10.5826/dpc.1303a163
text/xml
2160-9381
3076
en
Shari R. Lipner
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
outcomes
Cardiovascular Complications are Common in Patients with Juvenile Dermatomyositis in a Cross-Sectional Analysis of the 2016 Kids Inpatient Database
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2023-07-31
2022-12-13
2023-07-31
2023-08-24
e2023163
Dermatol Pract Concept
Shari R. Lipner
2023/07/31
10.5826/dpc.1303a163
e2023163
Weill Cornell Medicine, Department of Dermatology, New York, New York, USA
2160-9381
outcomes
en
e2023163
Cardiovascular Complications are Common in Patients with Juvenile Dermatomyositis in a Cross-Sectional Analysis of the 2016 Kids Inpatient Database
https://dpcj.org/index.php/dpc/article/download/3076/2222
          Cardiovascular Complications are Common
          in Patients with Juvenile Dermatomyositis
        in a Cross-Sectional Analysis of the 2016 Kids
                      Inpatient Database
                      Amar D. Desai1, Aman M. Patel1, Vraj P. Shah1, Shari R. Lipner2


1 Rutgers New Jersey Medical School, Newark, New Jersey, USA
2 Weill Cornell Medicine, Department of Dermatology, New York, New York, USA



Key words: dermatomyositis, pediatric, cardiovascular, management, outcomes
Citation: 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.1303a163
Accepted: February 1, 2023; Published: July 2023
Copyright: ©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.edu




Introduction                                                             by CVC status. Multivariable logistic regression identified
                                                                         predictors of CVCs (P < 0.05).
Juvenile dermatomyositis (JDM) is an uncommon inflam-
matory disease (incidence 2-4 per 1 million children) with
                                                                         Case Presentation
frequent cardiovascular complications (CVCs). Incidence
of CVCs in JDM varied widely from 2.9% to 37.5% in a                     There were 836 pediatric JDM cases and 222 (26.6%) had
meta-analysis of single-institution studies [1]. We aimed to             CVCs (Table 1). Patients with CVCs were older (14.0 versus
assess for differences in presentation, treatment, and out-              11.4 years) and more often Black (29.1% versus 21.6%),
comes of JDM pediatric inpatients with CVCs.                             Hispanic (35.5% versus 27.7%), in the lowest income
    The 2016 Kids Inpatient Database (KID), an all-payer                 quartile (36.1% versus 29.3%), and Medicaid-insured
in-patient pediatric database [2], was queried for JDM pa-               (52.3% versus 41.9%) (all P < 0.05). Overall treatment
tients using International Classification of Diseases, Tenth             nonadherence was more common for patients ages 14-20
Revision (ICD-10), Clinical Modification JDM code “M33.”                 (71.4% versus 44.2%), females (96.4% versus 78.1%), and
CVCs were collected using codes “A52.0/E08–E16/E66/E78/                  Blacks (48.3% versus 22.7%) (P < 0.025) (Supplementary
I00–I99/Q2/Z95.” Chi-squared tests compared frequencies                  Table 1).




Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163                                                                       1
          Table 1. Demographic data of dermatomyositis by cardiovascular complication status.
                                                            No Cardiovascular      Cardiovascular
                                                             Complications         Complications           Total
                                                             N = 613 (73.4%)      N = 222 (26.6%)        N = 836          P
 Age                             Age, years                      11.42                  14.04             12.12
                                                                                                                       < 0.001
                                 (mean [SE])                     [0.21]                 [0.31]            [0.18]
 Sex                             Male                            22.2%                  19.3%             21.4%
                                                                                                                         0.365
                                 Female                          77.8%                  80.7%             78.6%
 Race                            White                           44.5%                  29.1%             40.4%
                                 Black                           21.6%                  29.1%             23.6%
                                                                                                                         0.002
                                 Hispanic                        27.7%                  35.5%             29.8%
                                 Other                            6.1%                   6.4%              6.2%
 Median Income Quartile - 0 – 25%                                29.3%                  36.1%             31.1%
 Patient Zip Code         26 – 50%                               25.6%                  25.1%             25.5%
                                                                                                                         0.038
                                 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%
                                 Medicaid                        41.9%                  52.3%             44.7%
                                 Private Insurance               49.4%                  39.6%             46.8%
                                                                                                                         0.005
                                 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%
                                 Midwest                         22.5%                  15.8%             20.7%
                                                                                                                       < 0.001
                                 South                           40.6%                  40.1%             40.5%
                                 West                            21.7%                  36.0%             25.5%
 Severity of Illness             Minor LOF                       36.4%                  10.3%             29.4%
 Subclass                        Moderate LOF                    43.6%                  43.5%             43.5%
 (Loss of Function)                                                                                                    < 0.001
                                 Major LOF                       16.0%                  34.5%             20.9%
                                 Extreme LOF                      4.1%                  11.7%              6.1%
 Comorbidity                     Anemia                          15.2%                  27.5%             18.4%        < 0.001
                                 Fluid & Electrolyte             10.8%                  23.3%             14.1%
                                                                                                                       < 0.001
                                 Disorder
                                 Aphagia & Dysphagia              9.5%                   6.8%              8.7%          0.222
                                 Asthma                           7.7%                  10.8%              8.5%          0.154
                                 Heartbeat                        5.1%                  11.7%              6.8%        < 0.001
                                 Abnormalities at Initial
                                 Presentation
                                 Coagulation Defect               4.2%                  12.6%              6.5%        < 0.001
                                 Gastro-esophageal                3.4%                  11.3%              5.5%        < 0.001
                                 Reflux Disease
                                 Liver Disease                    1.6%                   6.8%              2.9%        < 0.001
                                 Perinatal Chronic                0.7%                   3.2%              1.3%          0.005
                                 Respiratory Disease
                                 Treatment                        1.6%                   8.1%              3.4%        < 0.001
                                 Nonadherence

LOF = loss of function; SE = standard error.




2                                                                    Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163
    JDM patients with vs. without CVCs had higher in-                 Similarly, in a national study of 16,097 pediatric inpa-
cidence of acute kidney injury (AKI) (5.8% versus 1.3%,               tients, Blacks vs. Whites were 20% more likely to die
P < 0.001) (Table 2). On multivariable analysis, CVCs were            within 30 days of surgery, which were attributed to lack
associated with increasing age (OR 1.12, 95% CI 1.07–1.16)            specialized care access, poor physician-parent communi-
and heartbeat abnormalities at initial presentation (OR 2.67,         cation, and systemic racism [3]. Therefore, social determi-
95% CI 1.37–5.17) (P < 0.005) (Supplementary Table 2).                nants likely influence the development of CVCs in JDM
                                                                      patients.

Conclusions                                                                We found that JDM patients with CVCs had greater total
                                                                      charges, LOS, and incidence of AKI, but no difference in the
We found that JDM inpatients with CVCs were most often                number of procedures performed, suggesting worse inpatient
Black or Hispanic, of lower income, and Medicaid-insured.             disease courses for JDM patients with CVCs. In a 10-year


        Supplementary Table 1. Demographics of dermatomyositis by treatment adherence status.
                                                                                   Treatment
                                           Treatment Adherence                    Nonadherence             Total
                                             N = 808 (96.6%)                      N = 28 (3.4%)          N = 836           P
 Age         0-6 years                             17.9%                               3.6%               17.5%
             7-13 years                            37.9%                              25.0%               37.4%          0.012
             14-20 years                           44.2%                              71.4%               45.1%
 Sex         Male                                  21.9%                               3.6%               21.3%
                                                                                                                         0.020
             Female                                78.1%                              96.4%               78.7%
 Race        White                                 41.1%                              20.7%               40.3%
             Black                                 22.7%                              48.3%               23.7%
                                                                                                                         0.006
             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           Cardiovascular
                                                          Complications              Complications         Total           P
 Total Charges               Charges ($)                        52,432.22              110,743.53        67,956.85     < 0.001
                             (mean [SE])                        [3,579.96]             [15,989.20]       [5,076.36]
 Length of Stay              Number of Days                        4.51                   8.54              5.58       < 0.001
                             (mean [SE])                          [0.34]                 [1.07]            [0.39]
 Number of Procedures        Number of                             1.27                   1.58              1.36         0.137
                             Procedures                           [0.08]                 [0.19]            [0.08]
                             (mean [SE])
 Time Until 1st              Number of Days                        2.00                   2.81              2.21         0.320
 Procedure                   (mean [SE])                          [0.46]                 [0.42]            [0.36]
 Sepsis                      Complication (%)                     4.7%                    4.0%             4.5%          0.670
 Respiratory Failure         Complication (%)                     3.1%                    5.0%             3.6%          0.202
 Urinary Tract Infection     Complication (%)                     2.4%                    4.5%             3.0%          0.123
 Acute Kidney Injury         Complication (%)                     1.3%                    5.8%             2.5%        < 0.001
 Hypoxemia                   Complication (%)                     0.5%                    2.7%             1.1%          0.006
 Mortality                   Complication (%)                     0.2%                    0.0%             0.1%          0.547
 Transfusion                 Procedure (%)                       20.2%                   17.1%             19.4%         0.320
 Imaging                     Procedure (%)                       10.1%                    7.7%             9.5%          0.284

SE = standard error.




Research Letter | Dermatol Pract Concept. 2023;13(3):e2023163                                                                    3
Supplementary Table 2. Binary logistic regression analysis of factors associated with cardiovascular
                              complications in dermatomyositis.
                                                                                             Odds Ratio            95% CI              Pa
 Age                                                                                              1.12            1.07-1.16        < 0.001
 West vs. Northeast                                                                               3.80            1.82-7.91        < 0.001
 Fluid and Electrolyte Disorders                                                                  1.93            1.14-3.28          0.015
 Heartbeat Abnormalities at Initial Presentation                                                  2.87            1.48-5.57          0.002
 Gastro-esophageal Reflux Disease                                                                 3.72            1.77-7.82        < 0.001
 Perinatal Chronic Respiratory Disease                                                            6.17           1.60-23.73          0.008
 Treatment Nonadherence                                                                           5.31           1.98-14.22        < 0.001

CI = 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               References
was 21.5% among 65 JDM patients, while overall incidence
of AKI was 2.5%. Therefore, CVCs may be contributory to                    1. Cantez S, Gross GJ, MacLusky I, Feldman BM. Cardiac findings
                                                                              in children with juvenile Dermatomyositis at disease presenta-
cost differences and LOS in JDM patients [4].
                                                                              tion. Pediatr Rheumatol Online J. 2017;15(1):54. DOI: 10.1186
    CVCs were associated with tachycardia, bradycardia,
                                                                              /s12969-017-0182-0. PMID: 28693511. PMCID: PMC5504613.
and palpitations at initial presentation. Continuous monitor-              2. Bliss-Holtz J. The Kids’ Inpatient Database (KID) and data min-
ing with wearables in multiple sclerosis patients accurately                  ing in nursing. Issues Compr Pediatr Nurs. 2012;35(1):1-3. DOI:
showed trend-based heart rate variability and general dys-                    10.3109/01460862.2012.648829. PMID: 22250963.
regulation [5]. Therefore, screening for heartbeat abnormal-               3. Chen C, Mpody C, Sivak E, Tobias JD, Nafiu OO. Racial dispar-
                                                                              ities in postoperative morbidity and mortality among high-risk
ities might detect undiagnosed CVCs and preventing disease
                                                                              pediatric surgical patients. J Clin Anesth. 2022;81:110905. DOI:
progression in JDM patients.
                                                                              10.1016/j.jclinane.2022.110905. PMID: 35696873.
    Older, female, and Black children had higher incidence                 4. Yen TH, Lai PC, Chen CC, Hsueh S, Huang JY. Renal involve-
of treatment nonadherence, which may explain the age and                      ment in patients with polymyositis and dermatomyositis. Int J
CVC association with multivariable analysis. Given the                        Clin Pract. 2005;59(2):188-193. DOI: 10.1111/j.1742-1241
predominance of United States White dermatologists [6],                       .2004.00248.x. PMID: 15854195.
                                                                           5. Hilty M, Oldrati P, Barrios L, et al. Continuous monitoring
physician/patient race discordance may be partially respon-
                                                                              with wearables in multiple sclerosis reveals an association of
sible for this nonadherence.
                                                                              cardiac autonomic dysfunction with disease severity. Mult
    Limitations include retrospective design and lack of                      Scler J Exp Transl Clin. 2022;8(2):20552173221103436. DOI:
data regarding medications administered and procedures                        10.1177/20552173221103436. PMID: 35677598. PMCID:
performed.                                                                    PMC9168869.
    We conclude that for JDM patients with CVCs, there                     6. AAMC. Data & Reports / Diversity in Medicine: Facts and
                                                                              Figures 2019 / Table 12. Practice Specialty, Females by Race/
are significant disparities in income, race, and insurance sta-
                                                                              Ethnicity, 2018. Available from: https://www.aamc.org/data
tus. Dermatologists treating pediatric JDM patients should
                                                                              -reports/workforce/data/table-12-practice-specialty-females
screen for CVCs with appropriate cardiologist referral to                     -race/ethnicity-2018. Accessed 27, 2022,
improve outcomes for these patients.




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