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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 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.