Mustafa Kurucay

Disease severity in adult patients of Turkish ancestry with familial mediterranean fever living in Germany or Turkey. Does the country of residence affect the course of the disease

BackgroundThe environment may affect the course of familial Mediterranean fever (FMF). ObjectiveThe objective of this study was to compare disease severity between adult FMF patients in Turkey (TR) and Germany (G). MethodsAdult FMF patients of Turkish ancestry on colchicine living in Turkey (n = 40) or G (n = 35) were compared. Disease severity, C-reactive protein (CRP), and erythrocyte sedimentation rate were assessed. ResultsGroups differed significantly in the following aspects: age at onset of disease (TR: 15.6, G: 10.8 years; P = 0.02), delay between onset and initiation of colchicine treatment (TR: 6.8 years, G: 14.9 years; P < 0.001), female gender (TR: 80%, G: 57.1%; P = 0.04), and duration of disease (TR: 14.4 years, G: 23.4 years; P < 0.001). There was no significant difference in colchicine treatment concerning average dosing and duration of therapy. No significant difference could be found between the 2 groups in CRP and disease severity as assessed by the score of Pras et al. (Am J Med Genet. 1998;75:216–219) even after adjusting for potential confounding variables. Mean erythrocyte sedimentation rate was significantly higher among patients living in G (TR: 13.2 mm/first hour, G: 26.3 mm/first hour; P < 0.001). Among patients living in Germany, there was a significant difference in age at FMF onset depending on their country of birth (born in TR: 14.9 years, born in G: 6.9 years; P = 0.0001). ConclusionsIn adult FMF patients living in Turkey or Germany, no difference in disease activity or CRP could be found. German patients were younger at onset of disease and had a longer delay between onset and initiation of colchicine treatment.

Anxiety and depression in adult patients with familial Mediterranean fever: a study comparing patients living in Germany and Turkey

To determine the prevalence of anxiety and depression among patients with familial Mediterranean fever (FMF) living in Germany or Turkey a prospective study was conducted.

P wave dispersion and QT dispersion in adult Turkish migrants with familial mediterranean fever living in Germany.

Background: Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease associated with subclinical inflammation, which includes atherosclerosis arising from endothelial inflammation, which in turn increases the risk of atrial or ventricular arrhythmias. Conduction abnormalities can be detected using the electrocardiographic (ECG) indices P and QT dispersion (Pdisp and QTdisp). Currently, it is unknown whether patients with FMF are more likely to have abnormalities of these ECG indices. Moreover, existing studies were conducted in countries with higher FMF prevalence. We therefore perform the first prospective study assessing Pdisp and QTdisp in adult FMF patients in Germany, where prevalence of FMF is low. Method: Asymptomatic FMF patients (n=30) of Turkish ancestry living in Germany and age-matched healthy controls (n=37) were prospectively assessed using 12-lead ECG. Results: Patients and controls were comparable in gender and body mass index, and patients had higher erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) and serum amyloid A (SAA) compared to controls (ESR: 23.7±14.3 vs. 16.1±13,3 mm/1sth, p=0.03, CRP: 0.73±0.9 vs. 0.26±0.4 g/dl, p=0.01, SAA: 3.14±4,8 vs. 0.37±0.3 mg/dl, p<0.01). No statistically significant difference between patients and controls respectively, for Pdisp (43.7±11.9 vs. 47.1±11.2ms, p=0.23), QTdisp (65.9±12.3 vs. 67.6±12.7 ms, p=0.58) or corrected QTdisp (cQTdisp: 73.9±15.0 vs. 76.0±13.3 ms, p=0.55) was found. No correlation could be found between Pdisp or QTdisp or cQTdisp and any of the biochemical markers of inflammation. Conclusion: FMF patients living in Germany show a Pdisp and QTdisp comparable to healthy controls, with no increased risk of atrial or ventricular arrhythmias indicated.

[Self-medication to treat pain in attacks of familial Mediterranean fever: aiming to find a new approach to pain management].

BACKGROUND Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by bouts of fever and serositis. Morbidity caused by bouts as well as self-medication were assessed among patients of Turkish ancestry living in Germany (D) or Turkey (T) in order to evaluate current analgetic concepts from a patients perspective. MATERIAL AND METHODS D and T were asked about the 3 months preceding the interview. RESULTS A total of 40 D and 40 T were included; 35/40 D and 40/40 T were on colchicine. In the last 3 months, 61.3 % had ≥ 1 bout and suffered from peritonitis (87.8 %), fever (61.2 %), myalgia (45 %), pleuritis (42.8 %), arthralgia (36.7 %), and cephalgia (32.6 %). Of the patients, 65.3 % were bedridden during bouts, 61.2 % sought the attention of a physician, 53.1 % were unable to work or attend school, and 38.8 % were hospitalized. The following drugs were taken: NSAIDs (45.6 %), NSAIDs and paracetamol (42.6 %), and combinations of NSAIDs with other analgesics. NSAIDs (58.6 %) and paracetamol (20.7 %) were considered the most potent substances. CONCLUSION FMF inflicts substantial morbidity. Patients most commonly rely on NSAIDs and paracetamol to relieve symptoms of FMF bouts.

Sonographic assessment of spleen size in Turkish migrants with Familial Mediterranean fever in Germany.

Familial Mediterranean fever (FMF) can be associated with splenomegaly. Prospective quantitative data are lacking. We performed a sonographic assessment of spleen size in patients with FMF and healthy control participants to assess its diagnostic value.

Schmerztherapeutische Bedarfsmedikation bei Attacken von familiärem Mittelmeerfieber

BACKGROUND Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by bouts of fever and serositis. Morbidity caused by bouts as well as self-medication were assessed among patients of Turkish ancestry living in Germany (D) or Turkey (T) in order to evaluate current analgetic concepts from a patients perspective. MATERIAL AND METHODS D and T were asked about the 3 months preceding the interview. RESULTS A total of 40 D and 40 T were included; 35/40 D and 40/40 T were on colchicine. In the last 3 months, 61.3 % had ≥ 1 bout and suffered from peritonitis (87.8 %), fever (61.2 %), myalgia (45 %), pleuritis (42.8 %), arthralgia (36.7 %), and cephalgia (32.6 %). Of the patients, 65.3 % were bedridden during bouts, 61.2 % sought the attention of a physician, 53.1 % were unable to work or attend school, and 38.8 % were hospitalized. The following drugs were taken: NSAIDs (45.6 %), NSAIDs and paracetamol (42.6 %), and combinations of NSAIDs with other analgesics. NSAIDs (58.6 %) and paracetamol (20.7 %) were considered the most potent substances. CONCLUSION FMF inflicts substantial morbidity. Patients most commonly rely on NSAIDs and paracetamol to relieve symptoms of FMF bouts.

Schmerztherapeutische Bedarfsmedikation bei Attacken von familiärem Mittelmeerfieber@@@Self-medication to treat pain in attacks of familial Mediterranean fever: Auf der Suche nach einem schmerztherapeutischen Ansatz@@@Aiming to find a new approach to pain management

BACKGROUND Familial Mediterranean fever (FMF) is an autoinflammatory disease characterized by bouts of fever and serositis. Morbidity caused by bouts as well as self-medication were assessed among patients of Turkish ancestry living in Germany (D) or Turkey (T) in order to evaluate current analgetic concepts from a patients perspective. MATERIAL AND METHODS D and T were asked about the 3 months preceding the interview. RESULTS A total of 40 D and 40 T were included; 35/40 D and 40/40 T were on colchicine. In the last 3 months, 61.3 % had ≥ 1 bout and suffered from peritonitis (87.8 %), fever (61.2 %), myalgia (45 %), pleuritis (42.8 %), arthralgia (36.7 %), and cephalgia (32.6 %). Of the patients, 65.3 % were bedridden during bouts, 61.2 % sought the attention of a physician, 53.1 % were unable to work or attend school, and 38.8 % were hospitalized. The following drugs were taken: NSAIDs (45.6 %), NSAIDs and paracetamol (42.6 %), and combinations of NSAIDs with other analgesics. NSAIDs (58.6 %) and paracetamol (20.7 %) were considered the most potent substances. CONCLUSION FMF inflicts substantial morbidity. Patients most commonly rely on NSAIDs and paracetamol to relieve symptoms of FMF bouts.

PW01-009 – Markers of inflammation in adult FMF patients

The therapeutic goal in Familial Mediterranean Fever (FMF) is to prevent attacks of clinically overt disease as well as to stop or reverse the development of amyloidosis and subsequent organ damage. The dosage of colchicine treatment is therefore generally adjusted according to clinical information and biochemical markers of subclinical inflammation.

THU0382 Familial mediterranean fever (FMF) - disease activity and quality of life in adult patients of turkish ancestry living in turkey or germany

Background Paediatric FMF-patients living in Turkey show a higher proportion of severe disease compared to Turkish children born and raised in Germany1. Objectives To assess differences in the course of FMF in adults living in Turkey and Germany. Methods Adult patients suffering from FMF according to the Livneh criteria2 with Turkish ancestry were prospectively included in the study in two university hospitals in Ankara, Turkey (TR) and Herne, Germany (D). A structured interview and the Turkish WHOQOL-BREF were performed. Results 61 patients were included (TR:31/D:30). The two groups were significantly different in the following aspects (mean±SD): percentage of patients on colchicine treatment (TR:100%/D: 83.3%,p=0.02), daily colchicine dosage in mg among patients taking colchicine (TR: 1.6±0.7/D:1.15±0.7,p=0.009), duration of disease (TR:15.5±9.5/D:22.4±8.2y,p=0.003). In other aspects examined there was no significant difference. The groups were similar in terms of age at study inclusion (TR:30.9±10.8/D:34.2±10.5y), age at onset of FMF symptoms (TR: 15.4±11.3/D:11.8±6.5y), female sex (TR: 74%/D:60%), FMF-severity according to the score by Pras et al.3 (TR: 6.6±2.3/D: 6.4±1.9) and the 4 domains of the WHOQOL-BREF (expressed on a 0-100 scale) physical health (TR:57.5±19/D:59.1±20.4), psychological health (TR: 61.3±17.4/D:66.4±19.2), social relationship (TR:66.4±21.7/D:67±23.3), environment (TR: 62.2±18.4/D:68.5±16.8). There was no relevant and significant correlation between FMF-severity and QOL. In most domains (exception: better psychological health of FMF-patients) QOL in the patients studied was comparable to that of Turkish patients on hemodialysis4. Conclusions 16.7% of FMF-patients of Turkish origin living in Germany included in our study are not taking colchicine (TR: 0%). The average colchicine-dosage in Germany is lower and patients examined in Germany had a longer duration of disease. However, there was no significant difference in disease activity (assessed by the Pras-score) and QOL (assessed by the WHOQOL-BREF) between FMF patients in the two nations. No relevant correlation between FMF disease severity and QOL could be found. References Özen S et al. Ann Rheum Dis 2008;68:246 Livneh A et al. Arthritis Rheum 1997;40:1879 Pras E et al. Am J Med Genet 1998;75:216 Sertoz OO et al. Ther Apher Dial 2009;13:366 Disclosure of Interest A. Giese: None Declared, A. Örnek: None Declared, M. Kurucay: None Declared, L. Kiliç: None Declared, S. Şendur: None Declared, S. Kiraz: None Declared, E. Lainka Grant/Research support from: Part of the study was supported by a grant from the German ministry of education and research (BMBF 01GM08104), B. Henning: None Declared