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Churg-Strauss syndrome

Churg-Strauss syndrome (also known as eosinophilic granulomatosis with polyangiitis) is a type of vasculitis that affects small and medium blood vessels. Symptoms of this disease are similar to seasonal allergies, such as runny nose, cough, sneezing. Health professionals diagnosis Churg-Strauss syndrome by examining eosinophil levels and pANCA antibody levels, in addition to the presence of granulomas from a tissue sample (biopsy). Created by Ian Mannarino.

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Video transcript

- [Voiceover] Churg Strauss Disease is another type of vasculitis. This vasculitis affects the small blood vessels and vasculitis is vessel inflammation, which can lead to many different tissues being damaged. Churg Strauss shares a lot of common damage to different organs as other vasculitides. For example, like wegener granulomatosis, you might see sinusitis, or kidney issues, or lung issues as well. Lung tissues may be damaged. Similar to polyarteritis nodosa, you might have intestinal issues, intestinal damage. Skin damage, nerve damage, like in the nerves of your feet, or even heart damage as well. So it shares a lot of different organs with some of the medium and small vessel vasculitides, but there's a couple key take-aways from Churg Strauss Disease that you can focus on. A lot of patients are first misdiagnosed with an allergy. They may have runny nose, coughing, sneezing, and these skin symptoms may be thought to be an allergic rash. Patients are also misdiagnosed with asthma very often. Now what's common in both allergy and asthma? You have one common blood cell type that's seen in allergic reactions. These are eosinophils. Eosinophils are white blood cells that react to allergies. Actually, under a microscope and with staining they kind of appear a pinkish color. Eosinophilia should normally be very low in the blood. However, with Churg Strauss, we see very high levels of eosinophils. So with damage to multiple organs, and allergy and asthma like symptoms, along with an increased amount of eosinophils in the blood, this distinguishes Churg Strauss from all the other vasculitides. Lastly, a blood test that you can perform is PANCA. ANCA stands for Anti-Neutrophilic Cytoplasmic Antibody. They're a type of antibodies that are seen in auto-immune diseases. With auto-immune diseases you have white blood cells releasing a lot of chemicals from inside of them. Some of these white blood cells are neutrophils and to create these chemicals that they're releasing they draw together components from their cytoplasm. With a large release of these immune peptides, these molecules, I can't stress enough how many dots I'm making here, your body gets confused and creates antibodies against these molecules from the cytoplasm of neutrophils. So these are ANCAs, Anti-Neutrophilic Cytoplasmic Antibodies. They can be elevated in auto-immune diseases. Of course, there isn't just one type of cytoplasmic protein or molecule. There are different types. So this P stands for one of the different types of ANCAs that you see. So once you find PANCAs in the patients blood, you can take a slice of tissue from one of these areas that are affected, maybe a nerve or somewhere that's not invasive to get to. This slice of tissue is known as a biopsy. A biopsy you look at under the microscope. In Churg Strauss Disease, will show a formation of granulomas. This should be a very similar thing that you've seen in other vasulitides. The immune system forms a protective barrier around something that it sees as foreign and so this is a granuloma. So in Churg Strauss Disease you see allergy, asthma, eosinophils, you get a blood test for a PANCA and see that that's elevated, and then you can do a biopsy and find granulomas.