Airway-centered interstitial fibrosis (ACIF) is a distinct type of lung interstitial fibrosis characterized by lesions centered on the airways. Several cases reported in the literature showed little to no effect of corticosteroids and a high mortality rate in the absence of lung transplantation. No other efficient approach is described for the treatment of this type of fibrosis. We report for the first time the case of a 44-year-old patient diagnosed with ACIF on surgical lung biopsy and stabilized with clarithromycin after failure of systemic corticosteroids. We need to confirm this benefit in other patients to ascertain the anti-inflammatory effect of macrolides, which are far less harmful compared to corticosteroids or immunosuppressant drugs.

Churg A, Myers J, Suarez T, Gaxiola M, Estrada A, Mejia M, Selman M: Airway-centered interstitial fibrosis: a distinct form of aggressive diffuse lung disease. Am J Surg Pathol 2004;28:62–68.
Gaxiola M, Buendia-Roldan I, Mejia M, Carrillo G, Estrada A, Navarro MC, Rojas-Serrano J, Selman M: Morphologic diversity of chronic pigeon breeder’s disease: clinical features and survival. Respir Med 2011;105:608–614.
Reghellin D, Poletti V, Tomassett S, Dubini A, Cavazza A, Rossi G, Lestani M, Pedron S, Daniele I, Montagna L, Murer B, Chilos M: Cathepsin-k is a sensitive immunohistochemical marker for detection of micro-granulomas in hypersensitivity pneumonitis. Sarcoidosis Vasc Diffuse Lung Dis 2010;27:57–63.
Hara Y, Kobayashi H, Osoreda H, Kanoh S, Motoyoshi K, Ozeki Y: Bird-related chronic hypersensitivity pneumonitis demonstrating the fluctuation of disease activity accompanied with environmental changes. Nihon Kokyuki Gakkai Zasshi 2008;46:1045–1049.
Yi XH, Chu HQ, Cheng XM, Luo BF, Li HP: Idiopathic airway-centered interstitial fibrosis: report of two cases. Chin Med J (Engl) 2007;120:847–850.
Fenton ME, Cockcroft DW, Wright JL, Churg A: Hypersensitivity pneumonitis as a cause of airway-centered interstitial fibrosis. Ann Allergy Asthma Immunol 2007;99:465–466.
Colombat M, Groussard O, Taille C, Marrash-Chahla R, Brugiere O, Mal H, Leseche G, Fournier M, Degott C: Lung transplantation in a patient with airway-centered fibrosis. Am J Surg Pathol 2004;28:1540–1542.
Serrano M, Molina-Molina M, Ramirez J, Sanchez M, Xaubet A: Airway-centered interstitial fibrosis related to exposure to fumes from cleaning products. Arch Bronconeumol 2006;42:557–559.
Altenburg J, de Graaff CS, van der Werf TS, Boersma WG: Immunomodulatory effects of macrolide antibiotics. 2. Advantages and disadvantages of long-term, low-dose macrolide therapy. Respiration 2011;81:75–87.
Altenburg J, de Graaff CS, van der Werf TS, Boersma WG: Immunomodulatory effects of macrolide antibiotics. 1. Biological mechanisms. Respiration 2011;81:67–74.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.