ANCA-associated vasculitis : towards patient-tailored therapy

Abstract

The work presented in this thesis concerns various, mainly clinicopathological, studies of ANCA-associated vasculitis. The first chapter provides a general introduction to the topic and the studies. Chapters 2 and 3 describe long-term patient and renal survival data concerning 535 patients. The emphasis in these chapters is on the results of multivariable models, developed to detect baseline patient characteristics that can provide reliable prognostic information to treating physicians. Chapter 4 comprises a clinicopathological study performed on renal biopsies of patients experimentally treated with a rituximab-based regimen. Specific attention is paid to the presence of B cell, T cell and plasma cell infiltrates in the diagnostic renal biopsy and the relation of these infiltrates to renal outcome under rituximab treatment. Chapter 5 reviews known disturbances in cellular immunity in vasculitis. In chapter 6 the presence of anti-plasminogen antibodies is described in two independent patient cohorts, one from the United Kingdom and one from the Netherlands. Chapter 7 illustrates that a simple classification schema comprising only four histological classes correlates well with renal outcome in a first validation exercise. Finally, the results described in this thesis are summarized and discussed in chapter 8.