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https://www.readbyqxmd.com/read/29345747/recurrent-iga-nephropathy-after-renal-transplantation-and-steroid-withdrawal
#1
Maria Cristina di Vico, Maria Messina, Fabrizio Fop, Antonella Barreca, Giuseppe Paolo Segoloni, Luigi Biancone
Immunoglobulin A Nephropathy (IgAN), is the most common primary glomerulonephritis; the reported recurrence rate of IgAN after renal transplantation is as high as 13-50%. The impact of immunosuppressive therapy and steroid withdrawal on the risk of recurrence of IgAN is still under debate. We performed a retrospective single center study, selecting 123 kidney transplants (rtx) in 120 patients, between January 1995 and December 2012, with IgAN on the native kidney. In 51/123 at least one post transplantation biopsy for clinical indication was performed; in 28/51 transplants IgAN recurrence (IgANr) was demonstrated...
January 18, 2018: Clinical Transplantation
https://www.readbyqxmd.com/read/29341047/german-guideline-for-idiopathic-pulmonary-fibrosis-update-on-pharmacological-therapies-2017
#2
Jürgen Behr, Andreas Günther, Francesco Bonella, Klaus Geißler, Dirk Koschel, Michael Kreuter, Antje Prasse, Nicolas Schönfeld, Helmut Sitter, Joachim Müller-Quernheim, Ulrich Costabel
Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease with a median survival of 2 - 4 years after diagnosis. Since the publication of the German IPF guideline in 2013 new treatment trials have been published, necessitating an update of the pharmacological therapy of IPF. Different from the previous guideline, the GRADE system was discarded and replaced by the Oxford evidence classification system which allows a more differentiated judgement. The following pharmacological therapies were rated not suitable for the treatment of IPF patients (recommendation A; evidence 1-b): triple therapy with prednisolone, azathioprine and acetyl-cysteine; imatinib; ambrisentan; bosentan; macitentan...
January 16, 2018: Pneumologie
https://www.readbyqxmd.com/read/29338679/therapeutic-role-of-methotrexate-in-pediatric-crohn-s-disease
#3
REVIEW
Zlatko Djurić, Ljiljana Šaranac, Ivana Budić, Voja Pavlović, Jelena Djordjević
The main role of therapy in Crohn's disease is to achieve long-term clinical remission, and to allow for normal growth and development of children. The immunomodulatory drugs used for the maintenance of remission in Crohn's disease include thiopurines (azathioprine and 6-mercaptopurine) and methotrexate. Development of hepatosplenic T-cell lymphoma in some patients with inflammatory bowel disease, treated with thiopurines independently or in combination with anti-tumor necrosis factor agents, resulted in a growing interest in the therapeutic application of methotrexate in children suffering from Crohn's disease...
January 16, 2018: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/29334924/longitudinal-observation-of-subretinal-fibrosis-in-vogt-koyanagi-harada-disease
#4
Chan Zhao, Fangtian Dong, Fei Gao, Xinshu Liu, Minghang Pei, Shanshan Jia, Meifen Zhang
BACKGROUND: Subretinal fibrosis (SRF) is a vision-threatening complication of Vogt-Koyanagi-Harada disease (VKH). It has long been recognized as a sequela of chronic inflammation. The developmental process of SRF, however, has not been described. The purpose of this study is to provide longitudinal observations of SRF in VKH. METHODS: Retrospective chart review of 10 VKH patients referred to our group between January 2008 and September 2015 at acute uveitic stage with SRF at presentation or who developed SRF during follow up...
January 15, 2018: BMC Ophthalmology
https://www.readbyqxmd.com/read/29332199/analysis-of-the-data-on-pregnancy-and-lactation-provided-by-patient-information-leaflets-of-anti-rheumatic-drugs-in-argentina
#5
Miguel Ormaza Sabando, Maira Arias Saavedra, Gabriel Sequeira, Eduardo Kerzberg
To analyse the level of consistency and updating of the information on pregnancy and lactation provided by patient information leaflets (PILs) of the antirheumatic drugs approved in Argentina. Inconsistencies between the 2016 EULAR Task Force recommendations on the use of anti-rheumatic drugs during pregnancy and lactation and the information provided by PILs of the same drugs approved in Argentina were analysed along with inconsistencies within the PILs of different registered trademarks of these drugs. Eighty-eight PILs of 32 drugs were analysed...
January 13, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29329602/abatacept-used-in-combination-with-non-methotrexate-disease-modifying-antirheumatic-drugs-a-descriptive-analysis-of-data-from-interventional-trials-and-the-real-world-setting
#6
Rieke Alten, Harald Burkhardt, Eugen Feist, Klaus Krüger, Juergen Rech, Andrea Rubbert-Roth, Reinhard E Voll, Yedid Elbez, Christiane Rauch
BACKGROUND: Methotrexate (MTX) remains the anchor drug in rheumatoid arthritis (RA) treatment, but is poorly tolerated or contraindicated in some patients. There is a wealth of data supporting the use of abatacept in combination with MTX, but data on alternative conventional synthetic disease-modifying antirheumatic drug (csDMARD) combinations with abatacept are scarce. METHODS: In this post-hoc exploratory analysis, efficacy and safety data were extracted from abatacept RA studies in which combination with csDMARDs other than MTX was permitted: three interventional trials (ATTAIN, ASSURE, and ARRIVE) and one real-world study (ACTION)...
January 2, 2018: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/29327413/itpa-activity-in-children-treated-by-azathioprine-relationship-to-the-occurrence-of-adverse-drug-reactions-and-inflammatory-response
#7
Antony Citterio-Quentin, Mustapha Moulsma, Marie-Paule Gustin, Alain Lachaux, Roselyne Boulieu
Azathioprine (AZA), a thiopurine drug, is widely used in the treatment of children with immunological disease such as inflammatory bowel disease (IBD) and autoimmune hepatitis (AIH); however, interindividual variability in the occurrence of adverse drug reaction (ADRs) and drug response is observed. This study investigated 1) the relationships between inosine triphosphate pyrophosphatase (ITPA) activity, an enzyme involved in thiopurine metabolism, and the occurrence of ADRs in children with immunological disease on AZA therapy, 2) the relationship between ITPA activity and the inflammatory activity observed in children with IBD...
January 11, 2018: Basic & Clinical Pharmacology & Toxicology
https://www.readbyqxmd.com/read/29320841/the-role-of-klotho-in-systemic-sclerosis
#8
R Talotta, S Bongiovanni, T Letizia, F Rigamonti, F Atzeni, M Benucci, T Vago, P Sarzi-Puttini
The aim was to evaluate the role of klotho in the pathogenesis of systemic sclerosis (SSc), through the measurement of its serum concentration in SSc patients compared to healthy controls, and to assess the association with cutaneous and visceral involvement. Blood samples obtained from both SSc patients and healthy controls were analysed by an ELISA assay for the detection of human klotho. SSc patients were globally evaluated for disease activity and assessed through the modified Rodnan's Skin Score, Medsger's scale, pulmonary function tests, 2D-echocardiography, nailfold capillaroscopy and laboratory tests...
December 21, 2017: Reumatismo
https://www.readbyqxmd.com/read/29319768/the-current-burden-of-cytomegalovirus-infection-in-kidney-transplant-recipients-receiving-no-pharmacological-prophylaxis
#9
Claudia Rosso Felipe, Alexandra Nicolau Ferreira, Adrieli Bessa, Tamiris Abait, Priscilla Ruppel, Mayara Ivani de Paula, Liliane Hiramoto, Laila Viana, Suelen Martins, Marina Cristelli, Wilson Aguiar, Juliana Mansur, Geovana Basso, Helio Tedesco Silva Junior, Jose Medina Pestana
Cytomegalovirus (CMV) infection in kidney transplantation has changed its clinical spectrum, mostly due to the current and more effective immunosuppression. In the absence of preventive strategies it is associated with significant morbi-mortality. OBJECTIVE: This study evaluated the incidence of CMV events and its effect on outcomes of kidney transplantation in recipients without pharmacological prophylaxis or targeted preemptive treatment. RESULTS: The study cohort comprised 802 recipients of kidney transplants between 04/30/2014 and 04/30/2015...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29316578/acute-hepatitis-as-a-prequel-to-very-severe-aplastic-anemia
#10
Christina Weiler-Normann, Johannes Hartl, Sören Weidemann, Ute-Marie von Pein, Walter Fiedler, Christoph Schramm, Florian Brinkert, Nicolaus Kröger, Maximilian Christopeit
Severe aplastic anemia is a rare and potentially life-threatening disease of the bone marrow often requiring allogeneic hematopoietic stem cell transplantation. Pathogenesis of the disease can vary and often remains enigmatic. Occasionally, severe aplastic anemia is associated with prior severe acute hepatitis. Differential diagnosis of acute non-viral hepatitis challenges the physician as pathogenesis remains unclear.We here present a case of a young patient presenting with acute hepatitis followed by severe aplastic anemia successfully treated with allogeneic hematopoietic stem cell transplantation...
January 2018: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/29316057/-a-promising-medium-term-follow-up-of-pediatric-sclerosing-cholangitis-mild-phenotype-or-early-diagnosis
#11
F Ferrari, G Ranucci, M Aloi, L Della Volpe, F Viola, E Miele, S Cucchiara, R Iorio
BACKGROUND AND AIMS: Sclerosing cholangitis (SC) is a chronic cholestatic liver disease whose diagnosis is being increasingly recognized in childhood. Long-term course and prognosis of pediatric SC is poorly described. METHODS: We reviewed data of pediatric SC patients, followed in two referral centers during a period of up to 20 years. We aimed to evaluate long-term outcomes according to SC phenotype. RESULTS: Among 45 patients (median age: 10...
January 9, 2018: Hepatology Research: the Official Journal of the Japan Society of Hepatology
https://www.readbyqxmd.com/read/29315147/nonmelanoma-skin-cancer-risk-in-patients-with-inflammatory-bowel-disease-undergoing-thiopurine-therapy-a-systematic-review-of-the-literature
#12
Joshua W Hagen, Melissa A Pugliano-Mauro
BACKGROUND: Azathioprine and 6-mercaptopurine (thiopurines) are common adjunct treatments for inflammatory bowel disease (IBD). Although thiopurine therapy in organ transplant recipients is known to increase nonmelanoma skin cancers (NMSCs), dermatologic literature yields less data regarding NMSC risk of thiopurine use in IBD. OBJECTIVE: The aim of this study was to systematically review current literature on NMSC risk in patients with IBD using thiopurine therapy...
January 8, 2018: Dermatologic Surgery: Official Publication for American Society for Dermatologic Surgery [et Al.]
https://www.readbyqxmd.com/read/29314776/chronicity-index-especially-glomerular-sclerosis-is-the-most-powerful-predictor-of-renal-response-following-immunosuppressive-treatment-in-patients-with-lupus-nephritis
#13
Dong-Jin Park, Sung-Eun Choi, Haimuzi Xu, Ji-Hyoun Kang, Kyung-Eun Lee, Ji Shin Lee, Yoo-Duk Choi, Shin-Seok Lee
AIM: Renal responses to immunosuppressive agents in patients with lupus nephritis (LN) differ depending on ethnicity, follow-up duration, disease severity and treatment. Thus, we evaluated predictors of complete remission during the first year following immunosuppressive treatment in patients with LN. METHODS: We retrospectively reviewed 79 patients who underwent kidney biopsy prior to the start of induction treatment and who were subsequently treated with immunosuppressive drugs for at least 6 months and followed-up for more than a year...
January 5, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29307132/current-epidemiology-and-clinical-characteristics-of-autoimmune-liver-diseases-in-south-korea
#14
Sook-Hyang Jeong
Autoimmune liver diseases including autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) are rare diseases. The aim of this review is to examine the epidemiology and clinical characteristics of AIH and PBC in South Korea. There were 4,085 patients registered as AIH in the Rare Intractable Disease Registry of Korea between 2009-2013, with a median age of 56 years and female-to male ratio of 6.4. The age-adjusted incidence and prevalence of AIH were 1.07/100,000/year and 4.82/100,000 persons, respectively...
January 5, 2018: Clinical and Molecular Hepatology
https://www.readbyqxmd.com/read/29305608/disease-course-and-treatment-responses-in-children-with-relapsing-myelin-oligodendrocyte-glycoprotein-antibody-associated-disease
#15
Yael Hacohen, Yu Yi Wong, Christian Lechner, Maciej Jurynczyk, Sukhvir Wright, Bahadir Konuskan, Judith Kalser, Anne Lise Poulat, Helene Maurey, Esther Ganelin-Cohen, Evangeline Wassmer, Chery Hemingway, Rob Forsyth, Eva Maria Hennes, M Isabel Leite, Olga Ciccarelli, Banu Anlar, Rogier Hintzen, Romain Marignier, Jacqueline Palace, Matthias Baumann, Kevin Rostásy, Rinze Neuteboom, Kumaran Deiva, Ming Lim
Importance: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) are consistently identified in a range of demyelinating disorders in adults and children. Current therapeutic strategies are largely center specific, and no treatments have been formally evaluated. Objective: To examine the clinical phenotypes, treatment responses, and outcomes of children with relapsing MOG-Ab-associated disease. Design, Setting, and Participants: This study prospectively collected demographic, clinical, and radiologic data from 102 patients from 8 countries of the EU Paediatric Demyelinating Disease Consortium from January 1, 2014, through December 31, 2016...
January 5, 2018: JAMA Neurology
https://www.readbyqxmd.com/read/29305138/musk-myasthenia-gravis-and-pregnancy
#16
Ernestina Santos, Antonio Braga, Denis Gabriel, Sara Duarte, Ana Martins da Silva, Ilda Matos, Marta Freijo, Joao Martins, Fernando Silveira, Goreti Nadais, Filipa Sousa, Carla Fraga, Rosa Santos Silva, Carlos Lopes, Guilherme Gonçalves, Clara Pinto, Jorge Sousa Braga, Maria Isabel Leite
Muscle specific kinase (MuSK) myasthenia gravis (MG, MuSK-MG) is a rare subgroup of MG affecting mainly women during childbearing years. We investigated the influence of pregnancy in the course of MuSK-MG and pregnancy outcomes in females with MuSK-MG. A multicentre cohort of 17 women with MuSK-MG was studied retrospectively; 13 of them with ≥1 pregnancy. MuSK-MG onset age was 35,4 years; 23,0% had other autoimmune disorder; 46,2% were treatment refractory. Thirteen women experienced 27 pregnancies, either after MG onset (group I) (n = 4; maternal age at conception = 29...
November 28, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/29298460/early-infliximab-yields-superior-long-term-effects-on-linear-growth-in-pediatric-crohn-s-disease-patients
#17
Jaeyoung Choi, Ben Kang, Min-Ji Kim, Insuk Sohn, Hae Jeong Lee, Yon Ho Choe
Background/Aims: Information regarding the efficacy of early infliximab treatment in pediatric patients with Crohn's disease (CD) is limited. We aimed to evaluate the impact of early combined immunosuppression on linear growth in pediatric patients with CD by performing step-up comparisons. Methods: This retrospective study included pediatric patients with moderate-to-severe CD, who received a combination therapy with infliximab and azathioprine for at least 3 years and sustained corticosteroid-free remission without loss of response...
January 5, 2018: Gut and Liver
https://www.readbyqxmd.com/read/29283382/thiopurine-drugs-repositioned-as-tyrosinase-inhibitors
#18
Joonhyeok Choi, You-Mie Lee, Jun-Goo Jee
Drug repositioning is the application of the existing drugs to new uses and has the potential to reduce the time and cost required for the typical drug discovery process. In this study, we repositioned thiopurine drugs used for the treatment of acute leukaemia as new tyrosinase inhibitors. Tyrosinase catalyses two successive oxidations in melanin biosynthesis: the conversions of tyrosine to dihydroxyphenylalanine (DOPA) and DOPA to dopaquinone. Continuous efforts are underway to discover small molecule inhibitors of tyrosinase for therapeutic and cosmetic purposes...
December 28, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29279846/autoantibodies-against-cyp-2c19-a-novel-serum-marker-in-pediatric-de-novo-autoimmune-hepatitis
#19
Maria Grazia Clemente, Roberto Antonucci, Claudia Mandato, Lucia Cicotto, Antonella Meloni, Bruno Gridelli, Stefano De Virgiliis, Michael P Manns, Pietro Vajro
Diagnosis of de novo autoimmune hepatitis (AIH) after orthotopic liver transplantation (OLT) is challenging especially in the absence of hyper-γ-globulinemia. Circulating autoantibodies are not sensitive nor specific in de novo AIH but when positive increase the diagnostic probability. We report the discovery of novel liver microsomal (LM) autoantibodies against CYP-2C19 in a 9-year-old boy with "de novo" AIH developed 7 years after OLT. Graft dysfunction presented with hypertransaminasemia (up to 400 IU/L), while serum γ-globulins remained within the normal range for age...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29276769/low-incidence-of-opportunistic-infections-in-lupus-patients-treated-with-cyclophosphamide-and-steroids-in-a-tertiary-care-setting
#20
Shubhasree Banerjee, Ann Biehl, Maryam Ghaderi-Yeganeh, Zerai Manna, Sarfaraz Hasni
Background: Infection is common cause of morbidity and mortality in systemic lupus erythematosus (SLE). Our objective was to determine incidence and types of infections, particularly opportunistic infections, in SLE patients receiving cyclophosphamide, and to identify contribution of variables like demographics, steroid, other immunosuppressives, white blood cell and absolute neutrophil count to infection risk. Patients and Methods: We did retrospective chart review of SLE patients in our institute over last 10 years, who received minimum six cyclophosphamide infusions...
March 2017: Medical Research Archives
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