ANSWERThe interpretation of BAL results is difficult as it is marred by the limited data available in the pediatric population. It can also be influenced by the sampling technique such as the size of the aliquots used etc. The cytology shows a marked eosinophilia. This is consistent with a diagnosis of CEP .The eosinophil count is usually normal in hypersensitivity pneumonitis. The lymphocyte count is also elevated. This can occur with sarcoidosis and hypersensitivity pneumonitis. The CD 4: CD 8 ratio is high. This is consistent with CEP. In hypersensitivity pneumonitis the CD4 to CD8 ratio is low.
At this point we feel the most likely diagnosis is CEP. A brief discussion of this condition will be followed by the management and follow up of this patient as well as a discussion regarding lung biopsy.
CEP was first described in 1969 by Carrington et al when he described a series of 9 women with a characteristic CXR consistent biopsy features and responsiveness to steroids. Since then there has been numerous case reports published in the literature.
There is a large age range with this condition although there is only one case report in a child. There are some adolescents included in some adult series. The mean age is 45 yeas with women more commonly affected (gender ratio 2:1). CEP is commoner in those who do not smoke and there is often a history of prior asthma.
The presenting features are usually non-specific systemic and respiratory symptoms including cough, malaise, dyspnea, night sweats and weight loss. Chest pain, hemoptysis, sputum, chronic rhinitis, sinusitis are rare presenting features.
Initial laboratory investigations typically reveal anemia, an elevated ESR and peripheral eosinophilia in two thirds of cases. There is often an elevated IgE. Pulmonary function tests show a restrictive pattern and a decreased diffusing capacity
The etiology of the condition is unknown but it is thought to be an exposure related disease mediated by a type I Hypersensitivity reaction. It is thought to be linked to asthma and Churg-Strauss Syndrome
This shows characteristic findings with intra-alveolar and interstitial infiltrates with eosinophils, macrophages, plasma cells and lymphocytes
CEP is characterized by a dramatic response to corticosteroids. The optimal length of treatment and prognosis is difficult to ascertain as there are not many cases reported in the pediatric literature. Rao et al reported a case of CEP in a 1 year old in 1975. This baby boy had been unwell for 6 months. He had bilateral hilar adenopathy, peripheral upper lobe infiltrates with no response to antibiotics. A lung biopsy showed acute and chronic inflammation and eosinophils and cultures were negative. Within 1 week of steroid treatment there was dramatic resolution of symptoms, examination and CXR appearances. His CXR at 6 months was normal. Steroids were weaned after eight months and discontinued after 1 year.
The prognosis in adults with this condition has been more closely studied. Pearson et al published a follow up study on the 9 patients originally described by Carrington et al. At 5.5 and 8 years only 2 patients were still on steroids. Naughton et al described follow up in 12 patients with mean follow up of 10.2 years. Multiple relapses when corticosteroids weaned or discontinued were found. Similarly Marchand et al who published the largest follow up study if 62 patients in 1988 found that most of the patients were corticosteroid dependent. This group also found that many of the patients had an obstructive pattern on spirometry and required inhaled corticosteroids for asthma. Two patients in this series had extra respiratory involvement including mononeuritis multiplex.
WOULD YOU CARRY OUT A LUNG BIOPSY IN THIS PATIENT?