EULAR recommendations for the management of familial Mediterranean fever

Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease, but many rheumatologists are not well acquainted with its management. The objective of this report is to produce evidence-based recommendations to guide rheumatologists and other health professionals in the treatment and follow-up of patients with FMF. A multidisciplinary panel, including rheumatologists, internists, paediatricians, a nurse, a methodologist and a patient representative, was assembled. Panellists came from the Eastern Mediterranean area, Europe and North America. A preliminary systematic literature search on the pharmacological treatment of FMF was performed following which the expert group convened to define aims, scope and users of the guidelines and established the need for additional reviews on controversial topics. In a second meeting, recommendations were discussed and refined in light of available evidence. Finally, agreement with the recommendations was obtained from a larger group of experts through a Delphi survey. The level of evidence (LoE) and grade of recommendation (GR) were then incorporated. The final document comprises 18 recommendations, each presented with its degree of agreement (0–10), LoE, GR and rationale. The degree of agreement was greater than 7/10 in all instances. The more controversial statements were those related to follow-up and dose change, for which supporting evidence is limited. A set of widely accepted recommendations for the treatment and monitoring of FMF is presented, supported by the best available evidence and expert opinion. It is believed that these recommendations will be useful in guiding physicians in the care of patients with FMF.

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POS1368 ANTI-IL-1 THERAPIES IN COLCHICINE-RESISTANT OR İNTOLERANT PATIENTS WITH FAMILIAL MEDITERRANEAN FEVER: SINGLE CENTER EXPERIENCE

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2021-eular.3257 ◽

2021 ◽

Vol 80 (Suppl 1) ◽

pp. 966.1-966

Author(s):

M. E. Derin ◽

B. Karakaş ◽

B. Karataş ◽

N. Çabuk Çelik ◽

İ. Yalçin ◽

...

Keyword(s):

Familial Mediterranean Fever ◽

Interleukin 1 ◽

Mefv Gene ◽

Gene Mutations ◽

Recurrent Fever ◽

Long Term Effects ◽

Single Center Experience ◽

Eular Recommendations ◽

Mediterranean Fever

Background:Familial Mediterranean Fever (FMF) is a hereditary auto-inflammatory disease characterized by recurrent fever and serosal inflammation (1). The goal of FMF treatment is to prevent the attacks and to minimize subclinical inflammation between attacks The main treatment of FMF is colchicine however anti-interleukin-1 treatments are recommended in colchicine resistant and/or intolerant FMF patients (2).Objectives:The aim of this study is to evaluate the efficacy of anti-interleukin-1 (anti-IL-1) agents in 81 FMF patients with resistant/intolareted to colchicine or complicated with amyloidosis.Methods:Between January 2014 and December 2020, eighty-one patients who were diagnosed as FMF according to the criteria of Tel-Hashomer that following-up at Cumhuriyet University Medical Faculty Rheumatology-Internal Medicine Department were included in to the study.Results:45 (55.6%) male and 36 (44.4%) female were included in the study. The median age of the patients was 25 years (min:17-max: 60) and the median age at diagnosis was 15 years (min 3-max 46). 44 patients (54.3%) used Anakinra (100 mg/day), and 27 (45.7%) canakinumab (150mg/8month) were used. 49 cases were resistant to colchicine,16 were intolerant to colchicine, 16 (20%) cases were comlicated with amyloidosis. 10 patients had renal transplantation. MEFV gene mutations are shown in Table 1. Median duration of anti-IL-1 agent use was 24 month (min:4-max 52). 9 patients were resistant to anakinra, 18 patients had side effects which anakinra related. After a median follow up 12 months overall clinical response was %95 (frequency of attacks <1/6months). median proteinuria decreased from 3500 mg /day to median 1500 mg /day (p: 0.04) (Table 2). IL-6 treatment was started in 4 patients because of ineffective canakinumab. Five pregnant patients were followed up with anakinra during pregnancy and there were no problems.Conclusion:Anti-interleukin-1 agents are effectively and safely in the treatment of FMF patients. There are still unanswered questions in FMF treatment such as other factors affecting the frequency of attacks, colchicine resistance is not defined precisely and the importance of some mutations. The effect of anti IL-1 agents on FMF patients with amyloidosis is not clearly. According to our experience, these treatments are effective in patients with glomerular filtration rate> 60 ml/min. For answers to these and similar questions, Large and long follow-up studies are needed for long-term effects.References:[1]Özen S, Batu ED, Demir S., Familial Mediterranean Fever: Recent Developments in Pathogenesis and New Recommendations for Management. Front Immunol. 2017 Mar 23;8:253. doi: 10.3389/fimmu.2017.00253. eCollection 2017.[2]Seza Özen ve ark. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis. 2016 Apr;75(4):644-51.Disclosure of Interests:None declared

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The orophilous communities of the Pino-Juniperetea class in the Central and Eastern Mediterranean area

Feddes Repertorium ◽

10.1002/fedr.4921120308 ◽

2001 ◽

Vol 112 (3-4) ◽

pp. 261-308 ◽

Cited By ~ 1

Author(s):

S. Brullo ◽

G. Giusso del Galdo ◽

R. Guarino

Keyword(s):

Eastern Mediterranean ◽

Mediterranean Area ◽

Eastern Mediterranean Area

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AB1065 VISIT COMPLIANCE IN PATIENTS WITH FAMILIAL MEDITERRANEAN FEVER: RESULTS FROM A GAZI UNIVERSITY FMF COHORT

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.4675 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1822.1-1822

Author(s):

R. Bilici Salman ◽

A. Avanoğlu Güler ◽

H. Satiş ◽

H. Karadeniz ◽

H. Babaoglu ◽

...

Keyword(s):

Logistic Regression ◽

Family History ◽

Familial Mediterranean Fever ◽

Predictive Factors ◽

Severity Score ◽

Comorbid Disease ◽

Rheumatology Clinic ◽

Mediterranean Fever ◽

One Year

Background:Follow-up in all rheumatologic patients is critical, particularly Familial Mediterranean Fever (FMF). Current recommendations for all experts by the EULAR state that patients with FMF should be evaluated 6-monthly intervals to monitore the character and frequency of the attacks and the acute phase response. Disease-related complications such as amyloidosis can beasymptomaticand need only a careful follow-up.Objectives:to quantify this phenomenon and to find predictive factors of visit compliance in patients with FMF.Methods:The study included 474 adult patients with a diagnosis of FMF who followed at the outpatient rheumatology clinic of tertiary university hospital, from January 2018 to December 2018. . Demographic, socioeconomic data, familiy history, comorbid disease, medication history, characteristics, the International Severity Score for FMF (ISSF),autoinflammatory disease damage index (ADDI) were recorded. Visit compliance was defined as the presence of two visits in the outpatient rheumatology clinic for FMF last one year for the purposes set out in EULAR suggestion.Those who had fewer than two visits in the last one year were considered noncompliant.Results:230 (48.5%) were compliant while 244 (51.5 %) patients were noncompliant with their rheumatology visit. Both compliant and noncompliant patients had similar median age and disease duration. Female sex and being married was increased the visit compliance.The results of the logistic regression model exploring factors associated with compliance indicated that presence of family history in parents, absence of family history in sibling, treatment with biologic agents, other drug using,presence of more than 2 attacks except fever and adequate medical care were important predictors of visit compliance.Conclusion:In conclusion, if FMF patients visit compliance increase, their functionality, medication adherence and quality of life will increase and flares and complication of disease can decrease. Thus, we highlight some recommendations for FMF specialist, patients and health care providers to improve outcomes.Table 2.Multivariate logistic regression analysis for predictive factors of visit compliance of the patients with FMF, n=430Adj. OR%95 CI**pFamily history in parents(positive history vs negative)1,81,0-3,10.03Family history in sibling(negative history vs positive)1,91,2-3,10.004Comorbid disease status1,30,7-2,50.32Treatment(anakinra&canakinumab vs colchicine)3,71,7-8,20.001Drug using(other drugs vs FMF drugs)2,21,1-4,40.01More than 2 attacks except fever2,31,2-4,00.004Chronic peripheral arthritis2,30,8-6,60.10Proteinuria2,20,7-6,70.14Adequate medical care1,91,2-3,10.003Number of index flare within last 12-month0,90,9-1,00.38ISSF severity score0,80,7-1,10,30Disclosure of Interests:None declared

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AB0968 Adrenomedullin Levels in Patients with Familial Mediterranean Fever: A Long Term Follow-Up

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2015-eular.6129 ◽

2015 ◽

Vol 74 (Suppl 2) ◽

pp. 1222.2-1222

Author(s):

A. Polat ◽

C. Saglam ◽

Y.G. Kurt ◽

G. Basbozkurt ◽

B. Sozeri ◽

...

Keyword(s):

Familial Mediterranean Fever ◽

Long Term Follow Up ◽

Mediterranean Fever

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Ceramic finds from the Byzantine fort of Gradina on the island of Žirje

Archaeologia Adriatica ◽

10.15291/archeo.3391 ◽

2021 ◽

Vol 14 (1) ◽

Author(s):

Ana Karađole ◽

Igor Borzić

Keyword(s):

Iron Age ◽

Eastern Mediterranean ◽

Mediterranean Area ◽

The Other ◽

Late Antique ◽

North African ◽

The North ◽

Other Hand ◽

Main Production ◽

Eastern Mediterranean Area

Repeated excavations of the area of the early Byzantine fort on Žirje, an island in the Šibenik archipelago, resulted in recovery of a substantial amount of movable finds, predominantly pottery. Most finds date to the period of Justinian's reconquista in the mid-6th century when the fort was used, but there are also some artifacts of earlier or later dating (Iron Age, Hellenistic and early Imperial periods; medieval and postmedieval periods) whose presence is explained by continuous strategic importance of the fort position. Late antique material has been analyzed comprehensively in terms of typology. Dating and provenance contexts of the finds have also been determined. Presence of pottery from the main production centers that supplied the eastern Adriatic at the time has been attested. This refers in particular to the north African and Aegean-eastern Mediterranean area providing fine tableware and kitchen pottery, lamps and various forms of amphorae. On the other hand, participation of local workshops in supply of the Byzantine soldiers stationed in Gradina probably relates to prevailing forms of kitchenware.

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Allium kyrenium (Amaryllidaceae), a new species from Northern Cyprus

Phytotaxa ◽

10.11646/phytotaxa.213.3.8 ◽

2015 ◽

Vol 213 (3) ◽

pp. 282 ◽

Cited By ~ 2

Author(s):

GIANPIETRO GIUSSO DEL GALDO ◽

CRISTIAN BRULLO ◽

Salvatore Brullo ◽

CRISTINA SALMERI

Keyword(s):

New Species ◽

Somatic Chromosome ◽

Eastern Mediterranean ◽

Conservation Status ◽

Diploid Species ◽

Mediterranean Area ◽

Somatic Chromosome Number ◽

Northern Cyprus ◽

Eastern Mediterranean Area ◽

A New Species

Allium kyrenium, a new species of Allium sect. Codonoprasum, is described and illustrated from northern Cyprus. It is a very circumscribed geophyte growing on the calcareous cliffs of the Kyrenia range. This diploid species, with a somatic chromosome number 2n = 16, shows close morphological relationships with A. stamineum, a species complex distributed in the eastern Mediterranean area. Its morphology, karyology, leaf anatomy, ecology, conservation status and taxonomical relationships with the allied species belonging to the A. stamineum group are examined.

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