33 yo female with PMH of reactive airway disease since childhood. She has been on corticosteroids for the last 15-20 years with frequent bursts/tapers. Her lowest dose was prednisone equivalent of 5 mg. She is seen in the ER at least 4-5 times/month for exacerbations at which she gets burst of steroids, NMT's x 2 or 3 and is discharged home. She has been admitted only 1 x in the last 5 years.
She had allergy testing as a teenager and was "allergic 4+ to almost everything they tested." Unfortunately, after multiple rounds of allergy shots, she still is not any better.
Her home is mostly wood floors and her father dusts weekly. She lives in a major metropolitan city. She does not work because of her asthma. She denies any domestic violence.
Meds: flovent, serevent, singulair, decadron (she likes this steroid the best), AllegraD, Protonix, Albuterol MDI PRN (uses 6-7 x per day with varying relief) and Xopenex Nebs Q 6 hours
PE: Cushoingoid obese African-American female in NAD
Has that upper airway wheeze which starts when I put on my stethoscope. Also with distant lung sounds. Unable to distinguish any other sounds in her chest because of her upper airway wheezing.
Studies:
PFT's
FEV1: 1.51 (62%)
FVC: 1.72 (60%)
FEV1/FVC 88 (103%)
Slow vital capacity (SVC): 2.02 (71%)
FEV1/ SVC 74%
DLCO: 15.39 (76%)
6 minute walk test: no desaturation (stayed stable at 99%); walked 1000 feet
Coughed during exercise
CXR: Normal
HRCT: Normal
CT with PE Protocol: no PE
Echo: normal with no TR jet to estimate RVSP
I sent her to the speech pathologist and she definitely has some laryngeal dyskinesia, but it is "not very severe and should be treated after 4-5 sessions."
Any thoughts?
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This is pretty classic for LD, although studies have shown that 33-50% of patients with LD also do have asthma. Obviously, her PFT's don't show obstruction, and the restrictive appearence is probably her obesity.
With regard to her "refractory" asthma (if we assume that this is not all her LD), the vast majority of these cases are due to non-compliance. There's good literature demonstrating that patients simply lie to us about the medication usage. {Bosley, ERJ 1994, Rand, AJRCCM 1992, Simmons, Chest 2000}
Other non-pulmonary mimics include CHF and mitral valve disease, so an echo may be useful. Also, consider GERD as a potential contributing factor. Psychiatric disease and "hyperventilation syndrome" accounted for 9/14 cases of "refractory asthma" in one small series (Thomas, Thorax 1999). Also, check out (Heaney, Thorax 2003)
There are, of course, some patients who are really steroid-resistant-some due to impaired binding of the steroid to the steroid-response elements in DNA.
An apporach to the refractory patient is outlined here in this paper: Proceedings of the ATS Workshop on Refractory Asthma, 2000. AJRCCM: 162;2341.
Alternative treatments that have been studies include: Methotrexate, Cylcosporine, Gold, IVIG and Xolair. There are no convincing data for the use of any of them (although Xolair hasn't really been studied in this population.) Finally, there are re-emerging data on the use of Macrolides-reinvention of an old theme, but it may be promising.
So--I would look for GERD, do a methacholine challenge to confirm hyperresponsiveness, get an echo, check serum IgE and eosinophils, and consider a trial of Macrolide while slowly tapering her steroids and obtaining a psychiatric evaluation.
Very comprehensive outline by Jeff H. I would add Aspergillus preciptins to the IgE/CBC and Diff. Though her "steroid-resistance" is most likely due to misdiagnosis of her LD, ABPA/ABPM may cause these steroid-relieved symptoms. It is also very easy to call her drugstore and inquire on how many times she has refilled her ICS and Singulair in the past 6 months (we sometimes get surprised).
Well, at first glance th ratio is not obstrucive, but with a slow vital capacity the ratio drops down to 74, so this to me indicates fairly significant airway obstruction. As for reactive airways vs LD, what did her flow-volme loops look like? What was her fef25-75? Was there flattening of the insp loop?
As for psychiatric, the PFT's are indeed abnormal so I would put this lower. As for mitral and CHF, the echo was normal as per Mike.
I would say this is mod-severe obstructive defect. Is there reversibility with post-bronchodilator challenge? If not, I would still say asthm but with fixed airway obstruction, perhps due to remodeling (if she had this since childhood chnces are she has gone a while without steroid inhalers which is a risk factor for fixed airways in asthma.
I agree that there is very high rate of noncompliance, especially with inhaled steroids. One study (maybe one that horowitz quoted above)showed that when physicians with a long reltinship with patient were absolutely sure of patient ompliance, they were right only about half the time.
Since this is severe asthma, maybe some steroid-paring drugs should now be tried (assuming you somehow deal with the noncompliance issue).
I agree with checking an IgE to see if she is a candidate for omalizumab .
I think ABPA is less likely with the normal HRCT.
Let me add that the dropped ratio with SVC could be consistent with LD *or* significant airtrapping; the flow volume loop morphology should sort this out though....
Use of the SVC is more sensative for obstruction, because the SVC will be greater than the FVC, therefore making the FEV1/SVC ratio lower than the FEV1/FVC ratio. That said, the ATS provides no guidelines or definition for obstruction based on the FEV1/SVC ratio. And in this case, it is still greater than 70%.
Nevertheless, I agree that there probably is obstructive disease present, and that it is probably mixed with a restrictive process due to obesity. A TLC and RV would be helpful--I'd suspect the TLC would be slightly high and that air-trapping would be present.
Additionally, I never stated that this was not, in fact, refractory asthma, or that it was psychiatric illness. I did say that 33-50% of patients with LD ALSO HAVE ASTHMA. But "refractory asthma" requires a search for alternative diagnoses, and the literature demonstrates that a large percentage of these are due to psyciatric illness. I don't make the rules, and I've given you the references.
I also purposefully left out the asthma mimics due to pulmonary disease (ABPA and CS) b/c I know Dr. L. has already considered these possibilities.
Finally, I'm hesitant (but not resistant) to use alternative therapies to asthma, as none have shown any demonstrable benefit. I'd have to be convinced that I have ruled out all alternative diagnosis, and non-compliance with inhaled steroids. Once that has been done, I'd use Methotrexate b/c it is the best studied alternative and there are some data supporting a steroid-sparing effect.
Her echo is completely normal without any evidence of MR.
She is too claustrophobic to sit in the box long enough to get full pft's so I have tabled that arguement for now.
Her serum IgE on decadron 1 mg daily is 300 (normal 0-120).
I actually went through JCH's presentation on refractory asthma (PP_Pulm still lives), and found some of those references to read.
I neglected to get fungal precipitans and an ANCA, and will do so next visit. I guess I forgot to consider these diagnoses. Thank heavens for the blog.
Have any of you given omalizumab?
Thanks for the help.
The elevated IgE, especially on decadron, is interesting. I havn't used Xolair, but if you have a low suspicion for ABPA, churg strauss, and Wegner's (none of which seem likely in your patient), it may be worth a try.
I have had mixed feelings/results with Xolair: I have a few patients that have come to me already on Xolair (usually started by an allergist), some have had a historic improvement but would probably have done just as well with more aggressive care of their GERD and post-nasal drip, and two of them still have frequent exacerbations and require ABTx and occasional courses of prednisone despite the Xolair.
I have not initiated Xolair Tx in anyone here yet.
Very Good article, this article make some interesting points.
airway dir
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