Background Lupus-nephritis (LN) is a serious organ involvement and important factor of morbidity and mortality in patients (pts) with systemic lupus erythematosus. As initial treatment of LN, high doses of glucocorticoids (GK), pulses of cyclophosphamide (PT CY) or mycophenolate mofetil (MMF) is recommended. Initial treatment is followed by subsequent (maintenance) therapy. There are few information about course and relapses of LN through the maintenance therapy. Methods In retrospective study, 36 pts with SLE and LN were hospitalized in National Institute of Rheumatic Diseases Piestany from 1996 – 2013 years. Effectiveness of initial treatment of PT CY on LN activity, course of LN and occurrence of relapses through maintenance therapy was evaluated. Results Total 36 patients (pts) with SLE and LN were treated with PT CY 0,5-1 g/m2 monthly for 6 month. There were 31 female and 5 male pts with with a mean age 38.7 yrs on the onset of SLE, with disease duration 9.62 yrs (2 to 25 yrs). Anti-dsDNA antibodies were positive in 25 pts at the onset of the therapy and in 12 pts persisted after PT CY. Hypocomplementemia before therapy was recorded in 34 of 36 pts. Renal failure with creatinine elevation at the beginning of the PT CY was in 6 of 36 pts. Overall SLE activity was evaluated by SELENA SLEDAI. After initial treatment of PT CY, complete renal response was reached in 34 pts, partial in 2 pts. Low complement was normalized in 64.7%. After that, four types of maintenance therapy was introduced: 12 pts PT CY 0,5-1 g/m2 each three months, 8 pts azathioprine from 50 to 100 mg a day, 6 pts MMF 2 g per day, 10 pts cyclosporine A 100 to 150 mg a day. Combination with belimumab had 1 pts and with hydroxychloroquine 10 pts. The dosage of GK through the maintenance therapy was from 10 – 25 mg of prednisone. In 16 pts suppression persist through the following up, in 20 pts relapse of LN appeared. Time to relapse after PT CY was 43 months in average (5 - 116 months). At AZA therapy, there were 11 relapses, 3 at MMF, 2 at cyclosporine A, 4 at GK maintenance therapy. In group of pts with LN relaps, 13 pts had anti-dsDNA autoantibodies. In another 5 pts were anti-dsDNA antibodies negative in time of LN reactivation. After LN relapse, PT CY was repeatedly introduced – again with complete or partial renal response. Conclusions Pulse CY therapy is effective almost in all pts with SLE and LN. Relapses in the phase of maintenance therapy occurred in more than 50% of pts. Relapses of LN can appear even in pts without anti-dsDNA antibodies. Tight control of SLE activity is necessary in all pts. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.3905

Background Low serum vitamin D concentrations have been reported in several autoimmune disorders. Objective To assess whether low serum vitamin D concentrations are related to disease activity of patients with systemic lupus erythematosus (SLE).Methods 378 patients from several European and Israeli cohorts were pooled and their disease activity was measured by two different methods: 278 patients had SLE disease activity-2000 (SLEDAI-2K) scores and 100 patients had European Consensus Lupus Activity Measurement (ECLAM) scores. In order to combine the two systems the scores were converted into standardised values (z-scores), enabling univariate summary statistics for the two variables (SLEDAI-2K and ECLAM). The commercial kit, LIAISON 25-OH vitamin D assay (310900-Diasorin) was used to measure serum concentration of 25-OH vitamin D in 378 patients with SLE.Results A significant negative correlation was demonstrated between the serum concentration of vitamin D and the standardised values (z-scores) of disease activity scores as measured by the SLEDAI-2K and ECLAM scales (Pearson's correlation coefficient r = -0.12, p = 0.018).Conclusions In a cohort of patients with SLE originating from Israel and Europe vitamin D serum concentrations were found to be inversely related to disease activity.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease, characterized by multisystemic involvement. Late onset SLE represents a specific sub-group of the disorder, beginning above 50-65 years of age. The incidence of late onset SLE ranges in the interval of 12-18% and the course of the disease is considered to be more benign. According to several authors, skin manifestations, photosensitivity, arthritis and nephritis, occur rarely in the elderly patients with late SLE onset; prevalence of serositis, lung involvement and Sjögren's syndrome were observed more often. Late onset SLE patients manifested higher rate of positive findings of rheumatoid factors, as well as of anti-Ro and anti-La antibodies; and the lower occurrence of anti-RNP antibodies and hypocomplementaemia. A slow onset of the disorder, non-specific manifestations at the beginning of the illness and less frequent prevalence of SLE in the elderly often result in late diagnosis. Treatment of the disease depends on its clinical manifestations. NSAID's, antimalarials or low doses of glucocorticoids are used for the less severe forms. Immunosuppressives and higher doses of glucocorticoids are the treatments of choice for more severe organ involvements and complications. A multidisciplinary approach is recommended for the treatment of late onset SLE patients.

INTRODUCTION:Due to ageing of population, gerontorheumatology becomes more and more important. Both polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) typically develop in later life and they have many other common features. The aim of our study was to explore diagnostic and prognostic markers and, prospectively, establish diagnostic and therapeutic algorithm for patients with PMR and GCA.SAMPLE AND METHODS:We examined 27 patients with suspected PMR or OBA. The diagnosis was verified in 22 patients. Three of them were in a long-term clinical remission. Besides examination for basic clinical and laboratory parameters, all other patients were subjected to ultrasonography of temporal artery and peripheral joints to detect any exudates. Also, they were examined for T-cell subpopulations in peripheral blood and HLA antigens.RESULTS:Exudate was confirmed in 7 patients; some of them had exudate in multiple joints. Puncture of synovial fluid was done in 4 patients. Increased resistance index of temporal artery was found in 2 patients with GCA and 4 patients with PMR. GCA patients showed lower level of T-cells and increased activation of CD8-cells. Decreased count of CD8+ T-cells was observed in 56 % of PMR patients. Analysis of HLA antigens indicates that GCA, rheumatoid arthritis and, probably, PMR are associated with HLA-DR4 antigen in Slovak population.CONCLUSION:The importance of assessment of disease activity and its prognosis in PMR or GCA patients via ultrasonographic evaluation of exudate in peripheral joints and resistance index of temporal artery as well as the analysis of T-cell distribution in peripheral blood and incidence of HLA-antigens has not been proved yet. Practical significance of monitoring the above-mentioned parameters can be verified only by further prospective study performed with a larger sample of patients.

Antibodies (Abs) against the structure specific recognition protein 1 (SSRP1) were reported in a small systemic lupus erythematosus (SLE) series but not in other systemic autoimmune diseases. The aim of the study was to confirm the selective presence of anti-SSRP1 Abs in a larger SLE series and to evaluate their relationship with disease activity and other immune markers. Anti-SSRP1 Abs were investigated by a 'home made' ELISA in: 120 SLE, 65 rheumatoid arthritis ( RA), 51 systemic sclerosis (SSc), 23 Churg-Strauss syndrome (CSS) and 40 idiopathic autoimmune urticaria (IAU) patients and 190 healthy controls. Sera from MRL lpr/lpr and Balb-c mice were also tested. Anti-SSRP1 Abs were detected in 43 SLE (35.8%), nine SSc (17.6%), eight RA (12.3%), six IAU (15%), three CSS (13%) patients and five healthy controls (2.6%). Antibody prevalence and titers were significantly higher in SLE patients than in sera from both normal and disease controls. Anti-SSRP1 Ab activity was also detected in sera from MRL lpr/lpr but not Balb-c mice. The antibodies did not correlate with the disease activity evaluated as the ECLAM index score and were more prevalent in patients without renal involvement. No correlation was found with other serum autoantibodies. Our results confirm that anti-SSRP1 Abs are associated with but not specific for the lupus disease.

: The aim of the study was to evaluate the efficacy and safety of disease-modifying drugs (DMARDs) in everyday clinical practice in Central European States (the Czech and Slovak republics). This was a retrospective, multicentre study. With the help of a special questionnaire, the medical files of 760 patients in 15 centres were analysed looking for reasons for DMARD discontinuation (e.g. insufficient efficacy, toxicity). The secondary endpoints were duration of therapy with individual DMARDs and the influence of other factors (demographic, disease specific, concomitant therapy) on duration of therapy. In 47.1 % of patients therapy was interrupted because of lack of efficacy, in 43.2 % because of adverse events, and in 9 % for undefined reasons. Toxic reactions leading to withdrawal were most common with gold (62.6 %) and methotrexate (62.5 %). Because of insufficient effect, treatment was most frequently interrupted with antimalarials (62.3 %) and penicillamine (53.2 %), but in only 22% treated with methotrexate. The mean duration of one treatment episode with DMARDs was 28.1 þ 48.9 months. Surprisingly, it was longest for cyclophosphamide (53.5 + 55.1 months) and shortest for cyclosporin (7.0 þ 6.7 months). The mean duration of treatment with methotrexate was only 14.9; þ 16.2 months. The mean duration of treatment with one DMARD was statistically longer in patients with positive rheumatoid factor, extra-articular disease and age lower than 50 years. There was no impact of sex, concomitant steroid treatment and high or low sedimentation rate on treatment duration. Considerable differences in everyday clinical practice with DMARDs between Central European states and published data from the US and western Europe have been found. More education about modern strategies in the treatment of RA is probably necessary for practising rheumatologists.

BACKGROUND:During the physiological ageing, function of individual factors of the immune system tend to decline. The aim of our study was to compare the ability of lymphocytes to respond to polyclonal mitogens and to the stimulation by antibodies anti-CD2/CD2R after an previous trypsinization.METHODS:17 adult (28 to 54-year-old) and 32 aged persons (63 to 90-year-old) were investigated. Lymphocytes were isolated from the peripheral blood, trypsinizated and subsequently incubated with polyklonic mitogens--phytohemaglutinin (PHA), concavalin (Con A), and a monoklonic antibody MoPr) anti-CD2/CD2R. The speed of recovery of the blastic transformation of trypsinizated lymphocytes was compared among the adult and aged persons.RESULTS:The stimulation values were in all studied time intervals significantly lower in aged persons then in adult ones (p < 0.001). In the group of aged persons, when the trypsinizated lymphocytes were stimulated by MoPr anti-CD2/CD2R, their ability of blastic transformation has not recovered to previous values.CONCLUSIONS:Our results indicate that function of T-lymphocytes diminishes in advanced age, however, lymphocytes still keep the ability to respond to specific stimulus. We found that the recovery of the blastic transformation of lymphocytes after a trypsinization requires in aged persons more than 20 hours. It probably corresponds with widespread decline of biological activities during ageing.

BACKGROUND:During physiological ageing changes of the immune system take place at several levels. The objective of the submitted work was to compare the ability of spontaneous restoration of selected differentiation antigens on lymphocytes in the peripheral blood stream after previous trypsin treatment in a group of healthy elderly and adult subjects.METHODS AND RESULTS:Twenty-four adults were examined (19-59 years) and 36 elderly subjects (60-90 years). Isolated lymphocytes from the peripheral blood stream were treated with trypsin and then incubated in a cultivation medium. The authors investigated the capacity of restoration of differentiation antigens CD2, CD4, CD8 and CD45RA. Antigen CD2 was not restored in any of the investigated groups to original levels. However the difference between its expression on lymphocytes before trypsin treatment and on lymphocytes after 16-hour incubation was higher in the elderly subjects 16% (p < 0.001) than in the group of adults 7% (p < 0.01). Restoration of antigen CD4 was in both investigated groups almost equal. The number of CD8+ T-lymphocytes was in elderly people lower (p < 0.05), spontaneous restoration of antigen CD8 did not differ among the investigated groups and reached in both instances the baseline value. Antigen CD45RA was restored more slowly in elderly subjects, the difference between groups was at borderline of statistical significance (p < 0.0595).CONCLUSION:From the results ensues that during physiological ageing the ability of spontaneous restoration of antigens CD2 and CD45RA declines but not of antigens CD4 and CD8. So far there is no unequivocal explanation why this change occurs, it is probably conditioned by several factors. Investigation of these changes and an attempt to influence them can help to understand age-conditioned immunological dysregulation, its consequences and the possibility to influence them by treatment.