ABPA

Question

I am 31 years old and have CF and diabetes. At 13, I had ABPA for the first time and was treated with cortisone. I had the second ABPA (RAST class 4) in fall 2006 and was treated with Decortin H® (prednisolone) for one year, starting with 50mg. My current IgE is at 519; in addition, the specific IgE shows RAST class 3 concerning. A.fumigatus. My FEV1 has dropped by 12% (now 71%), particularly the small airways by half (MEF 25 is at 25%, Sultanol®/salbutamol does not help much). An HRCT has not been done yet. I was told than an X-ray, which was done after a pneumonia that has worn off after an i.v. therapy, did not show any “discrete infiltrates.” Which therapy would be appropriate now? Would a cortisone pulse therapy be an option (50mg for three days)? Should I take Sempera® (itraconazole) as well? Many thanks in advance for your effort.

Answer

Dear questioner,

I can tell from your question that you have been diagnosed with ABPA twice and treated systemically with cortisone each time. Now the question is whether you have an ABPA relapse (recurring ABPA). The question of relapses is always very difficult and can certainly not be answered via the internet. It should always be the first goal to find other causes for a deterioration of lung function, e.g. infections? The information you provide does not rule out a recurring ABPA, but it is by no means definite enough to confirm an ABPA diagnosis. I can try to help by giving you some tips that could make a clarifying diagnostic conversation with your CF doctor easier.

In order to determine whether you do indeed have an ABPA relapse, the following aspects should be considered above all:

What are your current problems (noticeable shortness of breath)?
Is there a short-term decline in FEV1 that is not due to an infection?
Has the IgE increased (here the progression is extremely important, usually values over 1000 point to ABPA)? How has the specific IgE changed (RAST test progression). What is the result of the specific IgG?
Is aspergillus traceable in the sputum?
Are there new occurrences of radiologic changes (a CT of the thorax can be very helpful here)?

Only a synopsis of all test results can lead to a diagnosis. If ABPA is diagnosed, and after weighing benefits against side effects, a systemic cortisone and itraconazole (Sempera®) therapy should be started and carried out for an adequate time. Doing so, in your specific case, the diabetes mellitus should be taken into account. For patients prone to ABPA relapses, a long-term itraconazole therapy should be considered. Also, it should be checked regularly whether the patient lives in a largely mould-free environment.

After going through all these aspects with your CF doctor, I am confident that you will get adequate therapy.

Kind regards,
Dr. Christina Smaczny

16.01.2012