Metabolic syndrome is not only a risk factor for cardiovascular diseases in systemic lupus erythematosus but is also associated with cumulative organ damage: a cross-sectional analysis of 311 patients.

BACKGROUND/PURPOSE: Patients with systemic lupus erythematosus (SLE) have increased rates of cardiovascular disease (CVD) that are one of the major causes of mortality. The aim of this study was to determine the frequencies of metabolic syndrome (MetS) and CVD in SLE patients and investigate the link between these and clinical features of SLE. METHODS: A total of 311 SLE patients were consecutively assessed for cumulative organ damage (SDI/SLICC scores), history of CVD and MetS as defined by the National Cholesterol Educational Program Adult Treatment Panel III (NCEP ATP III). Clinical data of SLE patients were collected from the records. RESULTS: The mean age of the patients was 40.2 ± 13.4 years and 89% were female. The frequencies of CVD and MetS were 15.2% and 19%, respectively. In this SLE cohort increased age, cumulative damage, disease duration and CVD were associated with MetS. CVD was associated with disease duration, cumulative damage, pericarditis, hematologic involvement, lymphopenia, thrombocytopenia, neurological involvement and antiphospholipid antibody (aPL) positivity. Hydroxychloroquine (HCQ) use was found as a protective factor for CVD. CONCLUSION: In SLE patients, MetS was associated with CVD and both increased with disease duration. Patients who developed MetS and/or CVD had increased cumulative organ damage. Certain clinical features of SLE and the presence of aPL were also associated with CVD. There was a significant protective effect of HCQ from CVD. The prevention of MetS and long-term use of HCQ may be beneficial in improving the prognosis of SLE.

Adult-onset Still's disease.

BACKGROUND: Adult-onset Still's disease (AOSD) is a febrile disorder of unknown aetiology characterised by typical spiking fever, evanescent rash, arthralgia and leucocytosis. METHODS: According to the diagnostic criteria of AOSD, we identified 84 patients between 1990 and 2003. The aim of this study was to analyse the characteristics of AOSD in Turkish patients who were followed-up in a tertiary referral centre. RESULTS: Of 84 patients of AOSD, 59 (70.2%) were female, 25 (29.8%) were male. Arthralgia (96.4%), fever (95.2%), arthritis (69%), sore throat (65.5%) and typical rheumatoid rash (59.5%) were the most common findings. The mean value of laboratory findings were as follows; C-reactive protein level of 11.59 +/- 6.81 mg/dl, erythrocyte sedimentation rate (ESR) of 89.05 +/- 31 mm/h, leukocyte count of 16,234.51 +/- 7785.2/microl. Leucocytosis was present in 69 patients (84.15%). Forty-eight patients had a WBC count >or= 15,000/microl. Hypoalbuminaemia was present in 35 patients. Abnormal levels of aspartate aminotransferase and alanine aminotransferase were observed in 30 patients, whereas abnormal levels of alkaline phosphatase in 16 patients. Thirty-seven patients had an ESR value of more than 100 mm/h. Thirty-two patients had a ferritin value of more than 1000 ng/dl. CONCLUSION: High fever, sore throat, rheumatoid rash, polyarthritis, hyperferritinaemia (>or= 1000 ng/ml), leucocytosis with a neutrophilic predominance, anaemia and hypoalbuminaemia were remarkable observations in the initial examination.

Characteristics of patients with late onset systemic lupus erythematosus in Turkey.

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 > or =50. Twenty patients with late onset SLE (> or =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 by chi(2), Student's t-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 > or =50 years did not show significant differences from the control SLE patients with a younger age of onset.

Characteristics of patients with adult-onset familial Mediterranean fever in Turkey: analysis of 401 cases.

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 > or =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 > or =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.

Tuberculosis in Turkish patients with systemic lupus erythematosus: increased frequency of extrapulmonary localization.

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.

Eosinophilia and hyperimmunoglobulinemia E as the presenting manifestations of Wegener's granulomatosis.

Eosinophilia has long been known as a hallmark of Churg-Strauss syndrome but has rarely been reported in Wegener's granulomatosis (WG). Here we describe a patient with WG who had skin, kidney and lung involvement as well as striking peripheral eosinophilia and hyperimmunoglobulinaemia E (hyper-IgE). The patient's clinical picture was complicated by intra-alveolar haemorrhage resulting in severe anaemia and respiratory failure. The pulmonary symptoms recovered completely, but the renal involvement evolved into end-stage renal failure despite intensive immunosuppressive treatment, intravenous immunoglobulin and plasmapheresis. We suggest that the presence of eosinophilia and hyper-IgE might contribute to the development of different disease patterns in WG.

A weak association of HLA-B*2702 with Behçet's disease.

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.

Lack of association of HLA-B*51 with a severe disease course in Behçet's disease.

OBJECTIVE: To investigate the previously reported association of HLA-B51 with the manifestations and severity of Behçet's disease (BD). METHODS: The study group consisted of 148 consecutive BD patients (89 male, 59 female) with a minimum disease duration of 5 yr followed up at an out-patient BD clinic in a tertiary referral centre. The patients were classified into three severity groups (mild, moderate, severe) using a modified form of the BD total activity index. HLA-B alleles were determined by DNA amplification using the polymerase chain reaction and sequential hybridization with sequence-specific oligonucleotide probes. RESULTS: The frequencies of genital ulceration [odds ratio (OR)=3.1, 95% confidence interval (CI) 1.3-7.5], skin findings (erythema nodosum, folliculitis or acne-like lesions) (OR=4.4, 95% CI 1.1-17.7), a positive skin pathergy test (OR=3.4, 95% CI 1.1-10.9) and eye disease (OR=1.8, 95% CI 0.9-3.7) were all higher in B*51-positive patients. By contrast, no significant association was observed between B*51 positivity and a severe disease course, and B*51 homozygosity did not exhibit a prominent association with the severity of BD. Male sex was found to be the strongest determinant of the severity of BD by logistic regression analysis (OR=4.7, 95% CI 1.9-11.2). CONCLUSION: HLA-B*51 does not exhibit a strong association with a more severe disease course in BD. The involvement of other genetic and/or environmental factors seems to be required and to be more important than B*51 for the progression of BD.

Amyopathic dermatomyositis associated with cervical cancer.

We here report a 44-year-old female patient who is the first documented case of cervical carcinoma with amyopathic dermatomysitis (ADM). The association of ADM with malignancy is clearly stated in the literature. This patient first presented with classic cutaneous findings of dermatomyositis with no signs and symptoms of muscle involvement for the first 7 months following the diagnosis. When this case has been worked up for malignancy by invasive procedures such as cervical cone biopsy, an increase in muscle enzymes and exacerbation of skin findings were detected.

Familial aggregation of Behçet's disease in Turkey.

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.

The use of two different Health Assessment Questionnaires in Turkish rheumatoid arthritis population and assessment of the associations with disability.

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.

Antibodies reactive with HIV-1 antigens in systemic lupus erythematosus.

Antibodies reactive with retroviral gag proteins have been detected in patients with systemic lupus erythematosus (SLE). We investigated the immune responses against human immunodeficiency virus (HIV) 1 antigens in the sera of 44 Turkish patients with SLE. Serum samples were tested by using two different commercial enzyme immunoassay (EIA) kits and by Western blotting. EIA studies revealed positive results in 12 patients (27%) for HIV-1 antigens by one of the kits that coated with purified viral antigens. Immunoblot analysis showed antibodies mainly to retroviral gag proteins in 23 patients (52%). The most frequent reactivity was against the p18 gag protein (n = 9). Although antibodies reactive particularly with p24 antigen were described in the previous reports, antibodies to p24 were found in only two patients. These findings might reflect a serologic diversity in different ethnic groups and also suggest the involvement of different triggers in the etiopathogenesis of SLE.

Primary antiphospholipid syndrome associated with mesenteric inflammatory veno-occlusive disease.

We describe a 24-year old Caucasian man with gangrene of small bowels and intestinal resection due to mesenteric inflammatory veno-occlusive disease (MIVOD) who later developed deep vein thrombosis in his left leg. He had no clinical evidence of an underlying symptomatic connective tissue disease or Behçet's disease. An IgG anticardiolipin antibody titre above 60 GPL unit/mL and thrombocytopenia confirmed the diagnosis of primary antiphospholipid syndrome (APS). This is the first known case of APS associated with MIVOD.

Female genital tract giant cell arteritis associated with occult temporal arteritis.

Giant cell arteritis (GCA) of the female genital tract has been described as an incidental finding, but associated temporal arteritis (TA) has been rarely reported. We describe a case of female genital tract GCA associated with occult giant cell TA, which in the absence of cranial symptoms was confirmed by a random temporal artery biopsy. The patient remains asymptomatic at 12 month followup after treatment with prednisolone and azathioprine.

Paroxysmal dysarthria and ataxia in a patient with Behçet's disease.

The paroxysmal attacks which are frequently encountered in the course of multiple sclerosis (MS) are characterised by their sudden onset, short duration and frequent repetition. Such attacks have also been reported in some other diseases affecting the CNS, such as systemic lupus erythematosus. However, to our knowledge, they have not been reported in neuro-Behçet's disease (NBD). A patient with NBD who developed paroxysmal dysarthriaataxia attacks is presented, and the similarity of some clinical, laboratory, and neuroradiological aspects of NBD and MS are discussed with special emphasis on magnetic resonance imaging findings.

Comparative study of the skin pathergy test with blunt and sharp needles in Behçet's disease: confirmed specificity but decreased sensitivity with sharp needles.

OBJECTIVE: To compare the specificity and sensitivity of the skin pathergy test performed with blunt and sharp needles in patients with Behçet's disease. METHODS: The skin pathergy test was performed using the simultaneous four needle prick method with blunt and sharp, thick and thin needles in 92 patients with Behçet's disease, 64 healthy controls, and 128 patients without Behçet's disease. The test was evaluated at 48 hours. RESULTS: No positive skin pathergy test was found in healthy controls and patients without Behçet's disease. The frequency and intensity of the positive skin pathergy test with blunt needles were significantly higher than those with sharp needles. CONCLUSION: This study reconfirmed the specificity of a positive skin pathergy test for Behçet's disease using blunt and sharp needles and showed a decreased sensitivity and intensity of the reaction with sharp needles.