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non-systemic vasculitis
I have been diagnosed with non-systemic vasculitis (it is only affecting my peripheral nerves). So I have been reading about the set of diseases called the vasculitides.
As I understand it, the major types of *systemic* vasculitis (such as poyarteritis nodosa) have a 5-year mortality rate approaching 40-50%, even with treatment. However, most things I've read go on to say that the non-systemic version has "substantially better" prognosis.
That is reassuring, but far too vague. Can you tell me some actual numbers associated with non-systemic vasculitis? For instance, what is the 5- or 10-year mortality rate? What are the chances of going on to develop a systemic version of the disease?
Also, what is frequency of non-systemic vasculitis in the US or worldwide? It seems fairly rare.
And finally, more qualitatively, what sort of long-term course is likely for this disease? (Will it relapse, assuming I respond to the medication the first time? Will it get worse with each bout? What kind of long-term damage is being done to my cells?)
I am in my early 30s, and was an athlete before developing this.
As I understand it, the major types of *systemic* vasculitis (such as poyarteritis nodosa) have a 5-year mortality rate approaching 40-50%, even with treatment. However, most things I've read go on to say that the non-systemic version has "substantially better" prognosis.
That is reassuring, but far too vague. Can you tell me some actual numbers associated with non-systemic vasculitis? For instance, what is the 5- or 10-year mortality rate? What are the chances of going on to develop a systemic version of the disease?
Also, what is frequency of non-systemic vasculitis in the US or worldwide? It seems fairly rare.
And finally, more qualitatively, what sort of long-term course is likely for this disease? (Will it relapse, assuming I respond to the medication the first time? Will it get worse with each bout? What kind of long-term damage is being done to my cells?)
I am in my early 30s, and was an athlete before developing this.
My main symptom is axonal sensorimotor mononeuritis multiplex.
That means:
(1) The damage is primarily to the nerve axons, as opposed to the myelin sheath (determined by NCV/EMG tests). This is a big clue for vasculitis because most nerve diseases result in myelin damage.
(2) There are both sensory and motor components. In my case, the sensory part is pain, often localized along segments of major nerves, but also an aching pain in one knee, one elbow, and the muscles of my thighs and upper arms. The motor component is mostly weakess, now present in all 4 limbs, but also I have some function loss in my left leg that makes me limp. It is common to have both sensory and motor problems in vasculitis-- having just one might point toward something else.
(3) Mononeuritis multiplex means that the nerve symptoms developed asymmetrically (starting in my left leg, then my right leg a year later, then my arms a few months later). This is also a big clue because most nerve diseases present symmetrically. (Vasculitis *can* present symmetrically, but it is unusual).
The course of the disease for me was that symptoms developed acutely in a major nerve (tibial, peroneal, radial, median, ulnar)-- with pain. Then it would subside into weakness for a while with the pain returning upon overuse or sometimes just randomly. Then it would attack another nerve, etc. The result is a progressive restriction on how much you can move, how far you can walk, etc.
The 3 conditions above lead to a suspicion of vasculitis. It is confirmed by sural nerve biopsy, as noted in the previous comment. I also had blood tests to rule out diabetes and to check my ESR (erythrocyte sedimentation rate), rheumatoid factor, and immune complexes--- all negative. This is good because elevated ESR is likely in systemic vasculitis, but unlikely in non-systemic.
It all sounds neat and clean to write it up, but in fact it was a messy diagnosis. Because all of this took more than 18 months to develop, I was misdiagnosed 3 times and even had unnecessary surgery. I hear that is par for the course with vasculitic neuropathy, however. Standard treatment is the steroids, etc mentioned above, as far as I know. Let's hope it works.
That means:
(1) The damage is primarily to the nerve axons, as opposed to the myelin sheath (determined by NCV/EMG tests). This is a big clue for vasculitis because most nerve diseases result in myelin damage.
(2) There are both sensory and motor components. In my case, the sensory part is pain, often localized along segments of major nerves, but also an aching pain in one knee, one elbow, and the muscles of my thighs and upper arms. The motor component is mostly weakess, now present in all 4 limbs, but also I have some function loss in my left leg that makes me limp. It is common to have both sensory and motor problems in vasculitis-- having just one might point toward something else.
(3) Mononeuritis multiplex means that the nerve symptoms developed asymmetrically (starting in my left leg, then my right leg a year later, then my arms a few months later). This is also a big clue because most nerve diseases present symmetrically. (Vasculitis *can* present symmetrically, but it is unusual).
The course of the disease for me was that symptoms developed acutely in a major nerve (tibial, peroneal, radial, median, ulnar)-- with pain. Then it would subside into weakness for a while with the pain returning upon overuse or sometimes just randomly. Then it would attack another nerve, etc. The result is a progressive restriction on how much you can move, how far you can walk, etc.
The 3 conditions above lead to a suspicion of vasculitis. It is confirmed by sural nerve biopsy, as noted in the previous comment. I also had blood tests to rule out diabetes and to check my ESR (erythrocyte sedimentation rate), rheumatoid factor, and immune complexes--- all negative. This is good because elevated ESR is likely in systemic vasculitis, but unlikely in non-systemic.
It all sounds neat and clean to write it up, but in fact it was a messy diagnosis. Because all of this took more than 18 months to develop, I was misdiagnosed 3 times and even had unnecessary surgery. I hear that is par for the course with vasculitic neuropathy, however. Standard treatment is the steroids, etc mentioned above, as far as I know. Let's hope it works.
A related discussion, Acute vasculitis after C-section was started.
You're right about the prognosis of nonsystemic vasculitis. It's definitely much better than systemic vasculitis as its clinical manifestations are restricted to the nervous system. As for actual numbers, it's well documented in the literature that the course though slow and progressive, never becomes life threatening.
Reports are varied as to incidence. Some reports state that it's the most common vasculitic cause of peripheral neuroapthy (more common than the systemic vasculitides. Another report in the medical literature looked at 200 patients with vasculitic neuropathy and found that 35% had nonsytemic vasculitis.
As long as a complete and exhaustive search for systemic vasculitis was performed in your case and the physicians are confident that this is the right diagnosis, theoretically you should not go on to develop systemic vasculitis. But keep in mind that nothing in medicine or life for that matter is 100%.
Symptoms in most patients will respond to immune modulating agents such as prednisone which means you can hold off on the more intense immune agents such as chemotherapy with cytoxan. The clinical course is widely varied among different patients, so it's impossible to predict what course you'll have. Some people are able to keep the immune system in check with low dose prednisone on alternating days and then for some, they are able to taper off. Fortunately, peripheral nerves unlike your brain can repair and regenerate. It takes time and adequate therapy. Good luck.
Reports are varied as to incidence. Some reports state that it's the most common vasculitic cause of peripheral neuroapthy (more common than the systemic vasculitides. Another report in the medical literature looked at 200 patients with vasculitic neuropathy and found that 35% had nonsytemic vasculitis.
As long as a complete and exhaustive search for systemic vasculitis was performed in your case and the physicians are confident that this is the right diagnosis, theoretically you should not go on to develop systemic vasculitis. But keep in mind that nothing in medicine or life for that matter is 100%.
Symptoms in most patients will respond to immune modulating agents such as prednisone which means you can hold off on the more intense immune agents such as chemotherapy with cytoxan. The clinical course is widely varied among different patients, so it's impossible to predict what course you'll have. Some people are able to keep the immune system in check with low dose prednisone on alternating days and then for some, they are able to taper off. Fortunately, peripheral nerves unlike your brain can repair and regenerate. It takes time and adequate therapy. Good luck.
Do you mind expounding briefly on your symptoms and history for the benefit of the readers? Thanks and best wishes.
I too have had the same diagnosis. Mine was via a sural nerve biopsy, however have been told that it was not totally conclusive but that may be the nature of this disease. Mine currently only effects (only?, that's bad enough) the peripheral nerves as well. I have declined the Prednisone and Cyclophosphamide treatments due to their potentil severe side effects. I will be 52 years old soon. How were you diagnosed and what treatments were offered to you? I know of an individual with systemic vasculitis and it isn't pretty. My doc said I may have systemic in as much as all of my limbs are effected as well as the tops of my shoulders and the base of my neck.
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