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Systemic lupus erythematosus (SLE) is a multi-system autoimmune disorder mainly affecting young women. In this study, we aimed at investigating the clinical, laboratory and management characteristics of our SLE patients with an age of onset≥50.Twenty patients with late onset SLE (≥50 years) were identified from the records, on the basis of their first SLE-related symptoms (Group I). A hundred consecutive SLE patients with initial symptoms before the age of 50 were also selected as controls (Group II). Clinical, laboratory and management characteristics of the patients were recorded according to pre-defined protocol and compared byχ2, Student'st-test and Fisher's exact test.Comparison of the demographic findings between the Group I (F/M: 18/2) and the Group II (F/M: 90/10) were as follows: the mean age of disease onset was 53.9 ± 4.5 years vs. 26.3 ± 9.2 years, mean time of follow-up was 44.2 ± 40.5 months vs. 50.1 ± 47.4 months, mean damage index was 0.6 ± 0.6 vs. 0.58 ± 1.4. There was no significant difference between the two groups with regard to clinical, laboratory parameters, damage index and immunosuppressive treatment characteristics. SLE-related manifestations were similar in two groups except fever (10% in the Group I vs. 41% in the Group II; p = 0.01). The only two patients with pulmonary fibrosis were found in the Group I (p = 0.027).The clinical and laboratory characteristics and the disease outcome in SLE patients with an age of onset≥50 years did not show significant differences from the control SLE patients with a younger age of onset. [ABSTRACT FROM AUTHOR]

It has been generally accepted that the clinical onset of familial Mediterranean fever (FMF) begins before 20 years of age in most patients. In this study, we aimed to investigate the demographic and clinical characteristics of our FMF patients with an age of onset≥20.Records of 401 patients (female/male: 204/197) that followed up between 1990 and 1999 were reviewed according to a pre-defined protocol. All patients fulfilled the diagnostic criteria of Livneh et al. The demographic and clinical features of adult-onset FMF patients were compared to those of patients with a disease onset before 20 years of age.There were 57 patients (14%) who experienced symptoms of FMF at≥20 years of age; 34 of them (8.5%) reported their first attack between 20 and 29 years of age; 18 of them (4.5%) between 30 and 39 years of age and five patients (1.25%) had their first attack after 40 years of age. Arthritis (42 vs. 65%, p = 0.001) and erysipelas-like erythema (7 vs. 17%, p = 0.047) were significantly less frequent in patients with adult-onset FMF compared to patients with disease onset before 20 years of age.Arthritis and erysipelas-like erythema were less frequent in adult-onset patients compared to those with an earlier disease onset. Adult-onset FMF may be a form of disease with distinct clinical, demographic and molecular characteristics. Prospective clinical studies and investigation of genotypic features are needed to identify the characteristics of this phenotypic variant. [ABSTRACT FROM AUTHOR]

The objective was to investigate the frequency and characteristics of tuberculosis (TB) in patients with systemic lupus erythematosus (SLE). We reviewed the charts of 556 patients with SLE who were followed up between 1978 and 2001 in our lupus clinic. Patients who developed TB after the diagnosis of SLE were identified (SLE/TB+). Ninety-six consecutive patients with SLE who did not develop TB during the follow-up were evaluated as a control group (SLE/TB-). Clinical, laboratory and management characteristics of these two groups of patients were recorded according to a predefined protocol, and compared. Of the 556 patients evaluated, 20 patients (3.6%) with TB were identified. Nine of the 20 patients (45%) had extrapulmonary TB (vertebral involvement in three patients, meningeal in two, and joint and soft tissue in four). Arthritis and renal involvement were significantly high in the SLE/TB+ group (P = 0.045, P = 0.009, respectively). The mean daily dose of prednisolone before the diagnosis of TB and the cumulative dose of prednisolone were significantly higher in the SLE/TB+ group compared to the SLE/TB- group (27 ± 22 g versus 16 ± 16 g, 24 ± 45 mg versus 11 ± 8.5 mg, respectively). In conclusion, we found an increased frequency of TB infection and a high prevalence of extrapulmonary TB in a large cohort of SLE patients. The mean daily dose of prednisolone before the diagnosis of TB and the cumulative dose of prednisolone, which possibly related to disease severity, were important determinants for the increased risk of TB in these patients with SLE. [ABSTRACT FROM AUTHOR]

Objective: Familial aggregation of Behçet's disease has been reported previously. The current study aimed at investigating the sibling recurrence risk ratio (lambda s) for Behçet's disease, which is of value in the estimation of the magnitude of genetic factors in the pathogenesis of Behçet's disease.Methods: 170 consecutive unrelated index cases (98 male, 72 female) were interviewed with a detailed questionnaire to ascertain their family trees and the manifestations of Behçet's disease in their relatives. Subsequently, the immediately older sibling, or if an older sibling was not available, the immediately younger sibling, was selected as the second sibling for the evaluation. These siblings were contacted by telephone, and all subjects with recurrent oral ulcers were invited for examination.Results: 31 of the 170 index cases had 51 relatives fulfilling the International Study Group criteria. Among 166 second siblings, seven had Behçet's disease (six male, one female) and 22 siblings (eight male, 14 female) with recurrent oral ulcers were identified. Sibling recurrence rate-defined as the ratio of the risk of being affected among the siblings of patients and the risk of being affected in the general population- was found to be 4.2%, which gives a lambda s value for Behçet's disease of between 11.4 and 52.5 in Turkey.Conclusions: A high lambda s value supports a strong genetic background for Behçet's disease which will be helpful in designing genetic linkage studies. [ABSTRACT FROM AUTHOR]

Several criteria are being used for the classification of psoriatic arthritis (PsA) and there is a lack of consensus about PsA as a separate entity. Our aim is to investigate the clinical features of our patients with a clinical diagnosis of PsA, compare the sensitivities of different classification criteria, agreement between the criteria. In this study 86 PsA patients were investigated (48 females, mean age 44). Moll and Wright criteria was fulfilled by 91%, Vasey and Espinoza criteria by 94% and modified European SpA study group criteria by 59%, classification of PsA study group criteria by 86%, modified McGonagle criteria by 96%, Fournie et al. criteria by 84% and Gladman criteria by 95%. Significant agreement was present between criteria but generally kappa-values were less than 0.5. The pattern of PsA can differ with time and the implementation of the available classification criteria showed considerable differences.

Several criteria are being used for the classification of psoriatic arthritis (PsA) and there is a lack of consensus about PsA as a separate entity. Our aim is to investigate the clinical features of our patients with a clinical diagnosis of PsA, compare the sensitivities of different classification criteria and agreement between the criteria. In this study 86 PsA patients were investigated (48 female, mean age 44). Moll and Wright criteria were fulfilled by 91%, Vasey and Espinoza criteria by 94% and modified European SpA study group criteria by 59%, classification of PsA study group criteria by 86%, modified McGonagle criteria by 96%, Fournié et al. criteria by 84%, and Gladman criteria by 95%. Significant agreement was present between criteria but generally kappa values were less than 0.5. The pattern of PsA can differ with time and the implementation of the available classification criteria showed considerable differences.

Objectives: Ankylosing spondylitis is strongly associated with HLA-B27. However, the strength of the association with HLA-B27 and the clinical features may vary in different parts of the world. The aim of this study is to compare the clinical features of AS and the frequencies of HLA-B27 and its alleles in patients from Turkey with other series., Methods: One hundred and twelve patients (72 male/40 female) fulfilling the modified New York criteria for the classification of AS and 55 (27 male/28 female) healthy controls were typed for HLA-B27 and allele frequencies by sequence specific primer (PCR/SSP) method and assessed for clinical manifestations., Results: Male to female ratio was 1.8, mean age at disease onset was 23.5 and 24.1% of patients reported juvenile onset of symptoms. Peripheral arthritis was seen in 52.7% of patients. Family history (p=0.01) and peripheral arthritis (p=0.02) were more frequent in females and spinal involvement in males. HLA-B27 was found to be positive in 70% of patients and associated with younger mean age, uveitis and shorter time elapsed from symptom to diagnosis. The frequency of HLA-B27 alleles associated with SpA was not different between ankylosing spondylitis patients and healthy controls., Conclusion: Low frequency of HLA-B27 and clinical variations in ankylosing spondylitis may be due to different genetic and/or environmental factors in Turkey.

Objective: The aim of this study was to analyze and compare the demographic and clinical features and prognosis of patients with different systemic necrotizing vasculitides (SNV) in Turkey., Patients and Methods: Twenty-three patients with Wegener's granulomatosis (WG), 15 with polyarteritis nodosa (PAN), and two with Churg-Strauss syndrome were included in the study. The clinical and laboratory features of patients with WG and PAN were compared, and survival analysis was performed for the WG patients., Results: Twenty-one patients with WG had systemic disease involving kidneys, and two had localized disease. Fifteen patients were placed in the PAN group, 12 of whom were classified as having classic PAN and three with microscopic polyangiitis. Median follow-up time was 37 months (range 1-81) for WG patients and 41 months (range 5-132) for the PAN group. Upper respiratory tract, pulmonary, and renal involvement were significantly more frequent in the WG group than in PAN. Peripheral nervous system involvement was more frequent in the PAN group. In WG, survival was calculated as 59% at 35 months. High initial vasculitis damage index scores were found to be predictive for mortality., Conclusion: This study revealed that the most frequent type of SNV was WG in a tertiary rheumatology setting in Turkey. There was initial organ damage in most of the patients, frequently caused by severe renal involvement. In contrast to other published series, overt cardiovascular and gastrointestinal involvement were not observed in our patients with SNV.

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.

This study aimed to analyse the association of HLA-B alleles other than -B51 with Behçet's disease (BD). We also investigated the frequency of HLA-B alleles sharing the same natural killer cell immunoglobulin-like receptor (KIR) binding sequence with HLA-B51. Broad-genotyping of HLA-B locus by PCR-SSOP in 174 Turkish BD patients and 191 healthy controls confirmed the strong association of B*51 with BD (60.9% in BD patients, 24.6% in healthy controls, OR = 4.78). No other HLA-B allele was identified showing an association with BD after adjusting for multiple testing or by using relative predispositional effects (RPE) analysis after the deletion of B*51. HLA-B alleles reacting with the sequence specific oligonucleotide probe 23, which corresponds to the KIR binding site of B*51, were found to be positive in 127 BD patients (73%) and 90 controls (47%) (OR = 3.03, 95% CI 2-4.7). The repeated RPE analysis after separating HLA-B alleles carrying B51-KIR binding sequence as distinct alleles within a broad-type allele group revealed B*2702 allele as the only allele showing an association with BD after the deletion of B*51. Selective increase of B*2702, the only B*27 allele carrying the same KIR binding sequence with B*51, warrants investigation of the possibility of interaction of HLA molecules with KIRs on NK or other T cells in the pathogenesis of BD.

The aim of this study was to compare and evaluate the Health Assessment Questionnaire (HAQ) and Arthritis Impact Measurement Scale (AIMS) in our patient population with rheumatoid arthritis (RA) and also to find some associations with clinical assessment of disability. One hundred and twenty-three consecutive adult patients with RA were included in the study. Pain, and global assessments by patients and physicians were recorded using a 10 cm visual analogue scale. Each patient completed the HAQ and AIMS questionnaires. Correlations among tender and swollen joint counts, erythrocyte sedimentation rate, pain, and AIMS anxiety and depression scores were all investigated. Pearson correlation was used to assess the possible correlations between each questionnaire and clinical variables. Pain and the AIMS subscales of mobility, dexterity, social activity and activities of daily living correlated with global assessments by patients and physicians, and tender joint counts. Depression correlated with pain and disability (HAQ). It was also of note that we observed high intercorrelation between the global assessments of physicians and patients. It was concluded that a measure of functional status, patient global assessment and pain score should be considered as important in the evaluation of RA patients. Measuring psychological well-being also provides further information. The HAQ, with the addition of the anxiety and depression sections of AIMS (CLINHAQ), provides the advantage of a global evaluation of these chronically ill patients.

The association of amyloidosis with Behçet's disease has infrequently been reported in published works. Twenty four such cases have been observed in the world, of which 12 are from Turkey, including eight of ours. In all our eight cases renal biopsy showed amyloidosis of type AA. Behçet's disease of male preponderance, long duration, complete type, multiple organ involvement, and positive skin pathergy test were the main characteristics of all 24 cases of Behçet's disease with amyloidosis. We conclude that amyloidosis associated with Behçet's disease is a secondary AA amyloidosis occurring as an intrinsic manifestation of Behçet's disease.