Angelina Ratley

Ually modified after the original description by Ramirez in 1963 [7]. As a result, nowadays PAP is no longer considered a potentially lethal disorder. Descriptions of large series of PAP patients in the literature over the years have greatly extended the knowledge on the natural history of the disorder. Following the meta-analysis of 410 cases by Seymour and Presneill [8], who reviewed all PAP cases available in the literature, including 34 PAP PubMed ID:https://www.ncbi.nlm.nih.gov/pubmed/11011031 cases followed at the Mayo Clinic Center in Rochester [9], other 1-Oleoyl lysophosphatidic acid large multicenter series were published in Japan in 2008 (248 cases) [10] and China (241 cases) [11]. In 2011, Bonella and associates described the characteristics of a cohort of 70 PAP patients followed at a single center in Essen, Germany [12]. In this communication we report on a cohort of 81 PAP?2013 Campo et al.; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Campo et al. Orphanet Journal of Rare Diseases 2013, 8:40 http://www.ojrd.com/content/8/1/Page 2 ofpatients registered at the Pneumology Section of the San Matteo University Hospital of Pavia, Italy. It should be emphasized that the series of patients described in a single reference center, although less relevant than large meta-analysis or multicenter series, has however the advantage of including detailed experience accumulated over several years (decades, as in our case). This is of special relevance as many treatment aspects, such as WLL for PAP, have not been investigated in depth. In this report, we have focused on two aims : first, the description of the clinical characteristics of the disease at presentation in our series of PAP patients; secondly, to analyse some baseline functional and biochemical parameters in function of the long term follow up of patients, especially with respect to the need for WLL. The latter has not been covered in the literature so far.variables were normally distributed, the results were expressed as mean values and SD, otherwise median and interquartile range (IQR; 25th -75th percentile) were reported. The one-way ANOVA (or non parametric Kruskall-Wallis for skewed distributions), with Bonferroni correction for comparisons between two groups, was employed to investigate differences among the three study groups (PAP patients never lavaged, lavaged once or more times). Qualitative variables were summarized as counts and percentages and the Chi-square (2) test were used to compare gender among study groups. P < 0.05 was considered statistically significant and all tests were two-sided. Data analysis was performed with the STATA statistical package (release 11,1, 2010, Stata Corporation, College Station, Texas, USA).MethodsPatients' diagnosis and assessmentProgressive recruitment of patients with surfactant associated disorders started after the first WLL performed in 1989. Diagnostic tools were the presence of "crazy paving pattern" at the high resolution computed tomography scan images of the thorax and the presence of macroscopic milky fluid and/or the presence of amorphous, eosinophilic, PAS positive material, as well as lipid laden macrophages on bronchoalveolar lavage analysis [1]. Determination of serum level of autoantibodies antiGM-CSF (GMAbs) was performed in the Laboratory of the R.>