Clinical Features of Three Fatal Cases of Non-specific Interstitial Pneumonia.

Abstract

Wedescribe the clinical courses of the 3 fatal patients (2 females and 1 male) with idiopathic non-specific interstitial pneumonia (NSIP) among 24 patients with NSIP. Lung biopsies were diagnosed to be NSIP group II in all patients. The clinical courses from onset to death of these 3 patients were 41 months, 46 months, and 91 months. A follow-up chest CT demonstrated no apparent honey-combformation. Wefound that i) about 20%of patients with NSIP died of respiratory failure, ii) in the chest CT findings, apparent honey-combformation was rare even just before death, iii) prediction of the prognosis based on the histological findings was difficult. This is the first report to describe the clinical features of deceased patients with idiopathic NSIP; the incidence of fatal cases was considered to range from 10 to 20%.

Nonspecific interstitial pneumonia: a real clinical entity?

“…It seems difficult to detect significant findings as prognostic factors, however. Even in fatal NSIP cases, a follow-up chest CT demonstrated no apparent honeycombing [38]. Another report concluded that patients with interlobular interstitial thickening and traction bronchiectasis showed favorable response to therapy [28].…”

“…The presence of desaturation during a 6-minute walk test was associated with an increased hazard of death after adjusting for age, sex, smoking, baseline diffusion capacity for carbon monoxide, forced vital capacity (FVC), and resting saturation in patients with IPF/UIP (n = 83) and NSIP (n = 22) [38]. Changes in physiologic variables over 6 and 12 months may be more accurate prognostic information than baseline values alone [40].…”

BAL findings in idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia

“…(1-18)) and UIP patients (lymphocytes 6 (0-38), neutrophils 8.5 (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20), eosinophils 5 (0-11)). There was no statistical difference in the total and differential cell counts between the two groups (Mann-Whitney test p=NS for all cell types; fig.…”

“…However, although initial reports showed large differences in mortality between the two groups [7][8][9], later studies documented a high 5-and 10-yr mortality rate in fibrotic NSIP [5,10]. Cellular NSIP, by contrast, has a 10-yr survival of 100% [5,6].…”

Comparison of clinicoradiologic manifestation of nonspecific interstitial pneumonia and usual interstitial pneumonia/idiopathic pulmonary fibrosis: A report from NRITLD

“…Clubbing was reported in 10% of the patients with NSIP and in 65% of IPF cases, including UIP. [1213] It appears that the clinical presentation of NSIP and UIP are similar.…”