Successful treatment of idiopathic pulmonary capillaritis with intravenous cyclophosphamide.

Idiopathic pulmonary hemosiderosis (IPH), a subtype of diffuse alveolar hemorrhage is a rare condition, first described by Virchow in 1864. Historically, it manifests in children in the first decade of life with the combination of hemoptysis, iron deficiency anemia, and alveolar infiltrates on chest radiograph. More recently, diffuse alveolar hemorrhage has been classified by the absence or presence of pulmonary capillaritis (PC), the latter carrying a potential for a poorer outcome. While systemic corticosteroids remain the first line treatment option, other immune modulators have been trailed including hydroxychloroquine, azathioprine, 6-mercaptopurine, and cyclophosphamide with varying results. Our case demonstrates for the first time, the successful use of intravenous cyclophosphamide in the management of chronic idiopathic PC.

Chest radiographs for outcome assessment in cystic fibrosis.

We compare a chest radiographic scoring system developed by our group to spirometry in quantifying the longitudinal progression of lung disease among cystic fibrosis (CF) patients, and we evaluate the use of this radiographic scoring system in identifying the treatment effect of an inhaled antibiotic. Results suggest that longitudinally acquired chest radiographs, when scored using our scoring system, are at least as sensitive as lung function in detecting the progression of lung disease in CF patients.

CF: an X-ray database to assess effect of aerosolized tobramycin.

This paper assesses the effectiveness of aerosolized tobramycin (TOBI) on cystic fibrosis (CF) lung disease, using a radiologic tool. The published tool, the age-based severity curve (ABS), is derived from Brasfield scoring of chest X-rays (CXR). This study evaluates both the usefulness of the ABS as an assessment tool and the effectiveness of TOBI. Thirty-eight patients were treated with TOBI. Twenty-four treated with dornase alfa were excluded. Fourteen patients, aged 2 months to 22 years (mean, 17 months of TOBI treatment), comprised the study group. Radiographs were obtained over a mean of 7.8 years (SD = 6.5 years; range, 9 months-18 years). Two hundred and eighty-two CXR of TOBI patients were analyzed following the ABS protocol. Rate of decline in radiologic status of the TOBI group and ABS were compared. Also, TOBI was assessed by comparing rate of decline before and after initiation of treatment. The TOBI group's radiologic assessment was compared to its rate of decline in pulmonary function studies and published population data. Rate of decline in ABS was 0.175 Brasfield points/year vs. 0.150 points/year in the TOBI group (P < 0.001). Before treatment, the TOBI group's rate of decline was 0.169 Brasfield points/year; after treatment, it was 0.150 points/year (P = 0.02). Forced vital capacity revealed a statistically significant slowing in rate of decline on TOBI. Although not statistically significant, rate of decline in forced expiratory volume at 1 sec showed a similar trend. The degree of slowing in decline is similar to that previously reported for pulmonary function studies.