 82 Palombini B C, da Silva Porto N, Wallau C U, Camargo J J. Bronchopulmonary lavage in alveolar microlithiasis.  Chest . 1981;  80 242-243
    CrossrefPubMedGoogle Scholar
  • 83 Jackson K B, Modry D L, Halenar J, L'abbe J, Winton T L, Lien D C. Single lung transplantation for pulmonary alveolar microlithiasis.  J Heart Lung Transplant . 2001;  20 226
    PubMedGoogle Scholar
  • 84 Edelman J D, Bavaria J, Kaiser L R, Litzky L A, Palevsky H I, Kotloff R M. Bilateral sequential lung transplantation for pulmonary alveolar microlithiasis.  Chest . 1997;  112 1140-1144
    PubMedGoogle Scholar