scholarly journals Evaluation of tendon involvement with Sonoelastography in follow-up of Familial Mediterranean Fever

2015 ◽  
Vol 14 (1) ◽  
pp. 1
Author(s):  
Zeynep Yakut ◽  
Torel Ogur ◽  
Sukran Erten ◽  
Deniz Delibas ◽  
Meltem Yildirim ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Mediterranean Fever

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

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

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

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

Infliximab therapy for familial Mediterranean fever-related amyloidosis: case series with long term follow-up

2012 ◽  
Vol 31 (8) ◽  
pp. 1267-1271 ◽  
Author(s):  
Z. Birsin Özçakar ◽  
Selçuk Yüksel ◽  
Mesiha Ekim ◽  
Fatoş Yalçınkaya
Keyword(s):  
Familial Mediterranean Fever ◽  
Case Series ◽  
Infliximab Therapy ◽  
Long Term Follow Up ◽  
Mediterranean Fever

scholarly journals Evaluation of E148Q and Concomitant AA Amyloidosis in Patients with Familial Mediterranean Fever

2021 ◽  
Vol 10 (16) ◽  
pp. 3511
Author(s):  
Zehra Serap Arici ◽  
Micol Romano ◽  
David Piskin ◽  
Ferhat Guzel ◽  
Sezgin Sahin ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Clinical Phenotype ◽  
Clinical Manifestations ◽  
Histopathological Diagnosis ◽  
Close Monitoring ◽  
Aa Amyloidosis ◽  
Homozygous Genotype ◽  
Age Of Diagnosis ◽  
Mediterranean Fever

The aim of the study was to compare the clinical phenotype of patients with familial Mediterranean fever (FMF)-related AA amyloidosis, according to the age of FMF diagnosis and E148Q genotype. Patients with biopsy-confirmed FMF-related AA amyloidosis were included in the study. Tel-Hashomer criteria were applied in the diagnosis of FMF. All patients had detailed baseline assessment of clinical features, renal functions, genetic testing, histopathological diagnosis of amyloidosis, and treatment received. Multiple comparisons were performed according to the age of diagnosis, disease phenotype, mutation, and mortality. Our study included 169 patients with a diagnosis of AA amyloidosis. There were 101 patients diagnosed with FMF < 18 years of age and 68 patients diagnosed who were ≥18 years of age. The three most common clinical manifestations were fever (84.6%), abdominal pain (71.6%), and arthritis (66.9%). The most common allele among FMF patients was M694V (60.6%), followed by E148Q (21.4%), and M680I (10.3%). The most frequent genotypes were M694V/M694V (45.0%), M694V/E148Q (14.8%), and E148Q/E148Q (11.2%) among 169 patients in our cohort. During the follow-up period, 15 patients (10 male, 5 female) died, of whom 14 had M694V homozygous genotype and one was homozygous for E148Q. Clinicians should be aware of patients with homozygous E148Q genotype for close monitoring and further evaluation. The possible relationship between E148Q and AA amyloidosis needs to be confirmed in other ethnicities.

scholarly journals Adrenomedullin levels in patients with Familial Mediterranean Fever: a long term follow-up

2015 ◽  
Vol 13 (Suppl 1) ◽  
pp. P99
Author(s):  
A Polat ◽  
C Saglam ◽  
YG Kurt ◽  
G Basbozkurt ◽  
B Sozeri ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Long Term Follow Up ◽  
Mediterranean Fever

Comment on: Short-term follow-up results of children with familial Mediterranean fever after cessation of colchicine: is it possible to quit?: reply

Rheumatology ◽  
2019 ◽  
Vol 58 (10) ◽  
pp. 1886-1887
Author(s):  
Ayşe Tanatar ◽  
Şerife Gül Karadağ ◽  
Hafize Emine Sönmez ◽  
Mustafa Çakan ◽  
Nuray Aktay Ayaz
Keyword(s):  
Familial Mediterranean Fever ◽  
Short Term ◽  
Mediterranean Fever

Discontinuation of colchicine therapy in children with Familial Mediterranean Fever

2021 ◽  
pp. jrheum.201158
Author(s):  
Yonatan Butbul Aviel ◽  
Silman Rawan ◽  
Shafe Fahouma ◽  
Isaiah Wexler ◽  
Yackov Berkund
Keyword(s):  
Abdominal Pain ◽  
Familial Mediterranean Fever ◽  
Pediatric Patients ◽  
Predictors Of Success ◽  
Successful Termination ◽  
Select Group ◽  
Mediterranean Fever ◽  
Colchicine Therapy

Objective Colchicine has been considered a life-long therapy for Familial Mediterranean fever (FMF). Recent studies describe patients who discontinued colchicine, but data pertaining to predictors of success were not provided. The aims of our study are to describe a cohort of pediatric patients with FMF who discontinued colchicine therapy, and to identify factors predicting successful termination of colchicine. Methods This study describes a cohort of pediatric patients with FMF who discontinued colchicine therapy following a relatively prolonged attack-free period (≥6 month), and identifies factors predicting successful termination. Data collected included demographic, clinical, and laboratory characteristics of children diagnosed with FMF < 16 years who underwent a trial of colchicine discontinuation. Data from patients who successfully ceased colchicine therapy were compared to that of patients who relapsed. Results Of 571 patients with FMF, 59 (10.3%) discontinued colchicine therapy. The average attack-free period before enrollment was 1.4±0.97 years. Follow-up after ceasing colchicine was 5.0±3.05 years during which time 11 (20%) patients had an attack. The most common symptoms were fever (92%) and abdominal pain (84.6%). For those failing discontinuation, colchicine was restarted within 1.3 years (range: 0.3-5.0; median 0.7 years). A longer attack-free period prior to colchicine discontinuation predicted success. Myalgia and arthritis prior to colchicine cessation were more common among children who required renewal of colchicine. Conclusion Cessation of colchicine therapy should be considered following prolonged remission in a select group of patients. Patients with arthritis or myalgia are more likely to have an attack after ceasing colchicine therapy.

scholarly journals POS1372 EVALUATION OF E148Q AND CONCOMITANT AA AMYLOIDOSIS SECONDARY TO FAMILIAL MEDITERRANEAN FEVER AFTER ADJUSTED CLINICAL-DEMOGRAPHIC CHARACTERISTICS

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 968.1-968
Author(s):  
Z. S. Arici ◽  
M. Romano ◽  
D. Piskin ◽  
F. Guzel ◽  
M. Yilmaz ◽  
...  
Keyword(s):  
Family History ◽  
Familial Mediterranean Fever ◽  
Allele Frequency ◽  
Demographic Characteristics ◽  
Renal Amyloidosis ◽  
Histopathological Diagnosis ◽  
Aa Amyloidosis ◽  
History Of ◽  
Mediterranean Fever

Background:Amyloid A (AA) amyloidosis, previously known as secondary or reactive amyloidosis, is a long-recognized severe complication of some chronic inflammatory diseases. The pathogenesis and risk factors for amyloidosis in Familial Mediterranean Fever (FMF) remain partially understood (1). The development of AA amyloidosis is dependent on ethnicity and country of residence (2). In the pre-colchicine era, renal AA-amyloidosis was largely reported patients of Turkish (67%) and Sephardic Jewish ancestry (26.5%) (2,3). Currently it’s well known that the MEFV M694V variant associated with high risk of amyloidosis however, mutations on exon 2, specifically E148Q variant remained controversial.Objectives:To evaluate the E148Q mutation variant and concomitant AA Amyloidosis secondary to FMF after adjusted clinical-demographic characteristics.Methods:Patients were recruited from the renal unit at Epigenetic Health Center outpatient clinic in Turkey between September 2003 and February 2020. Patients who had biopsy confirmed FMF related AA amyloidosis were included the study. Tel-Hashomer criteria were applied in the diagnosis of FMF. The clinical characteristics of FMF patients and medication history were recorded by the physician at the time of registry entry. All patients had detailed baseline assessment of clinical features, renal functions, genetic testing, histopathological diagnosis of amyloidosis, and treatment received. We performed multiple comparisons according to the age of diagnosis, demographic features, disease phenotype, allele frequency, type of mutation and mortality. Statistical analysis was performed with Statistical Package of Social Science (SPSS) for Windows, version 15.0 (SPSS Inc, Chicago, IL).Results:Our registry consists of 195 patients with a diagnosis of AA amyloidosis. Complete information on 169 patients (lost to follow up, n=26) were included. The median age was 36 (19-49) years; male/female ratio was 1.6 (104/65). The median follow-up duration was 15.0 years (4-17 years). There were 101 patients diagnosed with FMF <18 years of age and 68 patients diagnosed ≥18 years of age. All participants developed renal amyloidosis before the age of 32 years. Family history of FMF was documented in 56 patients (33.1%) and family history of amyloidosis was present in 41 patients (24.3%). The three most common clinical symptoms were fever (84,6%), abdominal pain (71.6%) and arthritis (66.9%). During the follow-up, 5 patients started dialysis treatment and 9 patients had kidney transplantation. The most common allele frequency across patients was M694V (60.6%), E148Q (21.4%) and M680I (10.3%). The most frequent mutations were M694V/M694V (63.3%), M694V/E148Q (20.8%) and E148Q/E148Q (15.8%). During the follow up period, 15 patients (10 male, 5 female) died. In those that died, the mutations in 14 had M694V/M694Vand one demonstrated E148Q/E148Q.Conclusion:Patients with FMF related AA amyloidosis have an increased risk for mortality. This study confirmed the association between M694V and FMF-associated AA amyloidosis, which has been reported in many studies. Close clinical follow-up and further evaluation of patients with the E148Q mutation is warranted specifically if residing in FMF endemic areas. The possible relationship between E148Q and AA amyloidosis need to be confirmed in other cohorts.References:[1]Erer B, Demirkaya E, Ozen S, Kallinich T. What is the best acute phase reactant for familial Mediterranean fever follow-up and its role in the prediction of complications? A systematic review. Rheumatology international. 2016;36(4):483-7.[2]Touitou I, Sarkisian T, Medlej-Hashim M, Tunca M, Livneh A, Cattan D, et al. Country as the primary risk factor for renal amyloidosis in familial Mediterranean fever. Arthritis and rheumatism. 2007;56(5):1706-12.[3]Pras M, Bronshpigel N, Zemer D, Gafni J. Variable incidence of amyloidosis in familial Mediterranean fever among different ethnic groups. Johns Hopkins Med J. 1982;150(1):22-6.Disclosure of Interests:None declared

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