Undiagnosed - Clear scopes, high calprotectin

Hi guys,

I'm new to this forum and was hoping for a bit of advice. I posted this in introductions and I had a reply suggesting I post here, I hope that's okay.

I'm 25 and female. I have had a lot of clear results initially but it's the last one I had done I'm worried about and hoping for advice... I'm sorry this is a long post!!

For most of my life (since around 11) I have had episodes of abdominal pain after eating and severe weight loss. However things worsened quite badly around 2007ish and I got referred to a gastroenterologist.

Sept 2008 I had a clear colonoscopy and endoscopy. Marginally raised (and I mean pretty marginally) faecal calprotectin. High ESR. GI discharges me, IBS.

Less than a year later I develop chronic diarrhoea with bleeding and mucus. At first it was about 10 BM's per day, difficult and painful to pass with severe stomach cramping, went back to GI. I then had a flexible sigmoidoscopy which showed internal haemorrhoids, presumed from all the loose stools. GI determines this is where the bleeding is coming from.

At the last minute he decides that the high ESR needs investigating and does an autoimmune panel. It shows high rheumatoid factor and a visit to a rheumatologist later I am diagnosed with sjogren's syndrome. This is an autoimmune disease that normally attacks the moisture producing glands of the body. GI symptoms of this severity are very unusual for sjogren's syndrome, you can get a kind of IBS but as far as I'm aware no inflammatory type of thing. Neither GI nor rheumatologist knows what to do, GI sees me every few months. Repeat clear endoscopy. They try me on hydroxychloroquine for the sjogren's. By this point I also have abnormal liver function, high CK, low WCC, +ve anti-ro, anti-la etc. ESR comes down to normal in hydroxy, liver function near normal.

I struggle on getting worse. I lose 4 stone in weight and start to look very sickly, barely able to eat. Whenever I would eat I would be literally unable to walk or stand up. One day I pretty much collapsed at work and my boss (a doctor) sent me to A&E. They did bloods, urine etc. All normal (as the hydroxychloroquine had 'fixed' the high ESR etc) and discharged me with 2 paracetamol and told me to phone my GI.

I phoned my GI, told him what happened, he saw me for a consultation, decided I looked pretty sick. My stomach was literally covered in bruising as the only way to get any relief from the pain was to pinch my skin which to him indicated the severity of the pain. He found a bed and admitted me to hospital the next day.

I was in for three weeks. I was supposed to have NG/PEG feeding by this point as my weight was dropping stupidly fast but I could not stomach the thought of it and refused. When I was in hospital I was having NO diarrhoea I should note but having severe epigastric pain after eating. Pretty much continuous and through the night. Dihydrocodeine and paracetamol didn't even touch it. It was a struggle to walk to the toilet and I would often be near wetting myself scared of the pain of having to get out of bed.

While in hospital I had the following

1) Normal faecal calprotectin
2) CT scan showed fatty liver disease and being 'full of stool' but nothing else
3) Clear MRI thorax for lymphoma
4) Clear capsule endoscopy (without a prep though)
5) Blood test after blood test, only new thing was low complement

The focus was mainly on stabilising my weight which we did not really manage to do. I was told to come off hydroxychloroquine in case that was the cause. It did help, but I was still in pain every day. At this point it was presumed to be psychological (?!) but this has since been disproven after a years evaluation from a psychologist.

THEN... this is the 'interesting' part.

This summer I started to get super sick again. Continuous epigastric pain through the day and through the night. Noctural diarrhoea (which was new) 3 or 4 BM's per night. Completely unable to sleep but no weight loss.

GP does bloods which shows my ESR is the highest it has ever been by quite some way at 51.

As my GI just thinks I'm nuts and discharged me I called my rheumatologist and begged for help. I saw a diff doctor this time who upon telling him my symptoms said 'you might have serositis, we'll try you on steroids'

I go and have a steroid shot and the diarrhoea stopped virtually overnight, enabling me to sleep but the epigastric pain continued. I see my regular rheumatologist who then starts me on the following prednisolone regime

50mg 7 days
40mg 7 days
30mg 7 days
20mg 7 days
15mg 14 days
10/15mg alternate 14 days
then 10mg

I am currently on 15mg.

It has nearly cured everything!!!! I am able to eat full meals without pain but unfortunately am piling on the weight as a consequence I still have about 1 episode of diarrhoea a month which is very painful but generally over in one BM and controlled easily with one tab of imodium. A huge, huge, huge improvement.

HOWEVER...

They did a faecal caprotectin which was 227 and 'consistent with active GI inflammation'

All my consultant did was send me a copy of the result with NO explanation of the implications and I've not to see him for another 2 months!! And I've not to see my GI for over 6 months! I thought this test was related to crohns/colitis. I don't think it CAN be related to serositis/sjogren's.

I know 227 is not a phenomenally high result (Also it was done 4 days after the steroid shot which may have affected it) but my symptoms are certainly not NEARLY as bad as what I have read on here. I'm not going 40x a day and never have been.

I'm just a bit confused about what this result means considering everything had been clear so far. Bloods since the steroids show my ESR is 2! Yes 2!! and my liver function is great. I have never in my puff had an ESR of 2. Also I no longer have palpable lymph nodes and other telltale signs the inflammation has gone down.

You're a trooper if you've read this far and I really appreciate it!

Any thoughts?

Hi lsgs, welcome to the forum and the club. You've certainly been through a lot, poor thing! I don't have much in the way of advice but I do hope you get some answers soon. One thing that did spring to mind when reading your post, you certainly have something inflammatory since you had such a splendid response to pred - unfortunately though, pred is a systemic med, so it stops inflammation essentially anywhere in the body. So the source of your inflammation would still be a mystery and you could have been inflamed in multiple areas. So I'm sure your doctors are confused, it's great that pred worked but unfortunately doesn't really shed much light on anything, other than your illness is definitely inflammatory in nature.

One thing I will say, can you switch doctors and get a second opinion? It sounds like your current docs are throwing up their hands and saying it's all due to sjogren's/IBS/all in your head/whatever. Getting a fresh pair of eyes on your case could be beneficial as it certainly sounds like you're getting nowhere fast with your current team.

And if it's any consolation, I'm currently undiagnosed myself and I also responded really well to pred. That stuff is like magic and I'm pretty sure it gave me superpowers, I felt so amazing when I was on it. Anyway, you're not alone! I hope you can get some answers soon, hang in there and keep us posted.

Hi Isgs, given your severity and length of symptoms with a clear colonoscopy, normal colonoscopy and capsule endoscopy, I think it would very unlikely that Crohns would be driving those symptoms.

If you concentrate on your key symptoms - post prandial (after food) pain with and aversion to food with subsequent weight loss and diarrhoea, and then look at your CT which showed faecal loading. You're on narcotics and your symptoms were profoundly steroid responsive.

1. Your symptoms of post prandial pain and weight loss are typical of mesenteric ischaemia, however that is more common in elderly or those with significant vascular risk factors, and also, you wouldn't expect it to be steroid responsive. However, if the mesenteric ischaemia is caused by a vasculitis, which can be seen with autoimmune conditions (like Sjogrens), then this would be steroid responsive. Not very common though.

2. Another steroid responsive condition that might explain some of the findings : eosinophilic gastroenteritis, although no eosinophilia seen on bloods nor changes on endoscopy or capsule. Very rare also.

3. Given the faecal loading, could it be overflow diarrhoea, perhaps with IBS overlay?

4. I'm not sure how much narcotic you're on, but could you have Narcotic Bowel Syndrome - this would explain your CT findings and some of your symptoms, but not the steroid responsiveness.

Ignored the subsequent positive faecal calprotectin, might muddy the waters a little.

Difficult case. Good luck!

Cat-a-Tonic said:

Hi lsgs, welcome to the forum and the club. You've certainly been through a lot, poor thing! I don't have much in the way of advice but I do hope you get some answers soon. One thing that did spring to mind when reading your post, you certainly have something inflammatory since you had such a splendid response to pred - unfortunately though, pred is a systemic med, so it stops inflammation essentially anywhere in the body. So the source of your inflammation would still be a mystery and you could have been inflamed in multiple areas. So I'm sure your doctors are confused, it's great that pred worked but unfortunately doesn't really shed much light on anything, other than your illness is definitely inflammatory in nature.

One thing I will say, can you switch doctors and get a second opinion? It sounds like your current docs are throwing up their hands and saying it's all due to sjogren's/IBS/all in your head/whatever. Getting a fresh pair of eyes on your case could be beneficial as it certainly sounds like you're getting nowhere fast with your current team.

And if it's any consolation, I'm currently undiagnosed myself and I also responded really well to pred. That stuff is like magic and I'm pretty sure it gave me superpowers, I felt so amazing when I was on it. Anyway, you're not alone! I hope you can get some answers soon, hang in there and keep us posted.

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Hi! thanks for the reply. I agree it's very tempting to change doctors and VERY tempting to switch to the one who prescribed the steroids for me as my current rheumatologist likes to 'sit on the fence' as he admits himself. I may need to consider this. I am at that point where I feel like no one believes me and thinks I'm nuts and have lost hope of that changing. However with the recent reaction to pred and the high calprotectin results I'm hoping this won't be the case any longer. Also the psychologist who evaluated me has written to all doctors concerned to say that there is NO psychological basis for these symptoms, which is great.

Aussie said:

Hi Isgs, given your severity and length of symptoms with a clear colonoscopy, normal colonoscopy and capsule endoscopy, I think it would very unlikely that Crohns would be driving those symptoms.

If you concentrate on your key symptoms - post prandial (after food) pain with and aversion to food with subsequent weight loss and diarrhoea, and then look at your CT which showed faecal loading. You're on narcotics and your symptoms were profoundly steroid responsive.

1. Your symptoms of post prandial pain and weight loss are typical of mesenteric ischaemia, however that is more common in elderly or those with significant vascular risk factors, and also, you wouldn't expect it to be steroid responsive. However, if the mesenteric ischaemia is caused by a vasculitis, which can be seen with autoimmune conditions (like Sjogrens), then this would be steroid responsive. Not very common though.

2. Another steroid responsive condition that might explain some of the findings : eosinophilic gastroenteritis, although no eosinophilia seen on bloods nor changes on endoscopy or capsule. Very rare also.

3. Given the faecal loading, could it be overflow diarrhoea, perhaps with IBS overlay?

4. I'm not sure how much narcotic you're on, but could you have Narcotic Bowel Syndrome - this would explain your CT findings and some of your symptoms, but not the steroid responsiveness.

Ignored the subsequent positive faecal calprotectin, might muddy the waters a little.

Difficult case. Good luck!

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When I was in hospital last year I think they looked for vasculitis. Their main suspicion was pancreatitis but they couldn't find any evidence of it although my pancreatic amylase has been raised at times. They did bloods and I think one of the scans looked at it (vasculitis) and there was no evidence but again my symptoms were very different back then to what they are now. For example I have maintained a steady weight for over 6m and diarrhoea was not as predominant back then.

Interestingly I DO have eosinophilia... But again this CAN be a feature of sjogren's so has largely been ignored.

I have all sorts of weird and wonderful blood results which have never been pieced together. I wish they would communicate more with me. He mentioned briefly I had raised IgA (I think) normally found in the gut and seemed briefly interested but moved on swiftly.

I am generally not troubled with constipation but obviously have been using imodium for a number of years! The diarrhoea etc precluded the narcotics, I have only been on them for a year and even then very rarely took them as they didn't work. Now I'm on the steroids if I get any breakthrough pain they do work so I have been taking more, rheumatologist says this is because the pain is less severe now so the painkillers can touch it.

Well, I got nowhere with a GI perspective.

I still don't have an answer but the Rheumatology outlook has been much more productive.

I would recommend seeing a new Rheumy personally. A Dr who "sits on the fence" doesn't sound like they will help you get an answer.

StarGirrrrl said:

Well, I got nowhere with a GI perspective.

I still don't have an answer but the Rheumatology outlook has been much more productive.

I would recommend seeing a new Rheumy personally. A Dr who "sits on the fence" doesn't sound like they will help you get an answer.

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I am starting to think my rheumatologist is not the guy to help me to be honest. 4 years later i had to rely on him being on holiday and seeing a different doctor to get any sort of treatment.

I just don't know how to do that in the NHS or if it's even possible.

In the meantime I have given in and called to speak to someone at my local unit. The lower doses of steroid are not working quite so well and frankly 5 bowel movements in an hour is not normal and needs treated. I have had enough of sitting on the fence!!

Yes that is quite possible with the NHS.

There are varying routes to do that, one of the easiest is a letter to your Hospital's complaints service saying you're not satisfied with your current Rheumy and you want to see someone else instead should do it.

Hi guys,

Don't know if this update helps.

We may have some progress. Had a chat with rheumatologist on the phone this morning.

First point is he thinks the taper may have been too fast. To go back up to 20mg then taper down 1mg pw to try to get me down to 10mg. Which is great news as I felt fine on 20mg but this latest taper has killed me.

He is saying that they need to add in a second drug.

His problem is what to add in as he is not convinced it is sjogren's syndrome causing these symptoms. He said that sjogren's is rare in itself (so studies on it aren't as prevalent) and it's even rarer (i.e. it just doesn't happen) to have diarrhoea and abdo pain like this caused by sjogren's. He did however say that it IS an inflammatory condition so he can't rule it out. He is talking about adding in hydroxychloroquine again (although this was presumed to be making my abdo pain WORSE last year so I'm a little nervous) or sulfasalazine or azathioprine. Last time I saw him he talked about rituximab. He said he doesn't want me on methotrexate as I am young and it would increase my chances of lymphoma?

He thinks it could be inflammatory bowel disease and is going to write to my GI. However, my GI will laugh in his face if he suggests it after all the scoping he's done and also he said diagnosing it would be problematic given my response to pred, scoping wouldn't show anything now.

So what does he then prescribe as a second drug, something to treat sjogren's or something to treat inflammatory bowel disease?

*sigh*

I think from your post you are not satisfied with the options you have been presented with.

In which case perhaps a second opinion would be useful. Or a specialist in Sjogrens could evaluate you to decide once and for all.

As for rare diseases, they aren't mythical, they do happen. Might be 5 people per million- but for those 5 people it is real and shows it can happen.

People also can have more than one thing wrong with them- maybe it could be Sjogrens and something else like early onset IBD, so it's not showing up on tests yet.

Or there could be just one overall disease causing the various problems, it just hasn't been identified yet and the waters have been muddied with the other theories.

Either way if it was me I would be looking for additional opinions.

Hi. My son is also a difficult case too. In the beginning we saw a rheumi and he has positive Sjogrens blood test. When repeated it was negative. It was also suggested at one point it could be psychological and we too went for an eval and have a letter saying it was not. I am very glad they are talking about trying new medications since you should not be on high doses of prednisone for too long. Please keep us updated!

dannysmom said:

Hi. My son is also a difficult case too. In the beginning we saw a rheumi and he has positive Sjogrens blood test. When repeated it was negative. It was also suggested at one point it could be psychological and we too went for an eval and have a letter saying it was not. I am very glad they are talking about trying new medications since you should not be on high doses of prednisone for too long. Please keep us updated!

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How interesting. I read your thread on danny's story, what a lot you've been through! Has there been any suggestion it could be related to sjogren's? How is he doing now?

Has he been diagnosed with sjogren's? It's quite common to switch to being seronegative and back with sjogren's I think. Although being male is so uncommon for sjogren's, let alone a child!

I wish I could get off pred, I hate my moon face and night sweats etc. 20mg is not working for me atm, pain is less, it's not continuous as it was on 10mg, but diarrhoea still a huge problem during the night. Had a bad episode last night and noticed blood again, not a huge amount but never encouraging to see. Would be dropping weight like a stone if it wasn't the pred keeping my appetite up on days I don't have diarrhoea.

Starting to feel quite depressed and frustrated.