Volume 83, Issue 1 (April 2025)
Tehran Univ Med J 2025, 83(1): 66-70 |
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Shashaani N, Javadi Parvaneh V, Shiari R, Rahmani K. Concurrence of familial mediterranean fever and inflammatory bowel disease in a seven-year-old girl with henoch-schönlein
purpura: a case report. Tehran Univ Med J 2025; 83 (1) :66-70
URL: http://tumj.tums.ac.ir/article-1-13480-en.html
URL: http://tumj.tums.ac.ir/article-1-13480-en.html
1- Department of Pediatric Rheumatology, Mofid Children's Hospital, Faculty of Medicine, Shahid Beheshti University of Medical Sciences, Tehran, Iran.
Abstract: (1013 Views)
Background: Immunoglobulin A (IgA) vasculitis or Henoch–Schönlein Purpura (HSP) is a systemic vasculitis of small vessels associated with IgA deposition. It is the most common Vasculitis in childhood and presents with a wide spectrum of clinical manifestations, most commonly palpable purpura, renal involvement, and arthritis. However, its manifestations are not limited to these organs and may also involve other systems of the body. The coexistence of Henoch–Schönlein purpura with other autoimmune and autoinflammatory diseases has been reported. In particular, its association with Familial Mediterranean Fever (FMF), Inflammatory Bowel Disease (IBD), and Behcet Disease (BD) has been observed in different studies. Patients with Familial Mediterranean Fever who develop Henoch–Schönlein purpura usually exhibit more severe and prolonged inflammatory symptoms. Therefore, reporting the co-occurrence of these diseases can provide a better understanding of the spectrum of clinical manifestations and diagnostic-therapeutic challenges.
Case Presentation: This case describes a 7-year-old girl with initial manifestations of Henoch–Schönlein purpura, who, due to severe gastrointestinal symptoms, underwent further evaluations. In the performed investigations, the coexistence of Familial Mediterranean Fever and Inflammatory Bowel Disease was diagnosed, and incomplete Behcet Disease was also considered. The patient was placed under appropriate medical treatment. Finally, the patient was controlled with appropriate medical treatment.
Conclusion: This report shows that in children with Henoch–Schönlein purpura, especially in severe and recurrent cases, the possibility of associated autoimmune and autoinflammatory diseases such as Familial Mediterranean Fever, Inflammatory Bowel Disease, and Behcet Disease should be considered. These associated diseases can play a key role in the course of appropriate treatment.
Case Presentation: This case describes a 7-year-old girl with initial manifestations of Henoch–Schönlein purpura, who, due to severe gastrointestinal symptoms, underwent further evaluations. In the performed investigations, the coexistence of Familial Mediterranean Fever and Inflammatory Bowel Disease was diagnosed, and incomplete Behcet Disease was also considered. The patient was placed under appropriate medical treatment. Finally, the patient was controlled with appropriate medical treatment.
Conclusion: This report shows that in children with Henoch–Schönlein purpura, especially in severe and recurrent cases, the possibility of associated autoimmune and autoinflammatory diseases such as Familial Mediterranean Fever, Inflammatory Bowel Disease, and Behcet Disease should be considered. These associated diseases can play a key role in the course of appropriate treatment.
Keywords: familial mediterranean fever, henoch-schonlein purpura, iga vasculitis, inflammatory bowel disease.
Type of Study: Case Report |
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