ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1278

Growth and Puberty in Juvenile Dermatomyositis a Longitudinal Multinatinal Cohort Study

Ellen Nordal1,2, Angela Pistorio3, Marite Rygg3, Gabriella Giancane4, Michaël Hofer3, Jose Antonio Melo-Gomes4, Blanca Bica4, Ximean Norambuena4, Valda Stanevicha3, Rebecca ten Cate5, Olga Vougiouka4, Jurgen Brunner4, Guenther Dannecker4, Polyxeni Pratsidou-Gertsi4, Gabriele Simonini4, Helen Venning4, Serena Pastore4, Angelo Ravelli6, Alberto Martini7 and Nicolino Ruperto7, 1Department of Pediatrics, University Hospital of Northern Norway, Tromsø, Norway, 2Department of Clinical medicine, UIT, the Arctic University of Norway, Tromsø, Norway, 3Istituto Giannina Gaslini - Pediatria II, Reumatologia - PRINTO, Genoa, Italy, 4Istituto Giannina Gaslini - Pediatria II, Reumatologia - PRINTO, Genova, Italy, 5Pediatric Rheumatology, Leiden University Medical Center, Leiden, Netherlands, 6University of Genova, IRCCS Istituto Giannina Gaslini, Genova, Italy, 7Istituto Giannina Gaslini, Genoa, Italy

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: ,

Session Information

Date: Monday, November 6, 2017

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects Poster II: Lupus and Related Disorders, Myositis, Scleroderma and Vasculitis

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

 

Background/Purpose: Children with juvenile dermatomyositis (JDM) are at risk of growth failure and delayed puberty because of inflammatory disease activity and side effects of corticosteroid treatment. Knowledge on growth and pubertal development in JDM is very limited. Our aim was to study growth and puberty in a multicenter longitudinal prospective cohort of juvenile dermatomyositis (JDM).

Methods: Anthropometric and pubertal data in children ²18 years with recent onset or flare of JDM from 31 countries were studied. Growth failure was defined as parent-adjusted z-score < -1.5, and height deflection z-score < -0.25/year.

Results: Height and weight from four follow-up visits during two years in 196/275 (71.3 %) of the children included in the original JDM study were analyzed. There was a significant reduction in parent-adjusted height z score over time in females (p<0.0001) and males (p=0.001) with significant gender difference (p<0.05), but also catch-up growth at the final study visit were seen. Median BMI z score peaked at 6 months (p<0.0001) and was still significantly above baseline at the final study visit at a median of 26 months (p=0.007) with no gender difference. Females with a disease duration ³12 months after onset had significantly lower parent-adjusted height z score (p=0.002) with no catch-up growth. Delayed pubertal onset, pubertal duration and delayed menarche was found in a substantial number of the participants as shown in the table. Growth failure at base line was the main determinant of growth failure at the final study visit (OR: 53.4).

Conclusion: Children with JDM of long duration are at high risk of having a lower than expected height and a delayed pubertal development.

 

TABLE Growth and pubertal characteristics at the final study visit of 196 participants in a longitudinal JDM cohort

 

 

Girls (N=116)

Boys (N=80)

Growth failure

20/97 (20.6%)

11/73 (15.1%)

Height deflection

29/116 (25.0%)

25/80 (31.3%)

Age at B2 females(n=37)/ T2 males (n=23), mean (SD)

11.1 (1.9)

12.8 (1.6)

Menarche registered

31/55 (56.4%)

n.a.

Delayed menarche

7/31 (22.6%)

n.a.

Late pubertal onset

23/37 (62.2%)

15/23 (65.2%)

Delayed puberty

20/55 (36.4%)

11/27 (40.7%)

 

 

 

 

 

 

 

 

 

 

 

 

 

 

 

Numbers are frequencies (%) unless otherwise specified. Delayed menarche was defined as age of menarche >14 years,

delayed pubertal onset was defined as age at Tanner B2 >12 years or age at Tanner T2 >13 years, delayed puberty was defined

as a delay in pubertal onset, pubertal tempo or menarche.

Disclosure: E. Nordal, None; A. Pistorio, None; M. Rygg, None; G. Giancane, None; M. Hofer, Novartis and AbbVie, 5; J. A. Melo-Gomes, None; B. Bica, None; X. Norambuena, None; V. Stanevicha, None; R. ten Cate, None; O. Vougiouka, None; J. Brunner, None; G. Dannecker, None; P. Pratsidou-Gertsi, None; G. Simonini, None; H. Venning, None; S. Pastore, None; A. Ravelli, None; A. Martini, GASLINI Hospital, 3,Astellas, AstraZeneca, Bristol-Myers Squibb, Italfarmaco, and MedImmune, 8,AbbVie Inc., AstraZeneca, Bristol-Myers Squibb, Janssen Biologics B.V., Eli Lilly and Co., “Francesco Angelini”, GlaxoSmithKline, Italfarmaco, Novartis, Pfizer, Roche, Sanofi Aventis, Schwarz Biosciences GmbH, Xoma, and Wyeth Pharmaceuticals, 2; N. Ruperto, BMS, Hoffman-La Roche, Janssen, Novartis, Pfizer and Sobi, 2,Abbvie, Ablynx, AstraZeneca, Baxalta Biosimilars, Biogen Idec, Boehringer, Bristol Myers Squibb, Eli-Lilly, EMD Serono, Gilead Sciences, Janssen, Medimmune, Novartis, Pfizer, Rpharm, Roche, Sanofi., 5,Abbvie, Ablynx, AstraZeneca, Baxalta Biosimilars, Biogen Idec, Boehringer, Bristol Myers Squibb, Eli-Lilly, EMD Serono, Gilead Sciences, Janssen, Medimmune, Novartis, Pfizer, Rpharm, Roche, Sanofi., 8.

To cite this abstract in AMA style:

Nordal E, Pistorio A, Rygg M, Giancane G, Hofer M, Melo-Gomes JA, Bica B, Norambuena X, Stanevicha V, ten Cate R, Vougiouka O, Brunner J, Dannecker G, Pratsidou-Gertsi P, Simonini G, Venning H, Pastore S, Ravelli A, Martini A, Ruperto N. Growth and Puberty in Juvenile Dermatomyositis a Longitudinal Multinatinal Cohort Study [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/growth-and-puberty-in-juvenile-dermatomyositis-a-longitudinal-multinatinal-cohort-study/. Accessed .

« Back to 2017 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/growth-and-puberty-in-juvenile-dermatomyositis-a-longitudinal-multinatinal-cohort-study/