Idiopathic Nonspecific Interstitial Pneumonia: Prognostic Significance of Cellular and Fibrosing Patterns

Abstract

Nonspecific interstitial pneumonia (NSIP) has been proposed as a histologic subtype of idiopathic interstitial pneumonia with lung biopsy findings that are inconsistent with those of other idiopathic interstitial pneumonias. NSIP has a broad spectrum of histologic findings and a variable prognosis. The aim of this study was to determine whether it would be preferable to subdivide NSIP into cellular and fibrosing patterns. The authors classified lung biopsies from 101 patients with idiopathic interstitial lung disease as having histologic patterns of desquamative interstitial pneumonia (DIP), usual interstitial pneumonia (UIP), or cellular or fibrosing NSIP. Survival analysis was performed using the Kaplan-Meier method. Due to histologic, clinical, and survival similarities, the patients with idiopathic NSIP with lung biopsies that showed fibrosing as well as fibrosing and cellular patterns were combined into a single group of NSIP, fibrosing pattern. Of the 101 patients, 16 patients (9 women, 7 men) had idiopathic DIP; 56 patients (17 women, 39 men) had idiopathic UIP; 22 patients (7 women, 15 men) had idiopathic NSIP, fibrosing pattern; and 7 patients (2 women, 5 men) had idiopathic NSIP, cellular pattern. The patients had a mean age of 42, 51, 50, and 39 years respectively. Patients with idiopathic NSIP, cellular pattern had a better 5- and 10-year survival than those with idiopathic NSIP, fibrosing pattern (100% vs 90% and 100% vs 35% respectively, p = 0.027). Survival of patients with idiopathic UIP was worse than that of patients with idiopathic NSIP, fibrosing pattern (p = 0.014). The difference, however, was more evident at 5 years (43% vs 90%) than at 10 years (15% vs 35%). The 5- and 10-year survival of patients with idiopathic NSIP, cellular pattern and DIP was 100%, which was significantly better than that of patients with idiopathic UIP (p <0.0001). Based on these data, NSIP should be separated into cellular and fibrosing patterns, because these histologic patterns are associated with different clinical characteristics and prognoses.

Cyclosporin Treatment in Steroid-resistant and Acutely Exacerbated Interstitial Pneumonia

“…The prognoses after initiating CsA treatment in our series of patients as a whole are very similar to those reported in the previous studies (19)(20)(21)(22). Cellular-NSIP and OP showed better prognoses than fibrotic-NSIP, UIP or DAD.…”

Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment

“…1). [1][2][3][4][9][10][11][12] Honeycomb cysts are enlarged and distorted airspaces resulting from destruction of the normal alveoli. 4 Fibroblast foci are areas with palisades of fibroblasts and connective tissue located just beneath hyperplastic type 2 pneumocytes.…”

“…There are 7 distinct histological groups (see Table 1) with similar clinical presentations, but their clinical courses and responses to therapy differ. [4][5][6][7][8][9][10][11][12] …”

Cavitary Secondary Organizing Pneumonia: A Distinct Clinical Entity: Case Report

“…Difüz bilateral infiltratif patern daha nadirdir, idiyopatik pulmoner fibroz ve nonspesifik interstisyel pnömoni gibi diğer idiyopatik interstisyel pnömonilerle örtüşebilmektedir. 7 Fokal soliter opasiteler ise karakteristik olmayıp neoplastik hastalıklarla karışa-bilmektedir, bu lezyonlar PET-BT'de yanlış pozitif sonuçlara da yol açabilmektedir. 8 Soliter nodüller, daha sık üst loblarda yerleşmekte ve kaviteleşebil-mektedir.…”