Am stumped

[ericdamiansean]

A 27 year old woman has a dry cough for the past four months. She has no fever. She also has effort intolerance. Physical examination reveals vesicular breathing in both lungs with fine crepitations on inspiration. There is no cardiac disease. Her X-ray chest shows increased reticular shadowing in both lungs. Her SaO2 is normal at rest but decreases after a six minute walk. What disease is she likely to have?

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[Flobber]

Idiopathic pulmonary fibrosis?

 

[ericdamiansean]

I was thinking along the line of a restrictive lung disease too..probably idiopathic due to the young age, and lack of a history of exposure to any chemicals etc

 

[angel80]

Some type of interstitial lung disease. If she doesn't have the history of exposure to chemicals/drugs, the idiopathic pulmonary fibrosis is definately a possiblity.

 

[SOUNDMAN]

I was thinking restrictive lung disease as well. PFT's would tell you more and get things going in the right direction.

 

[Richie Truxillo]

You need some PFT's for sure. I totally agree with idiopathic pulmonary fibrosis but I'd like to throw in Alpha-1-antitrypsin deficiency as a differential as well.

Let us know what you find out! Good luck! I'm off to take my pediatrics exam

-Richie

 

[Becket]

Agree that an interstitial disease sounds likely, but she's a bit young for IPF. I'd be thinking more along the lines of sarcoid or some lung disease associated with a rheum disorder such as RA or scleroderma...high-res CT would be very helpful.

 

[QuinnB]

I think that it's restrictive; for alpha 1 antitrypsin would'nt she be too young to manifest disease (< 30 is fairly rare) and isn't this most often an obstructive picture (e.g., emphysema) which doesn't seem consistent with the physical exam, or at least the information provided.

 

[babyruth]

No mediastinal widening? I guess my vote is also sarcoid or another Rheum disorder as stated above. Is she African American (like the typical board question...)? Could be drug reaction too; is she on any meds?

 

[SaraMac13]

Yeah - I agree with the above. Also, check for pulmonary hypertension (primary or secondary)?

 

[Critical Mass]

I think that she didn't have enough surfactant in her lungs when she was born. It just took a long time for the symptoms to show up.

 

[Sureshot83]

My first thought given her age (~30) and symptoms was primary pulmonary HTN, as SaraMac said. Obviously could also be secondary, but then you'd need some other explanation for that.

But I'm just an M2, so what do I know....

 

[thewebthsp]

Demographics?
Smoker?
Exposure history (Animals, coal/asbestos/beryllium)?
Allergies?
Drugs history?
Where does she live?
Recent Infections?
Other medical problems?

On physical exam -- any rheum findings? GI problems? What's her skin look like? Heart murmurs?

Spirometry data
Plethysmographic data
Any CBC/lyte abnormalities?
Might want to consider ANA, IgE
Ultimate answer probably from bronchoscopy/biopsy... maybe not

 

[DarksideAllstar]

biopsy and send to path...

 

[Bikini Princess]

I think it sounds like interstitial lung disease, or else class 1 pulmonary hypertension, particularly since they mentioned the six minute walk (6MWD), a common scoring system for this disease.

Are the pulmonary arteries or right heart enlarged in the chest xray?

Another much rarer consideration is LAM (lymphangiomyomatosis), particularly for her age and gender. I think this is more common in caucasians.

1. Check cardiac ECHO
2. Check ANA
3. Check PFTs

 

[Tired]

biopsy and send to path...

Spoken like a true cutter. Excellent.

Was this a multiple choice question? Seems like the kind of question where they give you five disorders, but only one is a restrictive process, meaning you should select that one.

You can pretty easily get the category of the disease process going on here, but I think a definitive diagnosis is out of the question given the limited info available.

 

[ericdamiansean]

A peak flow was not done. She was treated as interstitial lung disease (case was from a smaller hospital)

 

[DropkickMurphy]

A peak flow was not done. She was treated as interstitial lung disease (case was from a smaller hospital)

Peak flows are of dubious value (because they are so effort and technique dependent), unless other PFT parameters are presented. The only use for them really is in pre- and post-bronchodilator therapy, and even this is up for debate (as occurred at a conference I attended last year....a very "spirited" argument involving several pulmonologists and a couple of RTs (myself not included as I was just a spectator to it))

Beyond normal lab studies:
-Full PFTs including N2 washout, He dilution and DLCO
-CXR ----> CT
-Bronchoscopy (with biopsies; if dx not established by less invasive means obviously)
-Echocardiogram

My DDx list would be:
-Idiopathic pulmonary hypertension
-Sarcoidosis
-Pulm HTN
-Hypersensitivity pneumonitis

 

[thewebthsp]

NEJM has a "Diagnosis medicine" section from time to time. They're fun...