1. Guest, 'News in Brief' for the week beginning 17th February 2020 - click here.
    Dismiss Notice
  2. Welcome! To read the Core Purpose and Values of our forum, click here.
    Dismiss Notice

I was diagnosed with a rare autoimmune disease called "Relapsing Polychondritis"

Discussion in 'Immune: Autoimmune and Mast Cell Disorders' started by TrixieStix, Nov 25, 2017.

  1. Alvin

    Alvin Senior Member (Voting Rights)

    Messages:
    3,311
    Likes Received:
    16,072
    yikes, sorry to hear that. Just so you know Vitamin K2 supposedly works in concert with calcium and has apparently been shown to help reduce osteoporosis risk due to prednisone (though i have not looked into that extensively).
     
  2. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    Hi Lucy. Now that I am taking both Prednisone (10mg) and Plaquenil (200mg) daily I thought I would say hi and ask you how you've tolerated them. How many months did it take for you to see improvement from the Plaquenil?

    In regards to Plaquenil I haven't noticed any side effects whatsoever thus far and hoping it stays that way. I'm aware it takes many months for it to start "working".

    As for Prednisone I continue to tolerate it well. No insomnia whatsoever and I'm not wanting to eat ALL the food in the house so that's good. When I did that 6-day Medrol (30mg) pack 4 months ago it made me crave carbs and sugar. I am enjoying the 5 hour or so increase in energy I get after I take my daily Prednisone dose (feels like a caffeine high without the horrible anxiety caffeine gives me). However I learned quickly that I can't let myself be fooled by this boost of energy. The other day at the grocery store I felt strong enough to walk around the grocery store instead of using one of the electric sit-down carts like I usually do. That evening I "crashed" hard (PEM).

    Update: As of 3/14 my doctor has increased my Plaquenil to 400mg daily.
     
    Last edited: Mar 15, 2018
  3. Samuel

    Samuel Senior Member (Voting Rights)

    Messages:
    338
    Likes Received:
    1,746
    if you mentioned angioedema, i missed it. were these tests a stab in the dark for your ear reddening?

    also i wonder why they test number and not just function.
     
    alktipping likes this.
  4. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    No those blood tests were done by an immunologist after it was discovered I had a complement deficiency. The doctor was unaware I was experiencing episodes of auricular chondritis. I think he ran the tests because I had told him I had experienced some episodes of sudden shortness of breath where it felt like my throat was partially closed off and I had also had some gastro issues in the past. He was indeed making sure I did not have C1-inhibitor deficiency.

    "The diagnosis of C1-inhibitor deficiency is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function." (they test both protein level and function)

    Even if I had mentioned the episodes of auricular chondritis to my doctors there aren't any blood tests they could have done do to detect the RP and the auricular chondritis alone wouldn't have been enough to diagnose me with RP. No biomarker exists for the disease. Diagnosis is purely based on symptoms (and response to steroids can help confirm). Also auricular chondritis like that seen in RP can also be caused by Wegener's Granulomatosis and also Lupus.

    As for a list of my symptoms. No I have not re-written out a timeline of my symptoms on this site. I will write one out one day.
     
    Last edited: Mar 14, 2018
  5. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    Looks like liver is a great source of K2. I've been wanting to start consuming beef liver from local pastured cows but have been putting it off. If what you say is true I guess that's even more reason to start eating it. I can buy it easily so no excuses! I've never eaten liver so I'm gonna have to figure out how I'm gonna get it down my throat. lol
     
  6. Alvin

    Alvin Senior Member (Voting Rights)

    Messages:
    3,311
    Likes Received:
    16,072
    It has a robust taste
    It is a good source but i don't know if you can eat it often enough to counter prednisone induced bone loss, you may need to supplement
     
  7. oldtimer

    oldtimer Senior Member (Voting Rights)

    Messages:
    206
    Likes Received:
    1,226
    I've been a vegetarian for 25 years but I remember cooking it with bacon and lots of onions and everyone liked it. I recall that it should not be eaten more than once or twice a week because of its high vitamin A content.
     
    Binkie4, alktipping, Inara and 2 others like this.
  8. Inara

    Inara Senior Member (Voting Rights)

    Messages:
    2,712
    Likes Received:
    11,040
    @TrixieStix, your story is so very exciting! I am very interested indeed in how it will continue. Thank you for keeping us up to date.
     
  9. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    I am one month into my RP treatment....

    -10mg of Predisone daily (though I did have to increase to 15mg daily for 6 days due to a sudden worsening of my nasal chondritis pain triggered by a stressful event)

    -400mg of Plaquenil daily (so far no side effects)

    -1,000mg of Calcium citrate w/500mg of Magnesium also (per NIH recommendations to help counter bone loss caused by the prednisone)

    - those are in addition to the things I was taking before the RP diagnosis which are: an oral opioid pain medication, Pilocarpine, Restasis, Vitamin D, and Sumatriptan as needed for migraines (along w/ ibuprofen).

    As for any changes in how I feel, the Prednisone is most definitely helping. I know it's nasty stuff, but boy am I thankful for it right now. It has made the pain caused by the nasal chondritis much more tolerable, and it also has me feeling less malaised (fatigue is still the same though).

    It's still too early to expect to see any improvement from the Plaquenil (I've read it can sometimes take up to a year for it to reach it's full effectiveness). I'm thinking it might be hard a little hard to tease out any improvements I might have from the Plaquenil (unless it's dramatic) as I've seen a lot of RP'ers say that they thought Plaquenil wasn't doing anything until the stopped taking it and realized it had been helping. It's a drug I'm happy to stay on long-term if it doesn't cause me any significant side effects (ie: retina damage).

    Something else I'm dealing with right now is a ramping up of the frequency of my migraine headaches. I've had more this month than I've ever had in a single month (I had to take Sumatriptan 10 times). However a few nights ago I learned about a condition called "Occipital Neuralgia", and I think there is a very good chance that a some of the headaches I've been calling migraines are actually occipital neuralgia . I messaged my neurologist yesterday and he got right back to me and said he could evaluate me for it, and perform a nerve block if I do have it. I hate to have to add yet another diagnosis to my list, but there are treatments for occipital neuralgia so perhaps if it's treated it will improve my quality of life a tad. I'm soooo scared of needles so the idea of having to get a nerve block (bunch of needles stuck in my neck and scalp) scares the crap outta me, but I know I will get thru if it's necessary.

    In the meantime I'm trying my best to avoid looking down (ie: at my cellphone), putting a cold pack on the area every couple of hours, taking ibuprofen ahead of when I go to bed since laying my head against the pillow makes it worse, and avoiding looking to the right which is also makes it worse. I'm almost tempted to buy one of those soft neck braces and see if immobilizing my neck for some days helps the nerve to calm down some, but maybe that's a bad idea?

    Anyone here suffer from Occipital Neuralgia?

    Happy Easter everyone!!!
     
    Binkie4, alktipping, Esther12 and 7 others like this.
  10. Daisybell

    Daisybell Moderator Staff Member

    Messages:
    1,920
    Likes Received:
    12,126
    Location:
    New Zealand
    So glad the prednisone is helping... fingers crossed for the Plaquenil!
     
    Binkie4, alktipping, Esther12 and 3 others like this.
  11. Little Bluestem

    Little Bluestem Senior Member (Voting Rights)

    Messages:
    1,447
    Likes Received:
    8,780
    You have my sympathy on that one. :eek: :hug:

    Could it hurt? Maybe ask your neurologist.
     
    Binkie4, alktipping, Esther12 and 3 others like this.
  12. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    Today marks 2 months since I began being treated for RP. I am now being treated with....

    - 10mg Prednisone daily

    -1,000mg of Calcium citrate w/500mg of Magnesium (per NIH recommendations to help counter bone loss caused by the prednisone)

    - 400mg Plaquenil daily

    *NEW - 10mg Leflunomide daily (I only started taking Leflunomide 5 days ago after I failed oral Methotrexate which caused me severe GI symptoms for an entire week after the 1st dose. My RP doctor felt it better to try Leflunomide before considering trying the injectable form of Methotrexate.) So far I have experienced no side effects. Whether or not it improves my RP symptoms is still to be seen and we won't know until I've been taking it for a few months. Like Methotrexate, Leflunomide can cause some very serious side effects and I will have to undergo monthly blood tests while taking it to monitor my liver function, kidney function, and blood counts. It is also going to suppress my immune system so there are risks in regards to that as well.

    - the above meds are in addition to the things I was already taking before the RP diagnosis which are: an oral opioid pain medication, Pilocarpine, Restasis, Vitamin D, and Sumatriptan as needed for migraines (along w/ ibuprofen).

    Thus far I've not noticed any improvement beyond that resulting from the daily Prednisone. It's been 2 months since I began the Plaquenil and 1 month since my dose was increased from 200mg daily to 400mg daily.

    I'm really really not looking forward to the monthly blood tests I must have while taking the Leflunomide. Perhaps having the same person do it each time would help? I wonder if there is a topical numbing agent that can be applied to my skin beforhand?

    Also I absolutely HATE those new retractable needles many places now use. They hurt like hell! At my last blood draw the phlembotomist chose not to use the retractable function because he didn't want to cause me more pain. I wish that guy could do all my blood draws, but unfortunately he is at the hospital where I see my RP doctor which is 4 hours away. Boo.

    Next week I have an appointment with my eye doctor to have baseline testing done due to the occular risks of Prednisone and Plaquenil. And then a month from today I have my 3 month follow-up appt with my RP doctor.

    Fatigue Meter: I have experienced no improvement in my fatigue.
     
    Last edited: Apr 28, 2018
  13. Gingergrrl

    Gingergrrl Senior Member (Voting Rights)

    Messages:
    637
    Likes Received:
    4,028
    Thank you for keeping us updated @TrixieStix and I was curious if you were having other improvements (separate from the fatigue not changing?) or is two months to soon to expect to see improvements?
     
  14. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    @Gingergrrl The only improvement I've experienced is that which I noted last month (resulting from the Prednisone)...

    "It has made the pain caused by the nasal chondritis much more tolerable, and it also has me feeling less malaised (fatigue is still the same though)."

    Unfortunately the dose of Prednisone I'm taking (nor the Plaquenil @ this point) has not improved the symptoms in my thyroid cartilage and trachea thus the reason my doctor and I made the call to put me on a stronger DMARD.

    As for the Plaquenil (aka: Hydroxychloroquine)...

    "With hydroxychloroquine, your symptoms may begin to improve in 1 to 2 months. However, it may take up to 6 months to a year before the drug achieves full effect."

    Even if the Plaquenil proves to not be all that helpful for my RP I do hope it will be helpful for my Sjogren's like symptoms.
     
    Last edited: Apr 29, 2018
  15. Daisybell

    Daisybell Moderator Staff Member

    Messages:
    1,920
    Likes Received:
    12,126
    Location:
    New Zealand
    Fingers crossed for you @TrixieStix that you get improvement...
     
    Binkie4, Inara, Gingergrrl and 3 others like this.
  16. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    I am a week away from hitting the 4 month mark since beginning treatment for RP.

    I am currently being treated with....

    - 10mg Prednisone daily

    -1,000mg of Calcium citrate w/500mg of Magnesium (per NIH recommendations to help counter bone loss caused by the prednisone)

    - 400mg Plaquenil daily

    - 20mg Leflunomide daily

    - at times... 400mg ibuprofen every 6 hours for flares of severe joint pain caused by the RP. (the past week this has been happening in my kneecap cartilage).

    - the above meds are in addition to the things I was already taking before the RP diagnosis which are: an oral opioid pain medication, Pilocarpine, Restasis, Vitamin D, and Sumatriptan as needed for migraines.

    I had my second appointment with my RP doctor almost 1 month ago. She doubled my dose of Leflunomide (from 10mg daily to 20mg daily) as my blood work showed no abnormalities thus far as a result of taking it. I see her again 3 months from now. She also referred me to the ENT (ear, nose & throat) she sends all her RP patients to.

    I had my appt with the ENT yesterday. The appointment went really well, and I liked him a lot. Thankfully he agreed with me that it wasn't necessary to "scope" me (up the nose & down to my voicebox) as it was done in October by my previous ENT. Basically it was just an appointment to establish care, ask a few questions, and refer me to the speech therapy my RP doctor had suggested may help reduce my pain when speaking, and feeling of strain I often feel when speaking. He said even though it's the inflammation in my throat/trachea that is causing these issues that learning some vocal exercises may help. I see the "speech & swallowing" doctor & speech therapist both on July 10th in a joint appointment.

    Next up is to establish care with an orthopedist (for joint issues due to RP). The past week I've had terrible pain and tenderness in my kneecaps for the first time ever so now I'm slightly concerned about the state of my cartilage in them. Damn cartilage is everywhere!!!!

    Tomorrow I have an appointment with my primary doctor to fill-out yet more paperwork for my private Long-term disability insurance (they still pay me a small monthly amount even though I receive SSDI). They make me jump thru the same paperwork hoops every 6 months. Most of it has to be filled-out by my doctor.

    I am also currently going thru my first ever SSDI review so I have to fill out that paperwork also and send it to SSA. I spoke with someone who also just went thru their first ever review and SSA made them go thru a psychological review and a physical with doctors SSA chose. I am hoping that now that I am diagnosed with RP that SSA won't deem such exams necessary in my case. Even though I'm confident my review will go just fine it's impossible not to feel some amount of stress/worry about it. I sure do how I miss being healthy and being able to work and make my own $. This past week marked 5 years since I had to stop working.

    As for whether I feel like the medications are improving things for me yet it's hard to tell. I already know the Prednisone helps and it continues to keep my nose pain at a tolerable level, and it has lessened the "malaise". One effect the Prednisone has had is to raise my "White Blood Cell" count to about 14,000 (high) which I've learned is a very common thing with Prednisone.

    As for the Plaquenil I was feeling like it has improved my skin rashes on my face, but a month ago I developed another rash (redness & flaking of skin) so now I'm not so sure. When my RP doctor looked at it she asked me if I had been staying out of the sun. So perhaps that's the culprit. I'm now being even more careful to avoid having sunlight hit my skin. So far riding in the car has proved the biggest challenge when it comes to this. I think perhaps I will just have to start riding in the trunk ;)

    And lastly it may be that I am starting to see some improvement in my fatigue (also not "crashing" as hard after activity & taking less time to recover) as a result of the Plaquenil, Leflunomide, or both in the past few weeks, but then again it's finally summer where I live, and I always experience some improvement in the summer. I guess the real test will come this winter.

    Fatigue Meter: slight improvement
     
    Last edited: Jun 21, 2018
    Binkie4, Skycloud, MEMarge and 9 others like this.
  17. Jonathan Edwards

    Jonathan Edwards Senior Member (Voting Rights)

    Messages:
    6,224
    Likes Received:
    63,109
    Interesting to hear this @TrixieStix.

    I am not in a position to give you medical advice but I think I can allow myself to think aloud.

    Plaquenil can produce sun sensitivity - quite often.

    Nobody really knows the immunological basis of RP but the way it moves from place to place without any obvsiou autoantibodies suggests that it is quite likely to be purely T cell mediated, like psoriasis. I don't think we have any theoretical reasons for thinking that plaquenil is helpful for T cell based problems. It is chiefly used in lupus, which involves B cell inflammatory mechanisms. I doubt there is any hard evidence from actual clinic use but I have not looked at the literature.

    Leflunomide was devised as an anti-T cell drug, even though it was marketed for RA, which turns out to be a B cell disease where T cells are involved as bystanders as far as we know. In RA responses are either quite clear cut or not at all and are usually obvious after 20mg daily for three months.
     
    TrixieStix, Binkie4, Hutan and 4 others like this.
  18. duncan

    duncan Senior Member (Voting Rights)

    Messages:
    872
    Likes Received:
    3,314
    It is used chiefly as an anti-protozoal, e.g. malaria and babesisois. It is also used for late stage Lyme (really PTLDS, for those who believe this is an actual thing), I think primarily for its anti-inflammatory properties. I think Aucott, well-known in the world of PTLDS and who is associated with Mark Davis through the Bay Lyme Group, recommends its use, although I cannot vouch it's for any T cell mediated purpose.

    I think it is also used to somehow affect dapsone when it is used.

    I have used it for all three (four) purposes.

    My eyes still see pretty much, usually...
     
    Last edited: Jun 21, 2018
    Binkie4, MEMarge and Inara like this.
  19. Little Bluestem

    Little Bluestem Senior Member (Voting Rights)

    Messages:
    1,447
    Likes Received:
    8,780
    @TrixieStix - it is so nice to hear that you are being referred to all of the medical specialist, now that you have a diagnosis. Getting treatment for what you actually have can be very useful. ;)
     
  20. TrixieStix

    TrixieStix Senior Member (Voting Rights)

    Messages:
    165
    Likes Received:
    1,212
    @Jonathan Edwards as always I very much appreciate your scientific analysis and input. Same also goes to everyone else who has commented & "liked" my posts :)


    I found this information online that says it is "current" & "updated" and my rheumy is one of the listed editors. I'm curious if after reading this you would revise any of your comments?


    "The etiology of relapsing polychondritis (RP) is unknown. Although few clues are evident, there appears to be a genetic susceptibility, an overlap with other disorders associated with immunologic abnormalities, and the potential for multiple inciting events including chemical insults. This hypothesis is supported by a series of observations which imply that RP is not a primary disease but a syndrome associated with multiple precipitating factors that appear in a genetically susceptible subject. The paraneoplastic myelodysplastic relationship suggests a potential pathophysiologic role for clonally expressed lymphoid stem cells in which functional T cell defects induce both autoimmunity and uncontrolled neoplastic hematopoietic clonal proliferation. However, a report of abrupt onset after recreational drug abuse implies that a direct biochemical insult also can induce the disease."

    https://www.uptodate.com/contents/e...lychondritis?topicRef=5587&source=see_link#H1

    The following is also in the article, but this portion is only accessible with an account...

    "PATHOGENESIS — Although the inciting cause may vary, increasing evidence derived from human and experimental animal studies has clearly implicated genetically preconditioned phlogistic and immunologic mechanisms that disturb connective tissue structure and cell function. In addition to the association with human leukocyte antigen (HLA)-DR4, the following observations are consistent with this hypothesis:

    ●The presence of autoimmunity, both antibody and cell-mediated, to extracellular matrix components of cartilage such as types II, IX and XI collagen, matrilin-1, and proteoglycan constituent fractions [10-12].

    ●Some observations support the contribution of a cell-mediated immune reaction to type II collagen [13]. As an example, T cell clones isolated from a DRB1*0101/0401 heterozygous patient with relapsing polychondritis (RP) showed specificity to a peptide derived from type II collagen that is an immunodominant epitope in a transgenic mouse model with features of RP. Of further interest are differences found in the epitope specificity for type II collagen antibody identified in patients with rheumatoid arthritis versus RP. Response in the rheumatoid patients was directed to an evolutionary conserved type II collagen domain that is also targeted by pathogenic autoimmune responses in murine models of arthritis but not RPC [14].

    ●The prominence of HLA-DR-positive antigen-presenting cells and CD4-positive T lymphocytes at lesional sites [15].

    ●Cartilage destruction may also be mediated by induction of apoptosis in chondrocytes. Chondrocyte expression of matrix metalloproteinase (MMP)–3 and cathepsin-K is strongly associated with this finding [16].

    ●The genetically governed experimental induction of features of RP following immunization of experimental animals with connective tissue antigens. (See 'Genetic predisposition in experimental models' below.)

    ●Selective decreases in the number and function of natural killer regulatory T cells (NKT cells), a novel lymphocyte lineage distinct from conventional T, B, or NK cells which are believed to be involved in control of expression of autoimmune disease by modulating the balance of cytokine expression [17].

    Findings such as these have led to the generation of a unifying hypothesis which takes into account genetic predisposition, the immunogenicity of cartilage, and specific immunologic pathways important in mediating the disease process."

    ---------------------------------------------------------------------------------------


    In regards to Plaquenil, I know that many other people with RP are also taking it, and I believe this may be due to the fact that with RP often comes other autoimmune issues. In my case biopsies show that I have severe Small Fiber Neuropathy (so much so that I have developed significant muscle weakness in my left leg that affects my ability to walk uphill and stairs). It is my understanding that SFN is not a symptom thought to be caused by RP. I also have significant sicca symptoms (decreased tear production & dry mouth) so I seem to also be experiencing Sjogren's-like symptoms (I am seronegative & not had a lip biopsy). It's my hope that if this is the case that the Plaquenil will perhaps slow down or stop further progression of the SFN. I will try and remember to ask my rheumy the question "why I am taking Plaquenil?" at my next appointment.

    I also have the whole genetic Complement deficiency thing as well and not sure how or if it may also be adding to my issues?

    As for the sun sensitivity, it actually started last summer (it's when I first noticed it) which is before I was on any of these recent medications. Last summer I began to notice that my fatigue, joint pain, and malaise would worsen in the hours/days after any considerable time in the sun. And my skin also began to get red, blotchy and painful like I had gotten a sun burn even with very brief exposures to the sun.

    Since I posted my update the other day I've had my most significant "flare" of my RP symptoms since beginning the Leflunomide. I remain hopeful as it's only been 1 month since I began taking 20mg daily. Fingers crossed I see a clear difference over the next 2 months. If not it will be on to the next drug.
     
    Last edited: Jun 29, 2018
    Binkie4, Inara, Daisybell and 3 others like this.

Share This Page