Thursday, February 16, 2006

Progressive Disease?

Quick case: a 55 year old woman was diagnosed with Sarcoidosis in 2000 based on a biopsy of a skin lesion. At the time, she was referred to my institution where saw a young, but talented fellow (CEOB). She had mild bilateral hilar adenopathy and some atypical diffuse interstitial markings. The biopsy was reviewed and the diagnosis confirmed. She has never received any treatment for sarcoidosis, and her pulmonary function remained normal for 4-5 years.

Co-morbidities include: asthma and fibromyalgia.

I began seeing her about year ago (after a second fellow who took over many of CEOB's patients). The first time I saw her, she had a new complaint of persistent chest tightness and a marked decline in her pulmonary function: DLCO decreased from a stable baseline of 77% to 60%. FEV1 and FVC dropped from 82% and 86% of predicted values to 72% and 74% respectively.

She was started on an inhaled corticosteroid and long acting beta-agonist, and we ordered a HRCT: I don't have the images, but compared to a CT done 2 years previously, there was:

1. Progressive areas of scarring/fibrosis in the lingula, middle
lobe, and right upper lobe, the latter associated with atelectasis.
2. Increasing parenchymal nodules, predominantly in the left upper lobe.
3. Stable mediastinal and upper abdominal lymph nodes.

We discussed bronchoscopy and immunosupressive therapy for treatment of sarcoidosis, but she was very resistant to therapy and we chose a "watchful waiting approach."

In follow-up (after 3 and 6 months), she has had consistent improvement in her pulmonary function, so that her FEV1, FVC, and DLCO are now the same (or better) as they were in 2000. She continues to complain of some persistent chest tightness, but otherwise feels much better and is not limited in any activity.

So, here are the clinical questions:
1)Would one treat for radiographic progression of Stage 4 sarcoidosis in the absence of a decline in pulmonary function?

2)Do you think that the persistent "band-like" chest tightness is related to either her sarcoidosis or to asthma?

4 comments - CLICK HERE to read & add your own!:

Mike L said...

1)Would one treat for radiographic progression of Stage 4 sarcoidosis in the absence of a decline in pulmonary function?

Depends on what you see radiographically. It seems from your description that there are some "reversible" changes (i.e. changes that are not scarred), I would consider it. Hydroxychloroquine would be a reasonable drug if she is against immunosuppressives.

However, your PFT's do not show any changes in 5 years (other than the one blip), so it would also be reasonable to watch Spiro and a DLCO every 3 months to observe any trends.

2)Do you think that the persistent "band-like" chest tightness is related to either her sarcoidosis or to asthma?

I have seen some patients like this, and I believe that it is secondary to altered pleural anatomy. Patients get some lung parenchyma scarring, and it causes some "tugging" on the parietal pleura which is perceived as pain. If it worsens when the patient gets a respiratory infection, it is almost certainly this entity.
I presume, of course, that you have ruled out other sources (cardiac, etc).

Baleeiro said...

Well, for starters, I wouldn't trust anything that first fellow said... But back to the current problem. I think the major difficulty is differentiating the asthma/bronchospasm from the progression of the sarcoid.
The decision to treat is not so obvious here because of the improvement. If we believe this is mostly bronchospasm (with the good response to ICS) you could make an argument to close short term follow-up since the patient has “asymptomatic” progression of sarcoid. Conversely, I don’t think you would be at fault for trying some steroids: she has had worsening respiratory symptoms with a decline in PFTs in the setting of radiographic progression.
I would also consider a bronch. It seems like it is also sarcoid but just because that fellow thought it was sarcoid a few years ago it doesn’t mean she has the same process. She may also have a chronic infection complicating the picture.

Jeff H said...

That former fellow (along with a current professor emeritus) had the pathology reviewed here. It was confirmed as sarcoidosis. As for the now, the patient is otherwise asymptomatic and has had no complaints suggestive of infection...

I will leave the discussion open for now, as I'm interested to hear what more people have to say, and then let you all know what I actually did....

DKeena said...

In terms of staging sarcoid and assessing the risk of progression, I'm always a little unsure what to make of the HRCT. Most of what we know about the natural history of sarcoid is based on chest X-rays, and I wouldn't be suprised if the interval CT changes you describe would be too subtle for detection on CXR.

I usually think of stage IV disease as a pretty gnarly looking X-ray with anatomical distortion etc. (Frito lung)

What does the CXR look like?

As Baleeiro said, the decline in lung function might represent an asthma exacerbation or relapsing-remitting sarcoid.

Because the CT shows evidence of progressive sarcoid, with some fibrotic changes- that probably corresponds to an increased risk of disease progression, but it depends on the magnitude of the changes.

I agree with Mike and would start plaquinil and continue close monitoring. As the PFT's improved with ICS/BD, and the Pt is asymptomatic I think you're justified in holding off on oral prednisone for now.

Chest pain is a pretty common complaint among patients with sarcoid, but I'm not sure about the pathophys.