So I’ve almost arrived at the final diagnosis after spending a week in the hospital surrounded by specialists and looks like it’ll be UCTD. The reason is my labs all point towards Scleroderma but my symptoms say Lupus and they assumed Lupus for a year now, but never popped positive on AntiDsDna and a couple other blood results that were needed. I am kind of ok with this but also it’s so weird to be feeling better knowing they don’t know if that makes sense hah. Anyhow, I’ll meet mid July for treatment plans. With this said; I see that 40% of UCTD does end up something eventually and in my case I’m guessing Lupus IF it doesn’t just stay UCTD (50% chance). Have any of you been diagnosed as UCTD? Did it eventually move to a more specific disease? How do you feel about the whole thing?
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I'm pretty much the same UCTD with lupus symptoms, but one odd complement blood result 'high C2' which can happen in Scleroderma.
I hoped vit D might correct it but realise had a lot of these symptoms for long while..
The new 2019 EULAR/ ACR SLE criteria, classification criteria intended for grouping patients in research, seem to have been applied to diagnosis - which maybe was not the intention.
I have most of the 11 symptoms previously referred to by ACR, so before 2019 may have got an SLE diagnosis
I tell people this so they understand things. Some people have heard of lupus, very few have heard of UCTD.
They, understandably, just switch off if I say I have been diagnosed with "Undifferentiated or Undefined Connective Tissue Disorder".
Hey
I’m so pleased your inpatient tests resulted in answers for you. It’ understandable that you’ll feel a sense of relief at knowing the score now the ‘beast’ has a name, so now it’ll be on to taming it! 😄 It’s the not knowing that is the worst.
A lot of us overlap to some degree, whether you’ve got Lupus or not. It’s whether the results get you to the Lupus threshold as per the Eular diagnostics list. At least you’ll soon have a plan for treatment going forward, as the initial treatments are the same. Your autoimmune markers may change once you’re receiving treatment 🤞🏻 and your symptoms lessen once you’re on the right meds too🤞🏻and hopefully then full antibodies to Lupus or Scleroderma won’t then develop.
Definitely look into pacing techniques, these will help in conjunction with meds. I’ll find a decent link and add it here. I think Lupus UK did a fab article on it recently (unsure whether it was in the magazine or online), can’t currently find the actual one… 🤔 Anyway, this will help manage fatigue as I recall this was a major issue for you.
Here’s a link for different article on pacing from Lupus UK-
Also, as you’ve the Lupus type of UCTD, then I’d definitely recommend checking out Lupus UK generally-all their articles will be relevant to you too. Plus we’re all here too of course!
This link below is from the ME Association, which explains pacing in greater detail - pacing is used in lots of chronic illnesses that have fatigue as a major symptom, so it’s still relevant, even though it refers to ME.
UCTD is almost pre Lupus and is treated as Lupus. In my view it is Lupus although I am not a doctor. I was crying to my phycologist at some point, quite few years back saying 'doctos keep saying I have UCTD but I think I have Lupus".
He smiled and said "Ms Lale, that is Lupus and your doctors know it'. So I would assume that you have it. Its auto immune disease without a Lupus is the umbrella name. Cover many auto immune disease. My view only and my doctors. 😇
I was diagnosed with MCTD and like you I had many weird illnesses for about 9 years, I was put on hydroxy. I had a bad flare with tenosynovitis and a horrible rash all over, managed to get an appointment within 3 weeks with my Rheumy, I was put on steroids and sent to a dermotologist for a biopsy. She diagnosed it as a lupus rash. I’ve had loads of rashes before, but this one just wouldn’t go away so it was finally good to see the right person while I had the rash. It’s such a hard one to get a diagnosis, I didn’t think I would ever get one, I was very surprised to finally get one.
Yes same, uctd with lupus features is my diagnosis and sjogrens. Hydroxychloquine .
Apparently it comes under the disability act but when I'm off ill with the disease the secretary can't log what my illness is as it isn't there on huge list of illnesses.
She actually showed me and there's arthritis, fibromyalgia etc but no lupus.
I'm in a school working for the Council so I find it very disappointing . Like I'm not recognised
weird…just wondering if the lower subtitle at that page’s header explains why there is nothing, ie SLE, UCTD etc are not common…they meet the criteria for classing as rare:
“There are over 370 topics, with focus on the most common and significant presentations in primary care.”
thanks for this v interesting discussion! Am following it closely because although I’ve long been officially diagnosed with SLE & Sjogrens, I’m seroneg…& unlikely to ever make the relevant antibodies due to my PID (the Primary Immunodeficiency Disease called panhypogammaglobulinaemia, aka Antibody Deficiency Disease)
You’ve probably found these already, but, for anyone reading your thread who hasn’t, here are 2 links I just found to official U.K. info re what qualifies as a rare disease
Thanks for sharing. I guess some of us, diagnosed recently with UCTD might also have other underlying additional differences that factor us out with the 2019 EULAR/ACR immunological tests results being attainable.
I can't understand why my C2 is high, as this might raise low C4 and C3 I'm unlikely to get low C4 and C3. It looks like a compensation process to me.
I'm going to read up on 'antibody affecting diseases', as know I have most of symptoms of SLE, and really relate to it.
you’re welcome! There’s an awful lot even the most cutting edge experts admit they don’t understand yet. Rheumatology & immunology need to admit they’re in an unacknowledged marriage! It’s so obvious we need both disciplines to at least run basic investigations on us All!
I think you already know of this 2022 paper re seroneg lupus:
With each set of blood results I calculate 'Neutrophil: Lymphocyte ratio and Immature granulocyte : Neutrophil ratio
Higher values are clearly corresponding to when I feel worse, and continue to go up with each flare.
When patients are first referred, and are not yet on immunosuppressive medication, why are they not looking at these sorts of markers? Much simpler too..
I'm sure there are other markers that we don't hear much of also, that might work..
so crazy cuz I read this article once and my ratio is quite high especially during flares even if they’re “normal” when measured as completely neutrophils etc ! But I go below into the red with neutrophils and lymphocytes often back and forth when one goes down the other goes high
If immunological criteria continue to focused on, and yet knowledge of the entire process is so limited, then there will be a big gaps and patients will get missed.
Hopefully those who come up with an update to EULAR / ACR 2019 will acknowledge these things, we can only hope.
and this paper specifically refers to seronegative AID in CVID patients (CVID is the type of PID I have) - see the section on autoimmune rheumatologic disease:
Yup! basically, lupus is an umbrella & the most complicated part of all CTD which in themselves are complicated…what’s often neglected is clear acknowledgement of the simple fact that all these conditions are also immune dysfunction conditions…that’s my mantra: our conditions should be cared for via the ‘wedding’ of rheumatology & immunology 🤷🏼♀️…a collaboration💞
Good question! in my case, rheumatology & immunology got together because:
1. my GP mistakenly referred me direct to the CUH chief @ the Metabolic Bone Unit which is part of the rheumatology Dept, instead of just messaging my rheumatologist direct. And the MBU Chief ordered an immunologic profile alongside a bone profile blood tests…which threw up red flags requiring both urgent bone cancer blood tests AND urgent referral to immunology
2. AND my rheumatologist was already well aware my immunoglobulin results were below normal range + that I had a lonnnnnng documented history (pre-imm7nomodulation meds) of multisystem complex persistent pattern treatment resistant infections, which of course indicated immunology needed to investigate my case..& lucky for me, my Univ hospital has a famous Immunology Dept
Possibly the biggest prob for us is that immunology depts are few & far between in th3 U.K., as are immunology consultants 🤷🏼♀️💞
As well as not getting the diagnosis we would have in the past it also means we can't get involved in research.
Yet we have the same symptoms and need the same treatment as those who still get a lupus diagnosis.
Where is the research on UCTD?
I guess research is concerned with drug trials etc, but not so much on investigating the immunology otherwise they would be wanting our involvement too. Quite sad really.
What makes you think the diagnosis has changed to such a degree? Some of the major changes in the lupus classifications over the years involved recognition of new antibodies like the anti-cardiolopin antibody that was formerly only represented by the false positive VDRL, the removal of duplication in the cutaneous realm, and a new weighted scale that allows recognition of lupus-like states and also severe lupus.
There is no agreement on a definition of undifferentiated connective tissue disease. Some rheumatologists prefer to call it latent lupus, autoimmune rheumatologic illness NOS, or lupus-like illness.
Studies do seem very limited with undifferentiated connective tissue disease. If it truly is stable UCTD it won’t likely be organ threatening. I guess they just use studies from lupus or Sjogren’s or whatever symptom is most prominent.
Studies have shown UCTD is associated with interstitial lung disease. They must be watching that with us because it is certainly serious.
Let’s hope more studies will be conducted in the future. I have never found that my diagnosis is an issue in terms of treatment. The one time IVIG was considered, the same conditions had to be meant whether it lupus or UCTD.
I did see a study on quality of life of patients with UCTD. In general, quality of life is similar to lupus. Patients struggle with confusing over diagnosis. I certainly did. If you are interested in seeing it, I will try to find it.
Try to remember that any serious symptom will be treated regardless of diagnosis. One of our members was in the hospital with serious inflammation in her eye. The doctor in the A & E didn’t say « not sure what this UCTD is, so no worry. » He saw what he thought was a problem, noted her autoimmune disease and got the ophthalmologist on call immediately. It is an important lesson for all of us. She is watched very closely by the ophthalmologist who stays in contact with her doctor.
Hi there, I was told I had UCTD a year ago when finally a rare antibody was found (anti-Ku). I've collected a whole bag of symptoms & pains over the past 10 years (including erythromelalgia & reynaud's) so it was a relief to get a diagnosis. I'm having a second try of HCQ soon, I gave up before due to gut problems (taken for 3 months) . As for evolving into something more defined, and potentially worse, I try not to think about it. It may well not happen - we don't know the future and what our health maybe like in 5 or 10 years. But it must be more of a worry when you are young. Good luck to you. x
I understand why you are concerned then. If your autoimmune disease is affecting your kidney, the name should not matter. When I had fever, red cells and protein my urine, I had weekly urinalyses and a 24 hour urine for protein. Your GP definitely needs to be alert. There is never a guarantee someone will stay undifferentiated and inflammation in the kidneys tips the scale.
The classifications have nothing to do with drug companies. The latest one was a multi year process with experts from every disciple from all over the world on the committee. They test and re-test for sensitivity and specificity. The classification may be research classification but it still informs opinion. Young doctors have to learn this. When I was diagnosed there was not even a test for certain antibodies. My young doctors are amazed when I tell them my blood was sent to someone’s lab.
The clinical picture is always the most important. Good rheumatologists can see a lupus case referred to them before an ANA is done in most cases. There are always the grey areas like us, though.
There are agreed experts in any field. They would be chosen based on status at an academic institution, their research, long-term interest in lupus. I don’t know how they are chosen, but just like the journal editors and research principals, they have to have stellar reputations.
As an example, one of my doctors is a leader in the field of preventative cardiology and lipids. He is the principal on a drug trial that could save millions of lives if the Novartis drug is successful. The academics at the top of their fields are known. It would be like Professor Hughes in lupus research and APS.
My rheumatologist would never be asked to be on a lupus classification committee. He just isn’t at that level academically.
It is not about being a great rheumatologist. It is about being an expert in lupus. I just saw two names that I recognized. One was a researcher and one is Daniel Wallace who wrote the Lupus Encyclopedia.
The names on the list are German, French, Spanish, Dutch .. These classifications are taken very seriously. It affects our understanding of what a disease is and whether research is valid.
Donald Thomas wrote my copy of The Lupus Encyclopedia He is very caring. So many good videos. He is passionate about really helping patients...hence the lovely book.
I'm not sure he gets onto these committees.
Think he would be brilliant as he is so concerned about patients not missing out on treatment. Which can happen when NHS is stretched and this research classification scheme is mistakenly used to decide who is treated and who gets discharged.
That was my experience of being 'uctd-ed' no treatment on NHS..
If they are clever, why don't they think about the implications ..I would be devastated if something I did meant patients were not helped who needed to be.
Also I believe childhood lupus can more difficult to diagnose using the 2019 criteria and early on people are not getting the right test results always. Someone with Lupus Nephritis who is ANA negative..does not even qualify.. so other 'serum negatives' should not be being excluded either..
The tests are not accessible enough or standardised enough. % positive can depend on test protocol.
I think even different ethnical groups may fair differently using 2019 EULAR/ACR..when last Googled the research..
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