Hello, Thank you again to the kind folks that commented on my last posts re: Sudden Rise in ESR. Here is the post-appointment with the rheumatologist update.
The rise of the ESR from 0 to 93 in 2 months time is a complete surprise to the treatment team and is not considered chronic. In addition to the ESR of 93, all other inflammatory markers have risen to their highest point since diagnosis 22 months ago.
These treatments and their magnificent side effects have been endured: High dose steroids; high dose methotrexate; plaquenil; and Rituxan. The rheumatologist reports there has been "zero response to treatments". As methotrexate, now discontinued, caused frequent vomiting and diarrhea, extreme fatigue, and almost total hair loss, cyclophosphamide is not being considered due its higher occurrence of side effects. Long term high dose steroids, being continued, have produced Cushing's Syndrome. Plaquenil is being discontinued due to retina issues and unbearable tinnitus. Rituxan is being continued.
Neurological evaluations including MRIs, extensive blood work, nerve conduction and other horrendously painful testing have been conducted at 2 separate facilities. Both exams have ruled out neurological disease, including demyelinating diseases.
Hopefully there is simply a long delay (22 months) in response to medications. Not responding at all is beyond frustrating; it is terrifying to the rheumatologist's "first patient in over 10 years to have no response to treatment". I feel like I am a ticking clock with the alarm going off at whatever time the clock choses. My greatest fear is to be diagnosed with refractory rheumatological inflammatory disease.
People with rheumatological and immunological issues understand how infuriating these diseases can be. My family and friends are dumbfounded and overwhelmed, as am I. Thank you for your support.
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Are doctors exploring potential additional diagnoses on top of vasculitis that might explain lack of responsiveness to treatment?
Rituximab has worked for me overall but I still get flares (usually sinus related but also occasional joint problems) and my last kidney tests showed a decline in function after all previous tests had shown an improvement, so I’m getting more tests in the next few weeks to see what’s going on. Hoping it was a blip. So not as frustrating as you in that I have responded to treatment, but understand the frustration that there aren’t just easy fixes that simply work and we’re treated, feels like a constant drain keeping on top of it.
Really hoping treatment starts working for you. Keep us updated.
Hi Grizz! I was hoping you would comment. Yes, the doctors have been exploring, testing, examining for everything! I have been tested/am being retested/and retested for every rheumatological disease under the sun. So far I'm positive/active for ANCA vasculitis MPO, systemic sclerosis scleroderma, mixed connective tissue disorder, sensorimotor axonal polyneuropathy, Hashimoto's, hypothyroidism, Raynaud's in hands, feet, and ears (!). I might be missing something...I don't have the list right here. There are a few minor neurological diagnoses but after extensive testing by the Cleveland Clinic, they have been ruled out as inflammatory in nature.
I have all my fingers and toes crossed that the treatments, especially the Rituxan, works. The only Rituxan side effects seem to be needing a few days of naps due to fatigue right after the infusion. In between infusions, there are sometimes I just plain run out of gas and just don't function. I am unsure if that is the diseases or the Rituxan. Some days are tougher than others but I am trying hard to remain positive but the 22 months of near constant appointments, labs, medications, and tests are starting to really wear me down. It would be OK if there was some positive response to treatment but the lack of result is tiring. The waiting for answers/results and the not knowing are the worst part of all of this. My life is on hold while this is all going on. I'm ready to move FORWARD!
It is all tiring, really sorry to hear about all these ongoing issues. I also get bad fatigue after rituximab infusions.
Keep us posted. I’m always doing research into various alternative options so if I come across anything where there doesn’t seem to be harm in trying it I’ll let you know.
One of the medications I looked at a while ago when I was having flares is low-dose naltrexone. It is used to suppress the desire for alcohol with addicts in higher doses, and has been trialled for some autoimmune diseases. There's some early evidence it has worked quite well in relapsing polychondritis for some patients. It is off-label so needs consultant approval, but might be worth looking into as a non-toxic option to try out if other things keep failing and your care team need to get more creative. It's been suggested for nearly 10 years and not really got anywhere, but there are often commercial reasons for that.
Might not lead to anything but sharing in case you'd like to look into it. There are a few behind paywalls and this one is an article/video explaining it: pharmacytimes.com/view/low-...
Thank you very much, Grizz. I actually saw something about that in my research. I will read what you sent me. The rheumatologist is "considering IVIG" and sent me a ton to read on that, all her professional stuff. It is kind of hard as a lay-person but I'm getting through it. I'll definitely review what you sent me. Thanks, Grizz, I appreciate it.
Oh Grizz, sorry but there is one more thing. You said you took naltrexone for flares so can it be assumed that you were in remission when you took it? (I am not in remission...) Is it a consideration for induction of remission? I reread your posts and if I read them correctly, Rituxan worked for you to achieve remission? And naltrexone added for when you had flares? Did I understand that correctly? Thank you.
I haven’t taken it yet as I managed to get flares under control but it was something I was considering and wanted to ask my consultant about. I have read about people who couldn’t get into remission with other autoimmune diseases who took this and it worked well for them. They had flares still but they were minimal and thus got them into remission.
I don’t think there’s any one treatment that works for everyone as autoimmune issues are complex, but this does look like an option for some people.
Blimey mate, right through the ringer! Poor lad. I wish I could give you some advice but I can’t because things have worked for me so far, however I thought last week when I had my scheduled meet with the Consultant that would be it no more treatment but she has decided to do another year with Rituximab and more or less said it up to you. I do get side effects with Rituximab unfortunately, usually about 4-6 weeks in. If I can’t give you advice maybe I can give you a little hope. My friend Dev who I worked with on the Railway way back in the 80s has GPA, diagnosed about 2 years before me in 2019. He, like you had bad side effects with Cyclophosphomide and Methotrexate made him really I’ll, the Rituximab wasn’t bringing down has marker and 18 months in they were at a loss. Well the Rituximab did kick in after nearly 2 years / 4 infusions, the medical people said even though it’s not supposed to make any difference the Rituximab had reacted with other medicines. Hang in there. Nick
Hi Nick, Thank you. I am willing to wait for the Rituxan to have a chance to work but after 22 months, super high inflammatory markers and the sudden ESR of 93 (from 0) the doctors are finally paying attention to me. That is what really makes me nervous because previous to this, I was an insignificant bug on their windshield. I will definitely DEFINITELY keep hanging. (Oh, I am not a lad, I'm a lassie... no worries there as long as my fiancé knows, I'm flexible.)
sorry about the gender confusion on my part. Apologies. I totally get where your coming from about the jump and your anxiety, I can’t imagine how that feels. When Covid came upon us they stopped my Cyclophosphomide as they didn’t want me near a hospital and put me on Azathioprine, it was like flying a plane from an aircraft carrier knowing you couldn’t get enough speed to take off. I was so anxious and I think the worry was contributory to my relapse. But I got through it……eventually, but had some dark thoughts. I am glad though people are now noticing you. Take care. Nick.
Hi Nick, Wow, I fully understand: Everyone in my "Rituxan infusion pod" got Covid last June. Then I got pneumonia. I recovered from the pneumonia but then covid again from my time in the hospital. Ridiculous. The infusion center has 55 to 65 of us packed like sardines in a little can. I wear a mask but the nurses/staff DO NOT! Your choices are insane: Rituxan and covid or no Rituxan. Complaints, in writing, are totally ignored. It's all about the financial gain (and it's a non-profit facility). Sure it is...what's the non-profit CEO make? Good thing I like sardines...
hi. Sorry I missed your previous posts. When you say high dose steroids do you mean 60mg prednisone?
My Inflanation markers don’t go up by much, but when they do I know something is going on ( as well as the way I’m feeling) high dose steroids usually start helping in days for me, and staying on them until the markers start to come down. Reducing them quickly causes more issues. Adding in the autoimmune suppressants helps further. I had methotrexate the first time (many years ago), but my liver wasn’t happy this time to we switched to mychlophenolate which (fingers crossed) is doing the job at the moment. Sorry not much help, but I wish you well in finding your answers.
Hi Smiley, Long term high dose prednisone that caused Cushing's syndrome and is currently being reduced. The high inflammatory markers and ride in ESR happened PRIOR to the start of the prednisone reduction. Prednisone produced the classic steroid face and a scar on my right cheek directly below my eye from a cut that received stitches when I was 4 years old swelled to the point of bursting. I hadn't even seen the scar, it was pencil line thin, for decades. The prednisone made my face swell and the scar GREW and BLISTERED. It was impressive. The hematological oncologist said "we have to reduce the prednisone right away". I also had the usual prednisone side effects: fat deposits, etc. Big FUN. Methotrexate, besides making me quite ill, also effected my liver. Nasty stuff. If methotrexate does not work for you, it does NOT work for you and goes out of its way to show its displeasure! Here's hoping the Rituxan is just being slow to be effective. Also, I have never been in remission in 22 months since diagnosis so the inflammatory markers are of an already active disease(s). Thanks for the Crossed Fingers. It shall be my new nickname.
I’m okay - just getting over a dose of pneumonia thanks for asking. I’m sure I’m in a better place than you are at the moment. Don’t give up hope you will get there eventually. 🤗
I can only tell you what worked for me. I was started on 50 mg Azathioprine and 25 mg Prednisone. After 9 months, I was given 4 infusions of Rituxan one week apart. Four months after that, I was given 4 more infusions of Rituxan one week apart. The other two oral drugs were continued for another year then stopped all together as it appeared I was in remission. I had kidney failure with my disease and my Nephrologist manages my GPA for me. He said he studied Wegeners/GPA when he was in Med school because of the affect it can have on kidneys. He told me he stays clear of Cyclophosphamide and Methotrexate because of the terrible side effects. I was corresponding with a UK doctor on Quora and he affirmed to me that in Europe, doctors prefer to use those two drugs but I can't understand why. There was a doctor at Cleveland Clinic that did a report years ago and it showed that the combo of Rituxan and Azathioprine are more effective than Cyclophosphamide and Methotrexate. Maybe it's because of cost or maybe they're just having a hard time changing to something new or different.
Thank you. I plan on continuing the Rituxan but will NEVER take methotrexate again! I am seeing a doctor at the Cleveland Clinic and have another appointment next month. I have a wonderful nephrologist and she has had 2 vasculitis clients prior to me so she has some experience. Other than that, I'm stagnant regarding treatment. I feel like a walking time bomb, never knowing when something is going to happen. I have had 2 pulmonary emergencies and I am tired of walking on egg shells. Thank you for sharing your experiences and insights.
Hi, Here is the Mayo Clinic definition:Sed rate, or erythrocyte sedimentation rate (ESR), is a blood test that can reveal inflammatory activity in your body. A sed rate test isn't a stand-alone diagnostic tool, but it can help your doctor diagnose or monitor the progress of an inflammatory disease.
When your blood is placed in a tall, thin tube, red blood cells (erythrocytes) gradually settle to the bottom. Inflammation can cause the cells to clump. Because these clumps are denser than individual cells, they settle to the bottom more quickly.
The sed rate test measures the distance red blood cells fall in a test tube in one hour. The farther the red blood cells have descended, the greater the inflammatory response of your immune system.
I hope that helps! There are a few posts here, too.
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