Background: Henoch-Schönlein purpura (HSP) is an acute systemic disorder characterized by IgA associated vasculitis. The available data indicate an inherited predisposition to disease with triggering autoimmune p...Background: Henoch-Schönlein purpura (HSP) is an acute systemic disorder characterized by IgA associated vasculitis. The available data indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe nephrotic/nephritic flares associated with non-crescentic nephritis in adult patients. Patients and methods: The regimen consisted of an initial induction phase of 3-month Prednisone and Mycophenolate followed by a maintenance phase of Mycophenolate alone for 2 years. Results: They were satisfactory with complete remission in 5 of 7 patients and partial in 2. Creatinine clearance was normalized in patients with complete remission and remained stable in the partially-responsive ones. Conclusion: Our study has shown the short- and long-term safety and efficacy of such autoimmune regimen directed towards the autoimmune triggering factors in severe forms of non-crescentic HSP.展开更多
文摘Background: Henoch-Schönlein purpura (HSP) is an acute systemic disorder characterized by IgA associated vasculitis. The available data indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe nephrotic/nephritic flares associated with non-crescentic nephritis in adult patients. Patients and methods: The regimen consisted of an initial induction phase of 3-month Prednisone and Mycophenolate followed by a maintenance phase of Mycophenolate alone for 2 years. Results: They were satisfactory with complete remission in 5 of 7 patients and partial in 2. Creatinine clearance was normalized in patients with complete remission and remained stable in the partially-responsive ones. Conclusion: Our study has shown the short- and long-term safety and efficacy of such autoimmune regimen directed towards the autoimmune triggering factors in severe forms of non-crescentic HSP.