Nonspecific Interstitial Pneumonia/Fibrosis: Clinical Manifestations, Histologic and Radiologic Features

Abstract

ObjectivesCustomarily used classification of IPF did not satisfy a sizable group of patients with IPF that in the past had been lumped with UIP and now currently has begun to be recognized as nonspecific interstitial pneumonia/fibrosis (NIP). There are few reports about the clinical features of NIP.MethodsThe pathologic slides of 66 patients having open lung biopsy (OLB) for the differential diagnosis of interstitial lung diseases (ILD) from 1984 to 1995 were reviewed. Seven cases were confirmed as NIP. The clinical record, pulmonay function tests (PFT), chest PA and HRCT were reviewed and analysed retrospectively.ResultsSix patients with NIP were female. The median age was 56 years. Mean duration of symptoms was 4 months. Five patients had systemic flu-like symptoms. the most common respiratory symptom was gradual dyspnea Two patients revealed a mild degree of anemia Four cases had leukocytois of more than 10,000/mm3. ESR and CRP O.K. elevated in all measured cases. Anti-nuclear antibody (ANA) was positive in three of six patients and ds-DNA antibody was positive in one of six patients Restrictive pattern of PFT was predominant. Diffusion capacity of carbonmonoxide (DLCO) decreased markedly. In bronchoalveolar lavage (BAL), total cell counts elevated about three times of normal value. On differntial counts of BAL cells, lymphocytes, neutrophils and eosinophils were higher than those of normal controls. The prominent finding of chest radiology was bilaterally patchy opacifications in parenchyme of lower lung zones. On HRCT, bilaterally patchy areas of ground-glass attenuation and/or areas of alveolar consolidation were commonly shown. The number of pathologic type was one case of group I, four cases of group II and two cases of group III. The average period from diagnosis to the last follow-up was 24.8 months. Five patients were clinically recovered to the previously well-being state.ConclusionPatients with NIP had different clinical features from UIP, AIP and DIP. They also had characteristic findings of radiology and their prognosis seems to be better than UIP.

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.…”

“…In light of the new classification, it can be argued that a BAL lymphocytosis may be suggestive of NSIP, as indicated in the recent ATS statement [3]. In small groups of NSIP patients, not necessarily characterised by the clinical features of IPF, a BAL lymphocytosis has been observed [16][17][18][19]. By contrast DANIIL et al [8] did not find a BAL lymphocytosis in a small cohort of NSIP patients.…”

The pathogenesis of pulmonary fibrosis: a moving target

“…Later, with the emergence of NSIP as a distinct entity [49], it was suspected that many patients with a BAL lymphocytosis, formerly diagnosed with IPF, did in fact have NSIP. This is supported by the fact that three major East Asian studies showed truly striking lymphocyte levels in NSIP [50][51][52]. It is suggested from high-resolution computed tomography scan observations that those patients might have organising pneumonia/NSIP overlap.…”

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

“…Discussion NSIP was first described by Katzenstein and Fiorelli in 1 994 (1). It has been reported that the prognosis of patients with this illness is significantly better than that for patients with UIP (1,5,7,8). Katzenstein and Fiorelli reported that almost 50%of patients completely recovered after steroid therapy and the overall mortality was 1 1 % (1).…”