Juvenile systemic scleroderma
- Alberto Martini MD
- … show all 1 hide
Rent the article at a discountRent now
* Final gross prices may vary according to local VAT.Get Access
Systemic scleroderma in children is very rare and is considered similar to adult-onset disease. In adults, new etiopathogenetic and therapeutic approaches have emerged in recent years. For instance, it has been shown that microchimerism could play a role in disease pathogenesis and that immunoablation followed by stem cell rescue could be of potential therapeutic benefit. There is also evidence that these new approaches can be of value for childhood-onset disease.
- Cassidy JT, Petty RE: Textbook of Pediatric Rheumatology. Philadelphia: WB Saunders; 1995.
- Tuffanelli DL, Winkelmann RK: Systemic scleroderma. Arch Dermatol 1961, 84:359–371.
- Uziel Y, Miller ML, Laxer RM: Scleroderma in children. Pediatr Clin North Am 1995, 42:1171–1203.
- Black CM: Scleroderma in children. Adv Exp Med Biol 1999, 455:35–48.
- Bell SA, Faust H, Mittermuller J, et al.: Specificity of antinuclear antibodies in scleroderma-like chronic graft-versus-host disease: clinical correlation and histocompatibility locus antigen association. Br J Dermatol 1996, 134:848–854. CrossRef
- Lo YMD, Lo ESF, Watson N, et al.: Two-way cell traffic between mother and fetus: biological and clinical implications. Blood 1996, 88:4390–4395.
- Bianchi DW, Zickwolf GK, Weil GJ, et al.: Male fetal progenitor cells persist in maternal blood for as long as 27 years postpartum. Proc Natl Acad Sci USA 1996, 93:705–708. CrossRef
- Nelson JL, Furst DE, Maloney S, et al.: Microchimerism and HLA-compatible relationships of pregnancy in scleroderma. Lancet 1998, 351:559–562. CrossRef
- Artlett CM, Smith BJ, Jimenez S: Identification of fetal DNA and cells in skin lesions from women with systemic sclerosis. N Engl J Med 1998, 338:1186–1191. CrossRef
- Artlett CM, Welsh KI, Black CM, et al.: Fetal-maternal HLA compatibility confers susceptibility to systemic sclerosis. Immunogenetics 1997, 47:17–22. CrossRef
- Evans PC, Lambert N, Maloney S, et al.: Long-term fetal microchimerism in peripheral blood mononuclear cell subsets in healthy women and women with scleroderma. Blood 1999, 93:2033–2037. A study showing persistent fetal microchimerism in peripheral blood mononuclear cell subsets in healthy women and women with systemic scleroderma.
- Lambert NC, Evans PC, Hashizumi TL, et al.: Persistent fetal microchimerism in T lymphocytes is associated with HLADQA1* 0501: implications in autoimmunity. J Immunol 2000, 164:5545–5548. This study suggests an additional route by which HLA genes may contribute to susceptibility to autoimmune diseases.
- Pollack MS, Kirpatrick D, Kapoor D, et al.: Identification by HLA typing of intrauterine derived maternal T cells in four patients with severe combined immunodeficiency. N Engl J Med 1982, 307:662–666. CrossRef
- Maloney S, Smith A, Furst D, et al.: Microchimerism of maternal origin persists into adult life. J Clin Invest 1999, 104:41–47. This study indicates that maternal cells can persist in immunocompetent offspring well into adult life. CrossRef
- Artlett CM, Cox LA, Jimenez SA: Detection of cellular microchimerism of male or female origin in systemic sclerosis patients by polymerase chain reaction analysis of HLA-Cw antigens. Arthritis Rheum 2000, 43:1062–1067. The study identifies the presence of microchimerism in male and female patients with systemic scleroderma and provides support for the hypothesis that persistent microchimerism plays a role in the pathogenesis of the disease. CrossRef
- Lee T-H, Paglieroni T, Ohto H, et al.: Survival of donor leukocyte subpopulations in immunocompetent transfusion recipients: frequent long-term microchimerism in severe trauma patients. Blood 1999, 93:3127–139.
- Foeldvari I, Zhavania M, Birdi N, et al.: Favourable outcome in 135 children with juvenile systemic sclerosis: results of a multi-national survey. Rheumatology 2000, 39:556–559. Results from this mail survey suggest a better outcome for juvenile systemic scleroderma. CrossRef
- Seely JM, Jones LT, Wallave C, et al.: Systemic sclerosis: using high-resolution CT to detect lung disease in children. Am J Roentgenol 1998, 170:691–697.
- Weber P, Ganser G, Frosch M, et al.: Twenty-four hour intraesophageal pH monitoring in children and adolescents with scleroderma and mixed connective tissue disease. J Rheumatol 2000, 27:2692–2695.
- Clements PJ, Furst DE, Wong WK, et al.: High-dose versus low-dose D-penicillamine in early diffuse systemic sclerosis: analysis of a two-year, double-blind, randomized, controlled clinical trial. Arthritis Rheum 1999, 42:1194–1203. A controlled trial questioning the efficacy of D-penicillamine in the treatment of systemic sclerosis. CrossRef
- Black CM, Silman AJ, Herrick AI, et al.: Interferon-alpha does not improve outcome at one year in patients with diffuse cutaneous scleroderma: results of a randomized, doubleblind, placebo-controlled trial. Arthritis Rheum 1999, 42:299–305. A controlled trial showing that interferon-alpha is of no value in the treatment of systemic scleroderma. CrossRef
- Enomoto DN, Mekkes JR, Bossuyt PM, et al.: Treatment of patients with systemic sclerosis with extracorporeal photochemotherapy (photopheresis). J Am Acad Dermatol 1999, 41:915–922. CrossRef
- Casteels K, Bouillon R, Waer M, et al.: Immunomodulatory effects of 1,25-dihydroxyvitamin D3. Curr Opin Nephrol Hypertens 1995, 4:313–318. CrossRef
- Hulshof MM, Bavinck JN, Bergman W, et al.: Double-blind, placebo-controlled study of oral calcitriol for the treatment of localized and systemic scleroderma. J Am Acad Dermatol 2000, 43:1017–1023. CrossRef
- Browne PV, Weisdorf DJ, DeFor T, et al.: Response to thalidomide therapy in refractory chronic graft-versus-host disease. Bone Marrow Transplant 2000, 26:865–869. CrossRef
- Oliver SJ, Moreira A, Kaplan G: Immune stimulation in scleroderma patients treated with thalidomide. Clin Immunol 2000, 97:109–120. CrossRef
- McKown KM, Carbone LD, Bustillo J, et al.: Induction of immune tolerance to human type I collagen in patients with systemic sclerosis by oral administration of bovine type I collagen. Arthritis Rheum 2000, 43:1054–1061. Describes the use of oral type I collagen in patients with systemic scleroderma to induce tolerance to collagen type I. Induction of tolerance to collagen type I is worth investigating as a possible new treatment of systemic sclerosis. CrossRef
- Badesch DB, Tapson VT, McGoon MD, et al.: Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease: a randomized, controlled trial. Ann Intern Med 2000, 132:425–434. An important study showing the efficacy of intravenous epoprostenol in the treatment of pulmonary hypertension in systemic scleroderma.
- Klings ES, Hill NS, Ieong MH, et al.: Systemic sclerosisassociated pulmonary hypertension: short- and long-term effects of epoprostenol (prostacyclin). Arthritis Rheum 1999, 42:2638–2645. Another study supporting the efficacy of epoprostenol in the treatment of pulmonary hypertension in systemic scleroderma. CrossRef
- Seibold Jr, Korn JH, Simms E, et al.: Recombinant human relaxin in the treatment of scleroderma: a randomized, double-blind, placebo-controlled trial. Ann Intern Med 2000, 132:871–819. Another important study in which administration of recombinant human relaxin, a pregnancy-related hormone that has tissue remodeling and antifibrotic effect, was associated with reduced skin thickening, improved mobility, and improved function.
- Marmont AM: New horizons in the treatment of autoimmune diseases: immunoablation and stem cell transplantation. Ann Rev Med 2000, 51:115–134. An excellent review on stem cell transplantation in autoimmune diseases including systemic scleroderma. CrossRef
- Locatelli F, Perotti C, Torretta L, et al.: Mobilization and selection of peripheral blood hematopoietic progenitors in children with systemic sclerosis. Haematologica 1999, 84:839–843.
- Martini A, Maccario R, Ravelli A, et al.: Marked and sustained improvement two years after autologous stem cell transplantation in a girl with systemic sclerosis. Arthritis Rheum 1999, 42:807–811. The first autologous hemopoietic stem cell transplant electively performed in childhood for the treatment of an autoimmune disease. CrossRef
- White B, Moore WC, Wigley FM, et al.: Cyclophosphamide is associated with pulmonary function and survival benefit in patients with scleroderma and alveolitis. Ann Intern Med 2000, 132:947–954. A retrospective study showing that lung function outcomes and survival are improved in patients with alveolitis who received cyclophosphamide.
- Juvenile systemic scleroderma
Current Rheumatology Reports
Volume 3, Issue 5 , pp 387-390
- Cover Date
- Print ISSN
- Online ISSN
- Current Medicine Group
- Additional Links
- Author Affiliations
- 1. Dipartimento di Scienze Pediatriche, Università di Pavia, IRCCS Policlinico S. Matteo, 27100, Pavia, Italy