Are Patients with Overlap Features Different from Patients without? Results from the Juvenile Systemic Scleroderma Cohort Www.Juvenile-Scleroderma.Com

Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Valda Stanevicha⁵, Maria Teresa Terreri⁶, Ekaterina Alexeeva⁷, Maria M. Katsicas⁸, Vanessa Smith⁹, Rolando Cimaz¹⁰, Mikhail Kostik¹¹, Thomas J. A. Lehman¹², Jordi Anton¹³, Walter A. Sifuentes-Giraldo¹⁴, Flavio Sztajnbok¹⁵, Tadey Avcin¹⁶, Mahesh Janarthanan¹⁷, Maria José Santos¹⁸, Dana Nemkova¹⁹, Cristina Battagliotti²⁰, Despina Eleftheriou²¹, Liora Harel²², Tilmann Kallinich²³, K Minden²⁴, Susan Mary Nielsen²⁵, Kathryn S. Torok²⁶, Yosef Uziel²⁷, Anne Stevens²⁸, Clarissa Pilkington²⁹ and Nicola Helmus¹, ¹Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, ²Epidemiology unit, German Rheumatism Research Center, Berlin, Germany, ³Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, ⁴Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey, ⁵Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, ⁶Pediatric Rheumatology Unit, Federal University of São Paulo (UNIFESP - Universidade Federal de São Paulo), São Paulo, Brazil, ⁷Children's Health of RAMS and IM Sechenov First Moscow State Medical University, Moscow, Russian Federation, ⁸Service of Immunology & Rheumatology., Hospital de Pediatría Prof Dr JP Garrahan, Buenos Aires, Argentina, ⁹Faculty of Internal Medicine, Ghent University, Ghent, Belgium, ¹⁰Pediatrics, Ospedale Pediatrico Anna Meyer, Florence, Italy, ¹¹Hospital Pediatrics, State Pediatric Medical University, Saint-Petersburg, Russia, ¹²Pediatric Rheumatology, Hospital for Special Surgery Weill Cornell Medical College, New York, NY, ¹³pediatric Rheumatology, University Childrenxs Hospital, Barcelona, Spain, ¹⁴Rheumatology, Hospital Universitario Ramón y Cajal, Madrid, Spain, ¹⁵Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁶University Children's Hospital, Ljubljana, Slovenia, ¹⁷Pediatric Rheumatology, Chennai, India, ¹⁸Reuma.pt, Almada, Portugal, Almada, Portugal, ¹⁹Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ²⁰Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, ²¹Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, ²²Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel, ²³Charité, Humbolt University Medicine Berlin, Berlin, Germany, ²⁴Charité – University of Medicine Berlin, Berlin, Germany, ²⁵Rigshospitalet, Copenhagen, Denmark, ²⁶Pediatric Rheumatology, University of Pittsburgh Med Ctr, Pittsburgh, PA, ²⁷Meir Medical Center, Kfar Saba, Israel, ²⁸University of Washington, Department of Pediatrics, Seattle, WA, ²⁹Paediatric Rheumatology, Great Ormond Street Hospital for Children NHS Trust, London, United Kingdom

Meeting: 2017 ACR/ARHP Annual Meeting

Date of first publication: September 18, 2017

Keywords: Juvenile sclerosis and pediatric rheumatology

Session Information

Date: Wednesday, November 8, 2017

Title: Pediatric Rheumatology – Clinical and Therapeutic Aspects III: Lupus, Dermatomyositis, and Scleroderma

Session Type: ACR Concurrent Abstract Session

Session Time: 11:00AM-12:30PM

Background/Purpose:

Juvenile systemic sclerosis (jSSc) patients with overlap features seems to have a better long term outcome¹. There is currently no data, where the clinical characteristics of patients with and without overlap features were compared.

Methods:

Patients, fulfilling the PRES jSSc-classification criteria were included. Organ involvement was assessed using the standardized assessment. Patients were classified to diffuse (dcjSSc) and limited (lcjSSc) subtype and grouped as patients with and without overlap features.

Results:

Till April 2016 eighty patients were enrolled. We present data at time point of the enrollment. 11 of the patients had overlap features (oJSSc). 6 of 58 (10%) in the dcjSSc and 5 of 22 (23%) in the lcjSSc. Most patients were Caucasian. Female/male ratio differed in the groups, in the nojSSc 3.9:1 and in the ojSSc 11:1 ( p=0.1). Mean disease duration was 3.6 years in the non-overlap features group (nojSSc) and 3.2 years in the ojSSc group. Patients with nojSSc were 35% Scl-70 positive and ojSSc group none (p=0.032). Decreased DLCO occurred in 48% in the nojSSc and in 100% in the ojSSc group (p=0.088). Renal involvement was more frequent in the ojSSc group with 18% compared to 4% the nojSSc (p=0.078). Swollen joints occurred in 63% in the ojSSc and in 29% in the nojSSc group (p<0.001). Muscle weakness combined with joint contractures occurred in 43% in the ojSSc and in 8% in the nojSSc group (p=0.011).

Conclusion:

Patients with overlap features have distinct characteristics compared with patients with nojSSc. This charactheristics were no Scl-70 positivity, higher portion of interstial lung disease, renal involvement, arthritis and muscle involvement accompanied by joint contractures. The future larger patient data collection in our cohort will help to make the differences more accentuated.

Literature

1. Foeldvari I, Nihtyanova SI, Wierk A, Denton CP. Characteristics of Patients with Juvenile Onset Systemic Sclerosis in an Adult Single-center Cohort. J Rheumatol 2010;37:2422- 6.

Disclosure: I. Foeldvari, None; J. Klotsche, None; O. Kasapcopur, None; A. Adrovic, None; V. Stanevicha, None; M. T. Terreri, None; E. Alexeeva, None; M. M. Katsicas, None; V. Smith, None; R. Cimaz, None; M. Kostik, None; T. J. A. Lehman, None; J. Anton, None; W. A. Sifuentes-Giraldo, None; F. Sztajnbok, None; T. Avcin, None; M. Janarthanan, None; M. J. Santos, None; D. Nemkova, None; C. Battagliotti, None; D. Eleftheriou, None; L. Harel, None; T. Kallinich, None; K. Minden, None; S. M. Nielsen, None; K. S. Torok, None; Y. Uziel, None; A. Stevens, None; C. Pilkington, 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, Smith V, Cimaz R, Kostik M, Lehman TJA, Anton J, Sifuentes-Giraldo WA, Sztajnbok F, Avcin T, Janarthanan M, Santos MJ, Nemkova D, Battagliotti C, Eleftheriou D, Harel L, Kallinich T, Minden K, Nielsen SM, Torok KS, Uziel Y, Stevens A, Pilkington C, Helmus N. Are Patients with Overlap Features Different from Patients without? Results from the Juvenile Systemic Scleroderma Cohort Www.Juvenile-Scleroderma.Com [abstract]. Arthritis Rheumatol. 2017; 69 (suppl 10). https://acrabstracts.org/abstract/are-patients-with-overlap-features-different-from-patients-without-results-from-the-juvenile-systemic-scleroderma-cohort-www-juvenile-scleroderma-com/. Accessed .

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