Sjogrens and small fiber neuropathiy

[Dryguy]

I received my SS diagnosis in October 2015. Simultaneously, my upper chest and neck felt like I had a bad sunburn. My face became very sensitive to shaving. My chest and neck feels as though burned. However, they never turn red and never itch. The only symptom is constant 24/7 pain that feels like a burn that I have felt for 18 months. Fortunately, the intensity of the pain varies: sometimes so mild I can ignore it; sometimes so severe I cannot concentrate.

After several months of testing and non-answers from my PCP, dermatologist , neurologist, and rheumy, a second opinion from another neurologist diagnosed small fiber neuropathy. I assume he is correct. He explains that SS patients can experience "extraglandular" complications or peripheral nervous system manifestations of SS with small fiber neuropathy causing the burning pain in chest and face.The bad news is there are no treatment options. He suggested I try hemorrhoid cream !

I have tried various ointments, creams, and gels, some prescription and some OTC, all of which make the pain worse.

Are there any others out there who experience the same or similar symptoms as me ? If so, what do you do and what specialist would you recommend ?

[Jasper]

Your Neurologist is incorrect when he says there are no treatments for SFN. 

There are several first line drugs for symptomatic relief (Lyrica, Gabapentin, etc.).

There are also immune suppressants and biologics that are more effective because they treat the inflammation (not just the symptoms). IVIG infusions and Rituximab infusions are the most effective treatments for SFN.

I have SFN and I had some similar symptoms like you have. In 2013 I had bilateral calf redness (bright red, like a sunburn) and extreme pain upon touch (like a very bad sunburn). This lasted 8 months and then subsided. For 5 months of that time I also had bilateral lower leg, ankle, and feet edema. That subsided at the same time the redness and pain subsided. I was new to Sjogren's at that time, diagnosed in July 2013. However, during and after the edema other symptoms appears ..... severe burning of the feet and ankles, odd sensations like cotton wadded up around my feet and toes, wadded up sock feeling, electric shocks, extreme sensitivity to touch on my toes, crawling sensations, etc. In 2014  I was diagnosed with sensory polyneuropathy/ganglionopathy and SFN. My Neurologist discussed IVIG but some of my symptoms (mainly the severe pain on touch) were improving and insurance posed a problem so I never had IVIG. However, he did consider it as a treatment. Had my symptoms worsened, he would have found a way  to order it.

I started Rituximab infusions in February 2016 and all of my SFN symptoms have vastly improved. In fact, some of them have mostly disappeared. I barely notice the neuropathy symptoms now. So, for me, Rituximab was very effective in treating the SFN.

Here is a link to one thread concerning SFN:

https://sjogrensworld.org/index.php?topic=30222.msg316637#msg316637

I tried to post a link to Dr. Anne Louise Oaklander's video lecture but the link does not work. However, if you type in "dr. oaklander, a name for the pain you tube" the topic will come up and you can click on it and the video will come up. It is about 55 minutes long and it is excellent. You need to add the you tube part of you won't get the video. One of the threads on the forum does have a correct link to the video so, if typing in the key words does not work, I can try to find that thread.

My suggestion would be to find a Neurologist who will treat your SFN. You may have to go to a physician who is affiliated with a university or another teaching facility.

I hope you find a doctor who will treat you because I know the pain is severe.

[SjoGirl]

Well, from what I have read the doc is correct, neuropathy can't be treated. However, the pain can be.

Did they do any skin biopsies to diagnose your SMN? That is how it is typically diagnosed. Nerve conduction testing will help determine if any of the neuropathy is large fiber.

There is a great book on this topic: Peripheral Neuropathy: When the Numbness, Weakness, and Pain Won't Stop, Norman Latov, MD, PhD.

I too take Gabapentin. The one issue that I've found it that it makes me spacey if I take the full 600 mg per day. I'm trying to cut back to 500 mg and am going to ask my neurologist if there is something different that I can try because I still work and need to function.

[Dryguy]

Thanks Jasper and Sjogirl for your replies. You gave me more info than I had. I will be prepared for my next neuro appointment. No, Sjogirl, he did not take a skin biopsy. After I was given a diagnosis, online research told me that some neuro doctors will diagnose using only the symptoms told him by the patient. I think a biopsy would be more reliable.

[irish]

I have to ask a question. Many people with neuropathy are treated with IVIG or intravenous immune globulin with good results. Doesn't IVIG work for small fiber neuropathy? Hopefully someone can answer this question. Thanks, Irish

[Jasper]

Irish ..... Yes, IVIG is used to treat SFN and it has shown good results. Of course, it does not work for everyone, but the studies have shown that it does work for a significant number of people and the improvement is significant in those people. 

[Dawnmist]

In terms of direct treatment for SFN, he's right...the only way to treat it is to find what it causing it and treat the cause.

In terms of symptomatic treatment, he's dead wrong - there's several treatment options to try to moderate the pain. They often do not eliminate it entirely (or if they do, pain will break through after a while as the amount of nerve damage worsens), but it can at least get you some relief. I've personally tried Lyrica, Gabapentin, Cymbalta (or its generic Duloxetine), Tramadol (the slow-release version lasts "12 hours", so you can take a dose morning & night keeping levels more stable than is possible with the shorter acting tablets), Mexiletine (oral lidocaine - didn't work for me, but does for some people), Lidocaine Patches on the worst places. Other medications sometimes used (but not normally appropriate when you have Sjogren's because they dry you out too much) are some of the tricyclic anti-depressants like Amitryptaline & Nortryptaline. On facebook there are several people that have reported good success with marijuana - that's not legal to use in Australia at this time, so I have no personal experience with that one. Several of the above medications can actually be used together (at least up to a point). He *should* have either proposed some of those options or sent you on to a pain management clinic to handle them...doing nothing at all is just plain wrong.

For treating the cause when the cause is autoimmune, you get into immune modification/suppression. IVIG has been the most successful/most tested treatment internationally, but Rituximab seems to be the next medication-of-choice. Then it falls off - there's been some reported success with Cellcept, and I think a little with Imuran. Of course, mucking around with the immune system increases your risk of infection/etc, which is something you need to keep a watch on.

[Carolina]

Dear DryGuy,

I have horrific SFN.  It felt like a sunburn, then stinging, burning, stabbing pain, and itching.   My face, eyelids, lips, tongue, neck, arms, hands and fingers, especially my finger tips.  Without Gabapentin (2400mg day) I live in a cage of pain that is my own skin.  I can still feel a bit of SFN in my face, but nothing to bother me.

I have IVIG every four weeks for my Immune Deficiency, but the treatment for neuropathy is much stronger and more often.  My Peripheral Neuropathy is profound and can't be 'undone' with any treatment.  I'm settled on Gabapentin for my SFN, and haven't asked about a different regimen of IVIG for it.

Lyrica or gabapentin will usually help a great deal with neuropathy pain and discomfort.

Hugs,  Elaine

[trc1962]

In 2003 before I had a diagnosis, I found a neurologist who really listened. He treated the terrible neuropathy symtoms I would get at night with gabapentin but said I needed the cause treated and sent me back to the rheumy. I literally begged the rheumy to have the nerve conduction test done that was just the little shocky things on my feet and hands and boy was he shocked as it showed the neuropathy. He started me on Imuran and 3 months later I was very good. Unfortunately the neuropathy came back and I am now on large doses of mycophenolate - which I think helps some, but not enough. My new rheumy is thinking rituxan ir IVIG but is waiting for my appointment in April at a hospital in Seattle to see what they think. I have a friend who suffered greatly for years and finally the doctors said lets try IVIG and it worked very well. She still takes 500 mg mycophenolate daily but now works full time, bikes and runs also - a new life she says. I am hopeful for IVIG because I teach school and am afraid rituxan might lower my immunity too much. Hoping you get results from the pain. I was briefly on a different generic form of mycophenolate and suddenly it felt like I had wetness on the bottom of my socks...weird. However, returning to the other brand of mycophenolate changed that after about 2 weeks. Such a very strange journey this is.

[SjoDry]

I know a Sjoggie who used to frequent this board who takes high dose IVIG for neuropathy and it greatly helps her. Also, I take Sub-Q IVIG for an immune deficiency. I do think that even in the smaller doses for my immune system that it helps my neuropathy as well.

SjoDry