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Concurrent protracted febrile myalgia syndrome in a child with diabetic ketoacidosis
Korcan Demir1, Kürşat B Carman, Belde Kasap
1Division of Pediatric Endocrinology, Children's Hospital, Gaziantep, Turkey. korcandemir@gmail.com
Abstract:
Familial Mediterranean Fever (FMF), characterized by recurrent attacks of inflammation in predominantly serosal and synovial membranes, is caused by MEFV gene mutations resulting in abnormal pyrin. Protracted febrile myalgia syndrome (PFMS), a kind of vasculitis requiring corticosteroid treatment, is associated with M694V mutation of MEFV gene. Here, we report a case where the patient developed PFMS leading to the diagnosis of FMF concurrently at the time of treatment for diabetic ketoacidosis (DKA) of new-onset type 1 diabetes mellitus and discuss the possible mechanisms of simultaneous DKA and FMF-associated PFMS. DKA-associated cytokine release may be a predisposing factor or trigger for FMF-associated PFMS.
Insights
Familial Mediterranean Fever (FMF) and its associated vasculitis, protracted febrile myalgia syndrome (PFMS), were diagnosed in a patient presenting with new-onset type 1 diabetes mellitus and diabetic ketoacidosis (DKA). Cytokine release from DKA may trigger FMF-associated PFMS.
Area of Science:
- Genetics and Immunology
- Endocrinology
- Rheumatology
Background:
- Familial Mediterranean Fever (FMF) is an autoinflammatory disease caused by MEFV gene mutations, leading to pyrin dysfunction and recurrent inflammation.
- Protracted Febrile Myalgia Syndrome (PFMS) is a vasculitic manifestation linked to the MEFV M694V mutation, often requiring corticosteroid therapy.
Observation:
- A case report details a patient diagnosed with FMF-associated PFMS concurrently with new-onset type 1 diabetes mellitus and diabetic ketoacidosis (DKA).
- The patient's PFMS diagnosis occurred during the management of DKA, highlighting a potential temporal link.
Findings:
- The study suggests that cytokine release associated with DKA might act as a trigger or predisposing factor for FMF-associated PFMS.
- This observation points to a potential interplay between metabolic and autoinflammatory conditions.
Implications:
- Understanding this association may improve diagnostic approaches for FMF in patients with DKA.
- Further research into the immunomodulatory effects of DKA could elucidate shared pathogenic mechanisms with autoinflammatory diseases like FMF.
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