Interstitial lung diseases: diagnosis and treatment

Abstract

Interstitial lung disease (ILD) is rare in childhood. The aim of this study was to describe the clinical, radiological and histological features and treatment responses in 9 patients enrolled between 1997 and 2002 in our clinic.

Methods: all patients underwent an extensive laboratory and immunological evaluation to detect systemic disorders, including microbiological studies using bronchio alveolarlavage (BAL). The diagnosis was made using lung biopsy. The biopsy site was selected from a thin cut CT chest scan and performed using video assisted thoracoscopy (VAT).

Results: the mean age at diagnosis was 5 years, 6 were girls. All patients had cough, tachypnoea, "velcro" crackles and chest X-ray and CT infiltrates. The average duration of symptoms was 3,2 years. Symptoms started at 3 months in 5 children. 3 children had a primary immunodeficiency (hair-cartilage syndrome, fascial-cardiac-hair syndrome and hyper-IgM syndrome), 1 had a collagen-vascular disease. There were no complications with the VAT biopsy, allowing sufficient material to be obtained for a diagnosis to be made. In 9 children with idiopathic ILD, the hystological pattern was chronic interstitial inespecific pneumonitis (CIIP) in 7, linfoid interstitial pneumonitis (LIP) in 1 and desquamative interstitial pneumonitis (DIP) in 1. The child with DIP received pulse methylprednisolone and one case of NICI prednisone alone. After 2 months of treatment both showed a complete remission. Patients with primary immunodeficiency inspite of treatment with prednisone or chloroquine developed extensive pulmonary fibrosis, respiratory insufficiency and finally died, after 2 years of progression. 1 child with biopsy proven fibrosis did not receive treatment. The other 3 patients remained stable, being treated with prednisone and chloroquine in 2 cases, and prednisone alone in 1.

Conclusion: In our series, VAT lung biopsy confirmed the diagnosis in all cases, the prognosis was not associated with histological pattern and immunodeficiency was associated with a worst prognosis with the development of pulmonary fibrosis.