ANSWERFindings are consistent with Crohns disease.
PROGRESSHe was commenced on Azathioprine, prednisone and Losec (omeprazole) with complete resolution of his respiratory symptoms. His PFTs returned to normal including his DLco (DLco 78, DLco/VA 81).
He had ongoing problems with GI symptoms, including fissures, particularly with cessation or weaning of his medications.
Whilst weaning prednisone he developed a dry cough, dyspnea with exercise and hypoxia with activity, similar to his initial presentation. His CXR was unremarkable. His PFTs demonstrated mild airflow limitation and his DLco values were normal (DLco 84, DLco/VA 73). Mycoplasma serology was negative. He improved on a course of Azithromycin.
Prednisone was weaned further. His symptoms improved slightly although mild dyspnea with exercise was still present. His PFTs were normal and his diffusion capacity was normal (DLco 78; DLco/VA 70).
main questions were:
1. What is the cause of his ongoing symptoms: is it an exacerbation of his pulmonary crohns disease or is it something else?
2. Should we monitoring these children and, if so, then what is the best way to do so?
the time, our main thoughts on these issues were:
1. The possible causes of his symptoms include exacerbation pulmonary Crohns disease, pulmonary complications of his medications, asthma, recent infection and march_03 infection. We also considered anxiety and anaemia as possible contributing factors for his presentation. We elected to continue weaning his prednisone and to assess his respiratory status a few weeks later both clinically and with DLco and PFTs. If there was any ongoing concerns at that point, or in the interim, we would then consider exercise PFTs and chest CTScan.
2. We decided that we should certainly monitor children with known pulmonary involvement of Crohns disease and that PFTs and DLco were the best way to do this.