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Pulmonary alveolar microlithiasis

Patrick Kosciuk, Cristopher Meyer, Kathryn A. Wikenheiser-Brokamp, Francis X. McCormack
European Respiratory Review 2020 29: 200024; DOI: 10.1183/16000617.0024-2020
Patrick Kosciuk
1Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, Cincinnati, OH, USA
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Cristopher Meyer
2Division of Radiology, University of Wisconsin, Madison, WI, USA
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Kathryn A. Wikenheiser-Brokamp
3Division of Pathology & Laboratory Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA
4Dept of Pathology & Laboratory Medicine, University of Cincinnati, Cincinnati, OH, USA
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Francis X. McCormack
1Division of Pulmonary, Critical Care, and Sleep Medicine, University of Cincinnati, Cincinnati, OH, USA
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  • ORCID record for Francis X. McCormack
  • For correspondence: frank.mccormack@uc.edu
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  • FIGURE 1
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    FIGURE 1

    Radiographic findings in pulmonary alveolar microlithiasis. a) Chest radiograph depicting a fine, sand-like micronodular pattern with basilar predominance. b, c) High-resolution computed tomography with posterior lower lobe and anterior upper lobe micronodules, interlobular septal thickening, subpleural emphysema with predominance of small cysts. d) Mediastinal windows reveal calcific burden in the parenchyma and most concentrated in a peripheral septopleural location.

  • FIGURE 2
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    FIGURE 2

    Pathologic findings in pulmonary alveolar microlithiasis (PAM). a) Lung explant from a 2-year-old boy transplanted for PAM showing lung regions with accentuation of the interlobular septa by microlith accumulations (arrow) and other regions with more diffuse accumulations of granular, gritty microliths (*). b, c) Histological sections showing microliths along interlobular septa (b, arrow) and areas with more diffuse microlith accumulations (c). d) Varying sized microliths are present both in the alveolar spaces and interstitium. e) The microliths are characteristically concentrically laminated calcified spherules. f) Calcium can be demonstrated in the microliths by Von Kossa stain. b–e) Haematoxylin and eosin stain; original magnifications ×20 (b, c), ×200 (d), ×1000 (e, f).

  • FIGURE 3
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    FIGURE 3

    Scanning electron microscopy (SEM) and energy-dispersive spectroscopy (EDAX) of pulmonary alveolar microlithiasis (PAM). a, b) Microliths isolated from explanted lungs demonstrating a) the characteristic spherical structure and b) inorganic elemental signature. Microlith (white arrow) in bronchoalveolar lavage (BAL) fluid with SEM (c) and EDAX characteristics that are similar to explant controls, demonstrating feasibility of using SEM and EDAX as a diagnostic BAL test for PAM. Note carbon spike associated with debris from lavage. Kα, Kβ and Lα represent X-rays emitted as electrons return to K and/or L electron shell. d) Inorganic elemental analysis of BAL microliths.

  • FIGURE 4
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    FIGURE 4

    Diagnostic algorithm for patients with suspected pulmonary alveolar microlithiasis (PAM). Obtaining a family and genetic testing history is key. Thereafter, diagnostic modalities progress from least invasive to most invasive. HRCT: high-resolution computed tomography; BAL: bronchoalveolar lavage; VAT: video-assisted thoracoscopic biopsy.

  • FIGURE 5
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    FIGURE 5

    Other examples of diffuse lung calcification. Computed tomography depicting examples of a, d) amyloidosis with calcified masses, lymphadenopathy and rare cysts, b, e) silicosis with peri-lymphatic calcified nodules, eggshell lymph node calcifications and conglomerate fibrosis, c) metastatic pulmonary calcification in chronic renal failure characterised by lobular ground-glass opacity with interlobular septal sparing and f) healed granulomas due to histoplasmosis with maximum intensity projection reformats.

Tables

  • Figures
  • TABLE 1

    Pathogenetic mutations

    ExonSequence involvedDefectFirst author [ref.]
    1–13195 kb deletionTruncation without synthesis (deletion)Stokman [15]
    1c.-6773_-6588delTruncation without synthesis (deletion)Corut [9]
    2–65.5 kb deletionTruncation (deletion)Ishihara [16]
    2insT (not specified area)Truncation (frameshift)Dogan [17]
    3c.114delATruncation (deletion)Corut [9]
    3c.212_224delTruncation (deletion)Vismara [18]
    3c.226 C>TSubstitutionCorut [9]
    4c.316 G>ASubstitution (missense)Jonsson [19]
    5c.IVS4+1452_IVS5+660delTruncation (deletion) of entire exon 5Dandan [20]
    6c.560 G>ASubstitution (missense)Jonsson [19]
    6c.575 C>ASubstitutionMa [21]
    7c.560 G>ASubstitution (nonsense)Jonsson [19]
    7insdel857-871Insertion/deletion with truncationHuqun [8]
    8IVS8+1 G>ATruncation by splicing failureHuqun [8]
    8c.906 G>ASubstitution (nonsense)Jonsson [19]
    8c.910 A>TTruncationZhong [22]
    10c.1136 G>ASubstitution (missense)Jonsson [19]
    11c.1238 G>ASubstitution (nonsense)Jonsson [19]
    11c.1327delCTruncation (deletion)Jonsson [19]
    11c.1328delTTruncation (deletion)Corut [9]
    12c.1333+1 G>ASubstitution (nonsense, frameshift splicing)Jonsson [19]
    12c.1342delGTruncation (deletion)Corut [9]
    12c.1363 T>CSubstitutionWang [23]
    12c.1390 G>CUnclear resultIzumi [24]
    12c.1393-1404delACCTruncation, threonine deletionJonsson [25]
    12c.1402-1404delACCTruncation, threonine deletionJonsson [25]
    12c.1456 C>TTruncationProesmans [26]
  • TABLE 2

    Lung transplantation in cases of pulmonary alveolar microlithiasis

    Single versus doubleAge at transplant yearsSexOutcomeComplication(s)First author [ref.]
    Single32MaleDeath, NRPGD, haemodynamic instabilityShadmehr [106]
    Single47FemaleAlive, 12 monthsPossible acute rejection, bronchial strictureRaffa [87]
    Single53MaleAlive, 12 monthsBronchial anastomosis granulation with stenosis, bacterial infectionRen [34]
    Single53FemaleAlive, 90 monthsNoneJackson [107]
    Single64FemaleAlive, 60 monthsNoneBorrelli [108]
    Double32FemaleDeath, 11 daysPGD, sepsisKlikovits [35]
    Double32MaleAlive, 18 monthsBronchial artery bleed, post-operative tracheostomy, CMV/fungal infectionsStamatis [27]
    Double34MaleAlive, 67 monthsNoneKlikovits [35]
    Double36FemaleAlive, 32 monthsNoneEdelman [33]
    Double45MaleAlive, 12 monthsMild PGDAlrossais [36]
    Double46FemaleDeath, 20 monthsBronchiolitis obliteransBonnette [109]
    Double48MaleAlive, 12 monthsPGD, haemodynamic instability, ARFSamano [57]
    Double49FemaleDeath, 3 monthsInfectionCoulibaly [110]
    Double52FemaleAlive, 35 monthsNoneKlikovits [35]
    Double52FemaleAlive, 74 monthsPGD, atrial fibrillationKlikovits [35]
    Double53FemaleAlive, 12 monthsNoneGucyetmez [111]
    Double54FemaleAlive, 12 monthsNoneJindal [112]
    Double56MaleDeath, 5 daysPost-operative bleedingEdelman [33]
    Double62FemaleAlive, 29 monthsAtrial fibrillationKlikovits [35]
    Double63FemaleAlive, 24 monthsNoneShigemura [113]

    NR: not reported; PGD: primary graft dysfunction; CMV: cytomegalovirus; ARF: acute renal failure.

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    Pulmonary alveolar microlithiasis
    Patrick Kosciuk, Cristopher Meyer, Kathryn A. Wikenheiser-Brokamp, Francis X. McCormack
    European Respiratory Review Dec 2020, 29 (158) 200024; DOI: 10.1183/16000617.0024-2020

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    Pulmonary alveolar microlithiasis
    Patrick Kosciuk, Cristopher Meyer, Kathryn A. Wikenheiser-Brokamp, Francis X. McCormack
    European Respiratory Review Dec 2020, 29 (158) 200024; DOI: 10.1183/16000617.0024-2020
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