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Clinical Radiology (2000) 55, 296–300<br />

doi:10.1053/crad.1999.0380, available online at http://www.idealibrary.com on<br />

<strong>Bronchocentric</strong> <strong>Granulomatosis</strong>: <strong>Computed</strong> <strong>Tomographic</strong><br />

<strong>Findings</strong> in Five Patients<br />

S. WARD*, L. E. HEYNEMAN*, J. D. A. FLINT†, A. N. LEUNG‡, E. A. KAZEROONI,<br />

N. L. MÜLLER*<br />

Departments of *Radiology and †Pathology, Vancouver Hospital and Health Sciences Centre,<br />

University of British Columbia, 855 W. 12 th Ave, Vancouver, BC, Canada V5Z 1M9; ‡Department of Radiology,<br />

Stanford University Medical Center, 300 Pasteur Drive, Stanford, CA 94305; and Department of Radiology,<br />

University of Michigan Hospitals, 1500 E. Medical Center Drive, Ann Arbor, MI 48109, U.S.A.<br />

Received: 17 June 1999 Revised: 31 August 1999 Accepted: 29 September 1999<br />

AIM: The aim of this study was to assess the CT manifestations of bronchocentric granulomatosis.<br />

SUBJECTS AND METHODS: The CT results of five patients with bronchocentric granulomatosis<br />

were retrospectively analysed. The patients ranged from 20 to 72 years of age and included three men<br />

and two women. The diagnosis of bronchocentric granulomatosis was made at lobectomy (n ¼ 2),<br />

open lung biopsy (n ¼ 2), and transbronchial biopsy (n ¼ 1). Only one of the five patients had asthma.<br />

RESULTS: The main findings consisted of a spiculated mass lesion (n ¼ 3) or lobar consolidation with<br />

associated mild volume loss (n ¼ 2). One of the two patients with consolidation had extensive mucoid<br />

impaction. The abnormalities involved predominantly an upper lobe in four patients and a lower lobe<br />

in one patient. In the four resected specimens, the macroscopic pathological appearance was<br />

consolidation (n ¼ 2) and mass lesion (n ¼ 2). Microscopically, the typical histology of airway-centred<br />

necrotizing granulomata was present in all cases. Aspergillus hyphae were identified in two cases.<br />

Nocardia sp. was cultured from the biopsy specimen in one case.<br />

CONCLUSION: The CT manifestations of bronchocentric granulomatosis consist of a focal mass<br />

or lobar consolidation with atelectasis. These reflect the presence of granuloma formation with<br />

or without associated bronchial obstruction. Ward, S. et al. (2000). Clinical Radiology 55, 296–300.<br />

2000 The Royal College of Radiologists<br />

Key words: bronchocentric granulomatosis, computed tomography, asthma, aspergillosis.<br />

<strong>Bronchocentric</strong> granulomatosis is a rare granulomatous disorder<br />

characterized histologically by granuloma formation and<br />

necrosis centred on bronchi and bronchioles [1,2]. Unlike the<br />

other granulomatoses, angiitis is not a major component.<br />

Vasculitis, when present, is incidental to inflammation in the<br />

adjacent conducting airways.<br />

In the original description by Liebow, the aetiology of<br />

bronchocentric granulomatosis was not identified, although<br />

two of the nine patients were noted to be asthmatic [1].<br />

Therefore, a hypersensitivity reaction was postulated. <strong>Bronchocentric</strong><br />

granulomatosis has also been reported in association<br />

with a variety of other conditions including Wegener’s granulomatosis,<br />

rheumatoid arthritis, heart–lung transplantation,<br />

bone marrow transplantation, red cell aplasia, obstructive<br />

pneumonia secondary to a proximal bronchogenic carcinoma,<br />

and infections such as histoplasmosis, blastomycosis, Mycobacterium<br />

avium intracellulare and tuberculosis [3–8].<br />

Author for correspondence: Dr Nestor L. Müller, Department of<br />

Radiology, Vancouver Hospital and Health Sciences Centre, 855 W. 12 th<br />

Ave, Vancouver, BC, Canada V5Z 1M9.<br />

Guarantor of study: Suzanne Ward, MRCP, FRCR.<br />

<strong>Bronchocentric</strong> granulomatosis is most commonly visualized<br />

on the chest radiograph as extensive areas of consolidation or<br />

as single or multiple nodules [1–3,5–7]. To our knowledge,<br />

there has been no previous report describing the CT<br />

appearances of bronchocentric granulomatosis in a series of<br />

patients.<br />

PATIENTS AND METHODS<br />

A retrospective review identified five patients with histologically<br />

proven bronchocentric granulomatosis who had undergone<br />

investigation at one of four institutions between October<br />

1989 and August 1997 (Table 1). The study group consisted of<br />

three men and two women aged 20–72 years (mean 41 years).<br />

One patient (patient 5) had a long history of asthma and a<br />

known diagnosis of allergic bronchopulmonary aspergillosis.<br />

The other four patients had no history of asthma. Pulmonary<br />

function tests performed in three of these four patients showed<br />

no evidence of reversible airflow obstruction. One patient had a<br />

previous history of tuberculosis and was noted to have an<br />

enlarging mass on the chest radiograph. One patient had<br />

undergone allogeneic bone marrow transplantation for acute<br />

0009-9260/00/040296+05 $35.00/0 2000 The Royal College of Radiologists


Table 1 – Summary of clinical data of five patients with bronchocentric granulomatosis<br />

lymphoblastic leukaemia. The respiratory symptoms consisted<br />

of cough (n ¼ 3), cough and shortness of breath (n ¼ 1), and<br />

haemoptysis (n ¼ 1). Two patients had constitutional symptoms<br />

of weakness and fatigue or weight loss.<br />

A diagnosis of bronchocentric granulomatosis was made<br />

histologically following lobectomy (n ¼ 2), open lung biopsy<br />

(n ¼ 2), and transbronchial biopsy (n ¼ 1). Aspergillus hyphae<br />

were identified in two of the specimens (cases 1 and 4, Table 1).<br />

In the other three specimens, no organisms could be identified,<br />

although Nocardia sp. was cultured from one of the biopsy<br />

specimens (case 3). Abundant tissue eosinophilia was noted<br />

within the specimen taken from the patient who had allergic<br />

bronchopulmonary aspergillosis (case 5).<br />

All patients underwent computed tomography. The median<br />

time interval between CT and the surgical procedure or biopsy<br />

was 18 days (range 7–41 days). Four patients had conventional<br />

CT with slice thicknesses between 5 and 10 mm. In one patient<br />

the conventional CT was supplemented by 1.5 mm sections<br />

targeted to the right upper lobe. The images were photographed<br />

at lung windows (W 800–2000; L ¹ 500 to ¹700 HU) and<br />

mediastinal windows (W 300–450; L 0–40 HU). One patient<br />

had high-resolution CT with 1 mm thick sections at 10 mm<br />

increments. The images were reconstructed using a high<br />

frequency spatial algorithm and photographed at lung windows<br />

(W 1500; L ¹ 700 HU).<br />

The CT results were analysed simultaneously by three<br />

radiologists and a final decision was reached by consensus.<br />

RESULTS<br />

In three patients (cases 1–3, Table 1), the main CT finding<br />

consisted of a spiculated mass or nodule ranging from 1 to 5 cm<br />

BRONCHOCENTRIC GRANULOMATOSIS 297<br />

Patient Age Sex History CT findings Mode of diagnosis Pathological features<br />

(years)<br />

1 72 F Enlarging LUL mass. 5 cm spiculated mass LUL Left upper lobectomy Consolidation. BCG.<br />

Cough Aspergillus hyphae. Vasculitis<br />

2 32 F Haemoptysis, Weakness, 2 cm cavitating nodule RLL. Open lung biopsy Consolidation surrounding<br />

fatigue. Joint pains 2 cm nodule LLL. Centrilobular mucoid-filled cavity.<br />

nodules RLL þ LLL. BCG. Foreign body-type<br />

Lymphadenopathy giant cells. Vasculitis.<br />

No organisms identified<br />

3 20 M Post-bone marrow transplant. 3 cm spiculated mass LUL. Open lung biopsy Parenchymal mass. BCG.<br />

Upper respiratory tract Ground-glass opacity LUL. Foreign body giant cells.<br />

symptoms. Dry cough Thickened interlobular septa Focal organizing pneumonia.<br />

Vasculitis.<br />

Nocardia sp. isolated from culture<br />

4 48 M Previous TB. Weight loss. Scarring and consolidation Right upper Inflammatory pseudotumour<br />

Cough. Smoker. RUL. Bronchiectasis in RUL lobectomy containing area of cystic<br />

Enlarging RUL mass and RLL. RLL nodules and bronchiectasis. BCG. Aspergillus<br />

patchy consolidation hyphae and aspergilloma<br />

identified<br />

5 35 M Asthma. Allergic Consolidation and atelectasis Transbronchial BCG. Palisaded histiocytes.<br />

bronchopulmonary LUL. Mucoid impaction LUL, biopsy Foreign body-type giant cells.<br />

aspergillosis LING, RUL. Patchy Focal organizing pneumonia.<br />

consolidation, RUL. No organisms identified<br />

Lymphadenopathy<br />

RUL, right upper lobe; RLL, right lower lobe; LUL, left upper lobe; LING, lingula; LLL, left lower lobe. BCG, <strong>Bronchocentric</strong> granulomatosis.<br />

in diameter and situated in the upper lobe (n ¼ 2) or superior<br />

segment of the lower lobe (Table 1) (Figs 1, 2, 3). One of these<br />

three patients had bilateral 2 cm nodules; one of which had<br />

cavitated and the other had a low attenuation, necrotic centre<br />

(Fig. 2). Two cases had minor associated findings. In one case,<br />

centrilobular nodules and a tree-in-bud pattern were seen<br />

adjacent to both lower lobe mass lesions (Fig. 2). In another<br />

case, air bronchograms were identified within the mass and<br />

areas of ground-glass opacification and smoothly thickened<br />

interlobular septa abutted the circumference of the lesion<br />

(Fig. 3).<br />

In the remaining two patients (cases 4 and 5, Table 1), the<br />

predominant finding was lobar consolidation with mild associated<br />

atelectasis (Figs 4, 5). One case exhibited dense consolidation<br />

of the upper lobe. The affected lobar bronchi were<br />

filled with dense, inspissated mucus (Fig. 4). Mucoid impaction<br />

was present in two other lobes and segmental consolidation was<br />

identified in the contralateral upper lobe. In the final case,<br />

scarring and volume loss were detected within an upper lobe<br />

(Fig. 5). This lobe showed evidence of traction bronchiectasis<br />

and a focal area of consolidation. Minor bronchiectasis, patchy<br />

airspace disease and multiple, less than 5 mm diameter nodules<br />

were present within the ipsilateral lower lobe.<br />

Mediastinal lymph node enlargement (short axis diameter<br />

greater than 10 mm) was seen in two cases.<br />

Pathological <strong>Findings</strong><br />

Macroscopically, two cases had the gross appearance of<br />

bronchopneumonia with no definitive mass lesion (Table 1).<br />

In one of these cases, a 1 cm cavity containing mucoid material<br />

was seen. The gross appearance in the two other resected specimens<br />

consisted of a parenchymal mass. In one an inflammatory


298 CLINICAL RADIOLOGY<br />

(a)<br />

(b)<br />

(c)<br />

Fig. 1 – 72-year-old woman with bronchocentric granulomatosis. (a)<br />

Contrast-medium enhanced CT (7 mm collimation) demonstrates a 5 cm<br />

irregular mass in the apical segment of the left upper lobe abutting the<br />

mediastinum. (b) Low power microscopy shows the bronchial wall (straight<br />

arrows) being replaced by granulomatous inflammation. Also noted are<br />

intraluminal debris and minimal inflammation of the accompanying pulmonary<br />

artery (curved arrow) (Haematoxilin and eosin stain, original<br />

magnification × 10, reproduced here at 50%). (c) High power view (original<br />

magnification × 40, reproduced here at 50%) of the bronchial mucosa shows<br />

replacement by pallisading histiocytes (straight arrows) and occasional<br />

giant cells (curved arrow). Also noted is luminal debris (open arrows).<br />

Fig. 2 – 32-year-old woman with bronchocentric granulomatosis. CT<br />

(5 mm collimation) reveals a 2 cm cavitating mass in the superior segment<br />

of the right lower lobe (large arrow). Also noted are small nodules in the<br />

superior segment of the left lower lobe (small arrows).<br />

Fig. 3 – 20-year-old man with bronchocentric granulomatosis following<br />

bone marrow transplant for acute lymphoblastic leukaemia. High-resolution<br />

CT (1 mm collimation) demonstrates a 2.5 cm spiculated mass in the<br />

apical segment of the left upper lobe. Areas of ground-glass opacification<br />

(curved arrow) and smooth interlobular septal thickening (straight arrow)<br />

are noted.<br />

Fig. 4 – 35-year-old man with allergic bronchopulmonary aspergillosis and<br />

bronchocentric granulomatosis. CT (8 mm collimation) demonstrates<br />

mucoid impaction (arrows) and consolidation in the left upper lobe and<br />

focal consolidation in the right upper lobe.


Fig. 5 – 48-year-old man with bronchocentric granulomatosis. Highresolution<br />

CT (1.5 mm collimation) reveals scarring, traction bronchiectasis<br />

and consolidation in the right upper lobe.<br />

pseudotumour containing two 1 cm cavities, representing areas<br />

of cystic bronchiectasis, was identified. One of these cavities<br />

contained a mycetoma.<br />

Necrotizing granulomatous bronchocentric inflammation<br />

was noted in all specimens (Fig. 1). This consisted of a<br />

mixed acute and chronic inflammatory infiltrate and was<br />

associated with partial to complete erosion of the bronchial<br />

mucosa. In one case (case 5), eosinophils were the predominant<br />

inflammatory cell. Palisaded histiocytes (n ¼ 2) and foreign<br />

body-type giant cells (n ¼ 3) were noted within the areas of<br />

inflammation (Fig. 1). Vasculitis involving arterioles was<br />

detected adjacent to areas of bronchocentric granulomatosis<br />

(n ¼ 3). In none of the cases was vasculitis identified away from<br />

regions involved by bronchocentric granulomatosis.<br />

The affected bronchi and bronchioles were impacted with<br />

necrotic material. In two cases, fungal hyphae consistent with<br />

Aspergillosis sp. were found within the necrotic debris. Distal<br />

to the more destructive bronchocentric lesions, patchy areas of<br />

organizing pneumonia were seen in two cases.<br />

DISCUSSION<br />

<strong>Bronchocentric</strong> granulomatosis is a rare disorder. There have<br />

been only a few reports describing the radiographic appearances<br />

BRONCHOCENTRIC GRANULOMATOSIS 299<br />

of bronchocentric granulomatosis. In his original description,<br />

Liebow referred to nine cases of bronchocentric granulomatosis<br />

[1]. Three cases had primarily lobar pulmonary infiltrates and<br />

atelectasis, and six cases showed a nodular or alveolar pattern<br />

on chest radiograph. The abnormalities were unilateral in<br />

five patients and bilateral in four. In subsequent series, the<br />

radiographic appearance of bronchocentric granulomatosis has<br />

consistently been divided into two main patterns: lobar consolidation<br />

or masses/nodules [2,3,5–7]. The mass lesions are<br />

unilateral in the majority of cases [1,2] and have only occasionally<br />

been reported to show cavitation [3,7]. The radiographic<br />

appearance is similar regardless of the aetiology of<br />

bronchocentric granulomatosis or the type of inflammatory cell<br />

predominant in the pathological specimen [2–7]. These observations<br />

are consistent with the findings in the current study.<br />

It has been debated whether bronchocentric granulomatosis<br />

is a separate disease entity or purely a pathological description<br />

of one of the limited ways in which bronchi and bronchioles<br />

respond to injury [9]. In the setting of asthma and allergic<br />

bronchopulmonary aspergillosis, bronchocentric granulomatosis<br />

is thought to develop as a result of a hypersensitivity<br />

response to endobronchial aspergillosis. This is reflected by<br />

the abundance of eosinophils at the site of inflammation in<br />

pathological specimens. However, an almost identical histological<br />

response can be seen secondary to other chronic pulmonary<br />

infections or chronic systemic inflammatory disorders.<br />

However, in these conditions, tissue eosinophilia is not a<br />

predominant finding. In our series, only one of the five patients<br />

had symptoms of asthma and a diagnosis of allergic bronchopulmonary<br />

aspergillosis. This finding reflects the increased rate<br />

of recognition of bronchocentric granulomatosis in nonasthmatic<br />

individuals, a significant minority of whom are<br />

immunocompromized [6,8,9]. This observation suggests a<br />

move away from the traditional thinking of bronchocentric<br />

granulomatosis as a specific disease entity and a step toward the<br />

acceptance of bronchocentric granulomatosis as a pathological<br />

description. This thereby emphasizes the importance of a<br />

rigorous search for an infectious aetiology for bronchocentric<br />

granulomatosis prior to the implementation of steroid therapy,<br />

the recommended treatment for bronchocentric granulomatosis<br />

in the setting of allergic bronchopulmonary aspergillosis.<br />

We confirm that the CT appearance of bronchocentric<br />

granulomatosis can be divided into two main patterns: mass<br />

lesions and lobar consolidation with atelectasis. However, the<br />

imaging features are non-specific and histological confirmation<br />

is required. In the original descriptions, 30–50% of cases were<br />

reported in asthmatic individuals. The present study emphasizes<br />

the greater prevalence in non-asthmatic patients.<br />

Acknowledgements. We would like to thank Dr Emily Folz from Wake<br />

Radiology, Raleigh, NC for contributing one of the cases.<br />

REFERENCES<br />

1 Liebow AA. The J. Burns Amberson Lecture – pulmonary angiitis and<br />

granulomatosis. Am Rev Respir Dis 1973;108:1–18.<br />

2 Katzenstein A-L, Liebow AA, Friedman PJ. <strong>Bronchocentric</strong> granulomatosis,<br />

mucoid impaction and hypersensitivity to fungi. Am Rev Respir Dis<br />

1975;111:497–537.<br />

3 Koss MN, Robinson RG, Hochholzer L. <strong>Bronchocentric</strong> granulomatosis.<br />

Hum Pathol 1981;12:632–638.


300 CLINICAL RADIOLOGY<br />

4 Clee MD, Lamb D, Clark RA. <strong>Bronchocentric</strong> granulomatosis: a review<br />

and thoughts on pathogenesis. Br J Dis Chest 1983;77:227–234.<br />

5 Myers JL, Katzenstein A-L. Granulomatous infection mimicking<br />

bronchocentric granulomatosis. Am J Surg Pathol 1986;10:317–322.<br />

6 Tazelaar HD, Baird AM, Mill M, Grimes MM, Schulman LL, Smith CR.<br />

<strong>Bronchocentric</strong> mycosis occurring in transplant recipients. Chest 1989;<br />

96:92–95.<br />

7 Yousem SA. <strong>Bronchocentric</strong> injury in Wegener’s granulomatosis: a<br />

report of five cases. Hum Pathol 1991;22:535–540.<br />

8 Martinez-López MA, Peña JM, Quiralte J, Fernandez MC, González JJ,<br />

Patron M, Vazquez JJ. <strong>Bronchocentric</strong> granulomatosis associated with<br />

pure red cell aplasia and lymphadenopathy. Thorax 1992;47:131–133.<br />

9 Myers JL. <strong>Bronchocentric</strong> granulomatosis. Disease or diagnosis? Chest<br />

1989;96:3–4.

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