Early-onset recurrent oral, genital and/or gastrointestinal ulcers are the hallmark feature of HA20. Frequency and intensity of other clinical manifestations varied highly. Treatment regimens should be based on disease severity, and cytokine inhibitors are often required to control relapses.

Objective 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.

A 10-year-old previously healthy boy presented to the emergency department with 6 days of persistent fever; 4 days of abdominal pain, emesis and diarrhea; and bilateral nonpurulent conjunctivitis and red cracked lips. Four weeks before presentation, the patient had had 3 days of headache with no respiratory symptoms, and he and his family had tested positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) on nasopharyngeal swab. On arrival at the emergency department, his blood pressure was 74/35 mm Hg and heart rate was 130 beats/min despite 60 mL/kg of fluid resuscitation. He was cool and had poor perfusion. He received inotrope support and empiric antibiotics and was transferred to the intensive care unit (ICU). Laboratory results on admission (Table 1) were clinically significant for leukocytosis, neutrophilia, lymphopenia, thrombocytopenia, elevated C-reactive protein (CRP), hyperferritinemia, hyponatremia, hypoalbuminemia, hypertriglyceridemia, acute kidney injury, transaminitis, coagulopathy, and markedly elevated troponin and N-terminal-pro-brain natriuretic peptide (NT-proBNP). Electrocardiography (ECG) showed conduction abnormality and an echocardiogram showed signs of myocarditis with no coronary artery changes or aneurysms. The patient's result for a nasopharyngeal swab for SARS-CoV-2 was negative on polymerase chain reaction (PCR) testing, but the result for serology testing was positive. We diagnosed pediatric inflammatory multisystem syndrome characterized by features of Kawasaki disease, cardiogenic shock, myocarditis, liver dysfunction, acute kidney injury and evolving macrophage activation syndrome. On admission to hospital, we provided the patient with concurrent treatment with intravenous pulse methylprednisolone (30 mg/kg/d) for 4 days, followed by a slow taper, and a dose of intravenous immunoglobulin (IVIG; 2 g/kg). Inotrope support was stopped by the fourth day after admission (DAA 4). Because of persistent fever and cytopenias, we added anakinra, a recombinant interleukin (IL)-1 receptor antagonist, at 100 mg/d (about 2.75 mg/kg/d) on DAA 7. His fever resolved on the same day. We started treatment with low-dose acetylsalicylic acid (antiplatelet dose of 3-5 mg/kg/d) when the patient's platelet count recovered by DAA 9. His renal function and troponin levels returned to normal on DAA 3 and 8, respectively. On DAA 10, clinical and biochemical

Kawasaki disease (KD) and systemic juvenile idiopathic arthritis (sJIA) are two distinct systemic inflammatory diseases of childhood. Each diagnosis is based on criteria, but numerous clinical features are overlapping. As no specific diagnostic tests are available, differentiation between both disease entities can be challenging. Here, we describe the disease course of patients with co-diagnosis of both KD and sJIA (KD/sJIA). All our KD (n = 1765) and sJIA (n = 112) cases were critically reviewed for co-diagnosis of KD/sJIA. Eight KD/sJIA cases were identified and their clinical presentation, treatment regimens, coronary artery outcome and complications are herein described. Each KD/sJIA patient fulfilled diagnostic criteria for KD and for sJIA. Ongoing fever, rash and arthritis were present in each patient. The KD/sJIA patients had recalcitrant KD requiring multiple doses of intravenous immunoglobulin and steroids. Five patients had coronary artery dilatation at KD diagnosis, which resolved in all by 6 weeks. Pericardial effusion was present in 5 patients. One KD/sJIA patient developed macrophage activation syndrome. In conclusion, a small proportion (0.5%) of our KD patients evolved into sJIA, and 7% of our sJIA population presented initially as KD. KD/sJIA patients were characterized by a recalcitrant KD course and a high prevalence of coronary artery dilatation. Patients with co-diagnoses may provide a clue to potentially shared immunopathology in KD and sJIA, leading us to posit that both entities may be part of the same clinical spectrum.