Malnutrition is very frequent in idiopathic pulmonary fibrosis (IPF) patients.  Low fat-free mass index (FFMI) assessed by bioimpedance analysis (BIA) is reported in 28% of patients.  Body mass index (BMI) and mid-arm circumference (MAC) are independently associated with low FFMI.  A two-step nutritional assessment based on BMI, MAC and BIA should be routinely performed in IPF patients.

Late results of abdominal aortic surgery in children, in our experience, are encouraging. Quality of life in adulthood was excellent. Insofar as possible, correction should be deferred until the child is 8 to 10 years old so that a prosthesis of sufficient diameter can be used.

The chemokine CXCL13 controls the normal organization of secondary lymphoid tissues and the neogenesis of ectopic lymphoid structures in nonlymphoid organs, particularly the lungs.The progression and severity of idiopathic pulmonary fibrosis (IPF), a fatal and irreversible interstitial lung disease, is predicted by the circulating blood concentrations of CXCL13.While CXCL13 is produced by pulmonary tissues, it has not been determined which cells are involved. This study examines CXCL13 production by lung tissue macrophages from patients with IPF and the signaling pathways controlling CXCL13 gene expression in human alveolar macrophages (AM) and monocyte-derived macrophages (MoDM). CXCL13 is found in CD68-and CD206-positive AM from patients with IPF, and the CXCL13 gene is induced in these macrophages and MoDM when they are stimulated with lipopolysaccharide. We found that TNF- and IL-10 control optimal CXCL13 gene expression in MoDM and possibly in AM by activating the NF-B and JAK/STAT pathways, respectively. We also found that blood TNF- and CXCL13 concentrations are significantly correlated in patients with IPF, suggesting that TNF- contributes to CXCL13 production in humans. In conclusion, the results of this study demonstrate that AM from patients with IPF produces CXCL13 and that the NF-B and JAK/STAT pathways are required to induce the expression of this major chemokine.

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