A submission from a reader: "34 year old, non-smoking African American female presenting with a recurring (for three years now), chronic (lasts for several weeks up to a few months) dry, non productive cough. No other symptoms (though the attached CT report says wheezing). No response to a variety of drug treatments over the years, including steroid shot, Levaquin, OTC Mucinex, OTC Claritin, Tussionex, Nasacort, OTC Prilosec, 6-day regimen of Metrol (4mg), Advair and Albuterol. First pulmonologist (who performed no diagnostic tests) diagnosed asthma and allergies. Nothing indicated on chest x-ray. Internal medicine doctor ordered CT scan (report attached). Second pulmonologist said subpleural cysts exist, which he indicated exist with UIP patients, but he said this was not UIP. Also said it's not tuberculosis or sarcoidosis. Prescribed 20 mg of Prednisone twice a day to get rid of cough, said we will proceed once cough is gone. Said a lung biopsy or thoracic biopsy may be in order.
CT reading:
Helical CT images through the chest were obtained following
intravenous contrast administration.
Within both lungs and involving both the upper and lower lung zones,
there are areas of subpleural macrocystic change with some
associated subpleural interstitial thickening. There are no
particular areas of ground-glass opacity associated with these
findings. No significant nodularity is seen. The central and midlung
zones are spared. No pleural fluid or lymphadenopathy is seen. These
findings can be seen in patients with usual interstitial pneumonia
(UIP) although this would be somewhat atypical in a patient of this
age group. Does the patient have a smoking history? Other
interstitial lung disease in its early stages may give a similar
appearance.
5 comments - CLICK HERE to read & add your own!:
UIP does not occur in this age group at all. It is exclusively in 50-60+ patients. Without having actualy CT, it is difficult to say because patterns on CT are crucial for certain ILD's. Sarcoid can give the appearance described and there does not need to be hi;lar adenopathy (this would be stage IV sarcoid). Given her age and ethnicity I would favor sarcoidosis. 20 mg of steroids is not sufficient for treating this (initially) and/or may warrant steroid-sparing drugs. She needs PFT's to characterize severity and a bronch with BAL and TBBX to confirm diagnosis.
Jeff, although I agree with you that it would be very rare for a person of this age to have "UIP" it is not correct to say that it never happens. We do see patients presented in our ILD conference in young age groups with a consensus diagnosis (radiology, pulmonary and pathology) ends up being UIP, and we have recently transplanted a 42 year old with UIP. That being said, the more likely outcome is that this is end stage hypersensitivity pneumonitis, and unless the history uncovers a relevant exposure, she may end up getting a surgical lung biopsy.
I'd stop the prednisone before it gets to be a big problem. In the end, I doubt it will make the cough go away.
Are those the patients with familial IPF? Anything different about them other than age?
Chronic (now fibrotic) HP is a good thought. I still favor sarcoid, but we'd really have to look at the CT and perhaps histology to say more...
The CT is essential, as are PFT's. Hard to imagine stage IV sarcoid or end-stage HP causing "only" a cough, without dyspnea and marked impairments in gas exchange. Another possibility (and likely the reason that smoking history is questioned in the CT report) would be eosinophilic granuloma. I'd favor that over LAM, but both should be in this differential.
Regardless, the patient needs an appropriate evaluation. Agree with having an experienced thoracic radiologist review the CT while getting full PFT's. After that, either a bronch with TBBX, which could diagnose EG or, possibly sarcoid. Next is a surgical biopsy. I'd hold the steroids at this point, pending the further evaluation.
Isn´t Langerhans Cell histiocytosis a diagnosis worth considering though quite uncommon?
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