ILD case

[nlax30]

Another recent gift from the ED.

Mid 50's male, c/o about 2 weeks progressive SOB, prod cough. Saw outpt Pulm 2 days prior to admission and put on PO Abx and prednisone for bronchitis. Unable to sleep because of dyspnea that acutely worsened and came to ER. In ED had SpO2 in 50's and immediately put on bipap 100% FiO2.

PMH: "Pulm Fibrosis" diagnosed couple years prior at another facility from what sounds like transbronchial biopsy. Also like has RA, DM, HTN though has no PCP and generally non-compliant. Not on any chronic meds. Has multiple RA findings (extensor nodules, ulnar deviation, Swann-neck deformity, etc...)

I pull up CXR and immediately decide to head to ED, shows b/l fibrotic changes and significant LLL consolidation.

Labs: WBCs 15, chem unremarkable, Trop neg, BNP 102, d-dimer elevated, ABG on 100% bipap 7.36/36/84/20

Walk in after he had been on bipap for little over an hour and he looks like crap.... tachycardic 130's, RR 40's, diaphoretic, using access muscles, etc... Immediately get ED doc back in there and tube the guy.

Units are full so spend a few hours managing him in ED, can't get sats above 88 on 100% FiO2 on the crappy ED vents and about to call for an ICU vent when we get a bed.

CTA chest on way to unit neg for PE though extensive honey combing and left consolidation.

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[KLPM]

Probably won't change much but I was just curious about the nature of his pulmonary fibrosis. Is it IPF? Or is it something else like fibrosis secondary to his RA?

 

[nlax30]

Started on steroids, Vanco/cefepime, had Levaquin in ED.

Next gas shows pCO2 in 70's, PaO2 50's. Not working well with vent and appears agitated even on high dose propofol. Decide to add nimbex and now able to ventilate better though best sats gets are ~85-90. CO2 improves to 50's and PaO2 60-70's on 100% Fio2, on AC/550/22/5. This all after playing with higher PEEPs and increasing I:E ratio with no improvement. Plateau pressures just under 30 and exceed it with any increase in PEEP.

We can do bi-level here but don't have oscillators available.

Need to do some more reading on ILD and specifically RA associated ILD due to his history.

 

[nlax30]

Probably won't change much but I was just curious about the nature of his pulmonary fibrosis. Is it IPF? Or is it something else like fibrosis secondary to his RA?

That's kind of the mystery. Pulm guy he had seen before hadnt been able to fully work him up. Trying to get a hold of his previous biopsy but was told it was very non-specific.

Personally I'm thinking RA associated, sounds and looks like he has had long standing RA and has never been treated for it. Was told he refused to see Rheum in the past and doesn't have great f/up.

 

[jdh71]

Probably won't change much but I was just curious about the nature of his pulmonary fibrosis. Is it IPF? Or is it something else like fibrosis secondary to his RA?

Can't call it IPF as long as the picture of complicated by RA, and given his RA symptoms this is likely fibrosis secondary to his RA.

 

[nlax30]

Flu A also came back positive.... put on Tamiflu just because of how sick he was but not sure it's going to make much difference.

 

[jdh71]

Started on steroids, Vanco/cefepime, had Levaquin in ED.

Next gas shows pCO2 in 70's, PaO2 50's. Not working well with vent and appears agitated even on high dose propofol. Decide to add nimbex and now able to ventilate better though best sats gets are ~85-90. CO2 improves to 50's and PaO2 60-70's on 100% Fio2, on AC/550/22/5. This all after playing with higher PEEPs and increasing I:E ratio with no improvement. Plateau pressures just under 30 and exceed it with any increase in PEEP.

We can do bi-level here but don't have oscillators available.

Need to do some more reading on ILD and specifically RA associated ILD due to his history.

http://journal.publications.chestnet.org/article.aspx?articleid=1654289

check it out at work or through the university library

 

[jdh71]

Flu A also came back positive.... put on Tamiflu just because of how sick he was but not sure it's going to make much difference.

He's probably toast.

 

[KLPM]

Can't call it IPF as long as the picture of complicated by RA, and given his RA symptoms this is likely fibrosis secondary to his RA.

Fair point. Exclusion of connective tissue disease is a prerequisite for diagnosing IPF.

 

[Hernandez]

Trying to get a hold of his previous biopsy but was told it was very non-specific. .

You sure that wasn't the result? NSIP? Realistically, besides LIP, PAP, COP, you aren't able to Dx IID on transbronchial and even COP has yield <40% so I don't do transbronchial looking for COP

 

[souljah1]

That is acute consolidation on a background of UIP in the setting of collagen vascular disease.

Not at all consistent with NSIP or any other ILD by CT.

Transbronchial biopsies for anything related to ILD is pretty low yield other than looking for granuloma. Far too often people go to transbronchs without realizing it wont help them at all. I'd also say that far too many send people to VATS for what is obviously radiographically UIP. All that does is increase the risk of acute hypoxemia peri-biopsy. We see lots of that transferred in from outside hospitals.