Pattern and diagnostic evaluation of systemic autoinflammatory diseases other than familial Mediterranean fever among Arab children: a multicenter study from the Pediatric Rheumatology Arab Group (PRAG)

2019 ◽  
Vol 40 (1) ◽  
pp. 49-56
Author(s):  
Sulaiman M. Al-Mayouf ◽  
◽  
Abdulaziz Almutairi ◽  
Safiya Albrawi ◽  
Basil M. Fathalla ◽  
...  
Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_5) ◽  
Author(s):  
W Messadi ◽  
A Salmi ◽  
N Cherif

Abstract Background Biotherapy result of spectacular advances in genetics and research in molecular and cellular biology, has considerably improved the management of rheumatic and autoinflammatory diseases in children. The Objective is to assess the efficacy and tolerance of biotherapy in pediatrics Methods A retro-prospective study was carried out in the Pediatric Department B at the pediatric rheumatology consultation, university hospital center. An operating sheet was drawn up including epidemiological, clinical, paraclinical and therapeutic data of the cases diagnosed as well as their evolution on biotherapy. Results 26 children received treatment with biotherapy. 73% presented with JIA (54% systemic, 19% polyarticular with positive RF, 19% severe uveitis, and 4% familial Mediterranean fever. 4% behçet disease The mean time to start biotherapy was 12 months [2–48]. There is a clear clinical and biological improvement for 93% of patients. Etanercept was effective in polyarticular-progressive JIA, Adalimumab in oligoarthritis with severe uveitis, Tocilizumab in systemic forms of JIA and Anakinra in familial Mediterranean fever resistant to colchicine and systemic arthritis. The adverse reactions noted were: one case of tuberculosis, hepatic cytolysis in 4 cases, and one case of psoriasis. Conclusion The introduction of biotherapy in pediatric has considerably improved the management of inflammatory rheumatic diseases and transformed the prognosis of these chronic diseases. The maintenance of the good response to treatment and the good tolerance of biotherapy must be evaluated prospectively over the long term.


2000 ◽  
Vol 16 (1-2) ◽  
pp. 72-74 ◽  
Author(s):  
H. A. Majeed ◽  
Khalil Ghandour ◽  
H. M. Shahin

2020 ◽  
Vol 29 (4) ◽  
pp. 49-60
Author(s):  
V.V. Rameev ◽  
L.V. Lysenko (Kozlovskaya) ◽  
M.V. Bogdanova ◽  
S.V. Moiseev

Autoinflammatory diseases are a group of disorders caused by a dysregulation of the innate immune system. Unlike autoimmune diseases, they are not associated with changes in humoral or cellular immunity. The authors review the current classification, clinical manifestations and treatment of various systemic autoinflammatory diseases, including cryopirinassociated periodic syndrome, familial Mediterranean fever, HIDS, and TRAPS.


2019 ◽  
Vol 14 (1) ◽  
Author(s):  
Georges El Hasbani ◽  
Ali Jawad ◽  
Imad Uthman

Abstract Background Familial Mediterranean Fever (FMF), an autoinflammatory disease, is characterized by self-limited inflammatory attacks of fever and polyserositis along with high acute phase response. Although colchicine remains the mainstay in treatment, intolerance and resistance in a certain portion of patients have been posing a problem for physicians. Main body Like many autoimmune and autoinflammatory diseases, many colchicine-resistant or intolerant FMF cases have been successfully treated with biologics. In addition, many studies have tested the efficacy of biologics in treating FMF manifestations. Conclusion Since carriers of FMF show significantly elevated levels of serum TNF alpha, IL-1, and IL-6, FMF patients who failed colchicine were successfully treated with anti IL-1, anti IL-6, or TNF inhibitors drugs. It is best to use colchicine in combination with biologics.


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