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Published on: October 12, 2012
Primary Biliary Cholangitis-Associated Granulomatous Interstitial Lung Disease: Pulmonary Epithelial-Interface Injury
Jiayao Liu1, Yuki Kamikokura1, Manami Hayashi1
1Department of Diagnostic Pathology, Asahikawa Medical University Hospital, Asahikawa, Japan.
Abstract:
Primary biliary cholangitis (PBC) can be accompanied by interstitial lung disease (ILD) manifesting as granulomatous interstitial lung disease, but the mechanism of granuloma formation remains unclear. We report a case of PBC-ILD coexisting with anti-synthetase syndrome (ASS) in a 49-year-old woman who presented with persistent cough, facial rash, and arthritis. Laboratory tests demonstrated cholestatic enzyme elevation, anti-mitochondrial M2 positivity, and anti-aminoacyl-tRNA synthetase antibody positivity, with elevated KL-6 and SP-D levels. High-resolution CT revealed lower-lobe-predominant ground-glass opacities. Bronchoalveolar lavage fluid showed lymphocytosis with high CD4/CD8 ratio. Liver biopsy confirmed PBC. Surgical lung biopsies revealed epithelial-interface injury and focal apoptosis of bronchiolar and alveolar epithelium accompanied by CD4-dominant T-cell infiltration and non-caseating granulomas (NCGs). Segment 2 showed cellular NSIP pattern and segment 9 showed fibrotic NSIP pattern. NCGs were more prominent in the cellular NSIP areas and decreased in the fibrotic NSIP areas. These findings suggest a possible pathological continuum from active inflammation by epithelial-interface injury to fibrotic remodeling. These observations may highlight the potential relevance of generalized autoimmune epithelitis in the pathogenesis of PBC-ILD.
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