Long-term outcomes in a cohort of patients with systemic vasulitides (sv) and kidney involvement

Abstract Background and Aims Many randomized trials address the issue of SV therapy. Nevertheless, individualized treatment in patients with SV and renal involvement requires long term data and is often center specific. We present the experience of our center, a tertiary University hospital in Crete, Greece. Method Medical records were reviewed for clinical signs and symptoms. Statistical analysis was performed using SPSS 20 (IBM). Data were tested for normality, statistical significance was set at <0,05. Chi-Square test, Student's T-Test, Multiple-Regression analysis were utilized for statistical analysis. Results We examined 58 patients (37 females) with a mean follow-up of 5 years (Range 1-31) and a mean age of 66 years (Range 12-102). Serology revealed 46 pANCA+ve (79%), 8 cANCA+ve (14%) and 4 p/c ANCA negative patients (7%). Nine patients lost renal function and 16 patients died during follow-up. All patients received immunosuppressive induction therapy (Cyclophosphamide N = 42, Rituximab N = 7, Steroid monotherapy N = 9). All patients with pulmonary hemorrhage underwent plasmapheresis. The severity of clinical manifestation was associated with need for plasmapheresis and rapid renal function deterioration. Plasmapheresis resulted in the remission of pulmonary hemorrhage in all patients. Renal death was associated with renal function decline in months 1 and 6 post diagnosis. Treatment with cyclophosphamide or rituximab did not affect outcome (death or renal death). Number of relapses (14 patients), surprisingly, was not associated with renal function deterioration. Death was not associated with renal function deterioration in months 1 and 6 post diagnosis. Loss of Follow up was associated with increased mortality. Conclusion In this cohort either cyclophosphamide or Rituximab does not seem to affect prognosis. plasmapheresis efficiently contains pulmonary hemorrhage and, as expected, loss of follow-up is associated with poor outcomes.