Update from the Juvenile Scleroderma Inception Cohort

Ivan Foeldvari¹, Jens Klotsche², Ozgur Kasapcopur³, Amra Adrovic⁴, Kathryn S. Torok⁵, Valda Stanevicha⁶, Flavio Sztajnbok⁷, Maria Teresa Terreri⁸, Ekaterina Alexeeva⁹, Jordi Anton¹⁰, Maria M. Katsicas¹¹, Vanessa Smith¹², Tadey Avcin¹³, Rolando Cimaz¹⁴, Mikhail Kostik¹⁵, Thomas J. A. Lehman¹⁶, Walter A. Sifuentes-Giraldo¹⁷, Simone Appenzeller¹⁸, Mahesh Janarthanan¹⁹, Dana Nemkova²⁰, Maria José Santos²¹, Cristina Battagliotti²², Lillemor Berntson²³, Blanca Bica²⁴, Juergen Brunner²⁵, Patrícia Costa Reis²⁶, Despina Eleftheriou²⁷, Liora Harel²⁸, Gerd Horneff²⁹, Tilmann Kallinich³⁰, Dragana Lazarevic³¹, Kirsten Minden³², Susan Mary Nielsen³³, Yosef Uziel³⁴ and Nicola Helmus¹, ¹Hamburg Center for Pediatric and Adolescent Rheumatology, Hamburg, Germany, ²Program Area Epidemiology, German Rheumatism Research Center, Berlin, Germany, ³Istanbul University, Cerrahpasa Medical School, Department of Pediatric Rheumatology, Istanbul, Turkey, ⁴Department of Pediatric Rheumatology, Istanbul University, Cerrahpasa Medical School, Istanbul, Turkey, ⁵Pediatric Rheumatology, Scleroderma Center of Pittsburgh, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, ⁶Pediatric cathedra, Riga Stradiņš University, Riga, Latvia, ⁷Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil, ⁸Pediatric Rheumatology Unit, Federal University of São Paulo (UNIFESP - Universidade Federal de São Paulo), São Paulo, Brazil, ⁹National Medical Research Center of Children's Health and Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russian Federation, ¹⁰Hospital Sant Joan de Deu, Barcelona, Spain, ¹¹Service of Immunology & Rheumatology., Hospital de Pediatría Prof Dr JP Garrahan, Buenos Aires, Argentina, ¹²Department of Internal Medicine, Department of Rheumatology, Ghent University Hospital, Department of Internal Medicine, Ghent University, Ghent, Belgium, Ghent, Belgium, ¹³University Children's Hospital, Ljubljana, Slovenia, ¹⁴Pediatrics, Ospedale Pediatrico Anna Meyer, Florence, Italy, ¹⁵Saint-Petersburg State Pediatric Medical University, Saint-Petersburg, Russian Federation, ¹⁶Pediatric Rheumatology, Hospital for Special Surgery Weill Cornell Medical College, New York, NY, ¹⁷Department of Rheumatology, University Hospital Ramón y Cajal, Madrid, Spain, ¹⁸Faculty of Medical Science State University of Campinas (UNICAMP), Sao Paulo, Brazil, ¹⁹Pediatric Rheumatology, Chennai, India, ²⁰Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, ²¹Reuma.pt, Almada, Portugal, Almada, Portugal, ²²Hospital de Niños Dr Orlando Alasia, Santa Fé, Argentina, ²³Dept. of Pediatrics, Uppsala University Hospital, Uppsala, Sweden, ²⁴"Chefe do Serviço de Reumatologia do Hospital Universitário Clementino Fraga Filho (HUCFF)Universidade Federal do Rio de Janeiro ", Rio de Janeiro, Brazil, ²⁵Division of Pediatric Rheumatology, Medical University Innsbruck, Innsbruck, Austria, ²⁶Pediatrics, Hospital De Santa Maria, Lisbon, Portugal, ²⁷Infection, Inflammation and Rheumatology, UCL Institute of Child Health, London, United Kingdom, ²⁸Schneider Children's Medical Center of Israel, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel, ²⁹Asklepios Kinderklinik St. Augustin GmbH, Sankt Augustin, Germany, ³⁰Charité Berlin Campus Virchow, Berlin, Germany, ³¹Dept of Pediatric Rheumatology and Immunology Clinical Center Nis, Nis, Serbia, ³²Children’s University Hospital Charite/German Rheumatism Research Centre Berlin, Berlin, Germany, ³³Rigshospitalet, Copenhagen, Denmark, ³⁴Meir Medical Center, Kfar Saba, Israel

Meeting: 2018 ACR/ARHP Annual Meeting

Keywords: Juvenile sclerosis, pediatric rheumatology and systemic sclerosis

Session Information

Date: Monday, October 22, 2018

Session Title: Pediatric Rheumatology – Clinical Poster II: Autoinflammatory Disorders, Scleroderma, and Miscellaneous

Session Type: ACR Poster Session B

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

Background/Purpose:

Juvenile systemic scleroderma (jSSc) is an orphan disease with a prevalence of 3 in 1 000 000 children(1). There is limited data published regarding the clinical presentation of jSSc. The juvenile systemic scleroderma inception cohort is a multinational cohort with a prospective standardized assessment of the patients. We present the clinical characteristics of the patients at time of the inclusion in the cohort.

Methods:

The juvenile scleroderma inception cohort is a prospective multicenter registry of patients with jSSc, who fullfill the adult classification criteria, are less than 16 years old at disease onset, and are less then 18 years of age at the time of inclusion in the cohort. We evaluated the patients characteristics at time of inclusion in the cohort.

Results:

One hundred and nine jSSc patients were enrolled and analyzed as of April 15th, 2018. The majority are female (79%) and Caucasian (90%). Diffuse cutaneous subtype was the most common (80%), with 15% having overlap features. The mean age of onset of Raynauds was 9.7 years in the diffuse subtype (djSSc) and 10.5 years in the limited subtype (ljSSc) (p=0.62). The mean age of non-Raynauds was 10.2 in the djSSc and 11.2 in the ljSSc (p=0.52). Mean disease duration at time of inclusion was 3.4 in the djSSc and 2.5 in the ljSSc group. Approximately 80% of all jSSc patients received DMARDs. ANA positivity was around 86% in both groups. Anti-Scl70 was 32% in djSSc and 39% in the ljSSc group. Anticentromere positivity was 4% in the djSSc and 11% in the ljSSc group(p=0.28). Mean Modified Rodnan skin score was 18.2 in the djSSc and 7.7 in the ljSSc (p=0.001). Gottron papulae were significantly more common the djSSc with 27% compared to 7% in the ljSSc group. History of ulceration was significantly more common in the djSSc with 57% compared to 29% in the ljSSc group (p=0.010). FVC<80% occured in 34% in the djSSc and 21% in the ljSSc group(p=0.295). DLCO <80% occured 44% in the djSSc and 43% in the ljSSc group. Pulmonary hypertension assessed by ultrasound occured 7% in both groups. No hypertension or renal crisis was reported or observed. Gastointestinal involvement occured 35% in the djSSc and 24% in the ljSSc (p=0.28). Number of swollen joints were observed around 20% in both groups. Muscle weakness wtih joint contractures were present in 12% in the djSSc and 28% in the ljSSc group. Tendon friction rub was present in 4 % in djSSc and 7% in the ljSSc group. djSSc patients had significantly worse scores for physician global disease activity (VAS 0-100), 41.6 compared to 30.7 ( p=0.041) and for physician global disease damage (VAS 0-100) 38.2 compared to 18.9 ( p=0.023).

Conclusion:

In this large cohort of jSSc patients there are surprisingly not many significant differences between djSSc and ljSSc. According to the physician global, the djSSc patients have significantly more severe disease.

Beukelman T, Xie F, Foeldvari I. Assessing the Prevalence of Juvenile Systemic Sclerosis in Childhood Using Administrative Claims Data from the United States. Journal fo Scleroderma and Related Disorders. 2018;3.

Disclosure: I. Foeldvari, Novartis, BMF, Bayer, Genentech, Sanofi, Abbvie, Chugai; Medac, BMS, Pfizer, 5, 8; J. Klotsche, None; O. Kasapcopur, None; A. Adrovic, None; K. S. Torok, None; V. Stanevicha, None; F. Sztajnbok, None; M. T. Terreri, None; E. Alexeeva, Roche, Abbott, Pfizer, Bristol-Myers Squibb, Centocor, Novartis, 2; J. Anton, None; M. M. Katsicas, None; V. Smith, None; T. Avcin, None; R. Cimaz, None; M. Kostik, None; T. J. A. Lehman, None; W. A. Sifuentes-Giraldo, None; S. Appenzeller, None; M. Janarthanan, None; D. Nemkova, None; M. J. Santos, None; C. Battagliotti, None; L. Berntson, None; B. Bica, None; J. Brunner, None; P. Costa Reis, None; D. Eleftheriou, None; L. Harel, None; G. Horneff, None; T. Kallinich, Novartis, 8; D. Lazarevic, None; K. Minden, Pfizer, Abbvie, Rche, Sanofi, Medac, MedCon, 2, 5; S. M. Nielsen, None; Y. Uziel, None; N. Helmus, None.

To cite this abstract in AMA style:

Foeldvari I, Klotsche J, Kasapcopur O, Adrovic A, Torok KS, Stanevicha V, Sztajnbok F, Terreri MT, Alexeeva E, Anton J, Katsicas MM, Smith V, Avcin T, Cimaz R, Kostik M, Lehman TJA, Sifuentes-Giraldo WA, Appenzeller S, Janarthanan M, Nemkova D, Santos MJ, Battagliotti C, Berntson L, Bica B, Brunner J, Costa Reis P, Eleftheriou D, Harel L, Horneff G, Kallinich T, Lazarevic D, Minden K, Nielsen SM, Uziel Y, Helmus N. Update from the Juvenile Scleroderma Inception Cohort [abstract]. Arthritis Rheumatol. 2018; 70 (suppl 9). https://acrabstracts.org/abstract/update-from-the-juvenile-scleroderma-inception-cohort/. Accessed March 21, 2023.

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