Diffuse and limited cutaneous systemic scleroderma.
Juvenile systemic scleroderma (jSSc) is a rare disease. Based on the first large data collection on this patient group, the disease course was demonstrated to differ from that in adults. The concept of persistence of maternal cells in patients with SSc remains pathogenetically fascinating, as does the resemblance with graft-versus-host-disease. In view of new therapeutic options, controlled trials have not established a gold standard for treatment, but autologous bone marrow transplantation may be considered a rescue therapy for selected patients. Palliative therapies have also improved markedly in recent years. The first controlled trials for patients with jSSc are being proposed.
Foeldvari
I
I
University Children's Hospital, Pediatric Rheumatology Unit, Hamburg, Germany. foeld-vari@uke.uni-hamburg.de
D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008431:Maternal-Fetal Exchange; D010166:Palliative Care; D011247:Pregnancy; D011379:Prognosis; D012595:Scleroderma, Systemic / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D012720:Severity of Illness Index; D013256:Steroids; D013792:Thalidomide / Q000627:therapeutic use
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D013256:Steroids; D013792:Thalidomide