PURPOSE: Little is known about the survival of idiopathic pulmonary fibrosis (IPF) patients with histological findings of usual interstitial pneumonia (UIP) and Wegener Granulomatosis (WG) in correlation with pulmonary hypertension (PH). Aims: To compare the survival between UIP and WG patients related to PH and other prognostic factors.
METHODS: 59 patients with UIP and 16 with WG (Mean age 63.9 ± 9.4 yrs. range 27–79, and 63.5 ± 10.4 yrs. range 48–78 respectively) with or without PH, enrolled between 1999 and 2004 have been followed up to 15th December 2006. Age, systolic pulmonary artery pressure (sPAP), gender, New York Heart Association (NYHA) functional class, Octreoscan uptake index (UI) have been considered as prognostic factors. All patients had histological confirmed diagnosis.
RESULTS: Median survival was 52 and 92 months among UIP and WG patients, respectively. The difference was statistically significant (p=0.004). PH was strongly correlated with diagnosis. No UIP patient had a sPAP lower than 35 mmHg and no patient with WG had a sPAP higher than 54 mmHg. No deaths occurred among patients with lower sPAP values, but all deaths occurred among patients with the highest values for each disease (35–54 for WG cases and >54 for UIP cases). Age class was significant among UIP patients (p=0.04). In both groups survival was not correlated with gender and smoker habits. Octreoscan UI was correlated to diagnosis, as it was increased in all WG patients and within normal range in all UIP patients.
CONCLUSION: The study highlighted that: i) PH can be considered as a good survival prognostic factor and correlated with diagnosis, ii) age class was significant prognostic factor only among UIP patients, iii) Octreoscan UI confirmed an accurate diagnostic power, showing a close correlation between UIP (normal) and WG (increased).
CLINICAL IMPLICATIONS: To enable clinicians to improve the management of pulmonary hypertension associated with interstitial lung disease in the clinical practice.
DISCLOSURE: Roberto Carbone, No Financial Disclosure Information; No Product/Research Disclosure Information