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Published on: January 11, 2018
Pulmonary alveolar proteinosis following cryptococcal meningitis: a possible cause?
Sarah Demir1, Nader Chebib1, Francoise Thivolet-Bejui2
1Department of Pneumology, Hospices Civils de Lyon, National Reference Center for Rare Pulmonary Diseases, Lyon, France.
Abstract:
Autoimmune pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease characterised by the presence of granulocyte macrophage colony-stimulating factor (GM-CSF) autoantibodies. A man with no history of infection developed cryptococcal meningitis and a right parahilar cryptococcal mass. Antifungal treatment led to infection control, although there was presence of neurological sequelae. After 3 years, thoracic CT revealed bilateral ground glass opacities and a crazy paving pattern. Transparietal needle biopsy showed proteinaceous alveolar deposits, confirming the diagnosis of PAP. A high titre of serum anti-GM-CSF autoantibodies was found. No specific treatment was started, and radiological lesions decreased progressively. Cryptococcal infection may occur in PAP and in patients with anti-GM-CSF antibodies without PAP. These antibodies dysregulate phagocytosis in monocytes and macrophages, possibly leading to opportunistic infections in previously healthy subjects.
Insights
Autoimmune pulmonary alveolar proteinosis (PAP) is linked to anti-GM-CSF antibodies. These antibodies may predispose individuals to opportunistic infections like cryptococcal meningitis, even in healthy subjects.
Area of Science:
- Pulmonology
- Immunology
- Infectious Diseases
Background:
- Autoimmune pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease associated with autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF).
- GM-CSF autoantibodies are implicated in the pathogenesis of PAP by impairing monocyte and macrophage phagocytic function.
Observation:
- A patient presented with cryptococcal meningitis and a pulmonary mass, later developing bilateral ground glass opacities and a crazy paving pattern on CT.
- Diagnosis of PAP was confirmed via biopsy showing proteinaceous alveolar deposits and high serum anti-GM-CSF antibody titers.
- Radiological lesions of PAP showed progressive decrease without specific treatment after cryptococcal infection control.
Findings:
- The case highlights a potential association between autoimmune PAP, anti-GM-CSF antibodies, and opportunistic infections, specifically cryptococcosis.
- The presence of anti-GM-CSF antibodies may compromise immune defenses, increasing susceptibility to infections in individuals without pre-existing conditions.
Implications:
- This suggests that anti-GM-CSF antibodies might play a broader role in immune dysregulation, potentially leading to opportunistic infections.
- Further research is warranted to elucidate the precise mechanisms linking anti-GM-CSF antibodies to increased susceptibility to infections and the management of such cases.
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