Abstract
Introduction: We aim to document the variability in survival across the range of diagnostic categories of ILD for patients treated at our hospital.
Methods: The total number of patients with interstitial lung disease referred to our department from 1999 to date is 620. The patients were retrospectively analyzed in this study using Kaplan-Meier survival analysis.
Results: The patients were subdivided into diagnostic groups: collagen vascular disease-associated ILD (CVD-ILD, n = 144), diffuse alveolar haemorrhage (DAH, n=36), idiopathic pulmonary fibrosis (IPF, definite; n = 92, probable; n = 107), non-IPF idiopathic interstitial pneumonia (IIP, n = 33), rare forms of ILD (n = 25) and sarcoidosis (n = 183). Of the patients diagnosed with IPF, 43 had had a surgical biopsy. Of the patients diagnosed with sarcoidosis, 174 had had a biopsy. From the whole group, 143 died, including 92 with a diagnosis of IPF but only 2 with sarcoidosis. Rare ILD and sarcoid were associated with excellent survivals by Kaplan-Meier analysis (means 201 and 180 months respectively), in contrast to the other groups (means 50 to 82 months). There was no significant difference in the survival between the definite and probable IPF groups. There was also no significant survival difference between those with CVD-ILD and those with other non-IPF IIP. Considering all IPF vs. CVD-ILD, there was a significant difference in survival (p = 0.047).
Conclusion: The need for biopsy to confirm the diagnosis varies across the range of ILD. There is great variation in survival, with IPF having significantly worse survival than other types of ILD, including CVD-ILD.
- Copyright ©ERS 2015