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Multicenter Study
. 2015 May 9;16(1):55.
doi: 10.1186/s12931-015-0213-7.

Airway-centered interstitial fibrosis: etiology, clinical findings and prognosis

Lilian Tiemi Kuranishi  1 , Kevin O Leslie  2 , Rimarcs Gomes Ferreira  3 , Ester Aparecida Ney Coletta  4 , Karin Mueller Storrer  5 , Maria Raquel Soares  6 , Carlos Alberto de Castro Pereira  7
Affiliations
Multicenter Study

Airway-centered interstitial fibrosis: etiology, clinical findings and prognosis

Lilian Tiemi Kuranishi et al. Respir Res. .

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.

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Figures

Figure 1
Figure 1
Clinical diagnoses of patients with airway-centered interstitial fibrosis (n = 68).
Figure 2
Figure 2
Radiologic findings of a patient with ACIF, showing diffuse ground glass opacities on peribronchovascular region, reticular infiltrates and traction bronchiectasias.
Figure 3
Figure 3
Histologic features of airway-centered interstitial fibrosis. (A) Airway-centered fibrosis with obliteration of bronchioles, (B) a detailed centrilobular fibrosis from figure a. (C) Peribronchiolar alveolar metaplasia. (D) Inflammatory peribronchiolar infiltration, obliterative focal organization on airways.
Figure 4
Figure 4
Kaplan-Meier survival curve for patients with airway-centered interstitial fibrosis (n = 68); median survival = 116 months (95% CI = 58.5 - 173.5).
See this image and copyright information in PMC

References

    1. American Thoraci Society; European Respiratory Society American Thoracic Society/European Respiratory Society international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2002;165:277–304. doi: 10.1164/ajrccm.165.2.ats01. - DOI - PubMed
    1. Colby TV. Pathological approach to idiopathic interstitial pneumonias: useful points for clinicians. Breathe. 2004;1(1):43–9.
    1. De Carvalho ME, Kairalla RA, Capelozzi VL, Deheinzelin D, Do Nascimento Saldiva PH, Carvalho CR. Centrilobular fibrosis: a novel histological pattern of idiopathic interstitial pneumonia. Pathol Res Pract. 2002;198(9):577–83. doi: 10.1078/0344-0338-00305. - DOI - PubMed
    1. Yousem SA, Dacic S. Idiopathic bronchiolocentric interstitial pneumonia. Mod Pathol. 2002;15(11):1148–53. doi: 10.1097/01.MP.0000037309.04985.B4. - DOI - PubMed
    1. Churg A, Myers J, Suarez T, Gaxiola M, Estrada A, Mejia M, et al. Airway-centered Interstitial Fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol. 2004;28(1):62–8. doi: 10.1097/00000478-200401000-00006. - DOI - PubMed

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