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https://www.readbyqxmd.com/read/29224302/-clinical-and-radiological-features-of-adult-patients-with-different-antisynthetase-syndrome-based-on-serum-anti-aminoacyl-trna-synthetase-antibody-type
#1
S Li, H Huang, X Y Song, K Xu, C Y Wu, Q Wang, Z J Xu
Objective: To describe the clinical and radiological characteristics of antisynthetase syndrome associated interstitial lung disease in patients with different serum anti-aminoacyl-tRNA synthetase antibodies. Methods: We conducted a retrospective analysis of 5 adult patients with antisynthetase syndrome associated interstitial lung disease in Peking Union Medical College Hospital. Their clinical and chest radiological data were analyzed and relevant literatures were reviewed. Results: Among these 5 patients, there were 1 male and 4 females, aged from 32 y to 67 y, with a mean age of 53 y...
December 12, 2017: Chinese Journal of Tuberculosis and Respiratory Diseases
https://www.readbyqxmd.com/read/29212510/factors-affecting-treatment-outcome-in-patients-with-idiopathic-nonspecific-interstitial-pneumonia-a-nationwide-cohort-study
#2
Sang Hoon Lee, Moo Suk Park, Song Yee Kim, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Soo Taek Uh, Choon Sik Park, Sung Woo Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Jong Wook Shin, Eun Joo Lee, Jin Hwa Lee, Yangin Jegal, Hyun Kyung Lee, Yong Hyun Kim, Jin Woo Song, Jong Sun Park
BACKGROUND: The effects of corticosteroid-based therapy in patients with idiopathic nonspecific interstitial pneumonia (iNSIP), and factors affecting treatment outcome, are not fully understood. We aimed to investigate the long-term treatment response and factors affecting the treatment outcome in iNSIP patients from a multi-center study in Korea. METHODS: The Korean interstitial lung disease (ILD) Study Group surveyed ILD patients from 2003 to 2007. Patients were divided into two groups to compare the treatment response: response group (forced vital capacity (FVC) improves ≥10% after 1 year) and non-response group (FVC <10%)...
December 6, 2017: Respiratory Research
https://www.readbyqxmd.com/read/29201326/a-challenging-entity-multiple-sclerosis-or-collagen-tissue-disorders-a-case-series-of-6-patients
#3
Raida Ben Salah, Yosra Cherif, Faten Frikha, Dammak Chifaa, Mouna Snoussi, Moez Jallouli, Sameh Marzouk, Mhiri Chokri, Zouhir Bahloul
Background: Multiple sclerosis and other demyelinating processes are sometimes difficult to differentiate from the neurological involvement in autoimmune diseases. Distinguishing multiple sclerosis from other lesions due to autoimmune diseases is crucial to avoid unsuitable or delayed treatments. Methods: Charts of 6 patients diagnosed with mimicking multiple sclerosis between 1996 and 2014 were retrospectively assessed. Results: The mean age at diagnosis was 35±7 years...
2017: Caspian Journal of Internal Medicine
https://www.readbyqxmd.com/read/29192160/a-novel-human-systemic-lupus-erythematosus-model-in-humanised-mice
#4
Merry Gunawan, Zhisheng Her, Min Liu, Sue Yee Tan, Xue Ying Chan, Wilson Wei Sheng Tan, Shubasree Dharmaraaja, Yong Fan, Chee Bing Ong, Eva Loh, Kenneth Tou En Chang, Thiam Chye Tan, Jerry Kok Yen Chan, Qingfeng Chen
Mouse models have contributed to the bulk of knowledge on Systemic Lupus Erythematosus (SLE). Nevertheless, substantial differences exist between human and mouse immune system. We aimed to establish and characterise a SLE model mediated by human immune system. Injection of pristane into immunodeficient mice reconstituted with human immune system (humanised mice) recapitulated key SLE features, including: production of human anti-nuclear autoantibodies, lupus nephritis, and pulmonary serositis. There was a reduction in the number of human lymphocytes in peripheral blood, resembling lymphopenia in SLE patients...
November 30, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29185130/increased-lipid-and-protein-oxidation-and-lowered-anti-oxidant-defenses-in-systemic-lupus-erythematosus-are-associated-with-severity-of-illness-autoimmunity-increased-adhesion-molecules-and-th1-and-th17-immune-shift
#5
Bruna Miglioranza Scavuzzi, Andréa Name Colado Simão, Tatiana Mayumi Veiga Iriyoda, Marcell Alysson Batisti Lozovoy, Nicole Perugini Stadtlober, Lorena Flor da Rosa Franchi Santos, Tamires Flauzino, Fabiano Aparecido de Medeiros, Marcelo Cândido de Sá, Luana Consentin, Edna Maria Vissoci Reiche, Michael Maes, Isaias Dichi
This study investigated nitro-oxidative stress in patients with systemic lupus erythematosus (SLE) in association with disease activity, immune-inflammatory biomarkers, and adhesion molecules. Two-hundred-four patients with SLE and 256 healthy volunteers were enrolled in this case-control study, which measured nitro-oxidative stress biomarkers, including lipid peroxides (LOOH), advanced oxidation protein products (AOPPs), nitric oxide metabolites (NOx), sulfhydryl (-SH) groups, products of deoxyribonucleic acid (DNA)/ribonucleic acid (RNA) oxidative degradation, and total radical-trapping anti-oxidant parameter (TRAP)...
November 29, 2017: Immunologic Research
https://www.readbyqxmd.com/read/29185086/interstitial-lung-disease-in-south-africans-with-systemic-sclerosis
#6
Philippa Ashmore, Mohammed Tikly, Michelle Wong, Claudia Ickinger
To investigate the frequency, severity and predictors of interstitial lung disease (ILD) in a cohort of South Africans with systemic sclerosis (SSc). Retrospective record review of SSc patients attending a tertiary Connective Tissue Diseases Clinic. Patients with ILD, defined by a combination of clinical findings, imaging, and lung function tests were compared to patients without ILD in terms of demographics, clinical features and autoantibodies. The majority (86.8%) of the 151 patients included were of Black ethnicity, 40% had ILD, of whom 39% had moderate-severe lung disease...
November 28, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29180749/clonal-evolution-and-antigen-recognition-of-anti-nuclear-antibodies-in-acute-systemic-lupus-erythematosus
#7
Shuhei Sakakibara, Takao Arimori, Kazuo Yamashita, Hideyuki Jinzai, Daisuke Motooka, Shota Nakamura, Songling Li, Kazuya Takeda, Jun Katayama, Marwa Ali El Hussien, Masashi Narazaki, Toshio Tanaka, Daron M Standley, Junichi Takagi, Hitoshi Kikutani
The evolutional process of disease-associated autoantibodies in systemic lupus erythematosus (SLE) remains to be established. Here we show intraclonal diversification and affinity maturation of anti-nuclear antibody (ANA)-producing B cells in SLE. We identified a panel of monoclonal ANAs recognizing nuclear antigens, such as double-stranded DNA (dsDNA) and ribonucleoproteins (RNPs) from acute SLE subjects. These ANAs had relatively few, but nonetheless critical mutations. High-throughput immunoglobulin sequencing of blood lymphocytes disclosed the existence of sizable ANA lineages shearing critical mutations intraclonally...
November 27, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29178913/identification-of-multiple-cancer-associated-myositis-specific-autoantibodies-in-idiopathic-inflammatory-myopathies-a-large-longitudinal-cohort-study
#8
Hanbo Yang, Qinglin Peng, Liguo Yin, Shanshan Li, Jingli Shi, Yamei Zhang, Xin Lu, Xiaoming Shu, Sigong Zhang, Guochun Wang
BACKGROUND: Cancer is a significant complication contributing to increased mortality in idiopathic inflammatory myopathies (IIMs), and the association between IIMs and cancer has been extensively reported. Myositis-specific autoantibodies (MSAs) can help to stratify patients into more homogeneous groups and may be used as a biomarker for cancer-associated myositis. In this study, we aimed to systematically define the cancer-associated MSAs in IIMs. METHODS: Serum from 627 patients with IIMs was tested for MSAs...
November 25, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29170979/predictors-of-changes-in-disease-activity-among-children-with-juvenile-dermatomyositis-enrolled-in-the-childhood-arthritis-and-rheumatology-research-alliance-carra-legacy-registry
#9
Divya Challa, Cynthia S Crowson, Timothy B Niewold, Ann M Reed
Determinants of changes in disease activity among patients with juvenile dermatomyositis (JDM) are unknown. Our objective was to develop predictive models to predict changes in disease activity using the CARRA Legacy Registry. The CARRA Legacy Registry included 658 subjects with definite or probably JDM with 297 subjects with a one follow-up visit after baseline, and we studied the 65 subjects with active disease at baseline. Linear regression models were used to build risk scores for changes in disease activity adjusted for baseline disease activity, age, sex, and disease duration...
November 23, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29169972/detection-of-circulating-igg-autoantibody-to-fc%C3%AE%C2%B5ri%C3%AE-in-sera-from-chronic-spontaneous-urticaria-patients
#10
Bastsetseg Ulambayar, Yi-Hsing Chen, Ga-Young Ban, Ji-Ho Lee, Chang-Gyu Jung, Eun-Mi Yang, Hae-Sim Park, Young-Min Ye
BACKGROUNDS: Chronic spontaneous urticaria (CSU) is a common skin disorder characterized by itchy wheals of at least 6 weeks in duration, wherein the autoimmune mechanism is involved to activate IgE receptors (FcεRIα) on mast cells. We aimed to assess levels of IgG autoantibody against FcεRIα in sera from CSU patients using dot-blot immunoassay. METHODS: We performed a hospital-based cross-sectional study of 125 CSU patients (64 ASST-positive, 61 ASST-negative) and 64 age-and sex-matched healthy controls...
November 14, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
https://www.readbyqxmd.com/read/29167450/mafb-is-a-critical-regulator-of-complement-component-c1q
#11
Mai Thi Nhu Tran, Michito Hamada, Hyojung Jeon, Risako Shiraishi, Keigo Asano, Motochika Hattori, Megumi Nakamura, Yuki Imamura, Yuki Tsunakawa, Risa Fujii, Toshiaki Usui, Kaushalya Kulathunga, Christina-Sylvia Andrea, Ryusuke Koshida, Risa Kamei, Yurina Matsunaga, Makoto Kobayashi, Hisashi Oishi, Takashi Kudo, Satoru Takahashi
The transcription factor MafB is expressed by monocytes and macrophages. Efferocytosis (apoptotic cell uptake) by macrophages is important for inhibiting the development of autoimmune diseases, and is greatly reduced in Mafb-deficient macrophages. Here, we show the expression of the first protein in the classical complement pathway C1q is important for mediating efferocytosis and is reduced in Mafb-deficient macrophages. The efferocytosis defect in Mafb-deficient macrophages can be rescued by adding serum from wild-type mice, but not by adding serum from C1q-deficient mice...
November 22, 2017: Nature Communications
https://www.readbyqxmd.com/read/29166758/clinical-significance-of-anti-dense-fine-speckled-70-antibody-in-patients-with-fibromyalgia
#12
Jisoo Jeong, Dong Hyun Kim, Gun Park, Suyeon Park, Hyun-Sook Kim
Background/Aims: Fibromyalgia (FM) is a common rheumatologic disease characterized by chronic widespread pain, along with various clinical manifestations including atypical autoimmune characteristics. Despite its high prevalence, there remain no approved laboratory tests to identify specific manifestations of FM, or to rule out FM from other rheumatic diseases. Anti-dense fine speckled 70 (anti-DFS70) antibodies were initially identified as a form of anti-nuclear antibodies in a patient with interstitial cystitis...
November 24, 2017: Korean Journal of Internal Medicine
https://www.readbyqxmd.com/read/29159696/reflex-testing-of-speckled-cytoplasmic-patterns-observed-in-routine-ana-hep-2-indirect-immunofluorescence-with-a-multiplex-anti-synthetase-dot-blot-assay-a-multicentric-pilot-study
#13
Maria Infantino, Boaz Palterer, Roberta Biagiotti, Fabio Almerigogna, Maurizio Benucci, Arianna Damiani, Valentina Grossi, Annalisa Azzurri, Patrizia Casprini, Giovanni Bacci, Maria Grazia Giudizi, Mariangela Manfredi
Immunofluorescence on HEp2-cells is the standard diagnostic assay for the detection of anti-nuclear antibodies (ANA). Cytoplasmic speckled patterns are a common finding, and are associated with various antibodies, including anti-synthetase antibodies. However, classic ENA testing generally identifies only anti-Jo-1. Moreover, anti-synthetase syndrome is increasingly recognized as a pleomorphic entity, possibly presenting as isolated arthritis or interstitial lung disease. Sera referred for routine ANA testing were selected on the basis of the presence of a fine dense speckled cytoplasmic pattern (254 samples) and compared to control sera with negative cytoplasm (239 samples)...
November 21, 2017: Immunologic Research
https://www.readbyqxmd.com/read/29151501/the-efficacy-of-corticosteroid-therapy-in-a-patient-with-nonalcoholic-steatohepatitis-overlapping-autoimmune-hepatitis-a-case-report
#14
Takuya Komura, Hajime Ohta, Takuya Seike, Yoshiaki Shimizu, Ryotaro Nakai, Hitoshi Omura, Takashi Kagaya, Satomi Kasashima, Atsuhiro Kawashima, Sakae Oba, Kennichi Harada, Shuichi Kaneko, Masashi Unoura
The overlap of multiple liver diseases can cause the disease activity and severity to worsen rapidly in some cases. We rarely see patients with non-alcoholic steatohepatitis (NASH) with overlapping autoimmune hepatitis (AIH). A 64-year-old woman who had been prescribed oral drugs to treat diabetes and hypertension (metformin 500 mg/day and voglibose 0.9 mg/day, and termisartan 40 mg/day and amlodipine 5 mg/day, respectively) was diagnosed with NASH with histological confirmation. At 68 years of age, her liver injury worsened with an IgG of 2,871 mg/dL and a high serum anti-nuclear antibody (ANA) level of 2,560...
November 20, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29130141/sj%C3%A3-gren-s-syndrome-initially-presented-as-thrombotic-thrombocytopenic-purpura-in-a-male-patient-a-case-report-and-literature-review
#15
REVIEW
Xiaohan Xu, Tienan Zhu, Di Wu, Lu Zhang
Thrombotic thrombocytopenic purpura (TTP) is a potentially lethal multisystem disorder which could be caused by autoimmune diseases. However, the concomitant occurrence of TTP and Sjögren's syndrome (SS) is an extremely uncommon scenario, especially in male patients. A 56-year-old Chinese male was admitted for the appearance of diffuse ecchymosis. Then he gradually developed transient slurred speech, progressive confusion, agitation, extremity weakness, and fever. Laboratory investigations suggested anemia, thrombocytopenia, significantly increased lactic dehydrogenase, schistocytes in peripheral blood smear, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers...
November 13, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29108204/-clinical-features-of-autoimmune-hepatitis-patients-with-poor-response-to-treatment
#16
H W Zhang, H M Liu, R Lin, L Zhou, J Zhang, W T Liu
Objective: To investigate the clinical features of autoimmune hepatitis (AIH) patients with poor response to treatment. Methods: A total of 61 AIH patients were enrolled, among whom 49 (80.33%) achieved complete response (good response group) and 12 (19.67%) had incomplete response (poor response group). The two groups were compared in terms of clinical manifestations, laboratory markers, abdominal ultrasound findings, pathological features by liver biopsy, and response to treatment. Continuous data were expressed as mean ± standard deviation (x±s), and the t-test was used for comparison between groups; categorical data were expressed as rates or percentages, and the chi-square test was used for comparison between groups; a binary logistic regression analysis was used to determine influencing factors...
October 20, 2017: Zhonghua Gan Zang Bing za Zhi, Zhonghua Ganzangbing Zazhi, Chinese Journal of Hepatology
https://www.readbyqxmd.com/read/29093268/microbiota-control-immune-regulation-in-humanized-mice
#17
Elke Gülden, Nalini K Vudattu, Songyan Deng, Paula Preston-Hurlburt, Mark Mamula, James C Reed, Sindhu Mohandas, Betsy C Herold, Richard Torres, Silvio M Vieira, Bentley Lim, Jose D Herazo-Maya, Martin Kriegel, Andrew L Goodman, Chris Cotsapas, Kevan C Herold
The microbiome affects development and activity of the immune system, and may modulate immune therapies, but there is little direct information about this control in vivo. We studied how the microbiome affects regulation of human immune cells in humanized mice. When humanized mice were treated with a cocktail of 4 antibiotics, there was an increase in the frequency of effector T cells in the gut wall, circulating levels of IFN-γ, and appearance of anti-nuclear antibodies. Teplizumab, a non-FcR-binding anti-CD3ε antibody, no longer delayed xenograft rejection...
November 2, 2017: JCI Insight
https://www.readbyqxmd.com/read/29070672/broad-susceptibility-of-nucleolar-proteins-and-autoantigens-to-complement-c1-protease-degradation
#18
Yitian Cai, Seng Yin Kelly Wee, Junjie Chen, Boon Heng Dennis Teo, Yee Leng Carol Ng, Khai Pang Leong, Jinhua Lu
Anti-nuclear autoantibodies, which frequently target the nucleoli, are pathogenic hallmarks of systemic lupus erythematosus (SLE). Although the causes of these Abs remain broad and ill-defined, a genetic deficiency in C1 complex (C1qC1r2C1s2) or C4 is able to induce these Abs. Considering a recent finding that, in dead cells, nucleoli were targeted by C1q and two nucleolar autoantigens were degraded by C1r/C1s proteases, we considered that C1 could help protect against antinuclear autoimmunity by broadly degrading nucleolar proteins or autoantigens...
October 25, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29056396/serology-in-autoimmune-hepatitis-a-clinical-practice-approach
#19
REVIEW
Benedetta Terziroli Beretta-Piccoli, Giorgina Mieli-Vergani, Diego Vergani
Serology is key to the diagnosis of autoimmune hepatitis (AIH). Clinicians need to be aware of which tests to request, how to interpret the laboratory reports, and be familiar with the laboratory methodology. If correctly tested, >95% of AIH patients show some serological reactivity. Indirect immunofluorescence on triple rodent tissue is recommended as first screening step, since it allows the detection of all liver-relevant autoantibodies, except for anti-soluble liver antigen (SLA) antibody, which needs to be detected by molecular based assays...
October 19, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/29037905/the-inter-observer-reading-variability-in-anti-nuclear-antibodies-indirect-ana-immunofluorescence-test-a-multicenter-evaluation-and-a-review-of-the-literature
#20
REVIEW
A Rigon, M Infantino, M Merone, G Iannello, A Tincani, I Cavazzana, N Carabellese, A Radice, M Manfredi, P Soda, A Afeltra
Recently there has been an increase demand for Computer-Aided Diagnosis (CAD) tools to support clinicians in the field of Indirect ImmunoFluorescence (IIF), as the novel digital imaging reading approach can help to overcome the reader subjectivity. Nevertheless, a large multicenter evaluation of the inter-observer reading variability in this field is still missing. This work fills this gap as we evaluated 556 consecutive samples, for a total of 1679 images, collected in three laboratories with IIF expertise using HEp-2 cell substrate (MBL) at 1:80 screening dilution according to conventional procedures...
October 14, 2017: Autoimmunity Reviews
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