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Macrophage activation syndrome induced by A/H1N1 influenza in cystic fibrosis

Rosaria Casciaro1, Federico Cresta, Federica Favilli

  • 1IRCCS G. Gaslini Cystic Fibrosis Center, Genoa, Italy. rosariacasciaro@ospedale-gaslini.ge.it.

Pediatric Pulmonology
|February 13, 2013
PubMed

Insights

Cystic Fibrosis (CF) patients can develop Macrophage Activation Syndrome (MAS), a severe condition. This case study shows H1N1 influenza triggering MAS in a CF patient, successfully treated with high-dose prednisone.

Area of Science:

  • Pulmonology
  • Rheumatology
  • Infectious Diseases

Background:

  • Bacterial and viral respiratory infections significantly impact cystic fibrosis (CF) pulmonary disease progression.
  • Macrophage Activation Syndrome (MAS) is a critical, life-threatening complication associated with rheumatic diseases, presenting with pancytopenia, hepatitis, hyperferritinemia, coagulopathy, and neurological symptoms.
  • MAS pathogenesis involves uncontrolled T lymphocyte and macrophage activation, leading to hemophagocytosis and cytokine release.

Observation:

  • A pediatric patient with cystic fibrosis (CF) presented with symptoms indicative of Macrophage Activation Syndrome (MAS).
  • The onset of MAS in this CF patient was strongly associated with infection by pandemic H1N1 influenza.
  • The patient exhibited typical MAS clinical manifestations, including pancytopenia, hepatitis, and neurological symptoms.

Findings:

  • Pandemic H1N1 influenza infection served as a trigger for Macrophage Activation Syndrome (MAS) in a patient with cystic fibrosis (CF).
  • High-dose prednisone treatment led to a positive clinical response in the CF patient experiencing H1N1-induced MAS.
  • This case highlights the potential for viral infections to precipitate severe complications like MAS in immunocompromised individuals.

Implications:

  • This case underscores the importance of recognizing MAS as a potential complication in cystic fibrosis patients, especially during viral outbreaks.
  • Early diagnosis and prompt treatment with corticosteroids, such as prednisone, are crucial for managing H1N1-triggered MAS in CF.
  • Further research may elucidate the specific mechanisms linking viral infections to MAS in CF, informing preventative and therapeutic strategies.
Keywords:
H1N1MAScystic fibrosis

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