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Anti-GM-CSF antibodies in paediatric pulmonary alveolar proteinosis
  1. P Latzin1,
  2. M Tredano2,
  3. Y Wüst1,
  4. J de Blic3,
  5. T Nicolai1,
  6. B Bewig4,
  7. F Stanzel5,
  8. D Köhler6,
  9. M Bahuau2,
  10. M Griese1
  1. 1Lung Research Group, Children’s Hospital of Ludwig Maximilians University, Munich, Germany
  2. 2Laboratoire de Biochimie et Biologie Moléculaire, Hôpital d’Enfants Armand-Trousseau, AP-HP, Paris, France
  3. 3Service de Pneumologie et Allergologie Pédiatriques, Hôpital des Enfants-Malades, Paris, France
  4. 4Internal Medicine, University of Kiel, Germany
  5. 5Internal Medicine, Asklepios Fachkliniken, Gauting, Germany
  6. 6Internal Medicine, Grafschaft, Germany
  1. Correspondence to:
    Dr M Griese
    Lung Research Group, Children’s Hospital of Ludwig Maximilians University, D-80337 Munich, Germany;


Background: Auto-antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF) may be central to the pathogenesis of adult sporadic pulmonary alveolar proteinosis (PAP). The role of anti-GM-CSF auto-antibodies in paediatric forms of PAP is as yet unclear.

Methods: Anti-GM-CSF auto-antibodies were determined with the help of an antigen capture assay using serum and/or bronchoalveolar lavage (BAL) fluid from 27 patients with PAP (nine adults, 15 children, three neonates) and from 185 children with different diseases as disease controls (various pulmonary conditions and patients with malignancies).

Results: Anti-GM-CSF auto-antibodies were detected in the serum of five of seven adult PAP patients. They were not found in the serum of any of the children or neonates with PAP nor in any of the disease control patients. Raised anti-GM-CSF titres were found in BAL fluid from three of four adult patients with PAP. Anti-GM-CSF auto-antibodies were detected in BAL fluid of only one of the 15 children (age at diagnosis 11 years, age at BAL 24 years) and in none of the neonates with PAP, nor in any of the disease control patients.

Conclusions: The presence of anti-GM-CSF auto-antibodies seems to define an autoimmune disease underlying most of the adult sporadic type of PAP, but age at diagnosis may cause an overlap with children in some rare instances. In most of the children and all of the neonates the anti-GM-CSF titres were not significantly increased, indicating that alternative explanations are needed for the pathogenesis of the disease in these patients.

  • ABCA3, human gene encoding ATP binding cassette transporter A3
  • BAL, bronchoalveolar lavage
  • CAP, congenital alveolar proteinosis
  • CD36, human gene encoding leucocyte differentiation antigen (scavenger receptor CD36)
  • CF, cystic fibrosis
  • CSF2, human gene encoding colony stimulating factor 2 (GM-CSF)
  • CSF2RA, human gene encoding GM-CSF receptor α subunit
  • CSF2RB, human gene encoding GM-CSF receptor β subunit (common to interleukins 3 and 5)
  • Csfgm, murine gene encoding GM-CSF
  • GM-CSF, granulocyte-macrophage colony stimulating factor
  • LPI, lysinuric protein intolerance
  • OLB, open lung biopsy
  • PAP, pulmonary alveolar proteinosis
  • PAS, Periodic acid Schiff
  • PPAR-γ, human gene encoding peroxisome proliferator-activated receptor gamma
  • rh-GM-CSF, recombinant human GM-CSF
  • SFTPB, human gene encoding SP-B
  • SFTPC, human gene encoding SP-C
  • SP-B, surfactant associated protein B
  • SP-C, surfactant associated protein C
  • TBB, transbronchial biopsy
  • children
  • pulmonary alveolar proteinosis
  • granulocyte-macrophage colony stimulating factor (GM-CSF)
  • auto-antibodies
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  • Supported by a grant from DFG Gr 970/7-1.

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