RESUMEN
INTRODUCTION: Primary biliary cirrhosis is a hepatic auto-immune disease which is rarely associated with respiratory complications. CASE REPORT: A 40-year-old woman had a 5-year history of primary biliary cirrhosis. Two years previously, she had begun to experience a chronic cough and dyspnea. On clinical examination, the patient had fever, jaundice and basal pulmonary crackles. Chest X-ray showed bilateral slowly migrating and spreading airspace consolidation refractory to antibiotics. Microbiological investigations evidenced no abnormalities. Routine biological studies revealed an inflammatory syndrome, a mild neutrophilic hyperleukocytosis without hypereosinophilia and a mild hepatitis picture with cholestasis. Antineutrophil cytoplasmic antibody testing and anti-nuclear factor testing were negative. Fibreoptic bronchoscopy showed diffuse bronchial inflammation with no specific pathological pattern at bronchial biopsy. The bronchoalveolar lavage revealed a mixed cellular pattern. The chest computed tomography revealed peribronchial pulmonary consolidation with septal and pleural thickening, suggestive of an organizing pneumonia. The patient was treated with oral corticosteroids which resulted in a prompt clinical improvement and complete resolution of radiographic pulmonary abnormalities within two months. Treatment continued for six months and no relapse was noticed during the six months following the end of treatment. CONCLUSIONS: This case of organizing pneumonia highlights the importance of physicians considering this condition in women with primary biliary cirrhosis complaining of respiratory symptoms in the context of unresolved pneumonia.