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Background:Autoantibodies permit to classify and subgroup connective tissue diseases (CTD) in homogeneous groups of patients in terms of phenotype and prognosis. Anti PM-Scl antibodies have been associated with different CTD categories such as: idiopathic inflammatory myositis (IIM), systemic sclerosis (SSc), Sjögren’s syndrome (SjS), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) or undifferentiated connective tissue disease (UCTD).Objectives:To determine clinical spectrum of anti-PM-Scl associated disease and if it an homogenous condition.Methods:This multicentric (four hospitals) observational and retrospective study included all consecutive patients with positive testing for anti PM-Scl antibodies on immunoblot assay and connective tissue disease (2011 -2020). Epidemiological, biological, clinical and radiological data were collected in standard form as well as patient’s outcome.Results:One hundred twenty height patients (female n=96;75%) were included. Median [quartiles] age at diagnosis was 50 [18;84] (IQR) and follow-up duration of 7 [3.75-12] years. Seventy-six (59.3%) patients were simple anti-Pm-Scl positive, and 40.7% were associated with other antibodies: anti-SSA/Ro52 (n=13; 10.92%), SSc associated antibodies (n=21; 16.4%), anti-dsDNA for (n=9; 7%), anti-RNP (n=6; 4.7%) and anti-CCP antibodies (n=6; 4.7%). Most patients had cutaneous involvement (n=106; 83%) with skin thickening (n=47; 36%), mechanics hands (n= 28; 22%), calcinosis (n=26; 20.3%) and subcutaneous edema (n=20; 15.62%). Vascular involvement was frequent with Raynaud phenomenon (n= 89; 69%), telangiectasia (n=36; 28%), skin ulcers (n=27; 21%), pulmonary hypertension (n=8/120; 6.7%) and scleroderma renal crisis (n=2; 1.5%). A majority of patients also displayed an interstitial lung disease (ILD) (n=83; 65.8%); nonspecific interstitial pneumonia (92.7%) and/or organizing pneumonia (25.3%). ILD was characterized by a subacute onset in 37/81 (45.7%); median [quartiles] forced vital capacity (FVC) and total lung capacity (TLC) at diagnosis of 88% [73-105] and 79.5% [68.5-101] respectively. Sixty patients (47%) had muscular sign including myalgia (47%), elevated CPK (n=51; 40%) and muscular weakness (Medical Research Council score Conclusion:Conducted on the largest cohort of Anti-PM-Scl patients, this study highlights two main phenotypes that determine different outcome and prognosis. One was associated with muscular disease and subacute onset ILD with more frequent relapses. The second with a vascular phenotype associated with chronic ILD, digestive involvement, chronic evolution and increased incidence of death. This could lead to a reclassification of PM-Scl associated auto immune diseases.Disclosure of Interests:None declared

Background In idiopathic pulmonary fibrosis (IPF) the distribution and spatial-temporal progression of fibrotic changes may be influenced by general or locoregional conditions. From this perspective, patients with asymmetrical disease (AIPF) may be unique. Methods This retrospective study included 32 patients (26 men, mean6SD age 6967 years) with AIPF, as defined by an asymmetry ratio (most affected e least affected fibrosis score)/(most affected + least affected fibrosis score) >0.2. The global fibrosis score was the average of the right and left scores. Patients with AIPF were compared with 64 matched controls with symmetrical IPF. Results Patients with AIPF did not differ from controls in global fibrosis score and forced vital capacity, but carbon monoxide transfer factor was less decreased (52619% vs 43613%, p¼0.009). The rate of gastro-oesophageal reflux and acute exacerbations was significantly higher in patients with AIPF (62.5% vs 31.3%, p¼0.006 and 46.9% vs 17.2%, p¼0.004, respectively). In patients with AIPF the right side was more likely to be involved (62.5%); the median asymmetry ratio was 0.5 (range 0.24e1). Although the global fibrosis score worsened significantly in all 23 patients with AIPF with serial high-resolution CT scans (p

Anthracofibrosis, defined as bronchial luminal narrowing with black pigmentation of the overlying mucosa, has been attributed to tuberculosis. Three patients with anthracofibrosis without mycobacterial infection are described who had previous occupational exposure to mixed dusts. CT scans showed calcified hilar lymph nodes in two patients. Surgical biopsy in one patient and autopsy in another revealed fibrotic lymph nodes with black pigmentation. Mineralogical analysis by transmission electron microscopy of pulmonary, hilar and/or bronchial samples found high levels of particle retention, raised percentages of free crystalline silica and mica in two patients, and free crystalline silica, kaolin and other silicates in the third. No evidence of any other contributory factor was found, suggesting that mixed mineral dust was the most probable cause. These observations suggest that exposure to mixed mineral dust should be added to the aetiology of anthracofibrosis.

Background Rheumatoid arthritis (RA) is a systemic autoimmune disease, the most severe manifestation being interstitial lung disease (ILD). Therapeutic options are undefined and ILD is one of the leading causes of mortality for RA patients. A better characterization of RA-ILD–associated factors may improve the risk stratification of RA patients. Objectives To identify variables associated with ILD occurrence in RA. Methods RA-ILD patients (RA-ILD+) in a French multicentric cohort were compared to monocentric RA cases without ILD (RA-ILD-). All patients fulfilled 2010 ACR/EULAR criteria for RA and had a HRCT chest scan for suspected ILD or during a pre-biologic assessment. ILD was defined by HRCT results. Because ILD occurrence increases with RA duration, RA-ILD+ and RA-ILD- patients were matched by disease duration (1:1). Demographic data, clinical features of RA and treatment modalities before the ILD diagnosis were collected. Results significant on univariate analysis (P Results Among 253 consecutive RA patients, 81 were male (32.02%), 112 (48.07%) were smokers, mean age at RA onset was 48.11 ± 16.33 years, and mean RA duration 11.69 ± 10.10 years; 199 patients (85.78%) were ACPA-positive and 171 (71.85%) had erosive status; 134 (60.63%) received MTX and 63 (28.25%) a TNF inhibitor. Among the 253 patients, 138 (54.55%) had ILD. Among the RA-ILD+ patients, 72 (74.23%) had UIP and 25 (25.77%) NSIP. On univariate analysis, ILD was associated with older age at RA onset (52.69 ± 15.00 vs 42.86 ± 16.26 years, P=0.0001), male sex (40.68% vs 21.74%, P=0.001), ACPA rate ≥60 N (20.63% vs 6.41%, P=0.012), less use of anti-TNF agents and MTX (14.55% vs 41.59%, P=0.001, and 50.94% vs 69.57, P=0.005, respectively) and lower MTX dosage (15.26 ± 4.44 vs 16.83 ± 4.31 mg/week, P=0.046). There was no difference for erosive status. On multivariate analysis, ILD was independently associated with older age at RA onset ( P P =0.0006), less use of MTX ( P =0.015) with lower dosage ( P =0.005) and less use of anti-TNF agents ( P =0.004). Conclusions As previously reported, ILD was independently associated with male sex as well as older age at RA onset and, of interest, less use of MTX and anti-TNF biologics before ILD diagnosis, even though patients did not differ in erosive status. Larger prospective studies are required to further elucidate the role of DMARDs and anti-TNF biologics in ILD occurrence and progression in RA patients. Disclosure of Interest None declared

Background Pulmonary fibrosis (PF) is a rare manifestation observed in patients with antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV), particularly microscopic polyangiitis (MPA). Objectives To report long-term outcome of a cohort of patients with the uncommon association of PF and AAV. Methods We performed a retrospective multicentric study of characteristics and outcome of patients with PF associated to AAV, fulfilling the ACR and/or Chapel Hill definitions. Results Forty nine patients [30 (61%) men, median age at diagnosis of AAV of 68 [58–73] years] with PF associated to AAV were identified. Forty (81.6%) patients had microscopic polyangiitis and 9 (18.4%) had granulomatosis with polyangiitis. The diagnosis of PF preceded the onset of vasculitis in 22 (45%) patients. Usual interstitial pneumonia was the main radiological pattern (n=18, 43%). ANCA were mostly of anti-myeloperoxydase specificity (88%). All patients were treated with steroids as induction therapy, combined with cyclophosphamide (CYC) (n=36, 73.5%) or rituximab (RTX) (n=1, 2%). Factors associated with mortality included occurrence of chronic respiratory insufficiency (HR 7.44, 95% CI (1.6 to 34.5), P=0.003), older age at PF diagnosis (HR 1.08, (1.02-1.13), P=0.005), and a higher eosinophil count at AAV diagnosis (HR 1.32, (1.07-1.63), P=0.0084). The 3-year survival rate in patients treated with steroids alone or combined with immunosuppressant (CYC or RTX) as induction therapy was of 64% (95% CI, 41-99) and 94% (95% CI, 86-100), respectively ( P =0.03, Fig. 1). After a median follow-up of 48 [14–88] months, eighteen (37%) patients died, including 11 related to respiratory insufficiency. Conclusions PF is a rare manifestation of AAV with a very poor prognosis. Induction therapy with CYC might improve the outcome. Disclosure of Interest None declared DOI 10.1136/annrheumdis-2014-eular.4159

Background The prevention of fragility fractures in patients with sarcoidosis (Sa) is a serious concern since risk factors are poorly understood and vitamin D (vitD) or calcium prescription is a matter of hesitation in these patients. Objectives To evaluate factors increasing the risk of osteoporosis (OP) in Sa. Methods We prospectively included 142 consecutive patients with histologically proven Sa. Biological and clinical parameters of Sa activity and severity, BMD (DXA, Lunar Prodigy) and vertebral fracture prevalence on lumbar and thoracic spine (with Genant’s semi-quantitative method of) were assessed. Results 80 women (51 menopaused) and 62 men, (age 51 ±11.9), with a mean Sa duration of 9.5±7 years were included. 28 patients had never received corticosteroids (CCS), 33 had received vitD supplements, and 46 bisphosphonates, within the 6 months preceding the study. Fragility fractures had occurred in 23.5 % of the patients, (vertebral fractures in 13.7%). 40.1% had BMD T-score Conclusions Sa patients have a high risk of fracture despite not lowered BMD. There could be a bimodal relationship between VitD levels and fracture risk. Predictive factors of fracture, independent from BMD and reflecting the severity of Sa, should be considered when a preventive treatment of OP is planned Acknowledgements This study was supported by the GRIO “group de recherche et d’information de l’osteoporose”. Disclosure of Interest None Declared

International audience; Two case histories are described of pleural and anterior mediastinal fibrosis presenting as a continuous fibrotic process with thick parietal pleural plaques extending from one pleura to the contralateral pleura through the retrosternal area, and with retroperitoneal fibrosis. Followup over 4 years in one case demonstrated rapid progression of disease, with pleural fibrosis preceding retrosternal and retroperitoneal fibrosis. Histopathological analysis in both cases showed non-tumoral fibrosis with broad fibrous bundles surrounding fibroblasts (and lymphocytes in one case). Possible causes such as infections and exposure to ergot derivatives were excluded. Both patients had been slightly or moderately exposed to asbestos. These cases represent an unusual new presentation of pleural and retrosternal fibrosis extending beyond the anatomical structures and associated with retroperitoneal fibrosis.