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Item CanVasc Consensus Recommendations for the Management of Antineutrophil Cytoplasm Antibody-associated Vasculitis: 2020 Update(The Journal of Rheumatology Publishing Co. Ltd., 2022-04) Mendel, Arielle; Ennis, Daniel; Go, Ellen; Bakowsky, Volodko; Baldwin, Corisande; Benseler, Susanne M.; Cabral, David A.; Carette, Simon; Clements-Baker, Marie; Clifford, Alison H.; Cohen Tervaert, Jan Willem; Cox, Gerard; Dehghan, Natasha; Dipchand, Christine; Dhindsa, Navjot; Famorca, Leilani; Fifi-Mah, Aurore; Garner, Stephanie; Girard, Louis-Philippe; Lessard, Clode; Liang, Patrick; Noone, Damien; Makhzoum, Jean-Paul; Milman, Nataliya; Pineau, Christian A.; Reich, Heather N.; Rhéaume, Maxime; Robinson, David B.; Rumsey, Dax G.; Towheed, Tanveer E.; Trudeau, Judith; Twilt, Marinka; Yacyshyn, Elaine; Yeung, Rae S. M.; Barra, Lillian B.; Khalidi, Nader; Pagnoux, Christian; Pediatrics, School of MedicineObjective In 2015, the Canadian Vasculitis Research Network (CanVasc) created recommendations for the management of antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) in Canada. The current update aimed to revise existing recommendations and create additional recommendations, as needed, based on a review of new available evidence. Methods A needs assessment survey of CanVasc members informed questions for an updated systematic literature review (publications spanning May 2014-September 2019) using Medline, Embase, and Cochrane. New and revised recommendations were developed and categorized according to the level of evidence and strength of each recommendation. The CanVasc working group used a two-step modified Delphi procedure to reach >80% consensus on the inclusion, wording and grading of each new and revised recommendation. Results Eleven new and 16 revised recommendations were created, and 12 original (2015) recommendations were retained. New and revised recommendations are discussed in detail within this document. Five original recommendations were removed, of which 4 were incorporated into the explanatory text. The supplementary appendix for practical use was revised to reflect the updated recommendations. Conclusion The 2020 updated recommendations provide rheumatologists, nephrologists, and other specialists caring for patients with AAV in Canada with new management guidance, based on current evidence and consensus from Canadian experts.Item Clinical Characteristics and Treatment Patterns of Children and Adults With IgA Nephropathy or IgA Vasculitis: Findings From the CureGN Study(Elsevier, 2018-08-03) Selewski, David T.; Ambruzs, Josephine M.; Appel, Gerald B.; Bomback, Andrew S.; Matar, Raed Bou; Cai, Yi; Cattran, Daniel C.; Chishti, Aftab S.; D’Agati, Vivette D.; D’Alessandri-Silva, Cynthia J.; Gbadegesin, Rasheed A.; Hogan, Jonathan J.; Iragorri, Sandra; Jennette, J. Charles; Julian, Bruce A.; Khalid, Myda; Lafayette, Richard A.; Liapis, Helen; Lugani, Francesca; Mansfield, Sarah A.; Mason, Sherene; Nachman, Patrick H.; Nast, Cynthia C.; Nester, Carla M.; Noone, Damien G.; Novak, Jan; O’Shaughnessy, Michelle M.; Reich, Heather N.; Rheault, Michelle N.; Rizk, Dana V.; Saha, Manish K.; Sanghani, Neil S.; Sperati, C. John; Sreedharan, Rajasree; Srivastava, Tarak; Swiatecka-Urban, Agnieszka; Twombley, Katherine; Vasylyeva, Tetyana L.; Weaver, Donald J.; Yin, Hong; Zee, Jarcy; Falk, Ronald J.; Gharavi, Ali G.; Gillespie, Brenda W.; Gipson, Debbie S.; Greenbaum, Larry A.; Holzman, Lawrence B.; Kretzler, Matthias; Robinson, Bruce M.; Smoyer, William E.; Flessner, Michael; Guay-Woodford, Lisa M.; Kiryluk, Krzysztof; CureGN Consortium; Pediatrics, School of MedicineIntroduction: The Cure Glomerulonephropathy Network (CureGN) is a 66-center longitudinal observational study of patients with biopsy-confirmed minimal change disease, focal segmental glomerulosclerosis, membranous nephropathy, or IgA nephropathy (IgAN), including IgA vasculitis (IgAV). This study describes the clinical characteristics and treatment patterns in the IgA cohort, including comparisons between IgAN versus IgAV and adult versus pediatric patients. Methods: Patients with a diagnostic kidney biopsy within 5 years of screening were eligible to join CureGN. This is a descriptive analysis of clinical and treatment data collected at the time of enrollment. Results: A total of 667 patients (506 IgAN, 161 IgAV) constitute the IgAN/IgAV cohort (382 adults, 285 children). At biopsy, those with IgAV were younger (13.0 years vs. 29.6 years, P < 0.001), more frequently white (89.7% vs. 78.9%, P = 0.003), had a higher estimated glomerular filtration rate (103.5 vs. 70.6 ml/min per 1.73 m2, P < 0.001), and lower serum albumin (3.4 vs. 3.8 g/dl, P < 0.001) than those with IgAN. Adult and pediatric individuals with IgAV were more likely than those with IgAN to have been treated with immunosuppressive therapy at or prior to enrollment (79.5% vs. 54.0%, P < 0.001). Conclusion: This report highlights clinical differences between IgAV and IgAN and between children and adults with these diagnoses. We identified differences in treatment with immunosuppressive therapies by disease type. This description of baseline characteristics will serve as a foundation for future CureGN studies.Item Diffuse Alveolar Hemorrhage(Springer Nature, 2019-07-24) Desai, Hem; Smith, Joshua; Williams, Mark Daren; Medicine, School of MedicineDiffuse alveolar hemorrhage[DAH] is a serious condition that can be life threatening. It can be caused by a constellation of disorders which presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Respiratory failure from DAH can be so severe that it has been called an ARDS mimic/imitator. Early recognition is crucial because prompt diagnosis and treatment are required for survival. DAH should be distinguished from other causes of pulmonary hemorrhage caused by localized pulmonary abnormalities and the bronchial circulation. Early bronchoscopy with bronchoalveolar lavage (BAL) is generally required to confirm the diagnosis of DAH and rule out infection. Progressively bloody bronchoalveolar lavage samples can distinguish DAH. Systemic vasculitis is one of the most common causes of DAH and can be pathologically defined by the presence of cellular inflammation, vessel destruction, tissue necrosis, and eventually, organ dysfunction. Corticosteroids and immunosuppressive agents remain the gold standard for the treatment. The following case illustrates a patient who was dependent on dialysis, then presented with hemoptysis. Bronchoscopy demonstrated progressively bloody bronchoalveolar lavage samples consistent with diffuse alveolar hemorrhage. Serologic testing was consistent with microscopic polyangiitis. The patient experienced a clinical remission with cyclophosphamide and corticosteroids.Item Early Enterococcus-associated acute postinfectious glomerulonephritis after kidney transplant.(Oxford University Press, 2014-08) Tandon, Teena; Mujtaba, M.; Mishler, Dennis; Phillips, Carrie; Sharfuddin, Asif; Department of Medicine, IU School of MedicinePostinfection as an etiology for glomerulonephritis (GN) is rarely described in post-transplant recipients and may be due to impaired immune response. It is also possible that such cases are not biopsied or not reported. There are rare case reports in the literature. We report here a rare first case of Enterococcus-related postinfectious GN in a transplant recipient seen in our center.Item The emerging role of cellular senescence in renal diseases(Wiley, 2020-02) Zhou, Bingru; Wan, Ying; Chen, Rong; Zhang, Chunmei; Li, Xuesen; Meng, Fanyin; Glaser, Shannon; Wu, Nan; Zhou, Tianhao; Li, Siwen; Francis, Heather; Alpini, Gianfranco; Zou, Ping; Medicine, School of MedicineCellular senescence represents the state of irreversible cell cycle arrest during cell division. Cellular senescence not only plays a role in diverse biological events such as embryogenesis, tissue regeneration and repair, ageing and tumour occurrence prevention, but it is also involved in many cardiovascular, renal and liver diseases through the senescence-associated secretory phenotype (SASP). This review summarizes the molecular mechanisms underlying cellular senescence and its possible effects on a variety of renal diseases. We will also discuss the therapeutic approaches based on the regulation of senescent and SASP blockade, which is considered as a promising strategy for the management of renal diseases.