Here's another anecdote on a patient started on prednisone/azathioprine/NAC for IPF. He was started on this for a variety of reasons, but one was that the biopsy had a bit more inflammatory changes even though there was ample fibroblastic foci and heterogeneity, so I thought a 3-6 month trial would be reasonable. By the time his imuran was up to 75 mg, I saw him. A repeat spiro was unchanged (FVC 43% predicted) but his DLCO went from 35% to 45% so I continued the meds. However he had mouth pain and the tongue showed possible thrush so I kept the Imuran at 75, gave some nystatin swish and sent him out to be followed up in 2 months and repeat the HRCT with the sprio/DLCO. However, his mouth pain did not go away and worsened, and he started developing malaise and a fever. I saw him in clinic that day. He looked fairly sick but vitals ok. He had a soft palate lesion. I got derm to KOH it and there are some non-budding hyphae so he's to get clotrimazole . Then almost as an afterthought I added on amylase and lipase to the repeat LFT's and low and behold the lipase is 800.
Of note his WBC was 13 and now down to 6....
This side effect is likely going to be self-limiting as he stays off the imuran. As I tell him to go light on PO intake, I am adding a creatinine to make sure he is not volume depleted (he's an outpatient).
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Poor guy. He's not any inhalers is he? Wonder how he might do on cellcept. Ever tried that on an IPF'er (vs NSIP)? Also, any sign of PH yet? We were wondering if those with PH shouldn't be started on NAC.
I have not tried cellcept. They do all sorts of weird wacky things over at the "big house" - maybe one of those guys can comment on that?
[wink doug-horowitz-other lurkers.....
its really frustrating that we do not have a proper treatment for this condition yet!it really frustrates me.just look at the patients fibrosis progressing........
I really find no evidence for the use of NAC in IPF - I wonder why it is used so much?
I give most patients who want it a 3 month trial of imuran and prednisone (in case we have the wrong diagnosis) I have at least one patient with biopsy proven IPF who responded very well and came off O2 but his CT was not typical for IPF so I suspect he did not have IPF.
I think if you are certain clinically that you are dealing with IPF you should simply accept that the treatment is in many cases worse than the disease and accept that if the treatment is not making these patients better (which all studies show it is not) then by definition it must be making them worse (side effects) - I am as guily of this as anyone but I think we need to stop the bias of "doing something is better than doing nothing" and simply sit on our hands. Its the best thing we can do for patients with IPF. We simply hurt too many of them with our concern and bias toward treatment
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