ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2393

Medication Use in the Juvenile Systemic Sclerosis Inception Cohort.  ARE There Differences in the Diffuse and Limited Subset Patients?

Ivan Foeldvari1, Jens Klotsche2, Ozgur Kasapcopur3, Amra Adrovic4, Valda Stanevicha5, Maria Teresa Terreri6, Ekaterina Alexeeva7, Maria M. Katsicas8, Rolando Cimaz9, Mikhail Kostik10, Thomas J. A. Lehman11, Walter A. Sifuentes-Giraldo12, Vanessa Smith13, Flavio Sztajnbok14, Tadey Avcin15, Maria Jose Santos16, Dana Nemkova17, Cristina Battagliotti18, Despina Eleftheriou19, Liora Harel20, Mahesh Janarthanan21, Tilmann Kallinich22, Jordi Anton23, Kirsten Minden2, Susan Mary Nielsen24, Kathryn S. Torok25, Yosef Uziel26 and Nicola Helmus1, 1Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, 2Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, 3Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, 4Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey, 5University Childrens Hospital, Riga, Latvia, 6Pediatric Rheumatology Unit, Federal University of São Paulo, São Paulo, Brazil, 7Rheumatology, Scientific Center of Children's Health, Moscow, Russia, 8Service of Immunology & Rheumatology. Hospital de Pediatrìa Prof Dr.Juan.P. Garrahan, MD, Buenos Aires, Argentina, 9Pediatrics, University of Firenze, Firenze, Italy, 10Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, 11Chief Div Ped Rheum PTD, Hospital for Special Surgery, New York, NY, 12Rheumatology, Hospital Universitario Ramón y Cajal, Madrid, Spain, 13Department of Rheumatology, Ghent University Hospital, Ghent University, Ghent, Belgium, 14Pediatric Rheumatology Division, Adolescent Health Care Unit, Universida de do Estado do Rio de Janeiro., Rio de Janeiro, Brazil, 15University Children's Hospital, Ljubljana, Slovenia, 16Rheumatology, Department of Rheumatology, Hospital Garcia de Orta, Almada, Portugal, Almada, Portugal, 17Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 18Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, 19Paediatric Rheumatology Department, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom, 20Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikvah, Israel, 21Pediatric Rheumatology, Chennai, India, 22Charite, University Medicine Berlin, Berlin, Germany, 23Unitat de Reumatologia Pediàtrica, Hospital Sant Joan de Déu, Barcelona, Spain, 24Rigshospitalet, Copenhagen, Denmark, 25Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, PA, 26Meir Medical Center, Kfar Saba, Israel

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords:

Session Information

Date: Tuesday, November 15, 2016

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects - Poster III: Systemic JIA, Autoinflammatory Syndromes, Scleroderma, Vasculitis, Miscellaneous

Session Type: ACR Poster Session C

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

Background/Purpose: Juvenile systemic sclerosis (jSSc) is an orphan autoimmune disease. Currently there is no data regarding use of medication in jSSc patients. Our project is the first project, where prospectively and with a standardized assessment data of jSSc patients are collected and the applied medication assessed.  We present the data of the medication us at the time point of entry into the cohort.

Methods: Patients with jSSc, according the ACR criteria, were recruited worldwide and were prospectively assessed, using the proposed standardized patient assessment protocol.

Results: Up till now 80 patients were enrolled. Sixty-six (82.5%) of the 80 patients were female. 58 (72.5%) had diffuse subtype (djSSc) and 22 had limited subtype (27.5% (ljSSc).  88% of the patients in the djSSc and 86% in the ljSSc patients received medication. Table 1.

Whole group Diffuse subtype Limited subtype
Number of patients 80 57  (71%) 23  (29%)
Sex female /male 66/14 => 4.7:1 47/10 => 4.7:1 20/4 => 5:1 0.924
Medication 62/71 (87%) 44/50  (88%) 18/21  (86%) 0.945
Corticosteroids 36/62 (58%) 27/44  (61%) 9/18  (50%) 0.667
Cyclophosphamide  5/62  (8%) 5/44  (11%) 0/18  (0%) 0.159
Chloroquine/Hydroxy-chlorqquine 10/62 (16%) 6/44  (14%) 4/18 (22%) 0.485
Methotrexate 35/62  (56%) 24/44  (54%) 11/18  (61%) 0.804
Mycophenolate 11/62 (18%) 8/44  (18%) 3/18  (17%) 0.905
Azathioprin 1/62   (2%) 1/44  (2%) 0/18  (0%) 0.524
Tocolizumab 1/62   (2%) 0/44   (0%) 1/18  (6%) 0.125
Rituximab 2/62  (3%) 1/44  (2%) 1/18  (6%) 0.523
Bosentan 11/62  (18%) 10/44  (23%) 1/18  (6%) 0.165
PDE5 inhibitors 4/62  (6%) 3/44   (7%) 1/18  (6%) 0.863
Prostanoids 1/62  (2%) 0/44  (0%) 1/18   (6%) 0.125

Conclusion: Interestingly the most frequently used DMARD was Methotrexate, followed by Mycophenolate. Only small number of patients received biologics.  Interestingly higher number of patients in the diffuse group received Bosentan.  Interestingly 9 patients did not received any DMARDS /biologics or medication again pulmonary hypertension.

Disclosure: I. Foeldvari, None; J. Klotsche, None; O. Kasapcopur, None; A. Adrovic, None; V. Stanevicha, Pfzer, 2,AbbVie, Roche, 5; M. T. Terreri, None; E. Alexeeva, None; M. M. Katsicas, None; R. Cimaz, None; M. Kostik, None; T. J. A. Lehman, None; W. A. Sifuentes-Giraldo, None; V. Smith, None; F. Sztajnbok, None; T. Avcin, None; M. J. Santos, None; D. Nemkova, None; C. Battagliotti, None; D. Eleftheriou, None; L. Harel, None; M. Janarthanan, None; T. Kallinich, None; J. Anton, None; K. Minden, None; S. M. Nielsen, None; K. S. Torok, None; Y. Uziel, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Stanevicha V, Terreri MT, Alexeeva E, Katsicas MM, Cimaz R, Kostik M, Lehman TJA, Sifuentes-Giraldo WA, Smith V, Sztajnbok F, Avcin T, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Janarthanan M, Kallinich T, Anton J, Minden K, Nielsen SM, Torok KS, Uziel Y, Helmus N. Medication Use in the Juvenile Systemic Sclerosis Inception Cohort.  ARE There Differences in the Diffuse and Limited Subset Patients? [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/medication-use-in-the-juvenile-systemic-sclerosis-inception-cohort-are-there-differences-in-the-diffuse-and-limited-subset-patients/. Accessed .

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