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Published on: June 27, 2011
Anti-GM-CSF antibodies in paediatric pulmonary alveolar proteinosis
1Lung Research Group, Children's Hospital of Ludwig Maximilians University, D-80337 Munich, Germany.
Insights
Auto-antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF) are key in adult pulmonary alveolar proteinosis (PAP). However, these auto-antibodies are not typically found in children with PAP, suggesting different causes for the disease in younger patients.
Area of Science:
- Pulmonology
- Immunology
- Pediatrics
Background:
- Auto-antibodies against granulocyte-macrophage colony stimulating factor (GM-CSF) are implicated in adult sporadic pulmonary alveolar proteinosis (PAP).
- The role of these auto-antibodies in pediatric PAP remains undetermined.
Purpose of the Study:
- To investigate the presence and significance of anti-GM-CSF auto-antibodies in pediatric PAP.
- To compare auto-antibody levels in adult and pediatric PAP patients with disease controls.
Main Methods:
- Serum and bronchoalveolar lavage (BAL) fluid were collected from 27 PAP patients (9 adults, 15 children, 3 neonates).
- An antigen capture assay was used to detect anti-GM-CSF auto-antibodies.
- 185 children with various pulmonary conditions or malignancies served as disease controls.
Main Results:
- Anti-GM-CSF auto-antibodies were detected in 5/7 adult PAP patients' serum and 3/4 in BAL fluid.
- No anti-GM-CSF auto-antibodies were found in the serum of children or neonates with PAP.
- One child with PAP had detectable auto-antibodies in BAL fluid, but none were found in neonates or disease controls.
Conclusions:
- Anti-GM-CSF auto-antibodies appear to be a hallmark of autoimmune adult sporadic PAP.
- The absence of these auto-antibodies in most pediatric PAP cases suggests alternative pathogenic mechanisms.
- Age at diagnosis is a critical factor, with rare overlaps observed in older children.
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.
