Does anyone else have Shrinking Lung Syndrome as a complication of their SLE Lupus

Does anyone out there sugger from a rare complication of SLE Lupus called Shrinkng Lung Syndrome. I have been diagnosed with this. My diagphram does not work and the base of my lungs are infarcted. Tests have also shown that I have been affected by TB at some point in the last few years (I work in social care so may have become infected by a client) so I have calcification in the upper right lung just to add to my breathing problems.

[Tigerlily4]

I'm so sorry to hear that Jinty - sounds like a total nightmare. No personal experience, nor have I heard of it before but this wretched disease seemingly never ceases to throw the most obscure curve balls. All I can do is sympathise and send you big hugs. Keep in touch. oxo

[KathinAyrshire]

My sympathies this I have never heard of despite the web, all I can say is that my original diagnosis was COPD. It sounds dreadful, you have my utmost sympathy as I have only three inhalers to take each day at various intervals. Although one of my close friends had TB there is no suggestion I have the latent version though apparently this is quite common and it flares with the chemo drugs if quiinoric doesn't work.

All I can do is to tell you that I feel for you, my breathing difficulties are minor compared to yours and I hope that like me you have some good days which relieve the difficulties.

My late husband was oxygen dependent so should it come to this in the future and you need to know the procedures on this, contact me.

Here's hoping tomorrow may be a good day. Chin up !!

[Sher78]

u have really drawn the short straw haven't u? I have heard of SLS but the only thing I know about it is it's so rare it literally only affects a very small number of lupus patients. As if u didn't have enough 2 deal with as it is! Seems there's nothing sacred when it comes 2 lupus :0( x

[lupiedeb]

I have shrinking lung syndrome and to start off with I was put on a very high dosage of steriods about 75mg a day stayed like this for about 18 months and then it was decided to try me with azathioprine and with that I was able to taper down very slowly on the steriods and I am now steriod free, it was a long process. I still my respirotry consultand once a year and if I need to see him at any time all I have to do is ring his secretary for an appointment. I am lucky in the fact that my lungs are stable now and have been for the past 3/4 years so hopefully I won't get any more problems.

[maazsk in reply to lupiedeb]

Hi, I live in Sydney AUS and my wife has SLE and SLS.

I wanted to get in touch with you and talk to you about the treatment of your LUPUS.

[Jujubeee in reply to lupiedeb]

Can u describe your symptoms? Thanks!

[Ghogh77]

Hi jinty I've never heard of this before but would explain a few things for me ill have a word with my consultant, thanks!!

A fellow social care worker we must be mad doing that with lupus...

[Hidden]

Thanks for the reply's I am just out of hospital after becoming neutropenic and a bone marrow biopsy revealed that this was caused by my Azathioprine so it has now been replaced with mycophenolate & my steroids have been increased to 30mg. For the first time since my diagnosis over a year ago I have started to suffer horrendous anxiety which is so debilitating. Concerns are that I am now on half pay & worry's about being able to pay my mortgage are prime followed by concerns that my condition is deteriorating. Sorry to sound such a grouch but it is honestly where I am at this point.

[Sher78 in reply to Hidden]

The only reason I'd heard about this condition is cos I was tested a few years back 4 it as I was suffering with my breathing something chronic & tests showed I had way too much calcium in my body & they wanted 2 rule it out. They didn't TELL me this was what they were testing me 4 (probably a good job as if I'd've known I probably would've started freaking out!) but I overheard my Rheumy discussing it with the Haemo so I asked him about it. May b an idea 2 speak 2 CAB or something 2 find out what financial help u can get. I take it u're claiming DLA?

[Hidden]

Hi Sher I am getting DLA at present but it's due to run out so it will be fingers crossed time to hope that it gets renewed (they way things are going with benefits now you can never be sure). I am just hoping I can manage to cope with working which just raises anxiety levels no matter how cool I try to be about it.

[Sher78 in reply to Hidden]

The way the benefits system goes these days is a joke, I've worked full time since I was 16 & never claimed a penny, yet I get signed off work by my consultant & GP due 2 a rapid decline in health & I have 2 fight like Hell 2 get anything. Sods law :0/ If u're able 2 carry on working then fantastic (I am at such a loss now I'm not working 4 the first time in my life) have u also put in 4 Disabled WORKING allowance? I THINK they still do it.

[anne59]

I don't have SLS but have developed interstitial lung disease as a complication of SLE, so I can well sympathise. It appears the treatment is similar and I take steroids, Myfenax, and n-acetylcysteine. I was motrified when I heard the progosis but as a positive note to anyone else who gets diagnosed;4 years on the medication is keeping the disease stable despite becoming an insulin dependant diabetic as a side effect of steroids. Try to keep positive :))

[CMac13]

Hi Jinty, I'm new to this site, tho I've had SLE ( as part of MCTD- as I've also got Dermatomyositis, vasculitis, Raynauds). You sound fairly miserable, thanks to all your chest problems, never mind having to worry about your pay too.

Anyway, just in case this sheds any light on your symptoms/ conditions, this is a synopsis of my chest problems. I was diagnosed as having asthma again about 14 years ago, I had it as a child originally. Then in 2006, I was really ill with what was diagnosed as Lupus pneumonitis. Since then, I've had 4-6 serious chest infections p.a, ending up in hospital.

I was started on azathioprine, in addition to steroids, Plaquenil, anti inflammatories etc, in 2004, after reduced lung function tests. This was increased, as were the steroids after my 1st hospitalisation due to the chest. The Respiratory centre I attend are treating this condition now as Bronchiectasis, with the base of my lungs both hit by Atelectasis (therefore v scarred/ solid). They told me that in particular my 'transfer tests' (part of the pulmonary tests) were dreadful, my spirometry is always very poor & I'm now on permanent oxygen. The Docs think that the disease attacked my lungs & weakened them so I've v reduced capacity. I'm now on permanent, prophylactic anti biotics, which has definitely reduce the no. of serious infections, tho I had pneumonia 3 times last year & then MRSA in the lungs. Not much fun.

Sorry if this sounds like a catalogue of woes, I just thought you might get a bit of comfort to know that when it's hard to breathe & things look bleak, things can improve, or at least that it may be tough going, but you'll still a have certain level of life - oh dear, I do go on!

The other main thing I wanted to say, was that the specialists never mentioned Shrinking Lung Syndrome to me, but my diaphragm doesn't work properly either & the lung bases are similarly affected... I can tell you that methotrexate is contraindicated with lung involvement, but I've found Rituximab to be effective.

Hope this is of some interest to you & not too confusing, feel free to ask me anything any way relevant. Take care & I hope you're not suffering too much.

[Cma9905]

Hi jinty, I am amazed to find someone else with shrinking lung syndrome. I was diagnosed with lupus 7 years ago. It took a year of tests etc biopsies, cameras etc. then they told me I have sls, which is very scary. I also have lung disease as well. My lung capacity decreased rapidly to start with and I had very high dose steroids then Rituximab every 6 months. I had the rituximab for 3 years and my lungs stabilised. Consultant said my body should have a break from the Rituximab. The first year off I felt really well. I then started going down hill with the lupus, chest infections etc although lungs were still stable. Consultant then put me forward for Rituximab again but that had to be authorised by a board as its very expensive, that took 1 year to be authorised. I have now been back on rituximab Since October. I get infections in my lungs about 4 times a year and the last one included pneumonia which is what I have now. My lung functions this year are now reducing "more than they would like to see". I had high dose steroid infusion a couple of weeks ago hoping that will stabilise my lungs. I have another lung function due beginning of june but I don't think there has been any improvement. I know it's very hard jinty, I get very low with it as well as scared. Do you get a letter from your consultant every year. I am under Addenbrookes hospital. I get one and when I was completing the ESA book I enclosed the letter from consultant. I have been granted ESA, although at first they did say I was fit for work. Just be honest on your form and tell them what it's like on a bad day. Sorry I have gone on a bit much about me, it's just very rare to find someone with sls. Carole

[Leanonme68]

I am just getting diagnosed with this. I'm seeking a Second opinion next week because my rheumy doesn't believe with all the rare disease I have that I have this too. I've had two pulmonary function tests done already. What treatment are you doing?

[Florence91]

Hi Hidden,

I was gold I have this last autumn 2017 at the Royal Brompton hosp in London after several yrs of increasing breathlessness. I also have a leaking aortic valve so nobody is sure how much is one or the other. Am seeing the cardiologidt for annual review in 10 days time. Was told it should be managed with the lupus so am still reducing my drugs as before with rheumatologist - who thinks breathlessness os lavk of fitness. I'm not over weight and walk etc but cant do as much as be.fore. Its all a pain but will see!

Keep positivre - thingd udually get sorted in the end.

All thd best

Florence91