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Pneumologie 2002; 56(7): 448-456
DOI: 10.1055/s-2002-32875
Serie: Seltene Lungenerkrankungen (2)
© Georg Thieme Verlag Stuttgart · New York

Alveolarproteinose

Pulmonary Alveolar ProteinosisH.  Teschler1 , T.  E.  Wessendorf1
  • 1Zentrum für Pneumologie und Thoraxchirurgie, Ruhrlandklinik, Essen
Herrn Prof. Dr. Karl-Heinz Rühle zum 60. Geburtstag gewidmet
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Publication History

Publication Date:
19 July 2002 (online)

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Definition

Die Alveolarproteinose ist ein seltenes alveoläres Füllungssyndrom, das 1958 von Rosen und Mitarbeitern beschrieben wurde [52]. Bei der Alveolarproteinose akkumulieren große Mengen von Phospholipiden und Lipoproteinen in den Azini und angrenzenden peripheren Lufträumen (Abb. [1]).

Abb. 1 Histologisches Bild der Alveolarproteinose (PAS-Färbung).

Das angereicherte Material enthält Bestandteile des pulmonalen Surfactant in pathologischer Zusammensetzung [22]. Von der primären (idiopathischen) wird die sekundäre Alveolarproteinose bei bösartigen Erkrankungen, Infektionen, nach Behandlung mit Zytostatika und nach Inhalation von Chemikalien, Metall- oder Quarzstäuben unterschieden [7] [11] [36] [39] [53]. Die kongenitale Form der Alveolarproteinose ist die häufigste Lungenparenchymerkrankung des Neugeborenen, wird autosomal rezessiv vererbt und ist auf das Fehlen des Surfactant-Protein B (SP-B) oder eine verminderte Expression des βc-Rezeptors für GM-CSF zurückzuführen [15] [35] [50] [54] [61].

Literatur

  • 1 Alberti A, Luisetti M, Braschi A. et al . Bronchoalveolar lavage fluid composition in alveolar proteinosis. Early changes after therapeutic lavage.  Am J Respir Crit Care Med. 1996;  154 817-820
    PubMedGoogle Scholar
  • 2 Arora P L, Rogers R M, Mayock R L. Alveolar proteinosis. Experience with trypsin therapy.  Am J Med. 1968;  44 889-899
    CrossrefPubMedGoogle Scholar
  • 3 Bakhos R, Gattuso P, Arcot C. et al . Pulmonary alveolar proteinosis: an unusual association with Mycobacterium avium-intracellulare infection and lymphocytic interstitial pneumonia.  South Med J. 1996;  89 801-802
    CrossrefPubMedGoogle Scholar
  • 4 Barraclough R M, Gillies A J. Pulmonary alveolar proteinosis: a complete response to GM-CSF therapy.  Thorax. 2001;  56 664-665
    CrossrefPubMedGoogle Scholar
  • 5 Bewig B, Wang X D, Kirsten D. et al . GM-CSF and GM-CSF beta c receptor in adult patients with pulmonary alveolar proteinosis.  Eur Respir J. 2000;  15 350-357
    CrossrefPubMedGoogle Scholar
  • 6 Bingisser R, Kaplan V, Zollinger A. et al . Whole-lung lavage in alveolar proteinosis by a modified lavage technique.  Chest. 1998;  113 1718-1719
    CrossrefPubMedGoogle Scholar
  • 7 Buechner H A, Ansari A. Acute silico-proteinosis. A new pathologic variant of acute silicosis in sandblasters, characterized by histologic features resembling alveolar proteinosis.  Dis Chest. 1969;  55 274-278
    PubMedGoogle Scholar
  • 8 Burkhalter A, Silverman J F, Hopkins M B. et al . Bronchoalveolar lavage cytology in pulmonary alveolar proteinosis.  Am J Clin Pathol. 1996;  106 504-510
    PubMedGoogle Scholar
  • 9 Clague H W, Harth M, Hellyer D. et al . Septic arthritis due to Nocardia asteroides in association with pulmonary alveolar proteinosis.  J Rheumatol. 1982;  9 469-472
    PubMedGoogle Scholar
  • 10 Clague H W, Wallace W C, Morgan W KC. Pulmonary interstitial fibrosis associated with alveolar proteinosis.  Thorax. 1983;  38 865-866
    PubMedGoogle Scholar
  • 11 Cordonnier C, Fleury-Feith J, Escudier E. et al . Secondary alveolar proteinosis is a reversible cause of respiratory failure in leukemic patients.  Am J Respir Crit Care Med. 1994;  149 788-794
    CrossrefPubMedGoogle Scholar
  • 12 Corrin B, King E. Pathogenesis of experimental pulmonary alveolar proteinosis.  Thorax. 1970;  25 230-236
    PubMedGoogle Scholar
  • 13 Danel C, Israel-Biet D, Costabel U. et al . Therapeutic applications of bronchoalveolar lavage.  Eur Respir J. 1992;  5 1173-1175
    PubMedGoogle Scholar
  • 14 Diaz J P, Manresa-Presas F, Benasco C. et al . Response to surfactant activator (ambroxol) in alveolar proteinosis.  Lancet. 1984;  5 1023
    PubMedGoogle Scholar
  • 15 Dirksen U, Nishinakamura R, Groneck P. et al . Human pulmonary alveolar proteinosis associated with a defect in GM-CSF/IL-3/IL-5 receptor common beta chain expression.  J Clin Invest. 1997;  100 2211-2217
    CrossrefPubMedGoogle Scholar
  • 16 Dranoff G, Crawford A D, Sadelain M. et al . Involvement of granulocyte-macrophage colony-stimulating factor in pulmonary homeostasis.  Science. 1994;  264 713-716
    CrossrefPubMedGoogle Scholar
  • 17 duBois R M, WAC A C, Branthwaire M A. Alveolar proteinosis: diagnosis and treatment over a 10 years period.  Thorax. 1983;  38 360-363
    PubMedGoogle Scholar
  • 18 Franquet T, Gimenez A, Bordes R. et al . The crazy-paving pattern in exogenous lipoid pneumonia: CT-pathologic correlation.  Am J Roentgenol. 1998;  170 315-317
    CrossrefPubMedGoogle Scholar
  • 19 Freedman A P, Pelias A, Johnston R F. et al . Alveolar proteinosis lung lavage using partial cardiopulmonary bypass.  Thorax. 1981;  36 543-545
    PubMedGoogle Scholar
  • 20 Gaine S P, O’Marcaigh A S. Pulmonary alveolar proteinosis: lung transplant or bone marrow transplant?.  Chest. 1998;  113 5-4
    PubMedGoogle Scholar
  • 21 Goldstein L S, Kavuru M S, McCarthy P C. et al . Pulmonary alveolar proteinosis. Clinical features and outcomes.  Chest. 1998;  114 1357-1362
    CrossrefPubMedGoogle Scholar
  • 22 Griese M. Pulmonary surfactant in health and human lung diseases: state of the art.  Eur Respir J. 1999;  13 1455-1476
    PubMedGoogle Scholar
  • 23 Hammon W E, McCaffree D R, Cucchiara A J. A comparison of manual to mechanical chest percussion for clearance of alveolar material in patients with pulmonary alveolar proteinosis (phospholipidosis).  Chest. 1993;  103 1409-1412
    CrossrefPubMedGoogle Scholar
  • 24 Hartung M, Salfelder K. Pulmonary alveolar proteinosis and histoplasmosis: report of three cases.  Virchows Arch A Pathol Anat Histol. 1975;  28 281-287
    PubMedGoogle Scholar
  • 25 Holbert J M, Castello P, Wei L. et al . CT features of pulmonary alveolar proteinosis.  AJR. 2001;  176 1287-1294
    PubMedGoogle Scholar
  • 26 Honda Y, Takahashi H, Shijubo N. et al . Surfactant protein-A concentration in bronchoalveolar lavage fluids of patients with pulmonary alveolar proteinosis.  Chest. 1993;  103 496-499
    PubMedGoogle Scholar
  • 27 Honda Y, Kuroki Y, Matsuura E. et al . Pulmonary surfactant protein D in sera and bronchoalveolar lavage fluids.  Am J Respir Crit Care Med. 1995;  152 1860-1866
    CrossrefPubMedGoogle Scholar
  • 28 Huffman J A, Hull W M, Dranoff G. et al . Pulmonary epithelial cell expression of GM-CSF corrects the alveolar proteinosis in GM-CSF-deficient mice.  J Clin Invest. 1996;  97 649-655
    CrossrefPubMedGoogle Scholar
  • 29 Ikegami M, Whitsett J A, Chroneos Z C. et al . IL-4 increases surfactant and regulates metabolism in vivo.  Am J Physiol Lung Cell Mol Physiol. 2000;  278 L75-80
    PubMedGoogle Scholar
  • 30 Ikegami M, Korfhagen T R, Bruno M D. et al . Surfactant metabolism in surfactant protein A-deficient mice.  Am J Physiol. 1997;  272 L479-485
    PubMedGoogle Scholar
  • 31 Kariman K, Kylstra J A, Spock A. Pulmonary alveolar proteinosis: prospective clinical experience in 23 patients for 15 years.  Lung. 1984;  162 223-231
    PubMedGoogle Scholar
  • 32 Kavuru M S, Sullivan E J, Piccin R. et al . Exogenous granulocyte-macrophage colony-stimulating factor administration for pulmonary alveolar proteinosis.  Am J Respir Crit Care Med. 2000;  161 1143-1148
    CrossrefPubMedGoogle Scholar
  • 33 Kitamura T, Uchida K, Tanaka N. et al . Serological diagnosis of idiopathic pulmonary alveolar proteinosis.  Am J Respir Crit Care Med. 2000;  162 658-662
    CrossrefPubMedGoogle Scholar
  • 34 Lee K N, Levin D L, Webb W R. et al . Pulmonary alveolar proteinosis: high-resolution CT, chest radiographic, and functional correlations.  Chest. 1997;  111 989-995
    CrossrefPubMedGoogle Scholar
  • 35 Lin Z, deMello D E, Wallot M. et al . An SP-B gene mutation responsible for SP-B deficiency in fatal congenital alveolar proteinosis: evidence for a mutation hotspot in exon 4.  Mol Genet Metab. 1998;  64 25-35
    CrossrefPubMedGoogle Scholar
  • 36 Littler W A, Kay J M, Hasleton P S. et al . Busulphan lung.  Thorax. 1969;  24 639-655
    PubMedGoogle Scholar
  • 37 Martin R J, Rogers R M, Myers M N. Pulmonary alveolar proteinosis: shunt fraction and lactic acid dehydrogenase concentration as aids to diagnosis.  Am Rev Respir Dis. 1978;  117 1059-1062
    PubMedGoogle Scholar
  • 38 Maygarden S J, Iacocca M V, Funkhouser W K. et al . Pulmonary alveolar proteinosis: a spectrum of cytologic, histochemical, and ultrastructural findings in bronchoalveolar lavage fluid.  Diagn Cytopathol. 2001;  24 389-395
    CrossrefPubMedGoogle Scholar
  • 39 Miller R R, Churg A M, Hutcheon M. et al . Pulmonary alveolar proteinosis and aluminum dust exposure.  Am Rev Respir Dis. 1984;  130 312-315
    PubMedGoogle Scholar
  • 40 Milleron B J, Costabel U, Teschler H. et al . Bronchoalveolar lavage cell data in alveolar proteinosis.  Am Rev Respir Dis. 1991;  144 1330-1332
    CrossrefPubMedGoogle Scholar
  • 41 Nagasaka Y, Takahashi M, Ueshima H. et al . Bronchoalveolar lavage with trypsin in pulmonary alveolar proteinosis.  Thorax. 1996;  51 769-770
    PubMedGoogle Scholar
  • 42 Nakajima M, Manabe T, Niki Y. et al . Serum KL-6 level as a monitoring marker in a patient with pulmonary alveolar proteinosis.  Thorax. 1998;  53 809-811
    PubMedGoogle Scholar
  • 43 Nishinakamura R, Nakayama N, Hirabayashi Y. et al . Mice deficient for the IL-3/GM-CSF/IL-5 beta c receptor exhibit lung pathology and impaired immune response, while beta IL3 receptor-deficient mice are normal.  Immunity. 1995;  2 211-222
    CrossrefPubMedGoogle Scholar
  • 44 Nishinakamura R, Wiler R, Dirksen U. et al . The pulmonary alveolar proteinosis in granulocyte macrophage colony-stimulating factor/interleukins 3/5 beta c receptor-deficient mice is reversed by bone marrow transplantation.  J Exp Med. 1996;  183 2657-2662
    CrossrefPubMedGoogle Scholar
  • 45 Prakash U B, Barham S S, Carpenter H A. et al . Pulmonary alveolar phospholipoproteinosis: experience with 34 cases and a review.  Mayo Clin Proc. 1987;  62 499-518
    CrossrefPubMedGoogle Scholar
  • 46 Ramirez J, Kieffer R F, Ball W C. Bronchopulmonary lavage in man.  Ann Intern Med. 1965;  63 819-828
    PubMedGoogle Scholar
  • 47 Ranchod M, Bissell M. Pulmonary alveolar proteinosis and cytomegalovirus infection.  Arch Pathol Lab Med. 1979;  103 139-142
    PubMedGoogle Scholar
  • 48 Parker L A, Novotney D B. Recurrent alveolar proteinosis following double lung transplantation.  Chest. 1997;  111 1457-1458
    PubMedGoogle Scholar
  • 49 Reed J A, Ikagami M, Cianciolo E R. et al . Aerosolized GM-CSF ameliorates pulmonary alveolar proteinosis in GM-CSF-deficient mice.  Am J Physiol. 1999;  276 L556-563
    PubMedGoogle Scholar
  • 50 Reed J A, Whitsett Y A. Granulocyte-macrophage colony stimulating factor and pulmonary surfactant homeostasis.  Proc Assoc Am Physicians. 1998;  110 321-332
    PubMedGoogle Scholar
  • 51 Riker J B, Wolinsky H. Trypsin aerosol treatment of pulmonary alveolar proteinosis.  Am Rev Respir Dis. 1973;  108 108-113
    PubMedGoogle Scholar
  • 52 Rosen S H, Castleman B, Liebow A A. Pulmonary alveolar proteinosis.  N Engl J Med. 1958;  258 1123-1142
    CrossrefPubMedGoogle Scholar
  • 53 Ruben F L, Talamo T S. Secondary pulmonary alveolar proteinosis occurring in two patients with acquired immune deficiency syndrome.  Am J Med. 1986;  80 1187-1190
    CrossrefPubMedGoogle Scholar
  • 54 Seymour J F, Dunn A R, Vincent J M. et al . Efficacy of granulocyte-macrophage colony-stimulating factor in acquired alveolar proteinosis.  N Engl J Med. 1996;  335 1924-1925
    CrossrefPubMedGoogle Scholar
  • 55 Shah P L, Hansell D, Lawson P R. et al . Pulmonary alveolar proteinosis: clinical aspects and current concepts on pathogenesis.  Thorax. 2000;  55 67-77
    CrossrefPubMedGoogle Scholar
  • 56 Stanley E, Lieschke G J, Grail D. et al . Granulocyte/macrophage colony-stimulating factor-deficient mice show no major perturbation of hematopoiesis but develop a characteristic pulmonary pathology.  Proc Natl Acad Sci. 1994;  91 5592-5596
    PubMedGoogle Scholar
  • 57 Steens R D, Summers Q A, Tarala R A. Pulmonary alveolar proteinosis in association with Fanconi's anemia and psoriasis. A possible common pathogenetic mechanism.  Chest. 1992;  102 637-638
    PubMedGoogle Scholar
  • 58 Sunderland W A, Campbell R A, Edwards M J. Pulmonary alveolar proteinosis and pulmonary cryptococcosis in an adolescent boy.  J Pediatr. 1972;  80 450-456
    PubMedGoogle Scholar
  • 59 Tan R T, Kuzo R S. High-resolution CT findings of mucinous bronchioloalveolar carcinoma: a case of pseudopulmonary alveolar proteinosis.  Am J Roentgenol. 1997;  168 99-100
    PubMedGoogle Scholar
  • 60 Tchou-Wong K M, Harkin T J, Chi C. et al . GM-CSF gene expression is normal but protein release is absent in a patient with pulmonary alveolar proteinosis.  Am J Respir Crit Care Med. 1997;  156 1999-2002
    PubMedGoogle Scholar
  • 61 Teja K, Cooper P H, Squires J E. et al . Pulmonary alveolar proteinosis in four siblings.  N Engl J Med. 1981;  305 1390-1392
    PubMedGoogle Scholar
  • 62 Teschler H, Ragette R. Other interstitial lung diseases. In: Grassi C (ed). Pulmonary diseases. McGraw-Hill: Maidenhead 1999: 259-270
    Google Scholar
  • 63 Teschler H, Ziesche R, Matthys H. et al . Nachweis aktivierter alveolärer Lymphozyten bei Alveolarproteinose.  Pneumologie. 1990;  44 306-307
    PubMedGoogle Scholar
  • 64 Wang B M, Stern E J, Schmidt R A. et al . Diagnosing pulmonary alveolar proteinosis.  Chest. 1997;  111 460-466
    CrossrefPubMedGoogle Scholar
  • 65 Wasserman K, Blank N, Fletcher G. Lung lavage (alveolar washing) in alveolar proteinosis.  Am J Med. 1968;  44 611-617
    CrossrefPubMedGoogle Scholar
  • 66 Wilson D O, Rogers R M. Prolonged spontaneous remission in a patient with untreated pulmonary alveolar proteinosis.  Am J Med. 1987;  82 1014-1016
    CrossrefPubMedGoogle Scholar
  • 67 Wright J R, Dobbs L G. Regulation of pulmonary surfactant secretion and clearance.  Annu Rev Physiol. 1991;  53 395-414
    CrossrefPubMedGoogle Scholar
  • 68 Yoshida M, Ikegami M, Reed J A. et al . GM-CSF regulates protein and lipid catabolism by alveolar macrophages.  Am J Physiol Lung Cell Mol Physiol. 2001;  280 L379-386
    CrossrefPubMedGoogle Scholar
  • 69 Zimmer W E, Chew F S. Pulmonary alveolar proteinosis.  Am J Roentgenol. 1993;  161 26
    PubMedGoogle Scholar

Prof. Dr. med. H. Teschler

Ruhrlandklinik, Zentrum für Pneumologie und Thoraxchirurgie

Tüschener Weg 40

45239 Essen

Email: Helmutt@t-online.de

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