PT  - JOURNAL ARTICLE
AU  - Barbara Bellofiore
AU  - Marialuisa Bocchino
AU  - Guiseppe Antinolfi
AU  - Antonio Ponticiello
AU  - Roberta Di Grazia
AU  - Alessandro Sanduzzi Zamparelli
AU  - Sepideh Nikfam
TI  - Could interferon-gamma 1b have a role in treatment of fibrosing NSIP?
DP  - 2011 Sep 01
TA  - European Respiratory Journal
PG  - p3743
VI  - 38
IP  - Suppl 55
4099  - http://erj.ersjournals.com/content/38/Suppl_55/p3743.short
4100  - http://erj.ersjournals.com/content/38/Suppl_55/p3743.full
SO  - Eur Respir J2011 Sep 01; 38
AB  - Aim: To detect the effectiveness of Interferon-gamma 1b in patients with fibrotic NSIP.Methods: We reviewed clinical records and pathologic findings of 11 patients with IPF (all males, mean age 54.2±6.3 yrs), who underwent surgical biopsy and were treated with IFN-gamma 1b between 2005 and 2007.In all patients, before therapy, after 6 and 12 (T2) months of treatment, the following parameters had been measured: lung volumes, DLCO, mPAP estimated at a doppler echocardiography. Biopsy slides were reviewed by two pathologists and reclassified according to the ATS/ERS consensus classification of interstitial pneumonia.Results: 5 out of 11 patients, previously diagnosed as Usual Interstitial Pneumonia, after the revision were identified as fibrotic NSIP. In ]real UIP subgroup, 2 patients died after 6 months of treatment, while all the 5 patients with fNSIP achieved at least 18 months of treatment with IFN gamma. Although the baseline values in NSIP group were better than those of UIP group, no significant difference in baseline lung function was observed between the two cohorts. In NSIP patients FVC (70.16±18,5% at T0 vs 72.4±24,8% at T2, p>0,05), TLC (64.2±8,7% at T0 vs 65.7±14.4% at T2, p>0,05) and DLCOsb (47.3±17.2% at T0 vs 47.8±17.2% at T2, p>0,05) showed a slight improvement trend over time. only the mPAP value showed a significant worsening after 12 months of treatment (27.18±8,7% at T0 vs 41.2±13,3% at T2, p =0,02).Conclusion: In this post-hoc analysis IFN-gamma 1b therapy showed a trend toward reduced disease progression in NSIP patients. The effects on the deterioration of the mPAP values need further investigation through more invasive procedures, such as the right heart catheterization.