Interstitial lung disease preceding primary biliary cirrhosis in a male patient
I.. Francoa,, , A.. Dubinib, S.. Piciucchic, G.. Casonid, V.. Polettid
a Department of Pulmonary Medicine, Centro Hospitalar Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
b Department of Anatomic Pathology, G.B. Morgagni L. Pierantoni General Hospital, Forl??, Italy
c Department of Radiology, G.B. Morgagni L. Pierantoni General Hospital, Forl??, Italy
d Department of Diseases of the Thorax, G.B. Morgagni L. Pierantoni General Hospital, Forl??, Italy

A 47-year-old male was admitted with subacute onset of dry cough and fever. Chest tomography demonstrated multifocal areas of consolidation and ground glass attenuation. Cytological analysis of bronchoalveolar lavage revealed lymphocytosis and eosinophilia and anatomopathological exam of transbronchial cryobiopsy showed poorly formed non-caseous granulomas associated to interstitial lympho-plasmocitary infiltrate. The diagnosis of idiopathic granulomatous lung disease (GLD) was assumed and the patient started oral prednisolone, presenting clinical, functional and radiological improvement. Two years later, the patient was diagnosed with primary biliary cirrhosis (PBC). At this time, it was possible to associate GLD with the autoimmune hepatobiliary disease.

Clinical, epidemiological and pathological aspects of this uncommon case of interstitial lung disease as first presentation of PBC in a male patient are discussed.

Primary biliary cirrhosis, Primary autoimmune hepatobiliary diseases, Granulomatous lung disease, Interstitial lung disease, Transbronchial cryobiopsy


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