Ciclofosfamida: eficaz no tratamento do quadro cutâneo grave da esclerose sistêmica

Abstract

Introduction: Systemic sclerosis (SSc) is a rheumatologic disease characterized by autoimmune inflammation, vasculopathy and tissue fibrosis of skin and various organs. There are few effective treatments available for a severe cutaneous involvement of diffuse SSc. Therefore, we evaluated the efficacy of cyclophosphamide in the treatment of severe diffuse SSc. Patients and methods: Nine diffuse SSc (American College of Rheumatology [ACR] criteria) patients with a modified Rodnan skin score (MRSS) > 30 (0-51) were submitted to treatment with cyclophosphamide (CPM), at a monthly dose of 0.5 to 1.0 g/m 2 IV for 18 months. MRSS was performed every six months during 18 months. Laboratory evidence of inflammatory activity and adverse events were also retrospectively assessed. Patients with severe pulmonary and cardiac involvement were excluded. Results: Most patients were female (77%) with mean age of 41.7 years and duration of disease of 2.2 years. There was a significant reduction in the MRSS from 37.7 ± 4.08 to 29.1 ± 8.13 after 12 months of treatment (P = 0.009). Seven patients completed 18 months of CPM and had persistent reduction of the MRSS (mean MRSS = 26.4. P = 0.01). There was also a reduction of C-reactive protein (CRP) (initial mean CRP = 8.9 mg/dL) compared with values after 18 months of treatment (mean CRP = 3.09 mg/dL, P = 0.04). There were no infections, urinary disorders and/or important hematological problems throughout the treatment. Conclusion: The treatment with cyclophosphamide was effective and should be considered an alternative for the treatment of severe cutaneous involvement of SSc.

Study of new onset cutaneous manifestations in Rheumatic Diseases

“…There was no significant improvement in MRSS after 3 months of treatment. A previous study done by Patrícia Andrade de Macedo et al [31] in Brazilevaluated effectiveness of Cyclophosphamide in the treatment of severe cutaneous involvement in systemic sclerosis showed that there was significant reduction in MRSS after 18 months of treatment. However, since the follow up period in our study was only 3 months, it may be inadequate to comment on the potential improvement in the long run in these patients on Cyclophosphamide therapy.…”