Differences associated with age at onset in early systemic sclerosis patients: a report from the EULAR Scleroderma Trials and Research Group (EUSTAR) database.

OBJECTIVE: The aim of this study was to analyse differences in clinical presentation in patients with early (< 3 years' duration) systemic sclerosis (SSc), comparing three age groups according to disease subsets. METHOD: Cross-sectional analysis of the prospective EULAR Scleroderma Trials and Research database (EUSTAR) was performed. Patients fulfilling preliminary American College of Rheumatology 1980 classification criteria for SSc, with < 3 years from the first non-Raynaud's SSc symptom at first entry, were selected. Patients with < 3 years from the first SSc symptom, including Raynaud's phenomenon, were also analysed. SSc-related variables, including antibodies, SSc subsets, and organ involvement, were examined. Age was categorized into ≤ 30, 31-59, and ≥ 60 years. We performed descriptive and bivariate analyses. RESULTS: The study included 1027 patients: 90% Caucasian, 80% women, and 40% with diffuse disease. In early stages of SSc, younger patients had significantly more anti-Scl-70 antibodies and diffuse disease. With increasing age, we observed more elevation of estimated pulmonary systolic pressure on echocardiography (5%, 13%, and 30%, respectively, in the three age groups), cardiac conduction blocks (6%, 6%, and 15%), and left ventricular diastolic dysfunction (4%, 12%, and 27%). The results were similar for 650 patients with < 3 years from first SSc symptom, including Raynaud's. CONCLUSION: In early stages of SSc, older patients showed data indicating more severe disease with greater cardiac involvement. The diffuse subset was more frequent in the younger subgroup. The identification of such differences may help in selecting appropriate management for individual patients in clinical practice.

Ovarian function and disease activity in patients with systemic lupus erythematosus.

OBJECTIVE: Menstrual cycle disturbances frequently occur during the onset or in exacerbation periods of systemic lupus erythematosus (SLE), suggesting a possible relationship. The aim of the study is to assess the ovarian function in SLE patients with active disease before the treatment with high doses of glucocorticoids (GC) and cytotoxic agents. METHODS: We evaluated 94 female SLE patients (mean age of 29.2+/-7.0 years). The mean SLEDAI score was 11.4+/-8.1. Seventy-nine patients had a current use of GC with a median dose of 10 mg/day (8-15). The other 15 patients were untreated. After examination and blood sample collection 40% of the patients were treated and high doses of GC (>30 mg/day); 68% from this group of patients were treated GC in combination with cyclophosphamide (CYC). Forty healthy women with the same mean age were evaluated as controls. A careful gynecological history and a gynecological examination were carried out in patients and controls. Hormonal serum levels of follicle-stimulating hormone (FSH), luteinizing hormone (LH), prolactin, estradiol (E2) and progesterone in SLE patients and controls were measured by enzyme-linked immunosorbent assay (ELISA). RESULTS: Menstrual cycle disorders with oligomenorrhea as dominant aspect were observed in 54% of SLE patients. The hormonal studies showed decreased progesterone level in 52% of patients, reduced E2 concentration in 25% of patients; increased levels of LH, FSH and prolactin were observed with the lower frequency (13%, 9%, 10% respectively). Menstrual cycle disorders and the hormonal unbalance such as decreased progesterone level and hyperprolactinemia were found related significantly to high SLEDAI score (p<0.05, p=0.001, p<0.05). In the group of non-treated SLE patients the menstrual and hormonal disorders were observed in the same spectrum and with the same frequency as in all the examined SLE patients. SLEDAI score was found correlated significantly with the frequency of menstrual cycle disorders in non-treated SLE patients (p<0.05). CONCLUSION: The reported study shows the disease activity as a major factor associated with menstrual cycle disorders in SLE patients before treatment with alkylating agents and high doses of GC. Therefore, SLE women might be considered as a risk group for altered ovarian function.

Lack of association between three vascular endothelial growth factor gene polymorphisms and systemic sclerosis: results from a multicenter EUSTAR study of European Caucasian patients.

INTRODUCTION: Systemic sclerosis (SSc) is characterised by disturbed vessel morphology and an overproduction of vascular endothelial growth factor (VEGF). The VEGF gene located on chromosome 6p21.3 has several polymorphisms. OBJECTIVE: To test the hypothesis that disturbed angiogenesis may be related to the genetic background of the VEGF gene. MATERIALS AND METHODS: EUSTAR centres included European Caucasian patients with SSc and matched controls with osteoarthritis. The VEGF gene was genotyped by polymerase chain reaction, followed by restriction enzyme analysis. The 634 C/T and 936 C/G mutations and an 18-base pair insertion/deletion at -2549 of the VEGF promoter region were tested. RESULTS: 416 patients with SSc and 249 controls were included in the study population. Of the patients with SSc, 42% had a diffuse cutaneous subtype, 16% had increased pulmonary arterial pressure and 61% had decreased carbon monoxide diffusion capacity. The genotype frequencies in the patients with SSc and in controls were in Hardy-Weinberg equilibrium. The allele and genotype frequencies of the polymorphisms did not differ between patients with SSc and controls. No association was found between these polymorphisms and disease phenotypes. CONCLUSION: This study shows that there is no association between the three selected functional VEGF polymorphisms and SSc.

The course of Lyme disease in different age groups.

Lyme disease is a multisystem infection affecting all age groups. In this study an attempt was made to determine whether the patient's age influences the course of the disease. One hundred and fifty patients with diagnosed Lyme disease were included in the study. Two serological methods were used to detect antibodies to Borrelia burgdorferi and to confirm the diagnosis: an indirect immunofluorescence assay (the Russian strain Ip-21) and Western blot. The course of Lyme disease did not differ from that seen in Europe and North America. However, a few clinical differences between groups were observed. In the first age group (0-15 years) the most common manifestation was flu-like symptoms with fever. Neither radiculoneuritis nor polyneuropathy was observed in this age group. Late manifestations were rare and the outcome of the disease was benign. The course of the disease in the second group (16-40 years) was most similar to that in childhood and the also outcome was similar. Carditis and erythema multiple were significantly more common in the second group (16-40 years) than in the other age groups. No differences were found between the third (41-60 years) and fourth (> than 60 years) group in the frequency of flu-like symptoms, erythema multiple and aseptic meningitis. However, the most important clinical sign in this group was involvement of the nervous system whereas in the third group this was joint damage. This feature deserves attention because, as a rule, the presence of an articular syndrome determines the prognosis of LD.