Impact of rheumatoid arthritis in Turkey: a questionnaire study.

OBJECTIVES: Unmet needs of rheumatoid arthritis (RA) patients regarding physician/patient communication, treatment preferences and quality of life issues were investigated in a Turkish survey study. METHODS: The study was conducted with the contribution of 33 rheumatologists, and included 519 RA patients. The study population included patients who had been on biologic therapy for >6 months and were still receiving biologic therapy (BT group), and those who were biologic naive, but found eligible for biologic treatment (NBT group). Of the RA patients, 35.5% initially had a visit to an internal disease specialist, 25.5% to a physical therapy and rehabilitation specialist, and 12.2% to a rheumatology specialist for their RA complaints. The diagnosis of RA was made by a rheumatologist in 48.2% of patients. RESULTS: The majority of RA patients (86.3%) visit their doctor within 15-week intervals. Most of the physician-patient communication focused on disease symptoms (99.0%) and impact of the disease on quality of life (61.8%). The proportion of RA patients who perceived their health status as good/very good/excellent was higher in the BT group than in the NBT group (74.3% vs. 51.5%, p<0.001). However, of those RA patients in the NBT group, only 24.8% have been recommended to start a biologic treatment by their doctors. With respect to dose frequency options, once-monthly injections were preferred (80%) to a bi-weekly injection schedule (8%). CONCLUSIONS: In conclusion, RA patients receiving biologic therapy reported higher rates of improved symptoms and better quality of life and seemed to be more satisfied with their treatment in our study.

Is there a real adrenal axis dysfunction in patients with amyloidosis associated with familial Mediterranean fever?

Systemic amyloidosis with AA-type amyloid deposition is the major complication of FMF, leading to end stage renal disease. There is no clear data on the prevalence of adrenal involvement in patients with FMF amyloidosis. The aim of this study is to determine the adrenal axis function in patients FMF with amyloidosis. Twenty patients with FMF with amyloidosis (F/M: 10/10, mean age; 38 ± 11 SD years), twenty without amyloidosis (F/M: 14/6, mean age 32 ± 10 years), and healthy controls (F/M: 12/8, mean age: 30 ± 7.6 SD years) were recruited. A dose of 250 mg tetracosactide (Synacthen) was then administered intravenously and further blood samples collected 30 and 60 min later. Blood samples were separated and collected at 4°C, and serum cortisol levels were measured. A normal cortisol response to Synacthen was defined as a post-stimulation peak cortisol value of >18 mg/d either at 30 or 60 min. sample. The mean disease duration was 8.8 ± 6 SD years, (range, 2-21) in FMF patients without amyloidosis compared to 16 ± 9.5 years (range, 0-30) in FMF with amyloidosis (P = 0.001). The cortisol concentrations increased significantly at 30 and 60 min compared to baseline after injection of synacthen in all groups. There were no statistically significant differences found among three groups, for basal, 30 and 60 min for cortisol levels (P = 0.154). FMF patients with amyloidosis do not exhibit overt adrenal insufficiency even though their basal cortisol levels were mildly lower.

The frequency of familial Mediterranean fever in an emergency unit.

Approximately 90% of patients with familial Mediterranean fever (FMF) complain of recurrent attacks of fever and abdominal pain of various severities. Prior to the diagnosis of FMF, the majority of patients are admitted to emergency units with a suspicion of acute abdomen pain and at least half of them undergo unnecessary abdominal interventions. The purpose of this study is to determine the frequency of FMF among the patients who are admitted to emergency units for acute abdominal pain. One hundred consecutive patients who were admitted to an emergency unit in Istanbul, Turkey, with acute abdominal pain were screened for FMF. When the definite cases were considered, a frequency of 2% was encountered which was significantly high compared to the frequency of FMF in Turkey. Physicians working in emergency units should include FMF in their differential diagnosis list when evaluating a patient with acute abdominal pain, especially in countries where the disease is prevalent.

Unusual central nervous system involvement of rheumatoid arthritis: successful treatment with steroid and azathioprine.

Central nervous system involvement of rheumatoid arthritis (RA) frequently develops in patients who had a long-term history of RA, irrespective of the disease activity of systemic arthritis, and it has a high mortality rate despite treatment. Since clinical symptoms and radiologic signs are rather nonspecific, in short of doing biopsy, the diagnosis of rheumatoid meningitis is one of exclusion. However, the strongly positive rheumatoid factor in the cerebrospinal fluid is quite specific. We here report a 70-year-old man who had not been diagnosed as RA before he was admitted with neurological findings, who was diagnosed as RA later and successfully treated with prednisolone and azathioprine.

A twin study in Behçet's syndrome.

OBJECTIVES: Case reports on monozygotic (MZ) twins with Behçet's syndrome (BS) have been few and we are not aware of formal twin studies. We sought the frequency of MZ and dizygotic (DZ) twin births in BS and compared it to a healthy population sample from the same geography. We also looked for the concordance rate among the MZ and DZ twins. METHODS: 1705 (1039M/666F) patients attending a dedicated BS outpatient clinic and 7761 (3848M/3913F) medical school students were asked about having a MZ or DZ twin sibling. MZ and DZ twins thus identified among both patients and controls were individually seen at the clinic. In addition, HLA, DNA microsatellite markers and blood groups were typed to further confirm twin- ship. All twins were contacted 8 years later for new emergence of disease. RESULTS: There were 14 (0.82%) patients with BS and 120 (1.55%) controls who had a twin sibling (p=0.022). Of these, 8 (0.47%) patients with BS and 92 (1.19%) controls had a DZ twin sibling (p=0.009). MZ twin frequency was similar between BS patients (6/1705; 0.35%) and control population (28/7761; 0.36%). The pairwise concordance rate for BS was 2/6 (95% CI: -0.21-0.88) for MZ and 1/8 (95% CI: -0.17-0.42) for DZ twins (p=0.538). Genetic effects accounted for 41% of the phenotypic variance for BS among twins. After 8 years of follow-up, 4 of 6 MZ and 6 of 7 DZ twin pairs were still discordant. CONCLUSIONS: The frequency of MZ twin births in BS is not different than that in the general population while the DZ twins were seen less frequently among the BS patients. The concordances for BS were higher in MZ compared with DZ twins, suggesting genetic predisposition. On the other hand, the persistence of discordance after 8 years of follow up among the remaining MZ twins demands further research to understand non- genetic factors in causation of BS.

Mycophenolate mofetil versus azathioprine in the maintenance therapy of lupus nephritis.

BACKGROUND: Renal involvement is one of the major determinants of the outcome in patients with systemic lupus erythematosus. Renal involvement contributes to both morbidity and mortality of the patients as well as indirectly through side effects of therapy directed at the renal lesions. The aim of the study was to evaluate the efficacy of mycophenolate mofetil (MMF) and azathioprine (AZA) in the maintenance therapy of lupus nephritis. METHODS: Thirty-two patients from our center with diagnosed lupus nephritis World Health Organization Class III, IV, V were treated with IVC (0.75-1g/month) for six months in addition to steroid therapy, and then with AZA (n = 15) or MMF (n = 17) as a maintenance therapy. The efficacy of two drugs was compared with changes in serum creatinine, creatinine clearance, 24 hour urine protein excretion, cholesterol, anti-dsDNA antibody, and urine sediment. RESULTS: Mean follow-up time was 41.5 + 7 months. The total remission occurred in 84% of patients (82% with MMF and 87% with AZA), with a complete remission rate of 59.3% (58% with MMF and 60% with AZA) and a partial remission rate of 25% (22% with MMF and 27% with AZA). The urinary protein excretion before MMF treatment was 1.9 + 1 g/dL and decreased significantly to 0.91 + 0.6 g/dL (p = 0.028) after treatment, and decreased from 1.58 + 0.7 g/dL to 0.4 + 0.23 g/dL in the AZA group (p = 0.04). The serum creatinine level decreased from 1.32 + 0.7 mg/dL to 1.12 + 0.68 mg/dL in the MMF group (p = 0.23), and decreased from 0.91 + 0.23 mg/dL to 0.88 + 0.23 mg/dL in the AZA group (p = 0.49). There was no significant change between two groups (p = 0.1). The serum cholesterol decreased from 229 + 57 mg/dL to 171 + 9 mg/dL (p = 0.002), and serum triglyceride level decreased from 228 + 116 mg/dL to 98 + 35 mg/dL (p = 0.004) in the MMF treatment, but no significant change was seen in AZA group. There was no significant difference between the two groups considering the rates of doubling of serum creatinine, progression to end-stage renal failure, relapses, and documented side effects, as well. CONCLUSION: Both therapeutic approaches with MMF or AZA, in combination with corticosteroids, are effective as a maintenance therapy for lupus nephritis.

[Increased pulmonary artery stiffness and its relation to right ventricular function in patients with systemic lupus erythematosus]. / Sistemik lupus eritematozusta pulmoner arter sertliginde artma ve sag ventrikül fonksiyonu ile iliskisi.

OBJECTIVES: Pulmonary hypertension and right ventricular (RV) dysfunction are severe complications of systemic lupus erythematosus (SLE). The role of increased pulmonary artery stiffness (PAS) has not been studied in RV dysfunction. We investigated the relationship between PAS and RV function in SLE patients without cardiovascular symptoms. STUDY DESIGN: The study included 32 patients with SLE (30 males, 2 females; mean age 34+/-9 years) and 30 age- and sex-matched healthy controls (28 males, 2 females; mean age 36+/-5 years). All the subjects underwent echocardiographic examination. Using Doppler echocardiography, PAS was calculated by dividing maximal frequency shift of the pulmonary flow by the acceleration time. To assess RV function, RV myocardial performance index (MPI) was determined by the sum of isovolumetric contraction and relaxation times divided by the ejection time. In addition, tricuspid annular plane systolic excursion (TAPSE) was measured on two-dimensional M-mode recordings. RESULTS: Compared to the control group, patients with SLE exhibited significantly higher PAS (p=0.004) and RV MPI (p=0.001), and lower TAPSE (p=0.001). In univariate correlation analysis, SV MPI was significantly correlated with PAS (r=0.60, p=0.001), age (r=0.48, p=0.003), SLE duration (r=0.51, p=0.002), and pulmonary artery systolic pressure (r=0.36, p=0.03). Multivariate linear regression analysis showed that PAS (95% CI 0.002-0.005; p=0.001) and SLE duration (95% CI 0.001-0.004; p=0.004) were independently associated with RV MPI. In addition, a significant inverse relationship was found between TAPSE and RV MPI (r= -0.48, p=0.005). Twenty-four SLE patients had normal RV function (TAPSE > or = 17 mm). Eight patients with RV dysfunction (TAPSE <17 mm) had significantly different RV MPI (p=0.001), PAS (p=0.002), age (p=0.04), and SLE duration (p=0.004). CONCLUSION: Our data suggest that increased PAS is strongly associated with the development of RV dysfunction in patients with SLE.

The carotid-femoral (aortic) pulse wave velocity as a marker of arterial stiffness in familial Mediterranean fever.

AIM: The purpose of the present study was to test the hypothesis that chronic inflammation impairs vascular function and leads to an arterial pulse wave velocity (PWV) increase in patients with familial Mediterranean fever (FMF). PATIENTS AND METHODS: Twenty-three patients (17 women) with FMF, and 23 age- and sex-matched controls were recruited. Aortic PWV was determined by using an automatic device (Complior Colson, Createch Industrie, France) that allowed on-line pulse wave recording and automatic calculation of the PWV. RESULTS: The PWV was slightly higher in patients with FMF than in control subjects (P=0.05). A significant correlation between PWV and age (P<0.001, r=0.67), body mass index (P<0.001, r=0.52) and leukocytes (P<0.001, r=0.66) was found in both groups combined and also in patients with FMF (P<0.001, r=0.73; P=0.01, r=0.52; P<0.001, r=0.69, respectively). CONCLUSION: The PWV was slightly higher in patients with FMF compared with control subjects. Colchicine, an anti-inflammatory drug treatment, may have reduced the expected increased level of PWV in FMF patients. PWV is influenced by age, body mass index and leukocytes.

Infliximab does not suppress the tuberculin skin test (purified protein derivative).

OBJECTIVE: Tuberculin skin testing with purified protein derivative (PPD) is part of tuberculosis (TB) screening in patients receiving infliximab. We assessed whether infliximab, a strong inhibitor of inflammation, suppressed dermal induration, the outcome of this test. We also reassessed the booster phenomenon and the interobserver variability in tuberculin testing. METHODS: Forty-seven patients with various diagnoses, who had had a PPD test before infliximab use, were retested after infliximab treatment. The test was also assessed cross-sectionally among 31 patients with rheumatoid arthritis (RA) after 8.6 [+/- 4.1 standard deviation (SD)] months of infliximab use and in 82 patients with RA who had never used this agent. Booster phenomenon and the interobserver variability of reading the test were reassessed among 163 infliximab-naive patients with RA and Behcet's disease (BD) and 47 healthy controls. RESULTS: Among the 47 patients who received infliximab, and for whom sequential data were available, the mean skin induration was 5.9 +/- 8.0 SD mm before and 6.1 +/- 7.5 mm after 4.8 +/- 3.7 months of treatment (p = 0.890). In the cross-sectional study the mean PPD induration was 7.8 +/- 8.4 mm among infliximab-naive patients with RA, while it was 6.6 +/- 2.1 mm in those receiving infliximab (p = 0.271). Booster phenomenon was observed in 14/49 (29%) of patients with RA, 7/31 (23%) of those with BD, and 1/10 of healthy controls. Interobserver variability of PPD reading was good (kappa = 0.92). CONCLUSION: Infliximab use does not suppress the skin reaction to tuberculin. We confirm the booster phenomenon and that the PPD skin test has an acceptable interobserver reliability for an in vivo test.