Thursday, August 4, 2011

Churgg-Strauss Syndrome (CSS)

Finally I get around to doing something I said I was going to!

On the list of Vasculitis, Churgg-Strauss Syndrome is another type of ANCA associated vasculitis which makes it a closer cousin to Wegner's than, say, TAK (which we will also get to eventually!).

CSS is also systemic (multiple systems) and shares some common symptoms with PAN (polyarteritis nodosa) which is another type of vasculitis. What makes this type different from PAN, though, is the presence of granulomas and eosinophils in the blood (an eosinophil is a type of white blood cell which usually only compromises about 5% or less of a person's total blood count, in a person with CSS they can make up as much as 60% of a person's blood count). Also, the ANCA's.

The "typical patient" (though we all know how this typical patient stuff works. If we went by that I should be a middle aged man of European decent) is a middle aged person, distribution between male and female is roughly equal, with new onset, or newly worsened asthma.

Now, for a long time my Wegner's also presented itself as asthma, but it is one of the defining features of CSS and CSS is the only type of vasculitis where asthma is a defining characteristic. This doesn't mean that everyone who has asthma has CSS, only a very small minority do, but everyone who has CSS has asthma. Other symptoms include nasal polyps and allergic rhinitis. After this it moves on to the presence of eosinophils in the blood. Finally, the disease will move into the vasculitis stage. Much like Wegner's, CSS can go anywhere in the body. Wegner's doesn't often go for the heart, but CSS can and does. The heart and kidney's seem to be CSS's big organs of choice (at least according to Johns Hopkins) with a smaller likelyhood of it causing damage to the lungs. Which seems odd to me considering that ASTHMA is one of its defining features but apparently only 1/3 of CSS patients get lung infiltrates with even less getting bleeding into the lungs and an even smaller amount than that getting lung disease.

Like most other forms of vasculitis, until doctors figured out that "Hey, Prednisone and chemo therapy, when used to together, induces remission!" which means that far fewer patients with vasculitis now die from their disease than before. Previously CSS, like Wegner's, was 100% fatal. It's good to know that we are improving in this area.

That's CSS in a nutshell!

No comments:

Post a Comment