How to handle the main drugs to treat autoinflammatory disorders and how we treat common autoinflammatory diseases

Koné‐Paut, Isabelle; Dusser, Perrine

doi:10.23736/s0392-0488.20.06708-5

Cited by 4 publications

(2 citation statements)

References 88 publications

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“…However, it is not approved in Europe and only available in the United States. 18 Currently, activity scores trials of assessment are in process. The AutoInflammatory Disease Activity Index (AIDAI) questionnaire is recommended by the international Single Hub and Access point for pediatric Rheumatology in Europe (SHARE) initiative but it is still under evaluation in order to unify the outcome measures to be objective in the assessment of activity of AIDs.…”

Section: Discussionmentioning

confidence: 99%

Efficacy of Anakinra Treatment in two Moroccan Patients With Mevalonate Kinase Deficiency

Souali,

Sakhi,

Bousfiha

et al. 2023

Global Pediatric Health

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Mevalonate kinase deficiency (MKD) is a rare hereditary autoinflammatory disease, with a widely variable clinical spectrum. It is characterized by febrile recurrent episodes and systemic inflammation. Data on therapeutic options for MKD are still limited and remain unknown in our country. We report Moroccan cases with MKD referred in our unit and treated with Anakinra, an interleukin-1 receptor antagonist. Through this study, we evaluate the efficacy of this bioagent, in our 2 MKD patients, in whom Anakinra has shown a complete clinical remission, with a remaining mild inflammation for one case, and normalization of growth with rare episodes of cervical adenopathies for the second case. Our experience provides an additional argument supporting the efficacy of Anakinra treatment, demonstrated previously but still lacks of objective data.

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Section: Discussionmentioning

confidence: 99%

Efficacy of Anakinra Treatment in two Moroccan Patients With Mevalonate Kinase Deficiency

Souali,

Sakhi,

Bousfiha

et al. 2023

Global Pediatric Health

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“…NSAIDs and systemic corticosteroids can be used for symptomatic relief during episodes. However, NSAIDs should not be used without concomitant IL-1 inhibition due to insufficient evidence that they will prevent organ damage if used alone [ 53 , 54 ]. Biological therapy in CAPS not only leads to the control of acute symptoms but also prevents long-term complications.…”

Section: Introductionmentioning

confidence: 99%

Pathophysiology, clinical manifestations and current management of IL-1 mediated monogenic systemic autoinflammatory diseases, a literature review

2022

Pediatr Rheumatol

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Background Systemic autoinflammatory diseases (SAIDs) are hyperinflammatory and immune-dysregulation conditions that present in childhood. This kind of disease is a rare disease with early-onset, severe condition and difficult diagnosis, which seriously affects the growth and development of children. Most children need a genetic diagnosis. However, with the limitation of access to genetic testing and the detection of somatic mutations, the diagnosis of SAIDs remains challenging. IL-1 is one of the important cytokines involved in the pathogenesis of SAIDs. Here we briefly review monogenic SAIDs mediated by aberrant IL-1 production, with the aim to further understand the pathogenesis, clinical manifestations and treatments of IL-1 mediated SAIDs. Methods Literature reviews were performed using “PubMed” and “Web of Science” by searching for the terms “autoinflammatory diseases” and “IL-1”. Results Monogenic SAIDs mediated by IL-1 include MKD, FMF, TRAPS, PAAND, PAPA, CAPS, DIRA, Majeed syndrome, NAIAD, NLRC4-MAS, PFIT, APLAID. Monogenic SAIDs have early onset, various clinical manifestations and difficult diagnosis, so early recognition and early treatment can reduce the complications and enhance the quality of life. Conclusions There are many kinds of IL-1 mediated SAIDs. Pediatricians should be alert to SAIDs in the face of the patients with repeated fever, repeated rash and poor effect of routine treatment. The patients should be carried out with gene testing and treatment in time.

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Rheumatologic manifestations of autoinflammatory diseases

Asnaashari

Rezaei

2023

Translational Autoimmunity

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How to handle the main drugs to treat autoinflammatory disorders and how we treat common autoinflammatory diseases

Cited by 4 publications

References 88 publications

Efficacy of Anakinra Treatment in two Moroccan Patients With Mevalonate Kinase Deficiency

Efficacy of Anakinra Treatment in two Moroccan Patients With Mevalonate Kinase Deficiency

Pathophysiology, clinical manifestations and current management of IL-1 mediated monogenic systemic autoinflammatory diseases, a literature review

Rheumatologic manifestations of autoinflammatory diseases

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