UpToDate
Official reprint from UpToDate®
www.uptodate.com ©2016 UpToDate®

Bronchocentric granulomatosis

Author
Talmadge E King, Jr, MD
Section Editor
Kevin R Flaherty, MD, MS
Deputy Editor
Helen Hollingsworth, MD

INTRODUCTION

Bronchocentric granulomatosis is a destructive, granulomatous lesion of the bronchi and bronchioles that is generally believed to represent a nonspecific response to a variety of types of airway injury [1-3]. Approximately half of all cases are associated with asthma and allergic bronchopulmonary aspergillosis (ABPA), and among these patients, bronchocentric granulomatosis may represent a histopathologic manifestation of fungal hypersensitivity [3-7]. (See "Clinical manifestations and diagnosis of allergic bronchopulmonary aspergillosis".)

The remaining cases of bronchocentric granulomatosis are usually idiopathic, although associations with mycobacterial and fungal infection, rheumatologic disease, granulomatosis with polyangiitis (Wegener’s), chronic granulomatous disease, glomerulonephritis, scleritis, diabetes insipidus, red cell aplasia, pulmonary echinococcosis, bronchogenic carcinoma, and influenza A virus have been reported (table 1) [3,8-24].

Because of the lack of a clear clinical syndrome associated with bronchocentric granulomatosis, the presence of this lesion should generally be considered a nonspecific manifestation of lung injury, not an etiologic diagnosis.

An overview of bronchocentric granulomatosis will be presented here. Pulmonary lymphomatoid granulomatosis, a different clinicopathological entity usually related to Epstein-Barr virus-associated lymphoma, and an approach to an adult with suspected interstitial lung disease, are discussed separately. (See "Pulmonary lymphomatoid granulomatosis" and "Approach to the adult with interstitial lung disease: Clinical evaluation" and "Approach to the adult with interstitial lung disease: Diagnostic testing".)

CLINICAL MANIFESTATIONS

The incidence and prevalence of bronchocentric granulomatosis are unknown. Bronchocentric granulomatosis is often divided clinically into two groups of patients based upon the presence or absence of asthma [1,2,5]. Patients with asthma tend to be younger (ages 20 to 40) and generally present with pulmonary symptoms such as cough, dyspnea or pleuritic chest pain. Additional features consistent with allergic bronchopulmonary aspergillosis (ABPA) may be seen, ie, fever, malaise, expectoration of brownish mucus plugs (picture 1) [25]. The nonasthmatic group is made up primarily of older patients, ranging in age from 30 to 70. Chest symptoms are rare, and nonspecific fatigue and malaise are more common.

        

Subscribers log in here

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information or to purchase a personal subscription, click below on the option that best describes you:
Literature review current through: Nov 2016. | This topic last updated: Thu Sep 03 00:00:00 GMT 2015.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2016 UpToDate, Inc.
References
Top
  1. Liebow AA. The J. Burns Amberson lecture--pulmonary angiitis and granulomatosis. Am Rev Respir Dis 1973; 108:1.
  2. Clee MD, Lamb D, Clark RA. Bronchocentric granulomatosis: a review and thoughts on pathogenesis. Br J Dis Chest 1983; 77:227.
  3. Myers JL. Bronchocentric granulomatosis. Disease or diagnosis? Chest 1989; 96:3.
  4. Cordier, JF. Eosinophilic pneumonias. In: Interstitial Lung Disease, 4th ed, King TE Jr, Schwarz MI (Eds), B.C. Decker, Hamilton, ON, Canada 2003. p.679.
  5. Katzenstein AL, Liebow AA, Friedman PJ. Bronchocentric granulomatosis, mucoid impaction, and hypersensitivity reactions to fungi. Am Rev Respir Dis 1975; 111:497.
  6. Koss MN, Robinson RG, Hochholzer L. Bronchocentric granulomatosis. Hum Pathol 1981; 12:632.
  7. Sulavik SB. Bronchocentric granulomatosis and allergic bronchopulmonary aspergillosis. Clin Chest Med 1988; 9:609.
  8. Berendsen HH, Hofstee N, Kapsenberg PD, et al. Bronchocentric granulomatosis associated with seropositive polyarthritis. Thorax 1985; 40:396.
  9. Bonafede RP, Benatar SR. Bronchocentric granulomatosis and rheumatoid arthritis. Br J Dis Chest 1987; 81:197.
  10. Den Hertog RW, Wagenaar SS, Wastermann CJ. Bronchocentric granulomatosis and pulmonary echinococcosis. Am Rev Respir Dis 1982; 126:344.
  11. Hellems SO, Kanner RE, Renzetti AD Jr. Bronchocentric granulomatosis associated with rheumatoid arthritis. Chest 1983; 83:831.
  12. Rohatgi PK, Turrisi BC. Bronchocentric granulomatosis and ankylosing spondylitis. Thorax 1984; 39:317.
  13. Rossi GP, Pavan E, Chiesura-Corona M, et al. Bronchocentric granulomatosis and central diabetes insipidus successfully treated with corticosteroids. Eur Respir J 1994; 7:1893.
  14. Wiedemann HP, Bensinger RE, Hudson LD. Bronchocentric granulomatosis with eye involvement. Am Rev Respir Dis 1982; 126:347.
  15. Warren J, Pitchenik AE, Saldana MJ. Bronchocentric granulomatosis with glomerulonephritis. Chest 1985; 87:832.
  16. Yano S, Shishido S, Kobayashi K, et al. Bronchocentric granulomatosis due to Aspergillus terreus in an immunocompetent and non-asthmatic woman. Respir Med 1999; 93:672.
  17. Yousem SA. Bronchocentric injury in Wegener's granulomatosis: a report of five cases. Hum Pathol 1991; 22:535.
  18. Myers JL, Katzenstein AL. Granulomatous infection mimicking bronchocentric granulomatosis. Am J Surg Pathol 1986; 10:317.
  19. Fannin SW, Hagley MT, Seibert JD, Koenig TJ. Bronchocentric granulomatosis, acute renal failure, and high titer antineutrophil cytoplasmic antibodies: possible variants of Wegener's granulomatosis. J Rheumatol 1993; 20:507.
  20. Houser SL, Mark EJ. Bronchocentric granulomatosis with mucus impaction due to bronchogenic carcinoma. An association with clinical relevance. Arch Pathol Lab Med 2000; 124:1168.
  21. Barrot E, Ortega-Calvo M, Borderas F, et al. Bronchocentric granulomatosis as a first clinical manifestation in an adult patient with p67phox deficiency. Respiration 1999; 66:547.
  22. Moltyaner Y, Geerts WH, Chamberlain DW, et al. Underlying chronic granulomatous disease in a patient with bronchocentric granulomatosis. Thorax 2003; 58:1096.
  23. van der Klooster JM, Nurmohamed LA, van Kaam NA. Bronchocentric granulomatosis associated with influenza-A virus infection. Respiration 2004; 71:412.
  24. Bes C, Kılıçgün A, Talay F, et al. Bronchocentric granulomatosis in a patient with rheumatoid arthritis. Rheumatol Int 2012; 32:3261.
  25. Li H, He J, Gu Y, Zhong N. Corticosteroid monotherapy in a case of bronchocentric granulomatosis with a two-year follow-up. J Thorac Dis 2013; 5:E207.
  26. Robinson RG, Wehunt WD, Tsou E, et al. Bronchocentric granulomatosis: roentgenographic manifestations. Am Rev Respir Dis 1982; 125:751.
  27. Ward S, Heyneman LE, Flint JD, et al. Bronchocentric granulomatosis: computed tomographic findings in five patients. Clin Radiol 2000; 55:296.
  28. Hurwitz LM, McAdams HP, Sporn TA. A 73-year-old woman with a cough. Chest 2005; 128:1018.
  29. Bosken CH, Myers JL, Greenberger PA, Katzenstein AL. Pathologic features of allergic bronchopulmonary aspergillosis. Am J Surg Pathol 1988; 12:216.
  30. Neff K, Stack J, Harney S, Henry M. The use of abatacept in debilitating cavitating lung disease associated with rheumatoid arthritis, bronchocentric granulomatosis and aspergillosis. Thorax 2010; 65:545.