A 70 year old has had about 15 years of (stable) arthralgias in the knees, feet and DIPs. 5 years ago a routine CXR suggested some scarring. Dyspnea on exertion with a flight occurred about a year ago. 5 mos ago it's a bit more progressive subjectively (but still a flight of stairs) and with a non productive cough. No fever/chills or other constitutional sx. His spiro 2 mos ago shos a TLC of 61%. Spiro today shos FVC 60%. An RF factor was 83 (<15). An HRCT showed some honeycombing at the bases and thickened septal lines elsewhere with some gnd glass; basically seemed to have NSIP appearance. His joint symtpoms have been stable but he still has am stiffness and takes motrin.
Question: this seems like rheumatoid lung to me. Would you treat the rheumatoid and follow, or continue with an official ILD workup? In other words, is there any reason to go with a bronchoscopy and maybe an open lung prior to empiric therapy for rheumatoid? Would any one give him prednisone and methotrexate and just follow?
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I would probably err on the side of getting more data: this may be as you suggested, simple rheumatoid lung but there may be an infectious component to the lung Dz or even a different path (i.e. BOOP) for which you may use a different regimen. So I would consider at least a bronch, get an ESR to assess activity of the RA (it will be up in IPF too, I know).
Having said that, I would also consider his functional status and how aggressive HE wants to be. He may prefer just to start MTX and pred and hope to feel better.
Another good option is to look for clinical trials. Is anybody recruiting IPF/rheum lung?
Well, I understand the bronch to rule out infection, but this is much less likely given the clinical presentation. It's more of a gradual dyspnea problem. As far as the other diagnoses, an open lung would be required. I feel that it would be reasonable to empirically treat as long as he does indeed have rheumatoid arthritis.
I sent him to rhem for their opinion. If they feel he has RA, I may presume that he has underlying RA-ILD and simply treat without an open lung Bx. Is this a reasonable approach?
I agree that TBBx are not as good as OLBx for Dx of ILD/IIPs. My point with the bronch is that, well, sometimes you get lucky (I've had a couple cases of very classic idiopathic BOOP diagnosed on TBBx alone) and as far as the infections, I was suggestion more atypical stuff (e.g. MAC and alikes) complicating (though not causing) the ILD.
My concern is that although there are no data specific to patients with RA-ILD, the prognosis and response to therapy probably varies as a function of the histopathological pattern, as it does with the IIPs in general and pathology may help planning.
Again, having said all that, it is reasonable with subacute diseases such as RA and IIPs to start Tx for RA and monitor the lung Fxn' closely, proceding with TBBx/OLBx only if you get a poor or unexpected response.
If he truly has a dx of RA, I would not bronch him at this time. I would get plain radiographs of his knees, hands and feet to see if a radiologist can make a call on them.
A subset of ILD patients will have (+) RF in the absence of rheumatoid arthritis.
Although there might be other diseases that present similarly, this is a pretty good story for CVD associated ILD. BOOP should look different that the pattern described on HRCT. Also, I think it would be more acute in presentation.
If rheum agrees, I would treat with MTX and prednisone and monitor. If they are inconclusive, I would bronch.
Then, if his spirometry abruptly changes or he gets more pulmonary symptoms, I would move forward with a bronch/BAL/TBBx and consider SLBx.
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