International expert consensus recommendations on standardised nomenclature of SSA/Ro (TROVE2/Ro60 and TRIM21/Ro52) autoantibodies in autoimmune diseases.

The Frequency of Rheumatic Disease Autoantibodies in Patients with ADAMTS13-Deficient Thrombotic Thrombocytopenia Purpura (TTP).

Objective To study the probability of other autoimmune diseases in primary biliary cirrhosis (PBC), autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) and explore its effects on the prognosis. Methods From January 1994 to March 2014, the data of 232 patients with autoimmune liver diseases (AILD) were collected. The type and case number of coexisting with other autoimmune diseases of patients with PBC, AIH and PSC were analyzed and compared. Cox regression model was performed to analyze the effects of coexisting with autoimmune diseases on the prognosis of AILD. Results Among 135 PBC patients, there were 64 cases that coexisted with Sjogren's syndrome (SS), seven cases with systemic lupus erythematosus (SLE), seven cases with rheumatoid arthritis (RA), nine cases with systemic sclerosis (SSc), three cases with polymyositis and/or dermatomyositis (PM/DM) and one case with Crohn's disease. Among 55 AIH patients, threre were 19 cases that coexisted with SS, 10 cases with SLE, one case with RA, two cases with SSc and two cases with PM/DM. Among 24 PSC patients, there were seven cases combined with ulceric colitis, one case with Crohn's disease and one case with RA. Among 18 patients with PBC-AIH overlap syndrome, there were five cases with SS and one case with RA. Compared with PBC patients, the risk of pulmonary interstitial fibrosis increased in PBC patiento coexisting with SS (OR=34.0, 95%CI 8.9 to 130.1). After gender, age, disease course and medicine intervention were adjusted, the prognosis of AILD which included death, liver transplantation and liver cirrhosis complications was not affected by the coexistence with other autoimmune diseases. Conclusions AILD patients coexisting with other autoimmune diseases is common, most of which are SS, SLE, SSc and RA. PBC patients coexisting with SS is the risk factor of pulmonary interstitial fibrosis, and coexisting with other autoimmune disease does not independently affect the prognosis of AILD. Key words: Hepatitis, autoimmune; Comorbidity; Autoimmune diseases; Prognosis

FRI0531 AIR POLLUTANTS AND DEVELOPMENT OF INTERSTITIAL LUNG DISEASE IN PATIENTS WITH AUTOIMMUNE DISEASES

Background Vitamin D plays a key role in calcium homeostasis and contributes to the regulation of the immune system. Furthermore, vitamin D deficiency has been reported to be associated with autoimmune diseases (AIDs), especially with multiorgan AIDs. Various multiorgan AIDs may be different based on the vitamin D status. This study aims to investigate the serum 25-hydroxyvitamin D (25(OH)D) levels in patients with different common multiorgan AIDs. Methods A total of 295 patients with multiorgan AIDs treated in our hospital from January 2012 to September 2018 were recruited, including 137 cases of rheumatoid arthritis (RA), 85 cases of systemic lupus erythematosus (SLE), 32 cases of Sjögren’s syndrome (SS) and 41 cases of mixed connective tissue disease (MCTD); 47 apparently healthy individuals were also recruited as controls. The serum 25(OH)D levels in patients with different multiorgan AIDs were measured with Roche electrochemiluminescence immunoassay and statistically analyzed the proportion of patients with normal, insufficiency and deficiency in 25(OH)D levels in different multiorgan diseases. The 25(OH)D levels of different multiorgan AID groups and healthy controls were also compared. Results Incidences of 25(OH)D deficiency in the RA, SLE, SS and MCTD groups were 21.2%, 35.3%, 25.0% and 22.0%, respectively, with significant inter-group differences (p < 0.05). The incidence in the SLE group was higher than in the RA, SS and MCTD groups, indicating severe 25(OH)D deficiency in patients with SLE. Significant inter-group differences (p < 0.05) were detected in the serum 25(OH)D levels in different multiorgan AID groups and in the healthy control group. Further pairwise comparison found a significantly higher level of 25(OH)D in the healthy control group than in the SLE, SS, RA and MCTD groups (p < 0.05). Moreover, the 25(OH)D status in the SLE group was significantly lower than that in the SLE, SS, RA and MCTD groups (p < 0.05). Conclusions Serum 25(OH)D deficiency and a low 25(OH)D status are commonly seen in patients with different multiorgan AIDs compared to healthy controls, warranting vitamin D supplementation. Severe 25(OH)D deficiency and a lower 25(OH)D status were found in patients with SLE.

Prior investigations demonstrated that autoantibodies recognizing cytosolic 5'-nucleotidase 1A (NT5C1A) are found in 33-76% of patients with inclusion body myositis (IBM) but are observed only rarely in patients with polymyositis (PM). Thus, anti-NT5C1A may help distinguish IBM from PM. Although 4-21% of patients with dermatomyositis (DM) were shown to be anti-NT5C1A antibody positive, the clinical features of anti-NT5C1A-positive patients with DM have not been described. Furthermore, the prevalence of anti-NT5C1A antibodies in other rheumatic conditions has not been reported. This study was undertaken to define the prevalence and clinical features of anti-NT5C1A-positive patients with DM, PM, IBM, or other systemic autoimmune diseases. We screened for anti-NT5C1A autoantibodies in patients with IBM, DM, PM, Sjögren's syndrome (SS), or systemic lupus erythematosus (SLE) and in healthy volunteers. Clinical characteristics were compared between patients who were anti-NT5C1A positive and those who were anti-NT5C1A negative. Anti-NT5C1A autoantibodies were detected in 71 (61%) of 117 patients with IBM, 2 (5%) of 42 patients with PM, 2 (5%) of 42 healthy volunteers, 24 (15%) of 159 patients with DM, 10 (23%) of 44 patients with SS, and 13 (14%) of 96 patients with SLE. No anti-NT5C1A antibody-positive patients with SS or SLE had muscle involvement. Anti-NT5C1A-positive patients with IBM had a lower prevalence of rimmed vacuoles (62% versus 83% of antibody-negative patients; P = 0.02). No differences in the clinical characteristics of antibody-positive and antibody-negative patients with DM, SS, or SLE were observed. Anti-NT5C1A is a common target of circulating autoantibodies, especially in IBM but also in several different autoimmune diseases. In SLE and SS, anti-NT5C1A autoreactivity is not associated with muscle disease.

Characterization of Clinical Features and Survival of Patients with Autoimmune-Associated Diffuse Large B Cell Lymphoma in a Large Institutional Cohort

Anticytokine autoantibodies occur across a range of hematologic, pulmonary, and infectious diseases. However, systematic investigation of their presence and significance in autoimmune diseases is lacking. This study was undertaken to examine the distinct functions of anticytokine autoantibodies in patients with systemic lupus erythematosus (SLE) compared to patients with other rheumatic diseases and healthy controls. Serum samples from patients with SLE (n = 199), patients with primary Sjögren's syndrome (SS) (n = 150), patients with rheumatoid arthritis (RA) (n = 149), and healthy controls (n = 200) were screened for 24 anticytokine autoantibodies using a multiplex bead-based assay. To evaluate the biologic activity of anticytokine autoantibodies, their ability to block cytokine-induced signal transduction or protein expression was measured. RNA sequencing was performed on whole blood in a subset of healthy controls and patients with SLE. Patients with SLE and those with SS had a striking excess of autoantibodies against interferons and the interferon-responsive chemokine interferon-inducible protein 10 (IP-10). Only autoantibodies against type I interferon, interleukin-12 (IL-12), and IL-22 exhibited neutralizing activity. In SLE, the presence of anti-interferon-γ autoantibodies was correlated with more severe disease activity, higher levels of anti-double-stranded DNA antibodies, and elevated expression of interferon-α/β-inducible genes. Conversely, in SLE patients with blocking anti-interferon-α autoantibodies, the type I interferon gene expression signature was normalized. Anti-type III interferon autoantibodies (λ2, λ3) and anti-IP-10 autoantibodies were newly recognized in SLE patient serum, and autoantibodies against macrophage-colony stimulating factor, IL-4, IL-7, IL-17, and IL-22, none of which have been previously identified in rheumatic conditions, were discovered. Anticytokine autoantibodies are associated with distinct patterns of disease in SLE, SS, and RA. Anti-interferon autoantibodies are overrepresented in patients with SLE and those with SS, and fall into distinct functional classes, with only a subset of anti-type I interferon antibodies exhibiting neutralizing activity. Anti-interferon-γ autoantibodies are correlated with increased disease activity and interferon-related gene expression, suggesting that such autoantibodies may contribute to the pathogenesis of SLE.

Characterizing Autoimmune Disease-Associated Diffuse Large B Cell Lymphoma in a SEER-Medicare Cohort

Background/PurposeSLE (Systemic Lupus Erythematosus) and SjD (Sjögren’s Disease) are similar diseases. Patients with these conditions share many overlapping features and some patients meet the classification criteria for both disease states....

status: Published

Systemic autoimmune diseases are a group of common diseases, including rheumatoid arthritis, systemic lupus erythematosus, spondyloarthropathy, Sjogren's syndrome, polymyositis, and dermatomyositis, etc. They are one of the leading causes of death and disability. With the use of glucocorticoid, immune suppression drugs and new developed biologics, the outcome of this group of diseases has greatly improved, but there is still no cure for them. Knowledge of the pathogenesis, diagnosis, and treatment of those diseases will lead to better understanding of the diseases and better care of patients. Based on this background, we assembled this special issue for a better understanding of the molecular pathology underlying systemic autoimmune diseases, the development of strategies to treat these conditions, and the evaluation of outcomes. In this special issue, several review articles discussed many important aspects about autoimmune disease. A. Mastrangelo et al. discussed the role of posttranslational protein modifications in rheumatoid arthritis. C. Gluhovschi et al. made a review about pregnancy associated with systemic lupus erythematosus. A. Kronbichler et al. reviewed the influence and role of microbial factors in autoimmune kidney diseases. T. Shizuma summarized clinical characteristics of concomitant systemic lupus erythematosus and primary biliary cirrhosis. Z. Wu and H. Nakanishi discussed the link between inflammatory bone disorders and Alzheimer's disease. K. R. Sigdel et al. made a review about the functions of long noncoding RNA in immune cells. L. Duan et al. made a review about treatment of bullous systemic lupus erythematosus. R. Hage-Sleiman et al. reviewed recent studies about the novel PKCtheta. L. Zhang et al. made a meta-analysis about interleukin-23R rs7517847 T/G polymorphism and the risk of Crohn's disease in Caucasians. Beside reviews, many original research studies about the pathogenesis or clinical characteristics of autoimmune disease were also included in this special issue. T. Elisa et al. studied the role of endothelin receptors in the pathogenesis of systemic sclerosis. A. Barbieri et al. analyzed the characterization of CD30/CD30L+ cells in peripheral blood and synovial fluid of patients with rheumatoid arthritis. G. F. Dong et al. researched the effect of leflunomide on the lipid rafts expression in SLE patients. P. Žigon et al. found that anti-phosphatidylserine/prothrombin antibodies were associated with adverse pregnancy outcomes. G. Sudzius et al. studied the distribution of peripheral lymphocyte populations in primary Sjogren's syndrome patients. J. Xu et al. showed that autoantibodies affect brain density reduction in nonneuropsychiatric systemic lupus erythematosus patients. B. Shen et al. found that body image disturbances have impact on the sexual problems in systemic lupus erythematous patients. G. Guo et al. and C. Zhao et al. studied mesenchymal stem cells in SLE and RA patients. Y. Liu et al. found a new serological marker in SLE patients. A. E. Ngono et al. found that frequency of circulating myelin oligodendrocyte glycoprotein B lymphocytes was decreased in relapsing-remitting multiple sclerosis patients. J. Amaya-Amaya et al. showed that GDF15 (MIC1) H6D polymorphism does not influence cardiovascular disease in a Latin American population with rheumatoid arthritis. A. D. Rocha-Munoz et al. demonstrated that anti-CCP2 antibodies are markers associated with the severity of RA- ILD. M. Lu et al. found that HMGB1 promoted systemic lupus erythematosus by enhancing macrophage inflammatory response. A. D. Rocha-Munoz et al. studied the influence of anti-TNF and disease modifying antirheumatic drugs (DMARDs) therapy on pulmonary forced vital capacity associated with ankylosing spondylitis. B. Kisiel et al. showed that methotrexate, cyclosporine A, and biologics protect against atherosclerosis in rheumatoid arthritis. L. Wang et al. analyzed clinical characteristics of cerebral venous sinus thrombosis in SLE patients. This special issue covers many important aspects in autoimmune diseases, which will surely provide us with a better understanding about the pathogenesis, diagnosis, and treatment of autoimmune diseases. Guixiu Shi Jianying Zhang Zhixin (Jason) Zhang Xuan Zhang

SAT0612 FREQUENCY OF POLYAUTOIMMUNITY IN A TERTIARY HOSPITAL

SAT0218 SINGLE NUCLEOTIDE POLYMORPHISMS LOCATED IN REGULATORY REGIONS OF GENES INVOLVED IN SYSTEMIC INFLAMMATION (MAMDC1, ITGAM, AND CRP) CORRELATION WITH THE CLINICAL PICTURE OF DISEASE AND ACTIVITY PARAMETERS IN SYSTEMIC LUPUS ERYTHEMATOSUS

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Nonstandard and adjunctive medical therapies for systemic lupus erythematosus