Kawasaki Disease Reconsidered New AHA Guidelines
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1 Kawasaki Disease Reconsidered New AHA Guidelines John Darby, MD Nisha Tamaskar, MD Stanford Shulman, MD Marietta DeGuzman, MD Kristin Sexson, MD, PhD Introductions John Darby, MD Texas Children s Hospital, Baylor College of Medicine Nisha Tamaskar, MD Texas Children s Hospital, Baylor College of Medicine Children s National Health System Page 1 xxx00.#####.ppt 7/19/2017 8:35:45 PM
2 Marietta DeGuzman, MD Texas Children s Hospital, Baylor College of Medicine Kristen Sexson, MD, PhD Texas Children s Hospital, Baylor College of Medicine Stanford Shulman, MD Ann & Robert H. Lurie Children s Hospital of Chicago, Northwestern McGaw School of Medicine Page 2 xxx00.#####.ppt 7/19/2017 8:35:46 PM Disclosures None of the presenters have any relevant financial relations to disclose We do not intend to discuss an unapproved or investigative use of a commercial product or device in our presentation Page 3 xxx00.#####.ppt 7/19/2017 8:35:46 PM
3 Objectives At the conclusion of this activity, participants will be able to: Compare Kawasaki Disease practice patterns and evaluate recently published clinical guidelines Recognize features of typical and atypical presentations of Kawasaki Disease Identify potential complications of Kawasaki Disease and of its treatment and prepare management plans Page 4 xxx00.#####.ppt 7/19/2017 8:35:47 PM Presentation Outline Introduction to new guidelines (10 minutes) Etiology and pathogenesis (10 minutes) Diagnosis (20 minutes) Management (30 minutes) Wrap up (5 minutes) Page 5 xxx00.#####.ppt 7/19/2017 8:35:47 PM
4 Survey Survey questions via audience response using the PHM App 1) Search for the session title in the mobile app using the search bar or in the agenda layout 2) Select the session to open the session page and select Live Polls 3) Select your desired answer and then select Finish to submit Page 6 xxx00.#####.ppt 7/19/2017 8:35:47 PM Survey Survey is part of a research project approved by Baylor College of Medicine IRB Aim: Evaluate hospitalist knowledge, practice patterns, and comfort level in caring for children with Kawasaki Disease Survey questions are completely optional and anonymous Participation in the survey implies consent to participate May opt out at any time Page 7 xxx00.#####.ppt 7/19/2017 8:35:48 PM
5 Audience Response Question 1 Which of the following best describes you? a) Hospitalist with 0 4 years of practice b) Hospitalist with 5 10 years of practice c) Hospitalist with > 10 years of practice d) Hospitalist fellow e) Other trainee (medical student, resident) f) Other (including outpatient pediatrics, subspecialty, advanced practice providers, etc.) Page 8 xxx00.#####.ppt 7/19/2017 8:35:48 PM Audience Response Question 2 Which of the following best describes your primary practice location? a) Outside the USA b) Northeast c) Mid-Atlantic / Southeast d) Midwest e) South / Southwest f) West / Pacific Northwest Page 9 xxx00.#####.ppt 7/19/2017 8:35:48 PM
6 Audience Response Question 3 Which of the following best describes your primary practice setting? a) Urgent Care b) Community hospital c) Community hospital with academic affiliation d) Tertiary care children s hospital e) Outpatient clinic f) Other Page 10 xxx00.#####.ppt 7/19/2017 8:35:48 PM Introduction to New Guidelines Page 11 xxx00.#####.ppt 7/19/2017 8:35:49 PM
7 Introduction to New Guidelines Dajani, et al. published first AHA Guideline in Pages Page 12 xxx00.#####.ppt 7/19/2017 8:35:49 PM Introduction to New Guidelines Newburger et al. published revision to AHA guideline in Pages Page 13 xxx00.#####.ppt 7/19/2017 8:35:49 PM
8 Introduction to New Guidelines McCrindle et al. published revision to AHA guideline in Pages Page 14 xxx00.#####.ppt 7/19/2017 8:35:49 PM Introduction to New Guidelines Guideline page count Page 15 xxx00.#####.ppt 7/19/2017 8:35:50 PM
9 Guideline Process Diverse group of content experts Representatives from North America, Taiwan, and Japan Careful review of the 2004 guidelines Background sections drafted to provide content for guidelines All recommendation statements reviewed by writing group and then submitted for peer review Page 16 xxx00.#####.ppt 7/19/2017 8:35:50 PM 2017 Stated Updates New evidence regarding underlying pathology An algorithm to ensure capture of incomplete KD during effective window of therapy Improved management of the acute illness that includes the use of additional therapies for IVIG refractory patients Page 17 xxx00.#####.ppt 7/19/2017 8:35:50 PM
10 2017 Stated Updates Greater use of Z-scores for classifying coronary artery involvement Greater specification of long-term management based on both initial and current coronary artery involvement Acknowledgement of care needs of growing population of adults with a previous history of KD and coronary artery aneurysms Page 18 xxx00.#####.ppt 7/19/2017 8:35:50 PM Summary Still do not know the cause of KD KD in infants can be very challenging Steroids likely have a role in the treatment of patients at high risk for IVIG resistance if given early Aspirin is likely safe to give at moderate dose and maybe even low dose Page 19 xxx00.#####.ppt 7/19/2017 8:35:51 PM
11 Summary A second dose of IVIG is our recommended therapy for refractory KD For patients with coronary artery abnormalities (CAA) we recommend early cardiology involvement Page 20 xxx00.#####.ppt 7/19/2017 8:35:51 PM Section 1: Pathophysiology Page 21 xxx00.#####.ppt 7/19/2017 8:35:51 PM
12 Section 1: Pathophysiology Moderator and audience questions (10 minutes) Page 22 xxx00.#####.ppt 7/19/2017 8:35:52 PM Section 1: Pathophysiology Moderator and audience questions (10 minutes) Page 23 xxx00.#####.ppt 7/19/2017 8:35:52 PM
13 Section 2: Diagnosis Page 24 xxx00.#####.ppt 7/19/2017 8:35:52 PM Audience Response Question 4 What is your comfort level in independently (without a consulting service) diagnosing incomplete Kawasaki Disease? a) Very comfortable b) Somewhat comfortable c) Somewhat uncomfortable d) Very uncomfortable Page 25 xxx00.#####.ppt 7/19/2017 8:35:52 PM
14 Audience Response Question 5 What is your comfort level in independently managing classic Kawasaki Disease? a) Very comfortable b) Somewhat comfortable c) Somewhat uncomfortable d) Very uncomfortable Page 26 xxx00.#####.ppt 7/19/2017 8:35:53 PM Audience Response Question 6 What is your comfort level in independently managing KD complicated by mild coronary artery dilation (no aneurysms) diagnosed prior to day 10 of fever? a) Very comfortable b) Somewhat comfortable c) Somewhat uncomfortable d) Very uncomfortable Page 27 xxx00.#####.ppt 7/19/2017 8:35:53 PM
15 Audience Response Question 7 Is any subspecialty/department typically consulted when a patient is diagnosed with classic KD without complications? a) No b) Yes, ID c) Yes, Cardiology d) Yes, Rheumatology e) Yes, Cardiology and ID f) Yes, Cardiology and Rheumatology g) Yes, other Page 28 xxx00.#####.ppt 7/19/2017 8:35:53 PM Audience Response Question 8 In addition to the PCP, who follows patients with classic KD without complications after discharge? a) None (PCP only) b) Cardiology c) ID d) Rheumatology e) ID and Cardiology f) Cardiology and Rheumatology g) Other Page 29 xxx00.#####.ppt 7/19/2017 8:35:54 PM
16 Diagnosis Moderator and audience questions (20 minutes) Page 30 xxx00.#####.ppt 7/19/2017 8:35:54 PM Diagnosis Page 31 xxx00.#####.ppt 7/19/2017 8:35:54 PM
17 Page 32 xxx00.#####.ppt 7/19/2017 8:35:54 PM Page 33 xxx00.#####.ppt 7/19/2017 8:35:55 PM
18 Is 2.5 the magic number? Bratincsak, et al. Pediatr Infect Dis J. 2012;31: Page 34 xxx00.#####.ppt 7/19/2017 8:35:55 PM Section 3: Management Page 35 xxx00.#####.ppt 7/19/2017 8:35:55 PM
19 Audience Response Question 9 In a patient with classic KD and a normal initial echocardiogram, what is your initial treatment? a) IVIG and high dose aspirin ( mg/kg/day) b) IVIG and medium dose aspirin (30 50 mg/kg/day) c) IVIG and low dose aspirin (3 5 mg/kg/day) d) IVIG alone e) IVIG and corticosteroids (with or without aspirin) f) Corticosteroids alone g) Other Page 36 xxx00.#####.ppt 7/19/2017 8:35:56 PM Audience Response Question 10 In a patient with classic KD and no complications who receives IVIG, how long do you typically observe following completion of IVIG prior to discharge? a) <24 hours b) About 24 hours c) About 36 hours d) About 48 hours e) >48 hours Page 37 xxx00.#####.ppt 7/19/2017 8:35:56 PM
20 Audience Response Question 11 In a patient with classic KD and no complications who received IVIG, which of the following most closely approximates the number of hours after the completion of IVIG when you would consider fever (>100.4 F) to indicate non-response to IVIG? a) 18 hours b) 24 hours c) 36 hours d) 48 hours e) Other Page 38 xxx00.#####.ppt 7/19/2017 8:35:56 PM Audience Response Question 12 You have a patient with classic KD who is found to have coronary artery dilation (but no aneurysm formation) on initial echocardiogram. Aside from giving aspirin, what is your most typical initial step? a) IVIG alone b) IVIG and corticosteroids c) IVIG and TNFα inhibitor (infliximab or etanercept) d) Corticosteroids alone e) TNFα inhibitor alone f) Other Page 39 xxx00.#####.ppt 7/19/2017 8:35:57 PM
21 Audience Response Question 13 You are caring for a patient with classic KD and no coronary artery changes who has recurrence of fever. You consider this recurrence to indicate a failed to response to an initial dose of IVIG. Which of the following is your most typical next step? a) Observe b) Second dose of IVIG c) IVIG and corticosteroids d) Corticosteroids alone e) TNFα inhibitor alone (infliximab or etanercept) f) IVIG and TNFα inhibitor e) Other Page 40 xxx00.#####.ppt 7/19/2017 8:35:57 PM Section 3: Management Moderator and audience questions (30 minutes) Page 41 xxx00.#####.ppt 7/19/2017 8:35:57 PM
22 Steroids: RAISE study (2012) Prospective, randomized, open-label, blinded endpoint trial 74 hospitals in Japan Patients selected for severe presentation IVIG and aspirin OR IVIG and aspirin and prednisolone 2 mg/kg over 15 days Page 42 xxx00.#####.ppt 7/19/2017 8:35:58 PM Steroids RAISE study Incidence of CAA significantly lower in steroid group 3% in steroid group vs 23% in control group Serious adverse events were similar Page 43 xxx00.#####.ppt 7/19/2017 8:35:58 PM
23 Steroids Chen et al. JAMA (2016) Relative Risk Reduction of 58% in CAA No benefit as rescue therapy High risk patients received greatest risk reduction No difference in adverse events Page 44 xxx00.#####.ppt 7/19/2017 8:35:58 PM Steroids Cochrane Systematic Review Wardle et al. January, studies (6 out of 7 were in JAMA Peds meta-analysis) Moderate quality evidence shows that the use of steroids in the acute phase of KD can be associated with improved CAA, shorter duration of stay and decreased duration of symptoms. Page 45 xxx00.#####.ppt 7/19/2017 8:35:58 PM
24 Steroids Cochrane Systematic Review Note that the most benefit may be in: Japanese populations Higher risk score patients Those receiving a longer course of steroids Acknowledge that there may be a confounding bias Treatment with a long course of steroids should be considered in all children diagnosed with KD until further studies are performed. Page 46 xxx00.#####.ppt 7/19/2017 8:35:59 PM Aspirin Aspirin does not appear to lower the frequency of development of coronary abnormalities. Japan and Europe use mg/kg/day US uses mg/kg/day There are no data to suggest that either does of ASA is superior. Page 47 xxx00.#####.ppt 7/19/2017 8:35:59 PM
25 Aspirin Cochrane review (2006) Until good quality RCTs are conducted, there is insufficient evidence to indicate whether children with KD should receive ASA. Page 48 xxx00.#####.ppt 7/19/2017 8:36:00 PM Aspirin June 2017 Multicenter, retrospective, nonrandomized cohort study in Canada 1213 children ages 0 to 10 with acute KD Low dose ASA was not inferior to high dose for reducing CAA Rate of fever recurrence and fever duration not significantly different Page 49 xxx00.#####.ppt 7/19/2017 8:36:00 PM
26 Other therapies Page 50 xxx00.#####.ppt 7/19/2017 8:36:01 PM Section 3: Management Moderator and audience questions (30 minutes) Page 51 xxx00.#####.ppt 7/19/2017 8:36:01 PM
27 Summary Still do not know the cause of KD KD in infants can be very challenging Steroids likely have a role in the treatment of patients at high risk for IVIG resistance if given early. Aspirin is likely safe to give at moderate dose and maybe even low dose Page 52 xxx00.#####.ppt 7/19/2017 8:36:01 PM Summary A second dose of IVIG is our recommended therapy for refractory KD For patients with coronary artery abnormalities (CAA) we recommend early cardiology involvement. Page 53 xxx00.#####.ppt 7/19/2017 8:36:01 PM
28 Page 54 xxx00.#####.ppt 7/19/2017 8:36:02 PM
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