Tuberc Respir Dis.  2009 Mar;66(3):230-235. 10.4046/trd.2009.66.3.230.

A Case of Idiopathic Bronchiolocentric Interstitial Pneumonia

• Kang JY
• Jung JI
• Lee KY
• Kim TJ
• Lee JW
• Lee WH
• Chung HS
• Yoon HK
• Song JS

• KMID: 1846386
• DOI: http://doi.org/10.4046/trd.2009.66.3.230

Abstract

Idiopathic bronchiolocentric interstitial pneumonia is one of idiopathic interstitial pneumonia, which has a relatively aggressive course and poor prognosis. It is characterized by diffuse centrilobular nodules radiologically with mainly bronchiolocentric inflammation and fibrosis associated with patchy alveolitis lacking interstitial granuloma histologically. This disorder is a recently classified disease category, and to our knowledge, there is no case report in Korea. We present a case of idiopathic bronchiolocentric interstitial pneumonia. A 62-year-old man presented with exertional dyspnea with a 1 month duration. The radiological findings showed extensive centrilobular lesions at both lungs. The surgical lung biopsy specimen demonstrated a centrilobular inflammatory process with small airway fibrosis and inflammation partially radiating into the interstitium. Therefore, the patient was diagnosed with idiopathic bronchiolocentric interstitial pneumonia. He was treated with immunosuppressants including steroids and azathioprine. However, his symptoms did not improve and he expired 7 months later due to an acute exacerbation of the interstitial pneumonia and probable infectious pneumonia.

Keyword

Bronchiolitis; Interstitial pneumonia; Interstitial lung disease

MeSH Terms

Azathioprine
Biopsy
Bronchiolitis
Dyspnea
Fibrosis
Granuloma
Humans
Idiopathic Interstitial Pneumonias
Immunosuppressive Agents
Inflammation
Korea
Lung
Lung Diseases, Interstitial
Middle Aged
Pneumonia
Prognosis
Steroids
Azathioprine
Immunosuppressive Agents
Steroids

Figure

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  Figure 1 Chest X-ray shows reticulonodular opacities at both lung fields.

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  Figure 2 Chest CT demonstrates innumerable tiny centrilobular nodules in whole lung fields (A, B). There are irregular linear densities or reticulations in both lower lobes, suggesting fibrosis (C).

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  Figure 3 Biopsy of the left lung shows centrilobular inflammatory cell infiltrations and fibrosis with extension to bronchovascular bundLe and perivascular zone of the distal pulmonary lobule, which distorts brochioloalveolar strutures with relative sparing subpleural area at low magnification (A, H&E stain, ×100). High power field image shows that the lymphocytic infiltrates occasionally involve alveolar wall and that foamy macrophages aggregation with fibroblastic plug formation is found in alveolar spaces (B, H&E stain, ×200).

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  Figure 4 Seven months later, follow up chest CT shows ground glass opacities with little interval change of centrilobular nodules, predominantly at both upper lobes (A, B). And some cavitary consolidations newly develop at right lower lobe (C, arrow).

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