What’s New in Churg Strauss Research

By Dr. David Jayne, Noted Researcher
and Medical Advisor to the CSSA.

The CSSA is very grateful to Dr. Jayne not only for the following article written just for the CSSA, but for his continuing interest and research pertaining to Churg Strauss Syndrome. In addition, Dr. Jayne has been instrumental in helping to organize the first ever UK Vascultis Foundation Conference to be held in September, 2007.

What’s new in Churg-Strauss research?

David Jayne MD FRCP
Cambridge, UK
March 2007

The treatment of Churg-Strauss syndrome (CSS) has been modelled on that of more common vasculitis syndromes such as Wegener’s granulomatosis (WG) or microscopic polyarteritis (MPA). However, the French Collaborative Research Group have a good tradition of studying CSS on its own and have completed the largest trials in CSS so far (www.vascularite.com). They have recently looked at the role of the ANCA blood test in 112 new CSS patients and found ANCA in 38%[Sable-Fourtassou, 2005 #11]. This is somewhat lower than previous studies, but of more importance they found that the patients with ANCA were more likely to have kidney and nerve involvement and were less likely to have involvement of the heart or a fever. This suggests that ANCA may be useful in dividing CSS into two subgroups and different treatment approaches may be required.

In Cambridge, we have been impressed by the improvements seen in some of our vasculitis patients with Rituximab (Mabthera in Europe, Rituxan in the US). Rituximab is a monoclonal antibody that has been designed to remove a type of white blood celleatment, B cells become undetectable in the blood for an important player in ds to subside. Fortunately we can cope without B cells reasonably well and this treatment may well be safer than current immunesuppressive drugs. Six CSS patients with disease that has proved difficult to control with the usual drugs have been treated with rituximab in Cambridge. Results in the first two patients were promising and have been published[Koukoulaki, 2006 #155]. These patients continue to do well although have required further courses of rituximab. We have also been encouraged by the results in the next four patients but it is too early to make any firm conclusions. It would appear that this success is similar to that seen with rituximab in WG and MPA and underlines the importance of bigger studies in CSS. The Mayo clinic in the USA have started such a study and we hope that a European study may start soon as well (www.clinicaltrials.gov).

Another drug that has attracted interest is Mepolizumab. Like rituximab, mepolizumab is also an antibody targeted treatment. Mepolizumab L-5 which is released by a subset of white blood cells, T cells, and stimulates eosinophils, the cell type closely involved in CSS. It has beeknow that IL-5 levels control and there are good reasons for thinking thaulitis and CSS is the study of gene expression in circulating blood cells. Newer scientific techniques, whether a genn other genes, can s on. This technique has beent the course of a disease and to pr drug. Patterns unique to vasculitis patients have now been described and we hope to find a CS