There are around 500 cases of this in the world, so is extremely rare, which means diagnosis becomes even more difficult.
CG as its referred to is a form of multi vessel vasculitis so symptons end up pretty broad.
Why so few cases is a lot of researchers believe there a lot of undiagnosed cases out there.
For those on this forum like myself struggling with both the disease and getting a diagnosis, this disease is worth looking into.
Unfortuneately there is scarce information out there and no studies have been carried out yet on this illness.
Additionally no standard treatment is used, but instead individual cases are treated patient specific by Rheumotologists, although with inner ear and eye involvement, ENT and OPTHAMOLOGISTS specialist have to be involved as well, again making diagnosis more difficult.
From 1980 CG was classified into TYPICAL and ATYPICAL sub types, with or without multi vessel vasculitis. Its a vague classification from what I have read.
Blood tests except for ESR/CSR all come back negetive, as does CT and MRI scans. Some positive results with PET.
In my case I have tested positive for HSP70 antigen, doesnt give a 100% diagnosis, but this result was ignored by 4 ENT specialists, who dismissed it entirely and one even commented it was just a fasle negative as did my Rheumatologist. I got this test done on my own back thru GP as we were looking into AIED at the time.
Would encourage anyone who is still struggling with diagnosis with symptoms not fitting the more common autoimmune illnesses to research CG and also share symptoms and experiences on this thread.
regards
OZ
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There seem to be more and more auto immune diseases appearing. I do suspect that a lot of them are close cousins of many of the others. Have you been given steroids? People with the more common auto immune diseases have problems with diagnosis as well. Some people waiting literally years.
According to the paper I've linked elsewhere in this thread, the recommended starting dose for Cogan's syndrome is 1 to 2 mg/kg/day. However, it doesn't say which corticosteroid that refers to, and potencies vary.
The autoimmune neurological disease MG (myasthenia gravis) has the same prednisolone starting dose per kg of body weight, up to a maximum of 100mg/day.
The normal starting dose for myasthena gravis is around 20-60 mg as a friend has it and we check with each other on our dose. For GCA - giant cell arteritis it is around 60 starting dose and can be higher. Over 40mg is considered a high dose. In my thinking 25mg is quite high and can cause some miserable side effects!
It depends on the severity of the MG symptoms and the mass of the patient in kg. The top-end doses (1 to 2 mg/kg/day up to 100mg/day) are for patients in imminent danger of a myasthenic crisis, which usually means the muscle weakness has reached the throat/diaphragm/chest muscles and is affecting breathing. Once the crisis is averted, the dose is then reduced (tapered) while keeping symptoms under control. Steroid-sparing drugs and/or AChE-inhibitors are often introduced in parallel, of course.
For non-emergency and milder cases, this 2022 paper (section 3.1) says a lower start and upward titration can also be used:
"Starting treatment with low-dose prednisone (≤25 mg) with gradual escalation has been suggested to avoid steroid-induced paradoxical weakness. As such, a low dose and slow [upward] titration approach in patients with mild to moderate oropharyngeal or respiratory symptoms is recommended in outpatient settings to avoid the transient weakness worsening—initial prednisone dose of 5–10 mg daily with a gradual dose escalation of 5 mg every 7–10 days up to 60–80 mg daily or until desirable symptom control is reached."
thanks ATOPIC, it was my blood sugar levels spiking to 20 on 25 mg that had me concerned. My GP is very careful, i will see if he would be happy with 5-10 mg, I am already on leflunemide 20 gm (a DMARD), MXT couldnt tolerate, next step would be biologics but does that mean I have to stop the Leflunemide ?
I don’t think people who don’t have very rare conditions grasp how isolating they are. I have Systemic Sclerosis, which took 12 years to diagnose correctly and unequivocally. 19,000 people in UK have SSc but only 4% of these will carry my rare scleroderma antibody and scleroderma antibodies very much dictate the course the disease will probably take. I was evaluated for AIED some years ago when my main diagnosis was Sjogren’s and I have profoundly deaf siblings - so I did come across Cogan’s in my research but didn’t realise how rare it is. The ENT phoned me recently when I was re-referred by my rheumatologist for worsening pulsatile tinnitus and vocal cord problems where my voice fades. He thinks it’s all part of my systemic sclerosis - which can attack any part of us. Interestingly my deaf brother in law sent me an article about comedian, John Bishop, and his son who has Cogan’s. I’ve just found it and thought it might be helpful in case you aren’t already aware of this: amp.theguardian.com/tv-and-...
I think you will find that even people with cancer can feel totally alone. It depends very much on your medical support and your friends and relations. You are the only one who really understands how you personally feel.
That isn’t what I’m taking about though. I mean that rare diseases often take much longer to diagnose because no one, including the majority of healthcare professionals, know/ recognise their signs.
And perhaps, most importantly from a day-to-day perspective, there are very few patient support organisations, including charities, for those with rare diseases and their families to turn to. Even rare forms of cancer come under the Cancer umbrella which means they can drop in to a Maggie’s Centre or qualify for MacMillan support. Also the more common the umbrella condition eg cancer, diabetes etc the more endowment funds and research into new treatments there is. Hospital rooms, treatment centres, drop in facilities, phone helplines and cancer wards around the UK tend to be much nicer, smarter and generally better, however rare the type. Yet MND and Huntington’s and my own disease, Systemic Sclerosis or rare types of Vasculitis or genetic/ hereditary conditions can be just as terrible to live with and die from. And I say this, thinking about the experiences of a local friend who has recently survived and been told he’s been fully cured of a very rare type of blood cancer. Within the same hospital and amongst his family and friends, he has had incredible support compared to myself or those I know with rare to very rare conditions. This is what makes them uniquely lonely whether it’s when a person phones 111 to answer a chain of questions about whether we have common conditions, assuming that if we don’t then we are less urgent, or whether when newly diagnosed and we have to tell our loved ones who have no idea at all of what this means and they have to Google or assume that if they haven’t heard of it then it can’t be too bad.
I do agree that less money is spent on development of drugs on rarer diseases by pharmaceutical companies as they go for their sales figures. However we also rely on someone making the correct diagnosis which does seem more hit and miss. I was reading only today that someone had died of cancer who had been diagnosed with anxiety by his doctor over quite a while. You hear this again and again. They reckon forty per cent have an incorrect diagnosis when they first visit their GP. In probably a lot of cases it does not matter as people get better anyway.
I know - not disagreeing that misdiagnosis is all too common - this is indisputable across the board. But I’m responding to the post which is about a very rare type of vasculitis, Cogan’s. And I still maintain that rare conditions are uniquely isolating - diagnosed or undiagnosed. Having spent 12 years being misdiagnosed with various conditions, now fully diagnosed with severe GI involvement requiring stoma on a fortified liquid diet. This has progressed aggressively in part due to misdiagnosis, I always empathise and relate most with the rare disease community. I also know from personal experience that it’s much, much harder to get support online and support from within the community for rare conditions. I have a few common conditions too such as arthritis, skin cancer and hypothyroidism so I do know what I’m talking about.
thanks TED, I also have GI involvement, 15 years ago had a hemicolectomy for lower half of bowel, and iliec small intestine biliary colic a few years ago, thes MRI or CT report mentioned mesentary vessel vascularity
I do agree it is great being able to talk to people who understand your illness. I have PMR and most people have never heard of it, neither had I for that matter. I have discovered people are not interested in other people’s illnesses unless they have the same. Diagnosis is important as at least you then know what the situation is. Misdiagnosis is also a worry. It became particularly bad during Covid as everything was on the phone.
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