Systemic idiopathic fibrosis with inflammatory pulmonary lesions

Systemic idiopathic fibrosis is characterized by non-suppurative inflammatory diseases of connective tissue, mainly adipose tissue. Although, in this condition, fibrosis may develop in several organs, there have been few reports of fibrosis involving the lung or pleura. We present a case of systemic idiopathic fibrosis associated with inflammatory pulmonary lesions, mimicking lung cancer with multiple pulmonary metastases. Chest CT and MRI showed a spiculated mass around the aortic arch with localized aortic wall thickening and nodules in the lungs. Abdominal CT showed a homogeneous mass around the abdominal aorta, consistent with retroperitoneal fibrosis. Transbronchial lung biopsy of the lesion in the right mid-lung field showed only a few scattered histiocytes accompanying inflammatory and fibrotic change. There was no evidence of malignancy, necrosis, angitis or mycobacterial infection. The lesions almost completely disappeared following prednisone treatment.