Churg Strauss Syndrome

[Kenia]

I am a 30 year-old female who began having symptoms 5 years ago after child birth. I Developed Leukocytoclastic vasculitis 2 years ago and was DX with CSS July of this year. My question is related to multiple flare-ups of the vasculitis.

During the past 2 years I never really got rid of the vasculitis it just waxed and waned from intense with full purpura, Bullous Leukocytoclastic vasculitis, and ulceration to mild petechiae. I have great difficulty sitting for longer than 5 min. before my legs go numb. At night sleeping becomes a chore as I have to keep getting up to get the feeling back in my legs. Has anyone had any similar problems with permanent vein damage, and does anyone know what my options are. At this point I don't even know which doctor to start seeing for this problem.

I am currently on 40mgs Prednisone, 100mgs Cytoxan, and Synthroid. I know I am lucky as my lungs are mostly unaffected, I have mild asthma. I do have mainly gastro, neural, and cutaneous involvement, but still I need some direction.

[dr_simran]

Hello Kenia,
Churg-Strauss syndrome (also known as allergic granulomatosis) is a medium and small vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable, non-transmissible and often mis-diagnosed. Churg-Strauss syndrome was once considered a type of Polyarteritis nodosa due to their similar morphologies.
Treatment for Churg-Strauss syndrome includes glucocorticoids such as prednisone and other immunosupressive drugs such as azathioprine and cyclophosphamide. In many cases the disease can be put into a type of chemical remission through drug therapy, but the disease is chronic and life long.
A systematic review conducted in 2007 indicated that all patients should be treated with high-dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy should be commenced, with 12 pulses leading to less relapses than 6. Remission can be maintained with a less toxic drug, such as azathioprine or methotrexate.
Refer: http://en.wikipedia.org/wiki/Churg-Strauss_syndrome
Best.

[reidweaver]

Hi Kenia,

I have cutaneous polyarteritis nodosa.  I have been able to achieve remission for 5 years with prednisone and Imuran (azathioprine).  My rheumatologist began me on prednisone (30-60 mg.  eventually 30 was enough) to control the flares and then once the ulcers began to recede, I was weaned off of the prednisone.  However, as soon as it was out of my system, the flare would begin again.  After a couple of prednisone trials, my physician added Imuran and began to wean me off of prednisone.  Because of my susceptibility to every sneeze around me (which landed me in the hospital each time with high fevers), he also added Bactrim 800 daily.  Imuran is mild for symptoms and most people had no idea I was on "chemo".  Within 18 mos I had been symptom free for a year other than nerve damage and was able to discontinue all medication.  I remained in remission for 5 years.  With my father's diagnosis of mesothelioma this fall and a number of other stressful events this year, I am now beginning to experience symptoms again.

Question:  have you have a nerve conductivity test or an EMG?  A neurologist can help you determine what type of nerve damage you may be experiencing from the vascullitis.  Be careful to keep your legs warm during the cold months.  Being outside with stockings on can exacerbate vasculitis.  The blood vessels with contract and expand, making your legs more painful and contributing to more ulceration.  I have found fleece socks and blankets to be very comforting to the pain and weird sensations I have (cold sheets and nylons feel like sandpaper).

I hope that helps!  Also, check out http://groups.yahoo.com/group/polyarteritisnodosa  We have people in the group with different types of vasculitis including CSS and Wegener's disease.  Very supportive and we also are part of the Vasculitis Foundation trying to raise awareness.

Barb