Airway-Centered Interstitial Fibrosis

Etiology, Clinical Findings and Prognosis

Lilian Tiemi Kuranishi; Kevin O Leslie; Rimarcs Gomes Ferreira; Ester Aparecida Ney Coletta; Karin Mueller Storrer; Maria Raquel Soares; Carlos Alberto de Castro Pereira

Disclosures

Respiratory Research. 2015;16(55) 

In This Article

Abstract and Introduction

Abstract

Background: Airway-centered Interstitial Fibrosis (ACIF) is a common pathologic pattern observed in our practice.

Objectives: The objectives of this study are to describe the causes associated with ACIF in a large sample of patients and its effect on survival.

Methods: A retrospective study in three centers of interstitial lung disease in São Paulo, between January of 1995 and December of 2012. The surgical lung biopsy specimens were reviewed by three pathologists. The clinical, functional and tomographic findings were analyzed by a standardized protocol.

Results: There were 68 cases of ACIF, most of them women. The mean age was 57 ± 12 yr. Dyspnea, cough, restrictive pattern at spirometry and oxygen desaturation at exercise were common. A reticular pattern with peribronchovascular infiltrates was found in 79% of the cases. The etiologies of ACIF were hypersensitivity pneumonitis in 29 (42.6%), gastroesophageal reflux disease in 17 (25.0%), collagen vascular disease in 4 (5.9%), a combination of them in 15 cases and idiopathic in 3 (4.4%). The median survival was 116 months (95% CI = 58.5 – 173.5). Lower values of oxygen saturation at rest, presence of cough and some histological findings - organizing tissue in the airways, fibroblastic foci and microscopic honeycombing - were predictors of worse survival.

Conclusions: ACIF is an interstitial lung disease with a better survival when compared with IPF. The main etiologies are HP and GERD. The oxygen saturation at rest, the presence of cough and some histological findings are predictors of survival.

Introduction

Since 2002 a new interstitial pneumonia centered on small airways and no granulomas has been described in small series.[1–4] This entity has been variably called centrilobular fibrosis, bronchiolocentric interstitial pneumonia, airway-centered interstitial fibrosis, and peribronchiolar metaplasia.[3–6] Lung biopsies from these cases showed a distinctive pattern of interstitial fibrosis centered and extending around the bronchioles, often with bronchiolar metaplasia of the epithelium. Due to involvement of small as well large airways,[5,7] the best name seems to be airway-centered interstitial fibrosis (ACIF). The prognosis is unknown due to small number of cases described.[2–4] The etiology in these series was unclear. Similar pathologic findings have been described in hypersensitivity pneumonitis (HP)[8–15] or be secondary to gastroesophageal reflux disease (GERD), isolated or associated with connective tissue diseases.[16,17] In our clinical practice this pathologic pattern is common in multidisciplinary case discussions.

The objectives of the present study were to describe the causes, the clinical, tomographic, functional and pathologic findings and their influence on survival in a large number of patients with histologic diagnosis of airway-centered interstitial fibrosis.

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