Pulmonary medium vessel vasculitis in an 11 year old boy: Hughes Stovin syndrome as a variant of polyarteritis nodosa?
2011

Pulmonary Vasculitis in an 11-Year-Old Boy

Sample size: 1 publication Evidence: moderate

Author Information

Author(s): de Vries Willemien, Koppelman Gerard H, Roofthooft Marc TR, Bootsma Hendrika, Leijsma Martha K, Armbrust Wineke

Primary Institution: Beatrix Children's Hospital, University Medical Centre, University of Groningen, The Netherlands

Hypothesis

Could Hughes Stovin syndrome be a variant of polyarteritis nodosa?

Conclusion

The patient achieved remission after treatment with cyclophosphamide and high-dose steroids.

Supporting Evidence

  • The patient presented with cough, fatigue, weight loss, and hemoptysis.
  • Diagnostic imaging revealed multiple aneurysms in the medium-sized arteries.
  • The patient developed pulmonary hypertension confirmed by cardiac catheterization.
  • Treatment included intravenous gamma globulin, prednisolone, and cyclophosphamide.

Takeaway

A boy had a rare lung condition that caused bleeding and high blood pressure in his lungs, but he got better after doctors gave him special medicine.

Methodology

The patient was treated with cyclophosphamide pulses and high-dose steroids after being diagnosed with medium-sized vessel vasculitis.

Limitations

Histological findings could not be obtained to support the diagnosis.

Participant Demographics

The patient was an 11-year-old boy with a Spanish mother and a Dutch father.

Digital Object Identifier (DOI)

10.1186/1546-0096-9-19

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