R. La Corte

Prolonged remission state of refractory adult onset Still's disease following CD34-selected autologous peripheral blood stem cell transplantation.

We report a 38-year-old patient affected by refractory adult onset Stills disease who achieved a prolonged remission following CD34-selected ABMT. The conditioning regimen was based on the use of CY and anti-thymocyte globulin. A 3.0 and 2.0 log reduction of T (CD3+) and B (CD19+) lymphocytes, respectively, was obtained using a Ceprate device to select CD34+ cells from PBSC. In the pre-transplant period (1994–1998) the patient had a chronic persistent disease course with frequent and recurrent systemic articular flares and loss of some functional abilities, despite daily prednisone, pulses of CY and immunosuppressive therapy (CYA or MTX). At the time of ABMT the patient had become non-ambulatory. Within 3 weeks of ABMT the patient showed a marked decrease in joint swelling, and morning stiffness. Joint pain and systemic symptoms disappeared, the patient was able to walk and run and gained general well being. ESR, C-reactive protein and WBC count were significantly decreased, while Hb level increased. This partial remission persisted for at least 1 year after ABMT, although at 15 months of follow-up a reappearance of moderate synovitis in the knees and wrists was noted. Our data further showed that both patient BM microenvironment and stem-progenitor cell function (as assessed by LTC-IC assay) were damaged even 1 year after CD34-selected ABMT, suggesting that the persistence of these alterations could have facilitated the favorable outcome of the disease following ABMT. Bone Marrow Transplantation (2000) 25, 1307–1310.

Bronchial hyperreactivity in systemic sclerosis patients: influence of associated Sjögren's syndrome.

OBJECTIVE--To determine the frequency and relative risk of bronchial hyperreactivity to methacholine in systemic sclerosis patients with or without associated Sjögrens syndrome. METHODS--A prospective study of 56 patients with systemic sclerosis (42 with the diffuse and 14 with the limited variant; 24 with associated Sjögrens syndrome), 57 with primary Sjögrens syndrome, and 61 healthy controls. RESULTS--Bronchial hyperreactivity (BH) was present in 6.5% of the healthy controls, 25% of the systemic sclerosis patients without associated Sjögrens syndrome, 42.2% of those with primary Sjögrens syndrome, and in 50% of those with systemic sclerosis with associated Sjögrens syndrome. The presence of BH did not correlate with age, disease duration, chest radiograph abnormalities, respiratory, and immunological data. The subgroup of subjects with the limited variant of systemic sclerosis more frequently had associated BH than did those with the diffuse variant of the disease; coexisting Sjögrens syndrome further increased this frequency. CONCLUSIONS--In agreement with previous studies, we have confirmed the high prevalence of bronchial hyperreactivity in primary Sjögrens syndrome; systemic sclerosis likewise appears to be associated with an increased frequency of bronchial hyperreactivity compared with healthy control subjects. There is evidence also that the coexistence of Sjögrens syndrome and systemic sclerosis further increases the frequency and the calculated relative risk of developing bronchial hyperreactivity.

Colon involvement in systemic sclerosis: Clinical-radiological correlations

SummaryAbout one third of all patients with systemic sclerosis (SS) presents colon abnormalities, although these may be underestimated because they frequently remain asymptomatic for a long time. Thirty-five patients (33 women and 2 men; mean age 56.5 years; mean disease duration 11.9 years) affected by SS (25 with limited and 10 with diffuse pattern of skin involvement) were investigated using barium enema to detect radiological changes in the colon, and to correlate them with other visceral involvement, autoantibody profile, abdominal symptoms and duration of the disease. Ten patients (28.6%) showed X-rays abnormalities (excluding isolated diverticula), wide-mouthed sacculations being the most frequent finding. Our data confirm that the colon is frequently involved in SS, even in the limited form of the disease. The most relevant finding was the dissociation between clinical symptoms and radiological features which proved to be more evident among the patients with limited SS. No correlations were found between the radiological picture and any other parameter, thus suggesting that careful evaluation of the colon should be performed in any patient suffering from the disease.

AB0595 Descriptive Analysis of A Single Center Series of 23 Patients with Positive PM-SCL Antibody

Background PM-Scl antibodies (abs) belong to the group of myositis-associated antibodies and typically determine a nucleolar or speckled pattern on immunofluorescence. These abs found in patients (pts) with Scleromyositis and, less frequently, in isolated forms of Systemic sclerosis (SSc), Polymyositis (PM) and Dermatomyositis (DM). In SSc pts PM-Scl +, pulmonary involvement seems less severe, while they show more frequent arthritis. Raynauds phenomenon (RP), digital ulcers (DU) and gastroesophageal involvement appear to be less frequently expressed. In myositis the association with esophageal and lung involvement is well known. Objectives To characterize demographic and clinical features of a single center series of PM-Scl positive patients. Methods Sera from 48 SSc and 60 idiopathic inflammatory myophaties (MII) attending local outpatient clinic, detected with EUROIMMUNE Myositis Profile 4 kit, were tested to search PM-ScL antibodies. Demographic, laboratory and clinical data (RP, arthritis, DU, muscular involvement, disphagia) were retrospectively retrieved from available clinical charts. The presence of interstitial lung disease (ILD), was evaluated by HRCT (measured by using score of Goh for the extension) and functional respiratory testing (FVC and DLCO). Severe ILD was defined by: Goh score of>20 and FVC and DLCO <75%. Results 23 Pts (18 F) resulted positive for PM-Scl. The mean age was 60.5±9.4 years and the mean disease duration was 145.9± 76.3 months. 1 patient had negative ANA, 1 showed ANA anticentromere and 1 dot pattern; the remaining 20 patients (86,9%) were ANA+ showing nucleolar pattern. By applying available formal classification criteria 5 (21.7%) pts were diagnosed as DM, 2 (8.7%) as DM amyopathic, 3 (13.05%) as overlap Scleromyositis, 3 (13.05%) as PM, 1 (4.4%) as cancer associated PM, 9 (39.1%) as SSc (2 diffuse, limited 6, intermediate 1). 13 (56.5%) pts had no evidence of cytopathic muscle involvement (defined as normal CPK, myoglobin and LDH). 5 pts (21.7%) presented a Goh score >20, 1 FVC <75%, and 7 (30.4%) DLCO <75%. 4 pts presented arthritis. 14 (60.8%) had RP, 6 (26%) DU, 8 (34.8%), dysphagia (see images 1). When compared with patients with SSc and MII negative for PM-Scl, MII PM-Scl + pts did not show significant greater risk of developing ILD (Goh>20)dysphagia or minor muscle involvement. SSc PM-Scl + pts seem to have a greater risk of developing arthritis and DU than their corresponding SSc PM-Scl negative pts (p<0.05) while no differences did emerged about ILD (Goh>20) and dysphagia. Conclusions The role of PM-Scl as a risk factor for ILD in MII pts is not confirmed, but should be considered the positivity in the control group of Jo1, representing per se a major risk factor for this complication (image 2). For patients with SSc is confirmed the role of PM-Scl as a risk factor for arthritis and digital ulcers but not for ILD. References Long-term out come of patients with polymiositis/dermatomyositis and anti PM/Scl antibody. Marie et al. Journal of Dermatology 2010; 162(2): 337–44 Disclosure of Interest None declared

SAT0310 Overlap Syndrome Systemic Sclerosis-Rheumatoid Arthritis and Pulmonary Involvement: Description of A Monocentric Series

Background the overlap syndrome Systemic sclerosis-rheumatoid arthritis (SSc-RA) is characterized by the presence in the same patient of clinical and laboratory features that meet the classification criteria for both diseases. The current literature shows how that lung involvement is more frequent and severe in SSc-RA than in SSc alone [1]. Objectives to describe the frequency of clinically significant interstitial lung disease (ILD), determining the reduction of the forced vital capacity (FVC) or the alveolar-capillary diffusion of carbon monoxide (DLCO) in a group of 25 patients with SSc-RA compared to a control group of 570 patients with SSc alone. Methods data were retrieved from a database and from medical records of patients followed at the outpatient clinic dedicated to SSc of the Rheumatology Unit of S. Anna Hospital, Ferrara. All patients fulfilled the classification criteria for SSc (ACR 1980 and LeRoy 2001) and RA (1987). For both groups (SSc and SSc-RA) the following seroimmunologic data were assessed: anticentromere antibodies (ACA), anti topoisomerase 1 (Scl 70), rheumatoid factor (RF) and anti-citrulline (ACPA). Pulmonary involvement was assessed by high-resolution chest CT scan using the score of Warrick, and spirometry including DLCO. A cut-off of Warricks score able to detect patients with ILD has been identified by the application of a regression curve between the total score at HRCT and spirometry data (FVC and/or DLCO <75% as predicted); the analysis identified a value>10 able to alter lung function. Results in the SSc control group FR was available in 38% of cases and ACPA only in 8.9% RF and ACPA were available in all 25 SSc-RA patients. Positivity for both FR and ACCP was detected in 23 SSc-RA patients whilst 2 were seronegative. ILD was more frequent in SSc-RA group compared to SSc (76% VS 33%, p<0.05, OR 6.43). The SSc-RA/ACA + group presented a higher frequency of ILD than in SSc/ACA + (54.5% VS 21.7%, p<0.05, OR 4:33) as if the known protective role of the ACA for the development of ILD was less relevant in SSc-RA. In the SSc group, the positivity of the RF seems to be associated with the risk of developing ILD only in patients ACA/Scl 70 negative (72% vs. 28%, p<0.05, OR 6.3). Due to missing data it was not possible to perform the analysis for the ACPA in SSc group. Conclusions an increased risk of ILD in SSc-RA compared to SSc was confirmed. In SSc-RA group, ACA seems to lose its role as a “protective” factor against the possible development of pulmonary fibrosis. If this information is confirmed, in SSc-RA patients, despite their ACA positivity, it could justify a more “tight” clinical-instrumental follow-up, to detect lung involvement early and to monitor its evolution. References Systemic sclerosis-rheumatoid arthritis overlap syndrome: a unique combination of features suggests a distinct genetic, serological and clinical entity. Szücs G, Szekanecz Z, Zilahi E et al. Rheumatology (Oxford). 2007 Jun;46(6):989-93 Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.5137