Osler-Rendu-Weber syndrome: congenital arteriovenous intrapulmonary fistula treated using a percutaneous Amplatzer plug.

Pulmonary arteriovenous fistulas (PAVFs) are rare vascular malformations (PAVMs) of the lung that could lead to severe hypoxiemia due to right-to-left intrapulmonary shunts. They may occur as isolated entities or associated with Osler-Rendu-Weber syndrome or hereditary haemorrhagic telangiectasia (HHT). We report a case of a 70 years old woman with Rendu-Osler-Weber disease and a large arteriovenous malformation involving the left pulmonary artery. We describe the successful transcatheter occlusion of the PAVF using an Amplatzer vascular plug. This work is an attempt to focus the attention on pulmonary arteriovenous malformations and on percutaneous treatment as an alternative to surgery, that consists of a conservative lung resection.

Colchicine, cyclophosphamide and prednisone in the treatment of mild-moderate idiopathic pulmonary fibrosis: comparison of three currently available therapeutic regimens.

STUDY OBJECTIVES: In this study we evaluated the role of three currently available therapeutic regimens in the treatment of early stages of idiopathic pulmonary fibrosis (IPF). PATIENTS: The study population consisted of 57 consecutive suspected individuals with IPF. Patients with interstitial pneumonias other than IPF and subjects with advanced disease or contraindication to therapy were excluded. We evaluated 30 subjects with mild-moderate IPF, homogeneous baseline characteristics and prognostic parameters that were assigned to 3 treatment regimens: group 1 (n = 11): prednisone 1 mg/kg/ day; group 2 (n = 9): prednisone 0.5 mg/kg/day plus cyclophosphamide 100 mg/day; group 3 (n = 10): prednisone 0.5 mg/kg/day plus colchicine 1 mg/day. We analysed response to therapy by analysis of a clinical-radiographic-physiologic (CRP) score before treatment and at 6 months intervals for 18 months. Side effects and three years survival rate were also investigated. RESULTS: Although our study was performed in a subset of patients with early disease's stages, these data showed that none of the regimens was able to interfere with IPF's course. However treatment with colchicine plus prednisone resulted in fewer side effects and re-evaluation parameters showed a significant decrease of dyspnoea (p < 0.01). No significant differences were observed in survival rate among the three groups. CONCLUSIONS: None of the regimens analyzed was effective even in the treatment of the early stages of IPF. The association colchicine/corticosteroids could be considered a safe and not expensive regimen that may be used in the treatment of IPF, especially in patients who have experienced adverse effects from immunosuppressive agents, while waiting for newer therapeutic strategies.