Left ventricular diastolic function evaluated with tissue Doppler imaging in children with familial Mediterranean fever

The aim of this study was to assess the left ventricular diastolic function using conventional echocardiography and tissue Doppler imaging in children with familial Mediterranean fever. This study included 29 (13 males and 16 females) patients and 30 healthy subjects as controls. Body mass index was calculated and arterial blood pressure was monitored. After an overnight fast, venous blood samples were taken and serum amyloid A protein, C-reactive protein, serum-fasting glucose, total cholesterol, triglycerides, high-density lipoprotein cholesterol, very low density lipoprotein cholesterol, and serum low-density lipoprotein cholesterol levels were measured. A complete 2-dimensional, M-mode, pulse wave Doppler, and pulse wave tissue Doppler echocardiographic examination was performed. There were no significant differences between the groups regarding age, body mass index values, systolic and diastolic blood pressures, heart rates, serum-fasting glucose, total cholesterol, triglycerides, high-density lipoprotein cholesterol, very low density lipoprotein cholesterol, and serum low-density lipoprotein cholesterol. Serum levels of inflammatory markers were higher in patients’ group (C-reactive protein and serum amyloid A protein levels were 10.84 mg/dl, 22.32 mg/l in patients’ group, respectively, and 4.11 mg/dl, 3.65 mg/l, respectively, in the healthy controls.) Peak mitral A wave, E and A wave ratio differed significantly in both groups. There were statistically significant differences regarding parameters observed by tissue Doppler imaging such as E′m, A′m, E′m, and A′m ratio between patients’ group and controls. Tissue Doppler imaging provided additional information on left ventricular diastolic function. While systolic functions were in normal range, some of the diastolic function parameters were impaired in patients with familial Mediterranean fever during childhood.     

Continuity of cytokine activation in patients with familial Mediterranean fever

Familial Mediterranean fever (FMF) is a recessively inherited inflammatory disorder, characterized by recurrent attacks of fever and polyserositis. It has been considered that miscellaneous cytokines take part in the pathogenesis of the disease. The aim of this study was to investigate serum levels of soluble interleukin-2 receptor (sIL-2R), interleukin-6 (IL-6), and interleukin-10 (IL-10) in patients with FMF. The study included 42 patients with FMF (3 females, 39 males, mean age: 24.43 years) and 20 healthy volunteers as the control group (18 males, 2 females, mean age: 23.2 years). The patients were chosen according to Eliakim criteria. After recording their history and performing an examination, leukocyte counts, erythrocyte sedimentation rates (ESR), C-reactive protein (CRP), fibrinogen, sIL-2R, IL-6, and IL-10 levels were measured before and during attacks. A significant increase was found in leukocyte (p<0.001), ESR (p<0.001), CRP (p<0.001), and fibrinogen (p<0.001) levels of the patient group in the attack period compared to those in the quiescent state. sIL-2R (p=0.019) and IL-6 (p<0.001) levels showed significant increases during attacks compared to the levels before an attack. There was no significant difference between IL-10 levels. The levels of the three cytokines were significantly high both before and during the attacks compared to the control group. As a result, the elevation of sIL-2R and IL-6 levels both before and during the attacks compared to control group suggests the existence of continuous cytokine activation in the patients. No significant increase in the IL-10 levels in spite of the significant rise of sIL-2R and IL-6 during attacks supports the notion of inflammation and also reveals that compensation by anti-inflammatory IL-10 does not seem to occur.     

Adrenal axis functions in patients with familial Mediterranean fever

Objective: Familial Mediterranean fever (FMF) is a hereditary disease characterized by recurrent attacks of fever with peritonitis, arthritis, pleuritis or erysipelas-like rash. It is unclear what effects of FMF itself on endocrine system and hormones are. None of the FMF patients without amyloidosis have been reported to have any endocrine disorders, except those who developed colchicine-induced diabetes insipidus. There is a large body of evidence to show that cytokines (IL-1, IL-6 and TNF-α) activate the hypothalamic-pituitary-adrenal (HPA) axis. We have designated this study to investigate the HPA axis in FMF patients without amyloidosis. Methods: Twenty-one patients with FMF were included. ACTH stimulation test was performed on the healthy subjects and during attack period in the patients. In the patient group, same test was repeated during remission period. Results: Peak cortisol levels were significantly higher in the attack period than those in the remission period of patients (p<0.05). Conclusion: The cytokines play a role on the activation of the HPA axis; we thought the axis would be affected in this disease. The response of cortisol to 250 μg ACTH was significant in attack period when compared with remission period. This result reveals that HPA axis is more activated in an FMF attack. Previous studies suggest that the adrenal hormones increase in acute inflammatory events, and eventually, the changes on these hormones are related to TNF and IL-6 levels. During the FMF attack, HPA axis may be stimulated by cytokines. It seems that HPA axis is regulated normally in FMF patients.An erratum to this article can be found at     

QT dispersion and transmural dispersion of repolarization in patients with familial Mediterranean fever

Abstract

Familial Mediterranean fever (FMF) is a disease characterized by sporadic, paroxysmal attacks of fever and serosal inflammation. QT dispersion (QTd) and transmural dispersion of repolarization (TDR), simple noninvasive arrhythmogenic markers, that can be used to assess homogeneity of cardiac repolarization, have not been studied in FMF patients before. The aim of our study was to evaluate the QTd and TDR in FMF patients without overt cardiac involvement. A total of 50 patients with FMF (30 men, 20 women, 29.4 ± 11.8 years) and 50 controls (30 men, 20 women; mean age 31.3 ± 11.9 years) were included. QTd, corrected QTd (cQTd), maximum QT (QTmax), maximum corrected QT (cQTmax), minimum QT (QTmin), and minimum corrected QT intervals (cQTmin) and TDR were measured from standard 12-lead electrocardiography (ECG). We found that QTd, QTmax, and TDR were greater in FMF patients than in the control group (36.0 ± 11.4 vs. 20 ± 11.2, P < 0.001 and 354.8 ± 30.9 vs. 342.8 ± 18.0, P = 0.02; 62.0 ± 16.0 vs. 49.0 ± 9.5 P < 0.001, respectively), as were cQTd and cQTmax (40.4 ± 13.5 vs. 21.9 ± 12.4, P < 0.001 and 397.7 ± 40.2 vs. 375.5 ± 25.4 P = 0.001). A modest positive correlation was found between cQTd and C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR) (r = 0.30, P < 0.001; r = 0.40, P < 0.001; respectively). QTd, which is an index of inhomogeneity of ventricular repolarization and an important predictor of cardiovascular mortality, and TDR, which is a better marker of cardiac repolarization, increased in FMF patients similarly as in other rheumatologic diseases.     

Improved left and right ventricular functions with trimetazidine in patients with heart failure: a tissue Doppler study

Downregulation of glucose and fatty acid oxidation occurs in heart failure (HF). Trimetazidine reduces fatty acid oxidation and increases glucose oxidation. In this single-blind study, trimetazidine, 20 mg three times per day (n = 51) or placebo (n = 36) was added to treatment of 87 HF patients receiving optimal HF therapy. Etiology of heart failure was coronary artery disease in 35 patients (68.6%) in the trimetazidine group and 22 (62.9%) in the placebo group. Fourteen (27.5%) patients in the trimetazidine group and 11 (31.4%) patients in the placebo group had diabetes. Peak systolic velocity (Vs), and the peak early diastolic (Vd) and late diastolic (Va) velocities of various segments left and right ventricles (RV) were obtained with tissue Doppler imaging (TDI) and averaged. Patients were re-evaluated three months later. Significant increases in mean left ventricular ejection fraction (LVEF) (33.3% ± 5.6% to 42.4% ± 6.3%, P < 0.001 and 30.6% ± 8.2% to 33.2% ± 6.6%, P = 0.021) and LV and RV myocardial velocities and mitral and tricuspid annular TDI velocities were observed in both groups. However, compared to placebo, increments in LVEF (9.1% ± 4.2% vs. 2.5% ± 1.4%, P < 0.001) and myocardial velocities were significantly higher with trimetazidine (P < 0.001 for LV Vs, Vd, Va; P = 0.035 for RV Vd; and P < 0.001 for RV Va and Vs). Increase in LVEF with trimetazidine was significantly correlated with presence of diabetes (r = 0.524, P < 0.001). With trimetazidine LVEF increased significantly more in diabetic patients compared to nondiabetics (P < 0.001). Also, patients having both diabetes and ischemic HF tended to have greater improvement in LVEF compared to ischemic HF patients without diabetes (P = 0.063). Addition of trimetazidine to current treatment of HF, especially for those who are diabetic, may improve LV and RV functions.     

P-wave duration and dispersion in children with uncomplicated familial Mediterranean fever

Objectives

This was a prospective controlled study to determine the P-wave duration (Pdu) and P-wave dispersion (Pd) in patients with familial Mediterranean fever (FMF).

Methods

The study group consisted of 26 children with uncomplicated FMF and 25 age- and sex-matched healthy controls. We performed electrocardiography (ECG) with Doppler echocardiography on patients and controls. All participants underwent 12-lead electrocardiography under strict standards. Pdu and Pd were computed from a randomly selected beat and from an averaged beat constructed from 12 beats, included in a 10-s ECG.

Results

The left ventricle (LV) dimensions, LV ejection fraction (LVEF), and LV fractional shortening (LVFS) values, left atrium dimension, and aortic dimension were in normal range in both groups. There were significant differences between the groups regarding LV-isovolumic relaxation time (IRT), LV-isovolumic contraction time (ICT), right ventricle (RV)-ICT, RV-IRT, and Pd (all p < 0.0001). However, highly significant positive correlation was detected between LV-ICT, LV-IRT, RV-ICT, RV-IVT, C-reactive protein (CRP), and Pd (r = 0.505, p < 0.0001; r = 0.483, p < 0.0001; r = 0.433, p = 0.001; r = 0.421, p = 0.001; r = 0.452, p = 0.001; r = 0.478, p < 0.0001, respectively).

Conclusions

Uncomplicated FMF children who are continuously treated with colchicine and do not develop amyloidosis have abnormal atrial dispersion and therefore seemingly have an increased electrocardiographic risk of atrial fibrillation.     

Colchicine treatment in children with familial Mediterranean fever

Prophylactic colchicine therapy has been shown to be a safe and effective method of eliminating the attacks and preventing the development of amyloidosis in patients with familial Mediterranean fever (FMF). However, information about effective dosages that control FMF attacks and prevent amyloidosis in childhood is not available. The aim of this study is to determine the effective colchicine dose for children in terms of body weight and surface area. Sixty-two (34 male, 28 female) children with FMF were selected and colchicine treatment was initiated by giving 0.5–1 mg/day to each patient. The dose was gradually increased up to a maximum 2 mg/day in unresponsive patients; mean duration of therapy was 45.6 ± 35.5 months. When the optimal effective dosage (i.e. the one that reduced the frequency of attacks and ESR, CRP and fibrinogen levels during the attack-free period) was achieved, the optimal effective dose was calculated according to the body weight and body surface area for each patient. Based on these values mean colchicine dose was computed for the study group and values for different age groups were evaluated. Mean colchicine doses according to the body weight and surface area of the whole group were found to be 0.03 ± 0.02 mg/kg/day and 1.16 ± 0.45 mg/m²/day, respectively. It was shown that children less than 5 years of age might need colchicine doses as high as 0.07 mg/kg/day or 1.9 mg/m²/day. These dosages are approximately 2.5–3 times more than the mean colchicine dose for children aged 16–20 years. These results clearly show that small children need higher doses of colchicine in order to control their attacks. Thus, we conclude that colchicine, when given according to body weight or body surface area, would be more effective in childhood.     

The expanded clinical spectrum of familial Mediterranean fever

The classical clinical features of familial Mediterranean fever (FMF) are recurrent self-limited attacks of fever and serositis. Amyloidosis of the AA type is the major complication of the disease. The diagnosis of FMF is still based on a history of typical acute attacks, ethnic background, and frequently, notable family history. Together with the discovery of MEFV gene, the clinical criteria for the diagnosis of the disease did not change. Although we have learned a great deal about the clinical features and the pathogenesis of FMF in the past few years, many atypical cases emerge, and caution should be exercised during diagnosis. In this report, we present three FMF patients not fulfilling clinical criteria for the diagnosis, discuss rare and unusual presentations of the disease, and emphasize the role of genetic analysis in these suspicious cases.     

Acute effects of biventricular pacing on right ventricular function assessed by tissue Doppler imaging.

AIMS: The benefits of cardiac resynchronization therapy (CRT) on functional status, left ventricular (LV) remodelling and survival in patients with drug-refractory congestive heart failure (CHF), LV systolic dysfunction, and wide QRS have been demonstrated in randomized trials. However, the impact of CRT on right ventricular (RV) function, an independent prognostic factor in CHF remains questionable. This study examined the acute effects of various pacing modes on RV function in recipients of CRT systems. METHODS AND RESULTS: Echocardiographic examinations were performed in 15 patients (median age: 67 years, range 49-78), to compare RV function during atrial (AAI), RV and LV pacing, and biventricular (BiV) pacing, in random order. At baseline, the median LV ejection fraction was 20% (range 10-35) and the median LV end-diastolic diameter was 78 mm (range 62-85). Right ventricular function was impaired, with a median 36% fractional shortening of RV surfaces (7-59). Tissue Doppler systolic peak of velocity (Sa) recorded at the tricuspid annulus increased significantly from 9.9 cm/s (range 4.7-16.5) during AAI pacing, 10 cm/s (range 5.4-20.3) during RV pacing, and 11.7 cm/s (range 4.6-16.7) during LV pacing to 12.6 cm/s (range 6.6-19.1) during BiV pacing (P < 0.01). Trends toward improvements in other indices of RV function, particularly myocardial performance index and systolic excursion of the tricuspid annulus, were also observed. CONCLUSIONS: This short-term study showed a significant improvement in RV systolic function during BiV pacing compared with AAI, RV, or LV pacing in CRT recipients.     

The relations between attacks and menstrual periods and pregnancies of familial Mediterranean fever patients

Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by short lived, febrile serosal inflammatory attacks. Although majority of patients have random pattern of attacks, some reports described precipitating factors. There are also contradictory reports relating FMF attacks with menstruation and the natural course of their pregnancies. Seventy-two female patients with FMF with a mean age of 34.9±12.4 were interviewed. A standardized questionnaire was used inquiring any associations of FMF attacks of the patients with their menstruations and pregnancies. Thirty-eight patients (53%) reported that their attacks frequently coincided with their menstrual cycles and 17 patients noticed pleuritic chest pain in addition to their abdominal attacks. One patient experienced only febrile pleural attacks during her menstrual cycles. Unlike dysmenorrhoea, none of these patients’ attacks responded to non-steroidal anti-inflammatory drugs. All of the patients could correctly differentiate their FMF attacks from dysmenorrhoea. Forty patients could give detailed information about the frequency and severity of their FMF attacks during 73 pregnancies: 25 patients (62.5%) experienced complete symptomatic remissions; the attacks were aggravated (7 patients), ameliorated (6 patients) or did not change (2 patients) in the rest of the pregnancies. Four patients continued to use colchicine during their pregnancies and delivered healthy babies. One patient gave birth to a child with Down’s syndrome although she was not on colchicine therapy. Although FMF attacks and discomforts of menstrual cycles do overlap frequently, patients can easily differentiated them. Patients can be reasonably assured that the period of pregnancy will be comfortable but abstaining from colchicine should not be recommended. Gynecologists must be aware of FMF in the differential diagnosis of dysmenorrhoea or endometriosis.     

Encapsulating peritonitis and familial Mediterranean fever

AIM: To investigate the relationship between encapsulating peritonitis and familial Mediterranean fever (FMF). METHODS: The patient had a history of type 2 diabetes and Iaparoscopic cholecystectomy was performed one year ago for cholelithiasis. Eleven months after the operation she developed massive ascites. Biochemical evaluation revealed hyperglycemia, mild Fe deficiency anemia, hypoalbuminemia and a CA-125 level of 2 700 IU. Ascitic evaluation showed characteristics of exudation with a cell count of 580/mm~3. Abdominal CT showed omental thickening and massive ascites. At exploratory laparotomy there was generalized thickening of the peritoneum and a Iaparoscopic clip encapsulated by fibrous tissue was found adherent to the uterus. Biopsies were negative for malignancy and a prophilactic total abdominal hysterectomy and bilateral salpingooophorectomy were performed. RESULTS: The histopathological evaluation was compatible with chronic nonspecific findings and mild mesothelial proliferation and chronic inflammation at the uterine serosa and liver biopsy showed inactive cirrhosis. CONCLUSION: The patient was evaluated as sclerosing encapsulating peritonitis induced by the iaparoscopic clip acting as a foreign body. Due to the fact that the patient had FMF the immune response was probably exaggerated.     

Pulsed Doppler tissue imaging can help to identify patients with right ventricular infarction

This study was planned to assess whether tissue Doppler imaging is a useful method for the detection of the right ventricular myocardial infarction. Forty-eight patients with acute inferior myocardial infarction and 24 age- and sex-matched healthy controls were included in this study. Twenty-four patients had electrocardiographic signs of inferior myocardial infarction without right ventricular infarction (group I), and the other 24 patients had electrocardiographic signs of inferior myocardial infarction with right ventricular infarction (group II). From the echocardiographic apical four-chamber view, peak systolic, early diastolic, and late diastolic velocities of the tricuspid annulus at the right ventricular free wall were recorded with the use of pulsed-wave Doppler tissue imaging. The tricuspid annular peak tissue Doppler imaging systolic velocity was significantly lower in group I (14.03 ± 2.57cm/s, P 0.005) and in group II (8.50 ± 0.84cm/s, P 0.005) than in controls (16.63 ± 2.31cm/s). The tricuspid annular peak systolic (8.50 ± 0.84cm/s vs 16.63 ± 2.31cm/s) and peak early diastolic (10.99 ± 3.28cm/s vs 19.39 ± 4.3cm/s) velocities were significantly lower in group II than in group I, as compared with controls (P 0.001). Peak early diastolic velocity of tricuspid annulus (10.99 ± 3.28cm/s vs 19.39 ± 4.3cm/s) was significantly lower in group I than in controls (P 0.001); however, late diastolic velocity was significantly lower in group II (15.98 ± 5.08cm/s, P 0.05) than in group I (18.21 ± 2.63cm/s, P 0.05) and in controls (19.02 ± 5.29cm/s). The results of this study indicate that tricuspid annular peak systolic and early diastolic velocities are reduced in patients with right ventricular infarction. The velocity of the tricuspid annulus by tissue Doppler imaging is simple and can be used to distinguish whether patients with inferior myocardial infarction have right ventricular infarction.This study was presented at the XXIII. Congress of the European Society of Cardiology, Stockholm, Sweden, 1–5 September 2001     

Magnetic resonance imaging can detect thoracic inflammation due to familial Mediterranean fever

A 32-year-old man presented to our hospital complaining of periodic fever and unilateral chest pain. We suspected that he had familial Mediterranean fever because of his symptoms. Magnetic resonance imaging (MRI) showed an increased intensity within the anterior chest wall, which was consistent with the site of his pain. Genomic analysis showed the patient to be heterozygous for the E148Q/M694I mutation in the MEFV gene, and we diagnosed familial Mediterranean fever. The ability of MRI to detect inflammatory changes could provide useful additional information for evaluating thoracic symptoms in FMF patients, and the detection of inflammatory changes using MRI may aid in early diagnosis, thus contributing to early and adequate treatment.     

Coexistence of familial Mediterranean fever and psoriasis in a patient with seronegative spondyloarthropathy

Familial Mediterranean fever (FMF) is a self-limited disease characterized by fever and polyserositis attacks. Arthritis caused by synovitis is either in acute monoarthritis or chronic mono-oligoarthritis form, usually affecting the lower extremities. Another potential but rare form of involvement is spondyloarthropathy (SSpA). Psoriatic arthritis (PsA) is inflammatory arthropathy of peripheral joints, spine and enthesis areas. Some PsA cases are classified as psoriatic spondyloarthropathy. A 43-year-old male patient with concomitant FMF and psoriasis presenting with bilateral sacroiliitis, chronic hip and knee arthritis has been presented along with follow-up findings and treatment options used.     

Plasma interleukin-10 and interleukin-12 levels in patients with familial Mediterranean fever

Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent attacks of fever, polyserositis and arthritis. A vast array of cytokines were analysed in these patients, however, little is known about the pro-inflammatory cytokine interleukin (IL)-12. Plasma IL-12 and IL-10 were measured in 24 patients with FMF (19 active, 5 inactive) and 18 healthy controls by ELISA. From 15 active patients blood was also drawn in attack-free period. Mean plasma IL-12 levels of the FMF patients (mean ± SEM, 6.84±3.59 pg/ml) were higher than the controls (0.13±0.09 pg/ml, P<0.001). Mean IL-12 levels of active (7.02±5.23 pg/ml) and inactive patients (6.89±5.61 pg/ml) were comparable, and they were higher compared to controls (P≤0.001). Mean plasma IL-10 levels of the total FMF patients (3.01±1.53 pg/ml) were also higher than the controls (P=0.024). Patients had higher IL-10 levels in attacks (3.83±2.02 pg/ml) compared to levels when they were in remission (1.86±1.59 pg/ml, P=0.046). Significantly elevated IL-12 levels in FMF patients regardless of activity may suggest the presence of a pro-inflammatory state also in the inactive period of FMF. Significant increase in IL-10 levels in FMF group may point to the compensatory suppression of inflammation in active periods of the disease.     

Tumor necrosis factor-alpha gene promoter polymorphism in patients with familial Mediterranean fever

The proinflammatory cytokine tumor necrosis factor-alpha (TNF-α) plays an important role on the course of disease in familial Mediterranean fever (FMF). TNF-α gene promoter polymorphism may be a marker of susceptibility and severity of FMF. The aim of this study is to evaluate both TNF-α/238 and TNF-α/308 genotypes and allelic distribution in patients with FMF. Forty-one FMF patients and 43 healthy volunteers were included in the study. Genomic DNA was extracted from EDTA-preserved whole blood of whole series of patients and controls. Polymorphism of TNF-α promoter at positions −238 and −308 were detected by using amplification refractory mutation system polymerase chain reaction. TNF-α/238 and TNF-α/308 genotype distributions and allele frequencies of FMF patients and healthy volunteers were found to be similar. Moreover, there was no association between TNF-α/238 and TNF-α/308 genotypes and the frequency of acute attacks in FMF. TNF-α/238 and TNF-α/308 promoter polymorphisms do not seem to be major genetic risk factors for susceptibility to FMF and severity of the disease.     

The evaluation of carotid intima-media thickness in children with familial Mediterranean fever

The aim is to investigate whether pediatric familial Mediterranean fever (FMF) patients have an increased risk of premature atherosclerosis and to determine the possible strength of association between atherosclerosis and Mediterranean fever (MEFV) gene mutation gene type. Demographic characteristics and MEFV mutations were defined in 49 children diagnosed with FMF (26 female, 23 male; mean age, 10.71 ± 3.69 years). Twenty-six age-, sex-, and body-mass-index-matched healthy children constituted the control group. We evaluated the blood counts and acute-phase proteins during attack-free periods. Mean C-reactive protein (CRP), serum amyloid-A (SAA), homocysteine (Hcy), lipoprotein-a (Lp-a), and common carotid artery intima-media thickness (CCA-IMT) were 10.75 ± 15.29 vs 4.03 ± 1.20, 23.22 ± 41.94 vs 3.53 ± 1.04, 10.36 ± 3.36 vs 8.64 ± 3.15, 20.84 ± 23.89 vs 8.56 ± 7.48, and 0.038 ± 0.007 vs 0.032 ± 0.004, respectively, and significantly higher than the mean values of control group (p < 0.05). However, no correlation was found between CCA-IMT and CRP, SAA, Hcy, and Lp-a. Twenty-nine patients had M694V mutation, and 13 patients had other mutations. There was no correlation between CCA-IMT and MEFV mutation subgroups. In conclusion, because of the nature of the disease, FMF patients should be considered to have an increased risk of early vascular alteration and atherosclerosis. For this reason, CCA-IMT measurement can be recommended as a noninvasive and early diagnostic method.     

A comparison of clinical findings of familial Mediterranean fever patients with and without amyloidosis

Objective This study investigates the clinical and demographic characteristics of familial Mediterranean fever (FMF) patients with and without amyloidosis.Patients and methods The clinical data of 503 patients with FMF (females:males 250:253) were reviewed. Fifty of these patients had amyloidosis (f:m 23:27).Results The ages of attack onset in patients with and without amyloidosis were 7.8±6.2 and 11.1±8.5, respectively (P<0.05). The time between disease onset and diagnosis was longer in patients with amyloidosis than those without (187.6±99.4 months and 132.5±110.2 months, respectively, P<0.001). More patients in the amyloidosis group had positive family histories of FMF (68% vs 54%, P<0.05). The frequencies of chest pain (78% vs 51%, P<0.001), arthritis ( 80% vs 60%, P<0.01), and erysipelas-like erythema (44% vs 16%, P<0.001) were higher in the amyloidosis group.Conclusion In the amyloidosis group, FMF-related manifestations of chest pain, arthritis, and erysipelas-like erythema are more frequent. Our results also support that long periods between disease onset and diagnosis are associated with a high risk of developing amyloidosis.     

Osteopoikilosis coexistent with ankylosing spondylitis and familial Mediterranean fever

Osteopoikilosis (OPK) is a rare benign sclerosing disease of the skeleton and inherited as an autosomal dominant trait. OPK is associated with inflammatory rheumatic disorders, such as rheumatoid arthritis, scleroderma, reactive arthritis and familial Mediterranean fever (FMF). We report a rare case of OPK coexistent with ankylosing spondylitis and FMF. The patient presented multiple sclerotic lesions within and around the sacroiliac joints and a series of radiological diagnostic challenges.