Thursday, August 11, 2005

Progressive dyspnea

DM is a 43-year-old dentist who was referred to our clinic with a presumptive Dx of lupus based upon a positive ANA and a constellation of physical findings and symptoms including arthralgias and skin rashes. These symptoms began about 3 years ago. About 2 months ago, he developed persistent redness in his eyes and was seen by Ophthalmology who diagnosed episcleritis. Most recently, he was started on Plaquenil at a dose of 200 mg b.i.d. while tapering prednisone.

In terms of his respiratory history, he notes that he first began experiencing fatigue and sensation of dyspnea about 3 years ago, but the dyspnea became more debilitating over the last year. He is an avid gardener and notes that each summer, it has become more difficult to perform his usual activity. He otherwise is not complaining of any chest pain, any inspiratory or expiratory pain with breathing, cough, fevers, or night sweats.

Medication list includes atenolol 50 mg p.o. daily, tramadol 50 mg p.o. daily,clonazepam 1 mg p.r.n., Clarinex 5 mg p.o. daily, Celexa 40 mg p.o. daily,Nasonex 50 mcg b.i.d., Lotemax 50 mg b.i.d., triamcinolone cream p.r.n.Albuterol prn. Allergy shots every week.

Social History: Quit Tob 4-1/2years ago.

Physical exam: his weight was 192, blood pressure 120/85, pulse 104,respiratory rate between 12 and 15, oximetry was 97% on room air.
No apparent distress at rest.
Lung exam revealed normal symmetrical excursion. Normal palpation and percussion. Auscultation revealed clear lungs without wheezes, crackles, or rhonchi. There is no forced expiratory wheeze. Heart exam revealed normal S1 and S2 without gallops, murmurs, or rubs. Extremities revealed no cyanosis, clubbing, or edema.

Data: Spirometry performed today reveal an FEV1 of 1.4 liters (55%), FVC of2.33 liters (54%), and FEV1/FVC ratio is 92% of predicted. Full pulmonary function tests recently performed revealed the following:A total lung capacity of 74%, residual volume 74%, FEV1 of 2.5 liters, 74%predicted, FVC of 3.1 liters 72% of predicted. He was unable to perform DLCO.

CXR performed in 2003 revealed the following:
1. Normal cardiomediastinal silhouette.2. Mild hypoinflation3. Lungs are clear with no evidence of abnormal opacities and nopleural effusions4. Normal bony structures of the chest5. A focal sclerotic lesion in the head of the left humerus, likelyrepresenting simple bony island.

More recent CXR performed this year revealed the following:
1. Minimal linear left basilar atelectasis. Lung volumes are somewhatunderinflated. Otherwise, no acute cardiopulmonary process.

What other information would you like (History, Exam, Tests?)
What is you differential?

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

Baleeiro said...

His weight is 192 and I may have missed a height. Is he pretty heavy?
He is a former smoker but his pattern seems more of a mild restriction. SLE (and it seems he may have associated Sjogren's) may cause a lot of different pulmonary manifestations... his CxR seems unremarkable for extensive ILD but a HRCT would be nice. Sjogren's has a good association with bronchiectasis but I did not see anything about chronic cough and sputum production.
Pulm HTN could explain some of the symptoms and SLE may lead to that. Maybe a 2-D-ECHO for starters?
Finally, since his CxR is quite clear, I will mention shrinking lung syndrome. I have always seen cases and reports on NEJM and other journals but have never seen a case... it would go along with the combination of SLE with dyspnea, clear lungs and decreasing volumes.

Mendez said...

ECHO: No effusion, normal chambers and LV function. Unable to estimate RVSP.

Findings: Inspiratory images demonstrate mosaic attenuation,
particularly in the upper lobes. There is no evidence of
bronchiectasis, pulmonary nodules, septal thickening, ground-glass,
emphysema, or peribronchial cuffing. Linear scar is noted in theinferior lingula. Expiratory images demonstrate diffuse air-trappingin the upper and lower lobes.
No pleural or pericardial fluid.
No thoracic lymph node enlargement.
Prominent mediastinal fat is identified incidentally.
Limited noncontrast evaluation of the upper abdomen is unremarkable.
No aggressive osseous lesions.
Impression: Air-trapping without evidence of interstitial lung disease which
may be consistent with asthma or reactive airway disease.

Jennings said...

Well, the spiro and the RV don't seem to support the apparent air trapping pattern on HRCT. What were his Mips and MEPS? Does he have any muscle pains to suggest a myositis? If not, maybe he could have a lupus-related myopathy((due to underlying vasculitis) which would explain the clear CXR with restriction. Obesity could be an explanation too of course which is why Baleeiro was wondering what the patient's BMI was. Lupus related to lung can involve pleur or a pneumonitis for the restriction, but there is no evidence of that here, so again I would check his weakness and proceed to emg if it's positive.

Does he have a history of prolonged steroid use for his lupus? Sometimes steroid-mypopathy can persist even after stopping the drug.

Mendez said...

His height is about 5'9-10. Mildly obese by appearance, but not impressively so.

MIPS/MEPS were normal. CPK nl. No specific weakness on neuro exam. Arthralgias and rashes much improved on therapy. Prednisone was weaned off, but symptoms of dyspnea/fatigue did not improve.

I'll post the remainder of the story by the end of the day, but will give others a chance to chime in to see if we get a consensus or thoughts on any other history or tests.

Mike L said...

I was wondering about BOOP. It could give a restrictive pattern on PFT's; however, a mosaic pattern on PFT's may be pushing it.

Per usual, CEOB has a great thought. Shrinking lung syndrome would be climbing on my differential. I suppose I would start with a SNIFF test and then move on to a diaphragmatic EMG.

You did not offer a MVV, which may be somewhat helpful.

Mendez said...

Looks like it's nearing the end of the day, so I'll brief you on the w/u and our Dx.

Our thoughts intially the more common lupus related ILDs - pleuritis/pneumonitis, but this was quickly ruled out by Hx, exam, HRCT. Likewise, steroid myopathy, or myopathy related to collagen vascular disease was a possibility. This was also r/o with exam and labs.
Hypercoaguability was entertained as well. However, patient was neg. for lupus anticoagulant and VQ scan was negative.

Probing into his Hx a bit further revealed dyspnea particulary in the supine position. He had poor sleep as well and snored.

An EMG revealed R. phrenic neuropathy. Sleep study revealed signifcant nocturnal desaturation (surprising,eh?).

Since his other symptoms of lupus were improved (scleritis, arthralgias, rashes), we opted not to reinstitute steroids. He was titrated for nocturnal CPAP as desaturation was likely a major contributor to his fatigue.
We will monitor his resp symptoms and PFTs, and decide on further therapy.
There isn't any convincing evidence out there regarding steroid for this specific indication. There is a decent review on this subject in Mayo Clin Proc V75(5), May 2000, p467.

As usual, CEOB hit the nail on the head immediately.