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https://www.readbyqxmd.com/read/29772037/a-review-of-fabry-disease
#1
A Cinats, E Heck
The class of medications known as Janus kinase inhibitors block cytokine-mediated signaling via the Janus kinase-signal transducer and activator of transcription (JAK-STAT) pathway, which plays an important role in immunoregulation and normal cell growth. This class includes the drugs tofacitinib, approved for the treatment of rheumatoid arthritis, and ruxolitinib, approved for the treatment of myelofibrosis and polycythemia rubra vera. The most common adverse events (AEs) reported in patients taking tofacitinib are infections, whereas the most common AEs in patients taking ruxolitinib are anemia and thrombocytopenia...
May 2018: Skin Therapy Letter
https://www.readbyqxmd.com/read/29768670/patients-affected-by-a-new-variant-of-endemic-pemphigus-foliaceus-have-autoantibodies-colocalizing-with-myzap-p0071-desmoplakins-1-2-and-arvcf-causing-renal-damage
#2
A M Abreu-Velez, M S Howard, H Yi, A A Florez-Vargas
BACKGROUND: We have previously reported that about 30% of patients affected by a new variant of endemic pemphigus foliaceus (EPF) in El Bagre, Colombia (termed El Bagre-EPF or pemphigus Abreu-Manu) have systemic compromise. In the current study, we focused on studying autoreactivity to the kidney and its pathological correlations. AIM: To investigate patients with El Bagre-EPF for renal compromise. METHODS: We performed a case-control study, enrolling 57 patients with El Bagre-EPF and 57 controls from the endemic area, matched by age, sex, race, work activity, demographics and comorbidities...
May 16, 2018: Clinical and Experimental Dermatology
https://www.readbyqxmd.com/read/29759047/histological-predictors-of-renal-outcome-in-lupus-nephritis-the-importance-of-tubulointerstitial-lesions-and-scoring-of-glomerular-lesions
#3
B Obrișcă, R Jurubiță, A Andronesi, B Sorohan, C Achim, R Bobeica, M Gherghiceanu, E Mandache, G Ismail
Introduction Lupus nephritis (LN) affects nearly 60% of patients with systemic lupus erythematosus and up to 30% of them will progress to end-stage renal disease (ESRD), despite receiving aggressive immunosuppressive therapy. The prognostic value of ISN/RPS classification is controversial. Therefore, we aimed to identify clinical and pathological predictors of outcome in LN patients independent of this classification. Material and methods Thirty-seven patients with LN who underwent percutaneous kidney biopsy between 1997 and 2016 were included in this study...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29745339/musculoskeletal-and-rheumatic-diseases-induced-by-immune-checkpoint-inhibitors-a-review-of-the-literature
#4
Devis Benfaremo, Lucia Manfredi, Michele Maria Luchetti, Armando Gabrielli
BACKGROUND: Immune checkpoint inhibitors are a new promising class of antitumor drugs that have been associated to a number of immune-related adverse events (AEs), including musculoskeletal and rheumatic disease. METHODS: We searched Medline reviewing reports of musculoskeletal and rheumatic AEs induced by immune checkpoint inhibitors. RESULTS: Several musculoskeletal and rheumatic AEs associated with immune checkpoint inhibitors treatment are reported in literature...
May 8, 2018: Current Drug Safety
https://www.readbyqxmd.com/read/29740923/long-term-renal-outcomes-in-multi-ethnic-southeast-asians-with-lupus-nephritis-a-retrospective-cohort-study
#5
Cynthia C Lim, Hui Zhuan Tan, Ying Hao, Yok Mooi Chin, Keng Thye Woo, Choong Meng Chan, Jason Cj Choo
BACKGROUND: Renal involvement is common among Asians with systemic lupus erythematosus and long-term renal outcomes have been described in homogeneous Caucasian and East Asian populations with lupus nephritis but data is scarce for other ethnicities. We aimed to evaluate the incidence and risk factors for progressive chronic kidney disease (CKD) in multi-ethnic South-east Asians with lupus nephritis. METHODS: Single-center retrospective cohort study of adults with biopsy-proven lupus nephritis diagnosed between May 2001 and May 2009...
May 9, 2018: Internal Medicine Journal
https://www.readbyqxmd.com/read/29730634/changing-patterns-in-clinical-histological-presentation-and-renal-outcome-over-the-last-five-decades-in-a-cohort-of-499-patients-with-lupus-nephritis
#6
Gabriella Moroni, Paolo Gilles Vercelloni, Silvana Quaglini, Mariele Gatto, Davide Gianfreda, Lucia Sacchi, Francesca Raffiotta, Margherita Zen, Gloria Costantini, Maria Letizia Urban, Federico Pieruzzi, Piergiorgio Messa, Augusto Vaglio, Renato Alberto Sinico, Andrea Doria
OBJECTIVES: To evaluate changes in demographic, clinical and histological presentation, and prognosis of lupus nephritis (LN) over time. PATIENTS AND METHODS: We studied a multicentre cohort of 499 patients diagnosed with LN from 1970 to 2016. The 46-year follow-up was subdivided into three periods (P): P1 1970-1985, P2 1986-2001 and P3 2002-2016, and patients accordingly grouped based on the year of LN diagnosis. Predictors of patient and renal survival were investigated by univariate and multivariate proportional hazards Cox regression analyses...
May 5, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29728506/abrogation-of-lupus-nephritis-in-somatic-hypermutation-deficient-mrl-lpr-mice
#7
Fengqi Hao, Miaomiao Tian, Yunpeng Feng, Chao Quan, Yixi Chen, Shuai Chen, Min Wei
Systemic lupus erythematosus (SLE) is an autoimmune disease posing threats to multiple organs in the human body. As a typical manifestation of SLE, lupus nephritis is characterized by a series of pathological changes in glomerulus as well as accumulation of pathogenic autoreactive IgG with complement in the kidney that dramatically disrupts renal functions. Activation-induced deaminase (AID), which governs both somatic hypermutation (SHM) and class-switch recombination (CSR), has been shown to be essential for the regulation of SLE...
May 4, 2018: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/29725698/scarf1-a-multifaceted-yet-largely-understudied-scavenger-receptor
#8
REVIEW
Daniel A Patten
BACKGROUND: As is a prerequisite of belonging to the scavenger receptor super family, SCARF1 (scavenger receptor class F, member 1) is known to play a key role in the binding and endocytosis of a wide range of endogenous and exogenous ligands. FINDINGS: Unlike most scavenger receptors, SCARF1 is an essential protein, as SCARF1-deficient mice exhibit a severe resting phenotype in which they develop systemic lupus erythematosus (SLE)-like disease, thus highlighting the importance of SCARF1-mediated clearance of apoptotic host cells in homeostasis...
May 3, 2018: Inflammation Research: Official Journal of the European Histamine Research Society ... [et Al.]
https://www.readbyqxmd.com/read/29723256/renal-protective-effect-of-antiplatelet-therapy-in-antiphospholipid-antibody-positive-lupus-nephritis-patients-without-antiphospholipid-syndrome
#9
Hironari Hanaoka, Harunobu Iida, Tomofumi Kiyokawa, Yukiko Takakuwa, Takahiro Okazaki, Hidehiro Yamada, Shoichi Ozaki, Kimito Kawahata
OBJECTIVE: We sought to evaluate the effect of antiplatelet therapy in addition to conventional immunosuppressive therapy for lupus nephritis (LN) patients positive for antiphospholipid antibodies (aPL) without definite antiphospholipid syndrome (APS). METHODS: Patients with biopsy-proven LN class III or IV were retrospectively evaluated. We selected patients positive for anticardiolipin antibody (aCL) or lupus anticoagulant (LA) who did not meet the criteria for a diagnosis of APS...
2018: PloS One
https://www.readbyqxmd.com/read/29707591/miliary-tuberculosis-presenting-with-meningitis-in-a-patient-treated-with-mycophenolate-for-lupus-nephritis-challenges-in-diagnosis-and-review-of-the-literature
#10
Precious Macauley, Mark Rapp, Sarah Park, Olaoluwatomi Lamikanra, Pratibha Sharma, Michael Marcelin, Kavita Sharma
Tuberculosis is one of the top 10 causes of death worldwide according to the World Health Organization. Central nervous system involvement is usually the least common presentation of tuberculosis occurring in about 1% of all cases but yet can have very devastating outcomes. Lupus nephritis is one of the most common complications of systemic lupus erythematosus with up to two thirds of patients presenting with some degree of renal dysfunction. The mainstay of treatment is glucocorticoids; however, to sustain remission, steroid sparing agents such as cyclophosphamide, azathioprine and mycophenolate mofetil are used...
January 2018: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/29700819/circular-rna-expression-profile-and-potential-function-of-hsa_circ_0045272-in-systemic-lupus-erythematosus
#11
Lian-Ju Li, Zhi-Wei Zhu, Wei Zhao, Sha-Sha Tao, Bao-Zhu Li, Shu-Zhen Xu, Jie-Bing Wang, Ming-Yue Zhang, Jun Wu, Rui-Xue Leng, Yin-Guang Fan, Hai-Feng Pan, Dong-Qing Ye
Circular RNAs (circRNAs) represent a class of non-coding RNAs that form covalently closed RNA circles with extensive expression and conservation in mammals. Circular RNAs regulate gene expression through acting as competitive endogenous RNAs (ceRNAs) and modulating gene transcription. Accumulating evidence supports the implication of circRNAs in a variety of human diseases, but studies of circRNA role in systemic lupus erythematosus (SLE) are lacking. The present study measured the circRNA expression profiles in T cells from patients with SLE and healthy controls with human circRNA microarray and identified 127 differentially expressed circRNAs in SLE patients...
April 26, 2018: Immunology
https://www.readbyqxmd.com/read/29692991/rapid-and-complete-remission-of-class-iv-lupus-nephritis-with-massive-wire-loop-lesions
#12
Tomo Suzuki, Ryutaro Matsumura, Hiroshi Kitamura, Yugo Shibagaki
Class IV lupus nephritis (LN) often has a poorer prognosis than other classes. However, class IV LN has various phenotypes, including not only segmental and global types but also others. We present the case of a 29-year-old woman with class IV-G LN who had an early response to glucocorticoid monotherapy. In addition, multiple lung nodules such as miliary tuberculosis (TB) were detected on computed tomography. All cultures of sputum, gastric fluid, and bone marrow were negative. A kidney biopsy revealed diffuse endocapillary proliferative glomerulonephritis with marked subendothelial deposition...
January 2018: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29691398/galectin-3-deficiency-drives-lupus-like-disease-by-promoting-spontaneous-germinal-centers-formation-via-ifn-%C3%AE
#13
Cristian Gabriel Beccaria, María Carolina Amezcua Vesely, Facundo Fiocca Vernengo, Ricardo Carlos Gehrau, María Cecilia Ramello, Jimena Tosello Boari, Melisa Gorosito Serrán, Juan Mucci, Eliane Piaggio, Oscar Campetella, Eva Virginia Acosta Rodríguez, Carolina Lucía Montes, Adriana Gruppi
Germinal centers (GC) are important sites for high-affinity and long-lived antibody induction. Tight regulation of GC responses is critical for maintaining self-tolerance. Here, we show that Galectin-3 (Gal-3) is involved in GC development. Compared with WT mice, Gal-3 KO mice have more GC B cells and T follicular helper cells, increased percentages of antibody-secreting cells and higher concentrations of immunoglobulins and IFN-γ in serum, and develop a lupus-like disease. IFN-γ blockade in Gal-3 KO mice reduces spontaneous GC formation, class-switch recombination, autoantibody production and renal pathology, demonstrating that IFN-γ overproduction sustains autoimmunity...
April 24, 2018: Nature Communications
https://www.readbyqxmd.com/read/29687651/tlr9-deficiency-breaks-tolerance-to-rna-associated-antigens-and-upregulates-tlr7-protein-in-sle1-mice
#14
Teja Celhar, Hiroko Yasuga, Hui-Yin Lee, Olga Zharkova, Shubhita Tripathi, Susannah I Thornhill, Hao K Lu, Bijin Au, Lina H K Lim, Thomas P Thamboo, Shizuo Akira, Edward K Wakeland, John E Connolly, Anna-Marie Fairhurst
OBJECTIVE: Toll-like receptors (TLRs) 7 and 9 are important innate signaling molecules with opposing roles in the development and progression of Systemic Lupus Erythematosus (SLE). While multiple studies support a dependency on TLR7 for disease development, genetic ablation of TLR9 results in severe disease with glomerulonephritis (GN) by a largely unknown mechanism. The present study was designed to examine the suppressive role of TLR9 in the development of severe lupus. METHODS: We crossed Sle1 lupus-prone mice with TLR9-deficient mice to generate Sle1TLR9-/- ...
April 24, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29675623/influence-of-autoimmunity-and-inflammation-on-endothelial-function-and-thrombosis-in-systemic-lupus-erythematosus-patients
#15
Kamil Bugała, Adam Mazurek, Krzysztof Gryga, Monika Komar, Grzegorz Kopeć, Jacek Musiał, Piotr Podolec, Carlo Perricone, Wojciech Płazak
The aim of this study is to assess the relationship between autoimmunity and endothelial activation/damage (ICAM-1 and vWF serum levels) and the degree of prothrombotic activity (thrombin-antithrombin complexes-TAT serum levels) in SLE. In 60 clinically stable SLE patients, levels of the following parameters were estimated in their serum: lupus anticoagulant (LA), anticardiolipin antibodies in both IgG and IgM classes (aCL-IgG and aCL-IgM, respectively), antiβ2GPI antibodies in both IgG and IgM classes (antiβ2GPI-IgG and antiβ2GPI-IgM, respectively), ICAM, von Willebrand factor (vWF), TAT, CRP, C3c, C4, and IL-6...
April 20, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29669532/pulmonary-lymphangioleimyomatosis-and-systemic-lupus-erythematosus-in-a-menopausal-woman
#16
Hong Hong, Ruiheng Yang, Xiuzhen Li, Mengjun Wang, Zhongchao Ma
BACKGROUND: Pulmonary lymphangioleimyomatosis (PLAM) is a rare disease involving lung. PLAM primarily affects young women, a characteristic it shares with systemic lupus erythematosus (SLE). Estrogen has long been assumed to play an important role both in PLAM and SLE. We report a menopausal woman, who was found to have PLAM 1 year after she was diagnosed with SLE. Her chest radiograph was normal in the early phase of SLE. CASE PRESENTATION: A 52-year-old Chinese woman was referred to our hospital in August 2014 because of swelling in both legs...
April 18, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29665754/the-clinical-manifestations-and-response-to-treatment-in-south-africans-with-lupus-nephritis
#17
P G Mody, G M Mody, A Assounga
There are varying observations on the influence of ethnicity on the clinical spectrum and response to treatment in lupus nephritis (LN). We studied a multiethnic South African LN cohort to determine the clinical manifestations, histological involvement and response to therapy. We reviewed the records of LN patients at Inkosi Albert Luthuli Central Hospital in Durban. There were 105 patients, 92.5% females and they comprised 49.1% Indians and 45.3% African Blacks. The mean age was 31.3 ± 12.5 years, and 41...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29661905/randomized-study-of-adjunctive-belimumab-in-participants-with-generalized-myasthenia-gravis
#18
Karen Hewett, Donald B Sanders, Richard A Grove, Christine L Broderick, Todd J Rudo, Ashlyn Bassiri, Marina Zvartau-Hind, Vera Bril
OBJECTIVE: To investigate the efficacy and safety of belimumab, a fully human immunoglobulin G1λ monoclonal antibody against B-lymphocyte stimulator, in participants with generalized myasthenia gravis (MG) who remained symptomatic despite standard of care (SoC) therapy. METHODS: Eligible participants with MG were randomized 1:1 to receive IV belimumab 10 mg/kg or placebo in this phase II, placebo-controlled, multicenter, double-blind study (NCT01480596; BEL115123)...
April 17, 2018: Neurology
https://www.readbyqxmd.com/read/29644061/interstitial-inflammation-and-interstitial-fibrosis-and-tubular-atrophy-predict-renal-survival-in-lupus-nephritis
#19
Parker C Wilson, Michael Kashgarian, Gilbert Moeckel
Background: This study examines the effect of interstitial inflammation and interstitial fibrosis and tubular atrophy on renal survival in lupus nephritis. Methods: Baseline characteristics, initial ( n = 301) and repeat biopsies ( n = 94) and clinical outcomes for patients with biopsy-proven lupus nephritis from 1998 to 2014 were retrospectively collected from the medical record. Clinical and morphologic variables were evaluated using a Cox proportional hazards model and multiple imputation to address missing data...
April 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29623013/human-leukocyte-antigens-drb1-03-is-associated-with-systemic-lupus-erythematosus-and-anti-ssb-production-in-south-tunisia
#20
Hend Hachicha, Arwa Kammoun, Nadia Mahfoudh, Sameh Marzouk, Sawsan Feki, Raouia Fakhfakh, Hajer Fourati, Samy Haddouk, Faten Frikha, Lilia Gaddour, Feiza Hakim, Zouheir Bahloul, Hafedh Makni, Hatem Masmoudi
Introduction: Systemic lupus erythematosus (SLE) is an autoimmune disease with various presentations. This variation is due to the interaction of hormonal, environmental, and genetic factors. Associations between human leukocyte antigens and SLE have long been recognized in different ethnic populations and have been suggested to represent the most important association. Objectives: The objectives of this paper were to determine susceptibility and protection human leukocyte antigens (HLA) Class II markers for SLE and to highlight, for the first time, associations between HLA alleles and clinical and serological features in South Tunisia...
January 2018: International Journal of Health Sciences
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