Polyarteritis nodosa and Henoch-Schonlein purpura nephritis in a child with familial Mediterranean fever: a case report

Girisgen I., Sonmez F., Koseoglu K., Erisen S., Yilmaz D.

RHEUMATOLOGY INTERNATIONAL, vol.32, no.2, pp.529-533, 2012 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 32 Issue: 2
  • Publication Date: 2012
  • Doi Number: 10.1007/s00296-010-1766-x
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.529-533
  • Keywords: Familial Mediterranean fever, Vasculitis, Genetic susceptibility, Intravenous immunoglobulin, DOSE INTRAVENOUS IMMUNOGLOBULIN, RESISTANT, CLASSIFICATION, VASCULITIS, MUTATIONS, CRITERIA, THERAPY, PATIENT, FMF
  • Bezmialem Vakıf University Affiliated: No


Familial Mediterranean fever is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis, and arthritis. Approximately 5% of individuals with familial Mediterranean fever have been reported to have Henoch-Schonlein purpura and about 1% to have polyarteritis nodosa. A 7-year-old girl presenting with complaints of purpuric rash, abdominal pain, arthritis, hematuria, and proteinuria and having IgA depositions on renal biopsy was diagnosed as Henoch-Schonlein nephritis. She had a history of recurrent fever, abdominal and joint pain and M694 V compound homozygote mutation. Colchicine treatment was started for the diagnosis of FMF. When constitutional symptoms such as myalgia, weight loss, fatigue, fever, and hypertension were added to the clinical picture, the diagnosis of polyarteritis nodosa HSP was thought and confirmed by the demonstration of microaneurisms on renal arteries. There was no response to corticosteroid and cyclophosphamide treatments; however, the symptoms were rapidly and dramatically reduced after the administration of intravenous immunoglobulin. In conclusion, polyarteritis nodosa and Henoch-Schonlein purpura can be seen together with familial Mediterranean fever. It is also suggested that IVIG might be an important adjunct therapy in selected patients with polyarteritis nodosa, especially in the lack of response to steroids and immunsuppressive drugs.