Follicular bronchiolitis and lymphocytic interstitial pneumonia in a Japanese man

Department of Pathology, Shizuoka City Shimizu Hospital, Shizuoka, Japan.
Diagnostic Pathology (Impact Factor: 2.6). 09/2011; 6(1):85. DOI: 10.1186/1746-1596-6-85
Source: PubMed


A 44-year-old Japanese man consulted to our hospital because of cough and sputum. Chest-XP and CT revealed diffuse reticular opacities in both lungs.
A transbronchial lung biopsy (TBLB) showed a moderate infiltration of lymphocytes in the alveolar septae. He was diagnosed as interstitial pneumonia, and treated by drugs. One year later, his condition deteriolated, and a large open biopsy was performed. It showed a diffuse severe infiltration of lymphocytes in the alveolar walls and a few epithelioid granulomas. No bronchiolitis was seen.
Immunohistochemical study denied lymphocyte monoclonality, and he was diagnosed as lymphocytic interstitial pneumonia (LIP). He was treated by steroid.
Six months later, TBLB showed peribronchial lymphocyte infiltration. A large open biopsy also revealed a severe lymphocytic infiltration around the bronchioles, sparing alveolar wall lymphocytic infiltration. Immunohistochemical study denied malignant lymphoma. He was diagnosed as follicular bronchiolitis (FB). One year later, TBLB showed little lymphocytic infiltration in the alveolar walls as well as peribronchial walls. Two years later, his condition became worse, and TBLB showed features of LIP. Later, his condition was stationary for 6 years with mild lung opacities for 6 years. These findings show that LIP and FB are interchangeable and overlapping, and suggest that LIP and FB belong to the same spectrum of benign lymphoproliferative disorders of the lungs.

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    • "These different patterns may be found in adjacent zones or even intermingled within one lung specimen [11]. In particular, LIP and follicular bronchiolitis belong to the same spectrum of benign lymphoproliferative disorders of the lungs [12] and often co-exist in CVID with pulmonary granulomatous diseases. For this reason they have recently been grouped together under the term granulomatous lymphocytic interstitial lung disease (GLILD) [13]. "
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    ABSTRACT: Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease that seems to have an immunological pathogenesis and that causes a severe progressive suppurative and obstructive respiratory disorder. Common variable immunodeficiency (CVID) is the most common serious primary immunodeficiency and it is often associated with respiratory diseases. Herein, we describe a case of DPB in a 41-year-old man affected by CVID. We examined the patient’s lungs, focusing on the characteristics of the inflammatory cells and of the foamy macrophagic nodules typical of DPB. Immunohistochemical typing of the lymphocytic infiltrate showed that B-cells were almost absent, matching the immunological profile of CVID. The case described is the first case reported in the literature of DPB in a patient affected by CVID. Moreover it seems to confirm the correlation between an immunodeficiency status and the development of DPB and provides more information on the accumulation of nodules of foamy macrophages in DPB. Virtual slides The virtual slide(s) for this article can be found here:
    Full-text · Article · Jan 2014 · Diagnostic Pathology
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    • "By this classification LIP was reintroduced, but non idiopathic cases have to be excluded faithfully [6]. In clinical practise overlap to follicular bronchiolitis has been found [7]. "
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