PURPOSE: To evaluate the clinical utility of various indicators of survival in cellular and fibrotic patterns of nonspecific interstitial pneumonia (NSIP).
METHODS: Between January 1995 and December 2006 we prospectively studied 29 NSIP patients (pts) subcategorized as cellular NSIP (n = 11) and fibrotic NSIP (n = 18). All cases were histologically diagnosed by surgical lung biopsy. Baseline characteristics included: gender, age at diagnosis, smoking habits, New York Heart Association class (NYHA), systolic pulmonary artery pressure (sPAP mmHg), Octreoscan uptake index (UI), HRCT score following ATS/ERS criteria, and treatment. Possible treatments were high-dose prednisone alone (1 mg/kg/d for 3 mo, with a subsequent taper) (P), or high-dose prednisone (P) in combination with cyclophosfamide (CYC). All cellular pattern pts were treated with P. Nine out of 11 fibrotic pattern pts treated with P–CYC and two with P. Survival analysis was performed using the Kaplan-Meier method and the prognostic effect of variables was evaluated using log-rank test. The endpoint was death.
RESULTS: No pts with a cellular pattern was deceased. By contrast, 7 pts (64%) deceased among fibrotic ones with a median survival time of 49 months. Age, HRCT, sPAP, and NYHA, with p<0.05, p<0.05, p<0,01 and p<0.01 respectively, were significant prognostic indicators of NSIP survival. Octreoscan U.I. was increased in all pts and the uptake was significantly higher in cellular pattern than in fibrotic pattern pts (p<0.01). No difference in survival was showed according to sex and smoking habits. Therapy was determined by histological NSIP pattern and no prognostic evaluation was feasible.
CONCLUSION: We conclude that: i) fibrotic NSIP yields a worse survival,ii) pulmonary hypertension evaluated by sPAP and NYHA seems to be the best indicator of survival, iii) by distinguishing histological NSIP, Octreoscan uptake should be a good marker of disease identifying patient outcomes.
CLINICAL IMPLICATIONS: The clinicians should be better able to recognize early NSIP diagnosis, optimizing treatment strategies, and to improve patient outcomes.
DISCLOSURE: Roberto Carbone, No Financial Disclosure Information; No Product/Research Disclosure Information