Hi everyone. I am a 48 year old female with a very long-standing dx of Sero neg Lupus/UCTD in the absence of any better suggestion. I have low C3C4, occ raised ESR but other markers are normal. One of my biggest problems is recurrent bouts of inflammation in my knees with pain, redness and swelling - often too severe to get my foot to the floor. My Rheumy has never seen my knees (just photos) but, although I definitely get some episodes of joint effusion, the more frequent bouts target the join in random places between my kneecaps and the tendons/ligaments of my upper leg (occasionally the border of my lower leg).

I have read on a UK forum about something called Enthesitis and I wonder if this is what I experience? If this is the case, would this fit with a dx of SLE/UCTD? It seems more typical of Spondylarthropathies from what I have read. If not, do I need to flag this up with my Rheumy - would the management change? Currently I take Hydroxychloroquine 200mg, 1.5g Mycophenolate and 6mg Prednisolone but the attacks keep coming - once or twice a month, worst between April - September.

Would be interested to hear your thoughts. Many thanks. Clare

Low complements occur in HAE... this is a more likely cause.

Here is some reading for you on this:

http://www.haea.org/patients/what-is-hae/

http://circ.ahajournals.org/content/95/5/1115.full

https://www.karger.com/Article/FullText/368404

If you use an ACE inhibitor for blood pressure, this will increase bradykinin and cause the swellings. People with HAE cannot use this family of drugs.

This condition was officially named in the mid- '70's. Many doctors don't know about it. But some continuing ed seminars are now being brought to some doctors today. My doctor went to one, and that was how I was finally diagnosed.

I had a swollen knee years ago and the orthopedic doctor went to drain it (after an MRI showed no tumor or cysts) and couldn't get the "fluid" out. Now we know why... the fluid was disseminated thru all the cells around and in the knee and there was no excess fluid in the joint space.

The attacks with more normal functions that cycle sound very much like hereitary Angioedema. The bradykinin builds up and then is released to cause swelling and pain, which resolves slowly by itself, until the next attack comes.

Bradykinin is related to serotonin and histamine, but is very different in its own way. Steroids and antihistamines do not work in general on it.

You would most likely find an immunologist more helpful than a rheumatologist.

Thank your for your reply, Mrs D. The Doctors and I have considered many other differential diagnoses but HAE was not one of them. I can see why you might suggest this although I don't think that I have any other features of this condition. Also, the pain of these attacks on my knees tends to start as a small, acutely painful red patch on the margin of my kneecap with associated stiffness which rapidly spreads to encompass the entire knee with diffuse swelling. I had attributed the greater occurrence during April-Sept to photo-sensitivity. I get low grade fever, malaise, fatigue, pleuritic pain etc too.

I was investigated extensively in 2013 by an internationally recognised Professor of Rheumatology (Lupus) with some immunology blood tests, PFT's, SFEMG etc with no glaringly obvious diagnosis other than SLE/UCTD to explain the clinical symptoms. After 25 years of this and now feeling old, I don't have the resilience and gritted determination to push through the disabling pain of these recurrent events in the way that I used to. My Rheumatologist (I no longer see the Prof) reassures himself that the Mycophenolate is doing its job well but sometimes I wonder hence my question about possible Enthesitis as part of an SpA where the treatment might be different.

Can I ask if you had other symptoms of the HAE other than a swollen knee?

Many thanks again. Clare

I react to any injury or bug bite with alot swelling.

My throat swells up sometimes and blocks my Eustachian tube resulting in ear problems.

Much of my HAE is GI centered. I cannot tolerate fresh fruit (esp apples), or gassy vegetables like cabbage. My abdomen will swell when I have an attack, and that is once or twice a month even when I watch what I eat. (lots of pain there) When I was little I used to throw up all the time with it.

When my throat swells up my larynx get tight and strange noises come out of it (squealing, rasping and unnameable sounds), and when this happens my lungs sound clear according the doctor who checks me out closely.

I had the knee swelling, and I have a chronic left ankle that swells then goes down, and cycles. Xrays show some spurs in it. I had an IV in my hand last year for an echocardiogram test, and my hand swelled up for 2 months! When I had my c-section I was BIGGER than when I was pregnant, and both arms swelled up from the IVs.

My mouth will swell up from any numbing injection at the dentist.

I had a HUGE reaction to the ACE inhibitors, which are off the table for me now forever. Whole body swelling, burning pain, and none of my clothing would fit me.

My whole life has been huge swelling attacks with any invasive thing. Injections, bug bites, surgeries (I've only had 2 thank goodness), minor injuries like a fall or sprain, led to long term swelling. I tried contacts when I was younger but they made my corneas swell up so they are out too. The eye doctor was mystified but now I know why that failed. After my doctor went to that seminar, we did a long history of all my "attacks" and she decided on this diagnosis. Mine was present in childhood....but you can acquire it with ACE drug use. In my case the symptoms were hugely additive with the lisinopril. Took several years to build up to the massive reaction though.

There is really no treatment for this, but some people get the new injectable drugs which are ferociously expensive. I just treat the abdominal pain/spasms with medication when it comes back. I have been lucky to not have a full blown throat blockage yet, but I carry an albuterol inhaler just in case.

My ANA's are normal... I've had them tested 3 times.
I had C4 and C1-inhibitor tested last year, and they were normal then. My doctor says best to test during an episode. One of the HAE varieties has normal complement and C1 levels. I have read that there is some thought that C1 inhibitor may be present but damaged and therefore not work properly but show up in testing. But I don't know how accurate that is.

Some women have higher testosterone levels, than others and this leads to suppressing some HAE symptoms. Once those levels fall with aging, HAE symptoms may appear later in life. Also some women who use birth control pills or HRT after menopause, show the symptoms..as female hormones seem to trigger symptoms in women.

My goodness Mrs D! That's a really big thing to live with. I had no idea what HAE was until yesterday but I don't have any of the things you mention and I do respond to higher doses of prednisolone. The reductions of the past year from 15mgs to 6mgs/day have not been problem free.

its interesting to note that, during my 25yr history of likely CTD and subsequent diagnosis with 3 monthly review appointments ever since plus second opinion in 2013, no-one has looked at my joints at all since that first appt in January 2001! Shocking really.

Many thanks for taking the time to reply. Clare