Abstract
Proceedings of the 24th Paediatric Rheumatology European Society Congress: Part three
Highlights
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non Mendelian autoinflammatory disorder until now considered to be limited to paediatric age
We report the case of a 17,5 years old girl presenting with recurrent episodes of fever starting at the age of 16
During episodes all the cultures were negative.Inflammatory markers were completely normal between attacks.The girl was diagnosed with PFAPA syndrome based on Marshall criteria revised by Thomas et al.,neglecting the item of disease onset before the age of five
Summary
Aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a non Mendelian autoinflammatory disorder until now considered to be limited to paediatric age. While again rising the steroid doses up to 60 mg/day and briefly to 80 mg/d our patient still presented with intermitting fever and high activity signs in the lab This brought us to change again the IL-1-antagonist to Canakinumab after about one year of therapy with Anakinra that caused strong local reaction due to the injections that were applied twice daily. Objectives: We present a patient with mosaic 47ΧΧΧ (90%) and 46ΧΧ (10%) who was diagnosed with SLE Methods: Case report Results: A 13-year old girl presented to our hospital with pain and swelling of her right hand and her left foot, asthenia, headaches, mouth sores and a rash on her legs. The introduction of adalimumab in the treatment regimen of children with JIA refractory to the basic immunosuppressive therapy allowed for the rapid disease remission while preserving the effect in a significant number of patients during the following year. Informed consent to publish has been obtained from the patient/parent/guardian
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