Prognostic factors and disease behaviour of pathologically proven fibrotic non‐specific interstitial pneumonia. Issue 11 (24th April 2018) - Yamakawa, Hideaki
ABSTRACT: Background and objective: Non‐specific interstitial pneumonia (NSIP) has heterogeneous characteristics in terms of background, disease behaviour and prognosis. This study of fibrotic NSIP (f‐NSIP) aimed to elucidate prognosis and disease behaviour from the viewpoint of clinical background and determine whether long‐term change of pulmonary function could provide useful prognostic information. Methods: We analysed the medical records of 157 consecutive patients diagnosed with f‐NSIP by surgical lung biopsy. Disease behaviour was categorized into two groups depending on long‐term change of pulmonary function: progressive type (relative ≥5%/year decline in the slope of forced vital capacity and/or relative ≥7.5%/year decline in the slope of %diffusing capacity of lung carbon monoxide) or stable type. Predictors of disease behaviour and prognosis were determined using logistic and Cox regression models. Results: Our f‐NSIP cohort included interstitial pneumonia with autoimmune features (IPAF) (36.9%), idiopathic (non‐IPAF) (22.3%) and connective tissue disease‐associated interstitial lung disease (40.8%). Multivariate analysis showed that idiopathic (non‐IPAF) f‐NSIP and progressive type disease were negative prognostic factors of mortality. Poor treatment response at 1 year was an independent predictor of progressive type disease, but was not related to survival. In terms of disease behaviour based on pulmonary function change, some patients with IPAF f‐NSIP showed a progressive course. Conclusion: Although an IPAF diagnosis was useful for identifying good prognosis in idiopathic f‐NSIP, some idiopathic f‐NSIP patients with or without IPAF showed progressive disease despite therapy. The definition of progressive type disease may be useful in clinical decision‐making when determining therapy for f‐NSIP. Abstract : Non‐specific interstitial pneumonia (NSIP) has heterogeneous characteristics in terms of background, disease behaviour and prognosis. Some patients with idiopathic fibrotic NSIP with or without interstitial pneumonia with autoimmune features (IPAF) showed progressive disease despite therapy.