Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)

Aim The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases. Materials and methods Familial Mediterranean fever patients enrolled in t...

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Published in:Clinical rheumatology 2024, Vol.43 (1), p.407-413
Main Authors: Ozdel, Semanur, Coşkuner, Taner, Demirkan, Fatmagül, Torun, Rüya, Aydın, Elif Arslanoglu, Bağlan, Esra, Yener, Gülçin Otar, Öztürk, Kübra, Demir, Ferhat, Karadağ, Şerife Gül, Çakan, Mustafa, Sönmez, Hafize Emine, Makay, Balahan Bora, Ünsal, Şevket Erbil, Bülbül, Mehmet, Ayaz, Nuray Aktay, Sözeri, Betül
Format: Article
Language:English
Subjects:
Amyloidosis
Arthritis
chest
Colchicine
Comorbidity
Erysipelas
Erythema
Familial Mediterranean fever
family
Fever
homozygosity
Inflammatory diseases
juveniles
Medicine
Medicine & Public Health
Original Article
Pain
Patients
Pediatrics
retrospective studies
Rheumatology
therapeutics
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container_title Clinical rheumatology
container_volume 43
creator Ozdel, Semanur
Coşkuner, Taner
Demirkan, Fatmagül
Torun, Rüya
Aydın, Elif Arslanoglu
Bağlan, Esra
Yener, Gülçin Otar
Öztürk, Kübra
Demir, Ferhat
Karadağ, Şerife Gül
Çakan, Mustafa
Sönmez, Hafize Emine
Makay, Balahan Bora
Ünsal, Şevket Erbil
Bülbül, Mehmet
Ayaz, Nuray Aktay
Sözeri, Betül
description Aim The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases. Materials and methods Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups. Results The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis ( n  = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 ( p  
doi_str_mv 10.1007/s10067-023-06802-6
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Materials and methods Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups. Results The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis ( n  = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 ( p  &lt; 0.05). Fever and abdominal pain were more frequently detected in Group 2 ( p  &lt; 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups. Conclusion To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date</description><identifier>ISSN: 0770-3198</identifier><identifier>EISSN: 1434-9949</identifier><identifier>DOI: 10.1007/s10067-023-06802-6</identifier><identifier>PMID: 37926798</identifier><language>eng</language><publisher>Cham: Springer International Publishing</publisher><subject>Amyloidosis ; Arthritis ; chest ; Colchicine ; Comorbidity ; Erysipelas ; Erythema ; Familial Mediterranean fever ; family ; Fever ; homozygosity ; Inflammatory diseases ; juveniles ; Medicine ; Medicine &amp; Public Health ; Original Article ; Pain ; Patients ; Pediatrics ; retrospective studies ; Rheumatology ; therapeutics</subject><ispartof>Clinical rheumatology, 2024, Vol.43 (1), p.407-413</ispartof><rights>The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR) 2023. Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.</rights><rights>2023. The Author(s), under exclusive licence to International League of Associations for Rheumatology (ILAR).</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c359t-14bbfcba3d4d39c1d59c7f72023d9429267207c8387f484b84c9ee29db78dac13</cites><orcidid>0000-0002-7698-235X</orcidid></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,27903,27904</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/37926798$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ozdel, Semanur</creatorcontrib><creatorcontrib>Coşkuner, Taner</creatorcontrib><creatorcontrib>Demirkan, Fatmagül</creatorcontrib><creatorcontrib>Torun, Rüya</creatorcontrib><creatorcontrib>Aydın, Elif Arslanoglu</creatorcontrib><creatorcontrib>Bağlan, Esra</creatorcontrib><creatorcontrib>Yener, Gülçin Otar</creatorcontrib><creatorcontrib>Öztürk, Kübra</creatorcontrib><creatorcontrib>Demir, Ferhat</creatorcontrib><creatorcontrib>Karadağ, Şerife Gül</creatorcontrib><creatorcontrib>Çakan, Mustafa</creatorcontrib><creatorcontrib>Sönmez, Hafize Emine</creatorcontrib><creatorcontrib>Makay, Balahan Bora</creatorcontrib><creatorcontrib>Ünsal, Şevket Erbil</creatorcontrib><creatorcontrib>Bülbül, Mehmet</creatorcontrib><creatorcontrib>Ayaz, Nuray Aktay</creatorcontrib><creatorcontrib>Sözeri, Betül</creatorcontrib><title>Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)</title><title>Clinical rheumatology</title><addtitle>Clin Rheumatol</addtitle><addtitle>Clin Rheumatol</addtitle><description>Aim The aim of this study was to investigate the frequency and type of FMF-associated inflammatory diseases in a large FMF pediatric patients and to compare them to those FMF patients without concomitant inflammatory diseases. Materials and methods Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups. Results The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis ( n  = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 ( p  &lt; 0.05). Fever and abdominal pain were more frequently detected in Group 2 ( p  &lt; 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups. Conclusion To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date</description><subject>Amyloidosis</subject><subject>Arthritis</subject><subject>chest</subject><subject>Colchicine</subject><subject>Comorbidity</subject><subject>Erysipelas</subject><subject>Erythema</subject><subject>Familial Mediterranean fever</subject><subject>family</subject><subject>Fever</subject><subject>homozygosity</subject><subject>Inflammatory diseases</subject><subject>juveniles</subject><subject>Medicine</subject><subject>Medicine &amp; Public Health</subject><subject>Original Article</subject><subject>Pain</subject><subject>Patients</subject><subject>Pediatrics</subject><subject>retrospective studies</subject><subject>Rheumatology</subject><subject>therapeutics</subject><issn>0770-3198</issn><issn>1434-9949</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><recordid>eNqFks1u1DAUhSMEokPhBVggS2ymi4D_EtvsRhWdViqiGsE6cuybGVdJPNhOpTwWOx6AB8PTKUViARtbsr97ru_xKYrXBL8jGIv3Ma-1KDFlJa4lpmX9pFgQznipFFdPiwUWApeMKHlSvIjxFmNMpSLPixMmFK2Fkovix9XY9XoYdPJhRsYPPrTOuuQgop_fR5R2gHodthAT2oN1OgVn0IUeXO90jz7lowQh6BH0iDq4g5BFdj6kD0ijYeqTMzBmAgVIwcc9mOTuAMU02Rn5Dt08am52MB2e0fvtjFZGWxhmtLyBzeqs3EAEHcwOrYOf9mi5WZ-9LJ51uo_w6mE_Lb5efPxyfllef15fna-uS8MqlUrC27YzrWaWW6YMsZUyohM0m2YVpwcbKBZGMik6LnkruVEAVNlWSKsNYafF8qi7D_7blG1oBhcN9H0e2U-xYaRiVaWE5P9FqZR1pSSnMqNv_0Jv_RTGPEhDFSE1p0QdetMjZbJ1MUDX7IMbdJgbgptDBJpjBJo8TXMfgabORW8epKd2APtY8vvPM8COQMxX4xbCn97_kP0Fwem_bA</recordid><startdate>2024</startdate><enddate>2024</enddate><creator>Ozdel, Semanur</creator><creator>Coşkuner, Taner</creator><creator>Demirkan, Fatmagül</creator><creator>Torun, Rüya</creator><creator>Aydın, Elif Arslanoglu</creator><creator>Bağlan, Esra</creator><creator>Yener, Gülçin Otar</creator><creator>Öztürk, Kübra</creator><creator>Demir, Ferhat</creator><creator>Karadağ, Şerife Gül</creator><creator>Çakan, Mustafa</creator><creator>Sönmez, Hafize Emine</creator><creator>Makay, Balahan Bora</creator><creator>Ünsal, Şevket Erbil</creator><creator>Bülbül, Mehmet</creator><creator>Ayaz, Nuray Aktay</creator><creator>Sözeri, Betül</creator><general>Springer International Publishing</general><general>Springer Nature B.V</general><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>3V.</scope><scope>7T5</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>7X8</scope><scope>7S9</scope><scope>L.6</scope><orcidid>https://orcid.org/0000-0002-7698-235X</orcidid></search><sort><creationdate>2024</creationdate><title>Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)</title><author>Ozdel, Semanur ; 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Materials and methods Familial Mediterranean fever patients enrolled in the Pediatric Rheumatology Academy (PeRA)-Research Group (RG) were included. The patients were divided into two groups according to concomitant inflammatory disease as FMF patients who had a concomitant inflammatory disease (group 1) and FMF patients who did not have a concomitant inflammatory disease (group 1). The clinical findings and treatments were compared between the two groups. Results The study group comprised 3475 patients with FMF. There were 294 patients (8.5%) in group 1 and 3181 patients (91.5%) in group 2. Juvenile idiopathic arthritis ( n  = 136) was the most common accompanying inflammatory disease. Arthritis, M694V homozygosity, and the need for biological therapy were more frequently observed in Group 1 ( p  &lt; 0.05). Fever and abdominal pain were more frequently detected in Group 2 ( p  &lt; 0.05). FMF patients with concomitant inflammatory diseas more frequently demonstrated colchicine resistance. There were no significant differences in the median attack frequency, chest pain, amyloidosis, erysipelas-like erythema, or family history of FMF between the two patient groups. Conclusion To the best of our knowledge, this is the largest pediatric cohort reviewed to date. FMF patients may have different clinical profiles and colchicine responses if they have with concomitant inflammatory diseases. Key points • FMF is associated with some inflammatory comorbidities diseases. • To the best of our knowledge, this is the largest cohort evlauated pediatric FMF associated inflammatory comorbidities diseases reviewed to date</abstract><cop>Cham</cop><pub>Springer International Publishing</pub><pmid>37926798</pmid><doi>10.1007/s10067-023-06802-6</doi><tpages>7</tpages><orcidid>https://orcid.org/0000-0002-7698-235X</orcidid></addata></record>
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subjects Amyloidosis
Arthritis
chest
Colchicine
Comorbidity
Erysipelas
Erythema
Familial Mediterranean fever
family
Fever
homozygosity
Inflammatory diseases
juveniles
Medicine
Medicine & Public Health
Original Article
Pain
Patients
Pediatrics
retrospective studies
Rheumatology
therapeutics
title Inflammatory comorbidities ın the largest pediatric Familial Mediterranean fever cohort: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)
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