Thursday, March 20, 2008

Acute ILD

This 77 year old man has had a bit of honeycombing at the bases since at least 2004. Radiographically c/w UIP. Biopsies not done and he has been stable for years (and FVC normal at 84% predicted).
In February he developed worsenign cough and SOB. He was admitted and was hypoxic with the CT shown below. A cath was done showing normal wedge, normal CO/CI.
A BAL showed only 184 cells half of which were macrophages. A TBBX was not done because he's on coumadin for afib and we didn't think it would add much to determining the diagnosis.
SH, desk job all his life. In January, routine maintenance change of the humidifier pipes in his furnace. This may be a red herring but throwing it out there. No one else in household sick.

Was going to send him for open lung/VATs, but what do you think are possible etiologies?
I also repeated autoimmune chemistries that had been negative in 2004.

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Anonymous said...

I'm not sure I buy the honeycombing on the old CT scan- possibly just not present on the cuts shown.

If we presume that he does have IPF, then the current CT is quite consistent with an acute exacerbation. Pathogenesis and etiology are not known in that case, but it's clear that up to 50% of patients with previously "stable" IPF will end up dying in the context of one. In any case, I'd treat empirically with antibiotics and, presuming the BAL cultures are negative in a few days I'd add pulse-doses of Solumedrol (1 g/d x 3 d) and hope for the best.

VATS now would probably show a UIP background with some diffuse alveolar damage. Regardless of what the VATS shows, the answer is either antibiotics or steroids (some would advocate cytoxan in these settings, treating more as an AIP). So a biopsy right now would not change management.

If the patient recovers, then a biopsy can be considered to confirm the underlying suspicion of UIP. I doubt HP is present here, but if it is the steroids would still be beneficial.