Obliterative bronchiolitis

Question

I have CF and was lung transplanted 2002. I have been doing fine until this spring. At that time the lung function values started to decline without any obvious reason. After two bronchoscopies in this autumn due to continuous decline of FEV1, my physicians have stated that I probably have “bronchiolitis obliterance”. I wonder if it is common for lung transplanted CF-patients and what the experience is regarding treatment and progress of OB?

Answer

Dear questioner,

I am glad to hear that you have had a successful lung transplantation 7 years ago and has had a good life with your new lungs. You have, however, now got some new symptoms in form of worsening of your lung functions, esp. FEV1, and your doctors have noticed “obliterative bronchiolitis (OB)” by bronchoscopy. To be able to answer fully on your question, I would need some further information about if the diagnosis has been made by a biopsy (a piece that is taken from the affected lung and analysed by microscopy) and how much your lung function has declined.
But generally I can say: OB is the characteristic marker for a chronic rejection of the transplanted lungs. It is usually diagnosed by a surgical biopsy
Similar symptoms can be found in a series of other causes –e.g. acute rejection (which might be occasional), infection or stenosis in the large airways. This is usually called “bronchiolitis obliterans syndrome (BOS)”. BOS is diagnosed when there is at least a 20% drop in FEV1. Unfortunately, BOS is common after lung transplantation, occurring in about 2/3 of patients by several years after transplantation. The rate of progression of BOS is quite heterogeneous; in some patients the lung function may remain stable for years. If you also have problems with gastro-oesophageal reflux disease (GERD), this should be treated aggressively. Azitromycin might also be of help for some.
I wish you all good luck
Hans Kollberg

04.01.2010