44y/o man referred to me for management of his COPD. He has become progressively short of breath over the last several years. Currently, he has difficulty walking up
hills and he gets short of breath walking approximately 1-2 flights of stairs. He additionally reports increased shortness of breath that is more pronounced when he wakes up in the morning and improves some with the use of an albuterol metered-dose
inhaler. He also notes occasional wheezing with exertion, but he denies any
nocturnal shortness of breath.
PMH: none
PSHx: none
Social: 16 pack year smoker-quit 2.5 years ago. + h/o cocaine use (smokeing and snorting), none for the past 10 years. + Heavy ETOH use, but quit a few months ago when he was told to stop because of "abnormal liver tests."
No sig family history.
PE: Tall and thin. Mild wheezing and prolonged expiratory phase. Otherwise unremarkable.
FEV1 of 2.6 L, which is 59% of predicted.
FVC is 6.55 L, which is 117% of predicted.
The FEV1/FVC is 38%.
His pulse oximetry is 98% on room air at rest.
The flow-volume loop is curved.
His exhalation time was 9 seconds.
CXR: is below
44 y/o with COPD
Ok, now what?
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I hope I'm not missing something obvious on the cxr. I think I just see emphysema. He's young and smokes and has obstruction. I would thus want to rule out alpha-1 AT deficiency first. If that's normal I would get a HRCT to see if there is a suggestion of something like bronchiolitis obliterans, with a mosaaic pattern. If so, he might need an open lung to confirm. I'll comment more later after I see what others think. No use in spewing out a long list for obstruction in a young person just yet....
I'm with JJ. Unless the resolution is off, all I see is hyperinflation. PAs are fairly smal and there are some increased markings in mid-lung fields (focal bronchiectasis?). I agree HRCT would be next on my list.
A1AT def is a concern. I would also check an HIV test: HIV+ patients also get early severe emphysema with less TOB exposure and he has an eventful SHx.
There was nothing on the SHx to suggest exposure to cadmium (heavy metal metallurgy, etc.) so I assume you're not trying to mislead us that way...
Our prominent radiologist (BG) paged me about the Xray before I ever saw the patient. The key to the CXR is that it is lower lobe emphysema, and he does indeed have alpha-1 antitrypsin deficiency. He has PiZZ, with a level of 17. He also has some abnormalities in his liver enzymes, but no frank cirrhosis.
So, the next question: in addition to standard treatment with pulmonary rehab, anticholinergics, and bronchodilators, would you start Prolastin?
Yes I would definitelt start the prolastin. He is young so any degree of "saving" the inevitable decline in FEV per year would benefit him (compared to say a 65 year old with the same PFT's). It might help also to see some serial PFTs to compute the rate of decline per year. If it is over 100 ml/year I would definitely start him on it. But even less than that I would because he already is into 59% predicted at age 44...
I would also start him on Prolastin. His level is very low and he already has emphysema but still some preserved function (as opposed to if his FEV1 were already 30% or less).
I recommended treatment with Prolastin. However, somewhere along the line, he was told that it was contraindicated in the presence of liver disease (I could find no such contraindication). Additionally, he did not like the idea of weekly or biweekly infusions (requiring either repeated placements of peripheral IV's or placement of a port/Hickman cath), and wanted to think about it. So, we will readdress it in a few months when he follows up, and after he has seen a hepatologist.
No its not contraindicated in liver disease. If the liver disease is because of hepatocyte death due to build up of the inclusions (mutated A1AT), giving supplemental A1AT infusions into the vein will have no bearing on that one way or the other.
The inconvience of the infusions is an issue for many patients, as you point out with this patient.
JJ is correct: replacement Tx will not worsen liver Dz. Furthermore if he has liver Dz, liver transplant eval should be considered and liver Txp would be "curative" for the progression of lung Dz since the new liver should produce normal levels of A1AT.
I agree, and discussed all of that with the patient. However, I though three months either way is not going to make a difference in the long run. We'll repeat the conversation when he comes back, and I'll try to convince him to deal with the infusion!
By the way, do you guys go with the weekly infusions, or do you stretch out the dosing interval?
If I remember correctly (from the readings I did for the A1AT talk I gave a few years ago), the weekly is more effective than the monthly. BUT there is no trial that compares them head to head; you have to infer the efficiacy by looking at each study alone - with that, the more frequent dosing schedule results in less FEV1 decline. But obviously it's more of a pain for the patient. But think of it like coumadin clinic which is also weekly....
I've heard about some every-other week dosing, but havn't found any studies...
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