Aman M. Patel

Amar Desai

Shari R. Lipner

Vraj P. Shah

Dermatology Practical & Conceptual

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Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163 1Cardiovascular Complications are Common in Patients with Juvenile Dermatomyositis in a Cross-Sectional Analysis of the 2016 Kids Inpatient DatabaseAmar D. Desai1, Aman M. Patel1, Vraj P. Shah1, Shari R. Lipner21 Rutgers New Jersey Medical School, Newark, New Jersey, USA2 Weill Cornell Medicine, Department of Dermatology, New York, New York, USAKey words: dermatomyositis, pediatric, cardiovascular, management, outcomesCitation: Desai AD, Patel AM, Shah VP, Lipner SR. Cardiovascular Complications are Common in Patients with Juvenile Dermatomyositis in a Cross-sectional Analysis of the 2016 Kids Inpatient Database. Dermatol Pract Concept. 2023;13(3):e2023163.  DOI: https://doi.org/10.5826/dpc.1303a163Accepted: February 1, 2023; Published: July 2023Copyright: ©2023 Desai et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.Funding: None.Competing Interests: Mr. Desai, Mr. Patel, and Mr. Shah have no conflicts of interest. Dr. Lipner has served as a consultant for  Orth-Dermatologics, Verrica, and Hoth Therapeutics.Authorship: All authors have contributed significantly to this publication.Corresponding Author: Shari R. Lipner, MD PhD, Department of Dermatology, Weill Cornell Medicine, 1305 York Avenue, 9th floor,  New York, NY 10021 Phone: 646-962-3376 E-mail: shl9032@med.cornell.eduIntroductionJuvenile dermatomyositis (JDM) is an uncommon inflam-matory disease (incidence 2-4 per 1 million children) with frequent cardiovascular complications (CVCs). Incidence of CVCs in JDM varied widely from 2.9% to 37.5% in a meta-analysis of single-institution studies [1]. We aimed to assess for differences in presentation, treatment, and out-comes of JDM pediatric inpatients with CVCs.The 2016 Kids Inpatient Database (KID), an all-payer in-patient pediatric database [2], was queried for JDM pa-tients using International Classification of Diseases, Tenth Revision (ICD-10), Clinical Modification JDM code “M33.” CVCs were collected using codes “A52.0/E08–E16/E66/E78/I00–I99/Q2/Z95.” Chi-squared tests compared frequencies by CVC status. Multivariable logistic regression identified predictors of CVCs (P < 0.05).Case PresentationThere were 836 pediatric JDM cases and 222 (26.6%) had CVCs (Table 1). Patients with CVCs were older (14.0 versus 11.4 years) and more often Black (29.1% versus 21.6%), Hispanic (35.5% versus 27.7%), in the lowest income quartile (36.1% versus 29.3%), and Medicaid-insured (52.3% versus 41.9%) (all P < 0.05). Overall treatment nonadherence was more common for patients ages 14-20 (71.4% versus 44.2%), females (96.4% versus 78.1%), and Blacks (48.3% versus 22.7%) (P < 0.025) (Supplementary Table 1).2 Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163Table 1. Demographic data of dermatomyositis by cardiovascular complication status.No Cardiovascular ComplicationsCardiovascular Complications TotalPN = 613 (73.4%) N = 222 (26.6%) N = 836Age Age, years(mean [SE])11.42[0.21]14.04[0.31]12.12[0.18]< 0.001Sex Male 22.2% 19.3% 21.4%0.365Female 77.8% 80.7% 78.6%Race White 44.5% 29.1% 40.4%0.002Black 21.6% 29.1% 23.6%Hispanic 27.7% 35.5% 29.8%Other 6.1% 6.4% 6.2%Median Income Quartile - Patient Zip Code0 – 25% 29.3% 36.1% 31.1%0.03826 – 50% 25.6% 25.1% 25.5%51 – 75% 24.6% 26.5% 25.1%76 – 100% 20.5% 12.3% 18.3%Primary Payer Status Medicare 0.2% 1.4% 0.5%0.005Medicaid 41.9% 52.3% 44.7%Private Insurance 49.4% 39.6% 46.8%Self-Pay 2.8% 0.5% 2.2%No Charge 0.2% 0.0% 0.1%Other 5.5% 6.3% 5.7%Hospital Region Northeast 15.2% 8.1% 13.3%< 0.001Midwest 22.5% 15.8% 20.7%South 40.6% 40.1% 40.5%West 21.7% 36.0% 25.5%Severity of Illness Subclass(Loss of Function)Minor LOF 36.4% 10.3% 29.4%< 0.001Moderate LOF 43.6% 43.5% 43.5%Major LOF 16.0% 34.5% 20.9%Extreme LOF 4.1% 11.7% 6.1%Comorbidity Anemia 15.2% 27.5% 18.4% < 0.001Fluid & Electrolyte Disorder10.8% 23.3% 14.1%< 0.001Aphagia & Dysphagia 9.5% 6.8% 8.7% 0.222Asthma 7.7% 10.8% 8.5% 0.154Heartbeat Abnormalities at Initial Presentation5.1% 11.7% 6.8% < 0.001Coagulation Defect 4.2% 12.6% 6.5% < 0.001Gastro-esophageal Reflux Disease3.4% 11.3% 5.5% < 0.001Liver Disease 1.6% 6.8% 2.9% < 0.001Perinatal Chronic Respiratory Disease0.7% 3.2% 1.3% 0.005Treatment Nonadherence1.6% 8.1% 3.4% < 0.001LOF = loss of function; SE = standard error.Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163 3Supplementary Table 1. Demographics of dermatomyositis by treatment adherence status.Treatment AdherenceTreatment Nonadherence TotalPN = 808 (96.6%) N = 28 (3.4%) N = 836Age 0-6 years 17.9% 3.6% 17.5%0.0127-13 years 37.9% 25.0% 37.4%14-20 years 44.2% 71.4% 45.1%Sex Male 21.9% 3.6% 21.3%0.020Female 78.1% 96.4% 78.7%Race White 41.1% 20.7% 40.3%0.006Black 22.7% 48.3% 23.7%Hispanic 29.8% 31.0% 29.8%Other 6.4% 0.0% 6.2%Table 2. Management and outcomes of dermatomyositis by cardiovascular complication status.No Cardiovascular ComplicationsCardiovascular Complications Total PTotal Charges Charges ($)(mean [SE])52,432.22[3,579.96]110,743.53[15,989.20]67,956.85[5,076.36]< 0.001Length of Stay Number of Days(mean [SE])4.51[0.34]8.54[1.07]5.58[0.39]< 0.001Number of Procedures Number of Procedures(mean [SE])1.27[0.08]1.58[0.19]1.36[0.08]0.137Time Until 1st ProcedureNumber of Days(mean [SE])2.00[0.46]2.81[0.42]2.21[0.36]0.320Sepsis Complication (%) 4.7% 4.0% 4.5% 0.670Respiratory Failure Complication (%) 3.1% 5.0% 3.6% 0.202Urinary Tract Infection Complication (%) 2.4% 4.5% 3.0% 0.123Acute Kidney Injury Complication (%) 1.3% 5.8% 2.5% < 0.001Hypoxemia Complication (%) 0.5% 2.7% 1.1% 0.006Mortality Complication (%) 0.2% 0.0% 0.1% 0.547Transfusion Procedure (%) 20.2% 17.1% 19.4% 0.320Imaging Procedure (%) 10.1% 7.7% 9.5% 0.284SE = standard error.JDM patients with vs. without CVCs had higher in-cidence of acute kidney injury (AKI) (5.8% versus 1.3%, P  < 0.001) (Table 2). On multivariable analysis, CVCs were associated with increasing age (OR 1.12, 95% CI  1.07–1.16) and heartbeat abnormalities at initial presentation (OR 2.67, 95% CI 1.37–5.17) (P < 0.005) (Supplementary Table 2).ConclusionsWe found that JDM inpatients with CVCs were most often Black or Hispanic, of lower income, and Medicaid-insured. Similarly, in a national study of 16,097 pediatric inpa-tients, Blacks vs. Whites were 20% more likely to die within 30 days of surgery, which were attributed to lack specialized care access, poor physician-parent communi-cation, and systemic racism [3]. Therefore, social determi-nants likely influence the development of CVCs in JDM patients.We found that JDM patients with CVCs had greater total charges, LOS, and incidence of AKI, but no difference in the number of procedures performed, suggesting worse inpatient disease courses for JDM patients with CVCs. In a 10-year 4 Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163References1. Cantez S, Gross GJ, MacLusky I, Feldman BM. Cardiac findings in children with juvenile Dermatomyositis at disease presenta-tion. Pediatr Rheumatol Online J. 2017;15(1):54. DOI: 10.1186 /s12969-017-0182-0. PMID: 28693511. PMCID: PMC5504613.2. Bliss-Holtz J. The Kids’ Inpatient Database (KID) and data min-ing in nursing. Issues Compr Pediatr Nurs. 2012;35(1):1-3. DOI: 10.3109/01460862.2012.648829. PMID: 22250963.3. Chen C, Mpody C, Sivak E, Tobias JD, Nafiu OO. Racial dispar-ities in postoperative morbidity and mortality among high-risk pediatric surgical patients. J Clin Anesth. 2022;81:110905. DOI: 10.1016/j.jclinane.2022.110905. PMID: 35696873.4. Yen TH, Lai PC, Chen CC, Hsueh S, Huang JY. Renal involve-ment in patients with polymyositis and dermatomyositis. Int J Clin Pract. 2005;59(2):188-193. DOI: 10.1111/j.1742-1241 .2004.00248.x. PMID: 15854195.5. Hilty M, Oldrati P, Barrios L, et al. Continuous monitoring with wearables in multiple sclerosis reveals an association of cardiac autonomic dysfunction with disease severity. Mult Scler J Exp Transl Clin. 2022;8(2):20552173221103436. DOI: 10.1177/20552173221103436. PMID: 35677598. PMCID: PMC9168869.6. AAMC. Data & Reports / Diversity in Medicine: Facts and Figures 2019 / Table 12. Practice Specialty, Females by Race/Ethnicity, 2018. Available from: https://www.aamc.org/data -reports/workforce/data/table-12-practice-specialty-females -race/ethnicity-2018. Accessed 27, 2022,Supplementary Table 2. Binary logistic regression analysis of factors associated with cardiovascular complications in dermatomyositis.Odds Ratio 95% CI PaAge 1.12 1.07-1.16 < 0.001West vs. Northeast 3.80 1.82-7.91 < 0.001Fluid and Electrolyte Disorders 1.93 1.14-3.28 0.015Heartbeat Abnormalities at Initial Presentation 2.87 1.48-5.57 0.002Gastro-esophageal Reflux Disease 3.72 1.77-7.82 < 0.001Perinatal Chronic Respiratory Disease 6.17 1.60-23.73 0.008Treatment Nonadherence 5.31 1.98-14.22 < 0.001CI = confidence interval. aMultivariable analysis with age, race, income quartile, primary payer status, hospital region, anemia, fluid and electrolyte disorders, heartbeat abnormalities, coagulation defects, gastro-esophageal reflux disease, liver disease, perinatal chronic respira-tory disease, and treatment nonadherence.single institution registry study, 1992-2002, AKI incidence was 21.5% among 65 JDM patients, while overall incidence of AKI was 2.5%. Therefore, CVCs may be contributory to cost differences and LOS in JDM patients [4].CVCs were associated with tachycardia, bradycardia, and palpitations at initial presentation. Continuous monitor-ing with wearables in multiple sclerosis patients accurately showed trend-based heart rate variability and general dys-regulation [5]. Therefore, screening for heartbeat abnormal-ities might detect undiagnosed CVCs and preventing disease progression in JDM patients.Older, female, and Black children had higher incidence of treatment nonadherence, which may explain the age and CVC association with multivariable analysis. Given the predominance of United States White dermatologists [6], physician/patient race discordance may be partially respon-sible for this nonadherence.Limitations include retrospective design and lack of data regarding medications administered and procedures performed.We conclude that for JDM patients with CVCs, there are significant disparities in income, race, and insurance sta-tus. Dermatologists treating pediatric JDM patients should screen for CVCs with appropriate cardiologist referral to improve outcomes for these patients.

Cardiovascular Complications are Common in Patients with Juvenile Dermatomyositis in a Cross-Sectional Analysis of the 2016 Kids Inpatient Database

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Cardiovascular Complications are Common in Patients with Juvenile Dermatomyositis in a Cross-Sectional Analysis of the 2016 Kids Inpatient Database

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