Is the performance of the International Severity Scoring System for Familial Mediterranean Fever in children better than other scoring systems?

Author(s):  
Gizem Ozcan ◽  
Semsa Cayci ◽  
Banu Celikel Acar ◽  
Ozge Basaran ◽  
Fatma Aydin ◽  
...  
2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1227.3-1228
Author(s):  
M. E. Tezcan ◽  
N. Şen ◽  
M. Yilmaz ◽  
Ö. Volkan ◽  
E. Tükel ◽  
...  

Background:Familial Mediterranean fever (FMF) is an auto inflammatory disease with recurrent attacks of serositis. Frequent attacks and disease related sequels may be associated with co-morbidities in FMF patients.Objectives:One of the tools for evaluating the FMF severity is the international severity scoring system for FMF (ISSF)1. This score includes disease related sequels, acute phase measurements, attack features and exertional leg pain. Therefore, more severe disease may be link with subclinical inflammation, amyloidosis and frequent, prolonged and widespread attacks. All these components may augment the frequency of non-disease related co-morbidities.Methods:We enrolled 158 FMF patients who fulfilled modifiedTel-HashomerDiagnosisCriteria2. The patients dichotomized based upon disease severity (mild disease or severe disease). Patients with ISSF scores lower or equal to 2 were accepted to have mild disease. Then, we compared frequency of non-disease related co-morbidities between the groups. These co-morbidities arehypertension, hypothyroidism, hyperthyroidism cardiovascular diseases, coronary artery diseases, cerebrovascular diseases, chronic renal disease (non-FMF related), chronic obstructive pulmonary diseases, and diabetes mellitus. This study was approved by the Local Research Ethics Committee and carried out in compliance with the Helsinki Declaration. All the patients gave written informed consent. P-value lower than 0.05 was considered as statistically significant.Results:Demographic features, disease duration, smoking history and body mass index (BMI) were similar between the groups. Frequency of co-morbidity in severe disease group was statistically higher than mild disease group (p=0.02). Most frequent co-morbidity was hypertension in both groups.Table.Features of mild and severe FMF groupsMild (n=135)Severe (n=23)pGender (M/F)47/8811/120.23Age36.4±11.336.5±14.30.68Smoking (%)38 (28.1)5 (21.7)0.52BMI (kg/m2)24.3±9.224.0±8.90.34Disease duration (year)7.7±11.38.6±14.30.09Amyloidosis (%)2 (1.4)3 (13.0)0.02Exon 10 homozygote (%)35 (25.9)9 (39.1)0.19Colchicine dosage (mg/day)1.2±0.41.4±0.50.02ISSF scores0.7 ±0.73.4±0.5<0.001Co-morbidity (%)25 (18.5)9 (39.1)0.02Conclusion:In our FMF patient cohort, we found that severity of the disease may be associated with higher frequency of co-morbidities. Therefore, clinicians should be aware of the high possibility of co-morbidities in patients with more severe FMF and addressed these co-morbidities timely and properly.References:[1]Demirkaya E, et al. Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF). Ann Rheum Dis 2016;75:1051-6.[2]Berkun Y, et al. Diagnostic criteria of familial Mediterranean fever. Autoimmun Rev 2014;13:388-90.Acknowledgments:NoneDisclosure of Interests:None declared


2015 ◽  
Vol 148 (4) ◽  
pp. S-1048-S-1049
Author(s):  
Sombat Treeprasertsuk ◽  
Puth Muangpaisarn ◽  
Panida Piyachaturawat ◽  
Naruemon Wisedopas ◽  
Piyawat Komolmitr ◽  
...  

2020 ◽  
Vol 1 (1) ◽  
Author(s):  
Aliyu D Waziri ◽  
Sani Awwalu ◽  
Ismaila N Ibrahim ◽  
Ibrahim U Kusfa ◽  
Aishatu M Suleiman ◽  
...  

Background: Sickle cell Anaemia (SCA) severity scoring has remained challenging. Scoring systems based on clinical and certain laboratory parameters are limited in simplicity and wide applicability. This is further worsened by dearth of exhaustive and accurate patient history. This study compared a simple, laboratory parameter-based severity scoring system to a pre-existing scoring system. Methodology: Derivation group (n = 45) and validation group (n = 300) were enrolled during steady state. Complications, haemoglobin (Hb) concentration and white blood cell counts (WBC) were documented. Severity scores were computed for each participant based on number of complications, Hb and WBC counts using a preexisting scoring system. Proposed scoring system


2008 ◽  
Vol 68 (2) ◽  
pp. 246-248 ◽  
Author(s):  
S Ozen ◽  
N Aktay ◽  
E Lainka ◽  
A Duzova ◽  
A Bakkaloglu ◽  
...  

Background:Worldwide, familial Mediterranean fever (FMF) is the most common autoinflammatory disease. It has been suggested that environmental factors affect the phenotype as some patients do not develop the complication of secondary amyloidosis.Objective:To analyse whether disease severity in Turkish children with FMF, living in Turkey and Germany is different.Patients and methods:A total of 55 Turkish children living in Turkey were compared with 45 Turkish children born and raised in Germany. Mean age among the group from Turkey and Germany was 42.2 and 44.29 months, respectively. M694V was the leading mutation in both groups. The severity scores were compared with two scoring systems, modified according to published paediatric data for dosage.Results:There was no significant difference between the mean C-reactive protein and erythrocyte sedimentation rate levels of the two groups. According to the modified Sheba Center score, 78.2% of patients from the group living in Turkey had a severe course compared with 34.1% from the group living in Germany. The modified score of Pras et al also showed more severe disease in the patients from Turkey. The difference between the two groups for both scoring systems were significant (both p<0.05).Conclusions:We believe the modified scores that we introduce can be widely used for children. Our results suggest that the environment affects the phenotype of a monogenic disease of the innate inflammatory pathway.


PEDIATRICS ◽  
2001 ◽  
Vol 108 (1) ◽  
pp. 215-215 ◽  
Author(s):  
N. Akar ◽  
E. Akar ◽  
F. Yalcinkaya; ◽  
G. J. Halpern ◽  
A. Mimouni ◽  
...  

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