Juvenile Systemic Sclerosis

What is Juvenile Systemic Sclerosis?

Juvenile systemic Sclerosis is a very rare disease in childhood, with an incidence of 0.05 /100000. Around 5 to 10% of all cases of Systemic Sclerosis evolve in childhood.

Currently we have only retrospective data regarding organ involvement and outcome of juvenile systemic sclerosis. This project should create a prospective cohort of new patients with juvenile systemic sclerosis, with an assessment protocol, which represents an international minimal gold standard for all participating countries in the care of these patients.This project should help to improve the care of these patients throughout the world.

If you are a patient or the parent of a patient, please ask your treating physician to participate in this project and contact us for the protocol. Thank you very much for your interest.

Member overview

Leading Investigator of the Project

International Advisory board for the project

Currently participating centers

Links to other websites

EUSTAR

The EULAR Scleroderma Trials and Research group (EUSTAR).

PRES

PRES (Paediatric Rheumatology European Society ) is a European scientific society for healthcare professionals in the field of paediatric rheumatology.

CARRA

The Childhood Arthritis and Rheumatology Research Alliance (CARRA) is a North American organization of pediatric rheumatologists who have joined together to answer critical clinical research questions. The purpose of CARRA is to create a robust multicenter organization that can support clinical trials and clinical research in childhood arthritis and pediatric rheumatology.

PRINTO

Pediatric Rheumatology INternational Trials Organisation (PRINTO).

Scleroderma Foundation

The Scleroderma Foundation is a national nonprofit health organization dedicated to a three-fold mission of Support, Education, and Research to help fight this challenging autoimmune disease.

The Juvenile Scleroderma Network (JSDN)

A support network helping children and their families with juvenile scleroderma.

Systemic Sclerosis Clinical Trial Consortium (SCTC)

An international organization dedicated to finding better treatment for scleroderma.

Deutsches Netzwerk für Systemische Sklerodermie (DNSS)

The German Network for Systemic Scleroderma is firmly convinced that cooperation between numerous clinics throughout Germany which already have special expertise in treating this disease will deliver long-term benefits to optimize diagnosis and care of these patients. One other important activity for the network is to build a national scleroderma registry with anonymized patient data.

Gesellschaft für Kinder- und Jugendrheumatologie

The Society for paediatric and adolescent rheumatology (GKJR) is a scientific society for healthcare professionals in the field of paediatric rheumatology which works in close cooperation with other national paediatric scientific associations and international paediatric rheumatology organisations. The majority of its more than 200 members are paediatricians who care for children and adolescents with rheumatic diseases.

European Society for Dermatology and Psychiatry

The primary purpose of the European Society for Dermatology and Psychiatry is to provide a forum of European physicians and psychologists working in the field of psychodermatology, psychosomatic dermatology, and dermatopsychiatry. The continuing aims of the European Society for Dermatology and Psychiatry are to exchange information and ideas, to expand the contacts of those working in the disciplines of dermatology, psychiatry, and psychology, to improve scientific research in this area, and to recruit the expertise of new members. The ultimate aim is to apply this knowledge to improve the quality of patient care.

Deutsche Dermatologische Gesellschaft

The German Dermatologic Society (DDG) is the scientific specialty society for German-speaking dermatologists.

Contact address of the coordinating study nurse

If you are interested in the study please feel free to contact the coordinating study nurse to receive the protocol and templates for the IRB approval.

Angela Wierk
Hamburger Zentrum für Kinder- und Jugendrheumatologie
Am Klinikum Eilbek
Dehnhaide 120

22081 Hamburg, Germany


a [dot] wierk [at] gmx [dot] de
sprechstunde [at] kinderrheumatologie [dot] de

Fon: +49 40 2092 3689
Fax: +49 40 2092 3693

Publications to juvenile systemic sclerosis

1. Jimenez SA, Hitraya E, Varga J. Pathogenesis of Scleroderma: Collagen. Rheum Dis Clin North Am 1996; 22, No.4: 647-674.
2. Furst DE, Clements PJ. Hypothesis for the pathogenesis of systemic sclerosis. J Rheumatol 1997; (suppl. 48): 53-57.
3. Gruschwitz M, Sepp N, Kofler H, et al. Expression of class II-MHC antigens in the dermis of patients with progressive systemic sclerosis. Immunology 1991; 182:234.
4. Kahaleh B. Immunologic aspects of scleroderma. Curr Opin Rheumatol 1993; 5:760-765.
5. Roumm A, Whiteside T, Medsger T Jr. Lymphocytes in the skin of patients with systemic sclerosis. Arthritis Rheum 1984; 27:645.
6. Reichmann L, Clark M, Waldmann H, Winter G. Reshaping human antibodies for therapy. Nature 1988; 332:323-327.
7. Evans PC, Lambert N, Maloney S et al. Long term fetal microchimerism in peripheral blood mononuclear cells subsets in healthy women and women with scleroderma. Blood 1999; 93:2033-7.
8. Nelson JL, Furst DE, Maloney S et al. Microchimerism and HLA compatible relationships of pregnancy in scleroderma. Lancet 1998; 351:559-62.
9. Foeldvari I, Zhavania M, Birdi N, et al. Favorable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology 2000; 39:556-559.
10. Kornreich HK, King KK, Bernstein BH, et al. Scleroderma in childhood. Arthritis Rheum 1977; 20, No.2: 343-350.
11. Silman AJ. Scleroderma-demographics and survival. J Rheumatol 1997; (Suppl. 48) 24:58-61.
12. Casas JA, Saway PA, Villarreal I et al. 5-Fluorouracil in the treatment of scleroderma: a randomised, double blind, placebo controlled international collaborative study. Ann Rheum Dis 1990; 49:926-928.
13. Clements PJ, Lachenbruch PA, Sterz M, et al. Cyclosporine in Systemic Sclerosis. Arthritis Rheum 1993; 36, No.1: 75-83.
14. Jiminez SA and Sigal SH. A 15-year prospective study of treatment of rapidly progressive systemic sclerosis with D-penicillamine. J Rheumatol 1991; 18:1496-1503.
15. Krasagakis K, Dippel E, Ramaker J, Owsianowski M, and Orfanos CE. Management of severe scleroderma with long-term extra corporeal photopheresis. Dermatology 1998; 196:309-315.
16. Matteson EL, Shbeeb MI, McCarthy TG, et al. Pilot Study of Antithymocyte Globulin in Systemic Sclerosis. Arthritis Rheum 1996; 39, No. 7: 1132-1137.
17. Polisson RP, Gilkeson GS, Pyun EH, et al. A multicenter trial of recombinant human interferon gamma in patients with systemic sclerosis: effects on cutaneous fibrosis and interleukin 2 receptor levels. J Rheumatol 1996; 23:654-658.
18. Pope JE, Bellamy N, Seibold JR, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum 2001; 44(6): 1351-1358.
19. Seibold JR, Korn JH, Simms R, et al. Recombinant human relaxin in the treatment of scleroderma. A randomized, double blind, placebo-controlled trial. Ann Intern Med 2000; 132 (11): 871-879.
20. Foeldvari I and Wulffraat H. Recognition and management of scleroderma in children. Paediatr Drugs 2001; 3(8): 575-583.
21. Binks M, Passweg JR, Furst D, et al. Phase I/II trial of autologous stem cell transplantation in systemic sclerosis: procedure related mortality and impact on skin disease. Ann Rheum Dis 2001; 60: 577-584.
22. Burt RK, Burns W, Hess A. Bone marrow transplantation for multiple sclerosis. BMT 1995; 16:1-6.
23. Fassas A, Anagnostopoulos A, Kazis A, et al. Peripheral blood stem cell transplantation in the treatment of progressive multiple sclerosis: first results of a pilot study. BMT 1997; 20 (8): 631-638.
24. Martini A, Maccario R, Ravelli, et al. Marked and sustained improvement two years after autologous stem cell transplantation in a girl with systemic sclerosis. Arthritis Rheum 1999; 42, No. 4: 807-811.
25. Martini A, Maccario R, Ravelli A, et al. Efficacy and safety of autologous peripheral stem cell transplantation in three children with systemic sclerosis and progressive pulmonary involvement. Arthritis Rheum 2000; 43, 9 (suppl.): S1538 (Abstract).
26. Snowden JA, Brooks PM, Biggs JC. Haemopoietic stem cell transplantation for autoimmune diseases. Br J Haematol 1997; 99:9-22.
27. Viganego F and Nash R. Bone marrow transplantation in the treatment of systemic sclerosis. Curr Rheumatol Rep 2000; 2(6): 492-500.
28. Wulffraat NM, Sanders LAM, Kuis W. Autologous hemopoietic stem cell transplantation for children with refractory autoimmune disease. Curr Rheumatol Rep 2000, 2(4): 316-323.
29. Wulffraat NM, Sanders LAM, Kamphuis SSM, et al. Prolonged remission without treatment after autologous stem cell transplantation for refractory childhood systemic lupus erythematosus. Arthritis Rheum 2001; 44, 3: 728-734.
30. Ellman, M. McDonald PA, Hayes FA. Etanercept as treatment for diffuse scleroderma: a pilot study. Arthritis Rheum 2000; 43,9 (suppl.): S1955 (Abstract).
31. Isaacs JD, Hazleman BL, Chakravarty K, et al. Monoclonal antibody therapy of diffuse cutaneous scleroderma with Campath-1H. J Rheumatol 1996; 23:1103-1106.
32. Hale G, Xia MQ, Tighe HP, Dyer MJ, Waldmann H. The CAMPATH-1 antigen (Cdw52). Tissue Antigen 1990; 35(3): 118-127.
33. AHFS Drug Information 2002, 897-899.
34. Flynn JM, Byrd JC. Campath-1H monoclonal antibody therapy. Curr Opin Oncol 2000; 12:575-581.
35. Kottaridis PD, Milligan DW, Chopra R, Chakraverty RK, et al. Invivo Campath-1H prevents graft-versus-host-disease following nonmyeloablative stem cell transplantation. Blood 2000; 96:2419-2425.
36. Weinblatt, ME, Madison PJ, Bulpitt KJ, et al. Campath-1H, a humanized monoclonal antibody, in refractory rheumatoid arthritis. Arthritis Rheum 1995; 38:1589-1594.
37. Moreau T, Coles A, Wing M, et al. CAMPATH-IH in multiple sclerosis. Multiple Sclerosis 1996; 1(6): 357-65.
38. Reiff A, Shaham B, Bernstein BH, Weinberg K, Parkman R: Campath-1H administration as immunoablative therapy for a patient with treatment refractory polymyositis. Arthritis Rheum 2002; 43, 9 (suppl.): (Abstract).
39. Rebello PR, Hale G, Friend PJ, Cobbold SP, Waldmann H. Anti-globulin responses to rat and humanized Campath-1 monoclonal antibody used to treat transplant rejection. Transplantation 1999; 68(9): 1417-20.
40. Clements PJ, Wong WK, Hurwitz EL, et al. Correlates of the disability index of the health assessment questionnaire: a measure of functional impairment in systemic sclerosis. Arthritis Rheum 1999; 42(11):2372-2380.
41. Weinberg K, Blazar B, Wagner JE, et al. Factors affecting thymic function after allogeneic hematopoietic stem cell transplantation. Blood 2001; 97:1458-1466.