Comparative proteomic analysis of bronchoalveolar lavage of familial and sporadic cases of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by progressive deterioration of the alveolar integrity. Among IPF identified phenotypes, that of familial (f-)IPF is usually associated with several gene mutations which are seldom observed in sporadic (s-)IPF. This study aimed at investigating the molecular patterns and variability in f-IPF and s-IPF patients through a differential proteomic analysis.

A functional proteomics approach to the comprehension of sarcoidosis.

Pulmonary sarcoidosis (Sar) is an idiopathic disease histologically typified by non-caseating epitheliod cell sarcoid granulomas. A cohort of 37 Sar patients with chronic persistent pulmonary disease was described in this study. BAL protein profiles from 9 of these Sar patients were compared with those from 8 smoker (SC) and 10 no-smoker controls (NSC) by proteomic approach.

A system biology study of BALF from patients affected by idiopathic pulmonary fibrosis (IPF) and healthy controls.

Background: Idiopathic pulmonary fibrosis (IPF) is a devastating interstitial lung disease characterized by progressive loss of the alveolar integrity, recruitment, and activation of myofibroblast, and excessive collagen deposition that resulted in loss of parenchymal architecture and lung function. Although etiology is unknown, major risk factor of disease development is represented by cigarette smoke or exposure to dust.

Aims: Aim of this proteomic study was to compare broncho alveolar lavage fluid protein profiles of IPF patients, never-smoker healthy control (nonsmoker control) and smoker control subjects in order to investigate proteins potentially related to disease progression and pathogenesis.

Towards a functional proteomics approach to the comprehension of idiopathic pulmonary fibrosis, sarcoidosis, systemic sclerosis and pulmonary Langerhans cell histiocytosis.

Unlabelled: Bronchoalveolar lavage fluid of patients with four interstitial lung diseases (sarcoidosis, idiopathic pulmonary fibrosis, pulmonary Langerhans cell histiocytosis, fibrosis associated to systemic sclerosis) and smoker and non smoker control subjects were compared in a proteomic study. Principal component analysis was used to statistically verify the association between differentially expressed proteins and the conditions analyzed. Pathway and functional analysis by MetaCore and DAVID software revealed possible regulatory factors involved in specific "process networks" like regulation of stress and inflammatory responses.

Utility of spiral CAT scan in the follow-up of patients with pulmonary Langerhans cell histiocytosis.

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease that occurs almost exclusively in smokers, generally young adults between 20 and 40 years old. Prognostic biomarkers of the disease are lacking. This study describes the clinical-radiological features of a group of PLCH patients and applies a semi-quantitative CT score of the chest to verify the prognostic value of radiological findings in this disease.

Mesalazine-induced multi-organ hypersensitivity.

Mesalazine therapy for ulcerative colitis has been reported to be effective and safe. Rare cases of mesalazine-induced renal, pancreatic, myo-pericardial, pleuro-pulmonary and haematological toxicity have been described separately. We report a case characterized by the simultaneous presence of fever, pericarditis, peripheral eosinophilia, eosinophilic pneumonia, anaemia and haematuria (together with proteinuria and leukocyturia) due to mesalazine treatment in a patient with ulcerative colitis.

Analysis of macrophage migration inhibitory factor (MIF) in patients with idiopathic pulmonary fibrosis.

By proteomic approach we previously characterised bronchoalveolar lavage (BAL) protein profiles of patients with idiopathic pulmonary fibrosis (IPF), sarcoidosis and systemic sclerosis. Among differently expressed proteins we identified macrophage migration inhibitory factor (MIF), a multi-function pleiotropic cytokine. This study was performed to validate our findings by a further proteomic approach and ELISA in a larger population of patients and controls.

CD4+CD25+ lymphocyte subsets in chronic graft versus host disease patients undergoing extracorporeal photochemotherapy.

Extracorporeal photochemotherapy (ECP) has been used successfully for the treatment of chronic Graft versus Host Disease (cGvHD). However, the mechanism by which ECP exerts its protective effects remains elusive. Some recent observations have suggested a possible role of certain subsets of T lymphocytes with immunosuppressive properties (T-regulatory cells) that coexpress CD4 and high levels of the interleukin-2 receptor chain: CD4+CD25+ T lymphocytes.

Chitotriosidase analysis in bronchoalveolar lavage of patients with sarcoidosis.

Background: Sarcoidosis is a systemic granulomatous disease characterised by T-helper cell/macrophage alveolitis. Activated macrophages release mediators, such as cytokines, chemokines, oxygen radicals, and enzymes. In a previous paper we found higher levels of chitotriosidase, a macrophage derived enzyme, in serum of patients with sarcoidosis than in controls.

Chitotriosidase activity in patients with interstitial lung diseases.

Background: In previous papers, we found significantly higher activity of chitotriosidase, a macrophage derived enzyme, in serum and BAL of patients with sarcoidosis, especially in those with progressing disease and lung involvement, than in controls. Locally and systemically produced chitotriosidase activity was correlated with radiological stage and also with degree of lung infiltration, suggesting that this enzyme may play a role in the pathogenesis of sarcoidosis and may be used as a marker of disease severity.

Aim: To analyse chitotriosidase activity in serum and bronchoalveolar lavage of patients with idiopathic pulmonary fibrosis and pulmonary fibrosis associated with systemic sclerosis and to compare it with chitotriosidase activity in controls and sarcoidosis patients.

Analysis of carbonylated proteins in bronchoalveolar lavage of patients with diffuse lung diseases.

Diffuse lung diseases (DLD) are a heterogeneous group of diseases with different etiopathogenesis, clinical course, and prognosis. It has been demonstrated that oxidative stress can contribute to the pathogenesis of these diseases. In the present study we measured carbonylated protein concentrations in the BAL of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis, idiopathic pulmonary fibrosis, and for the first time in patients with chronic eosinophilic pneumonia and extrinsic allergic alveolitis.

Cytokine profile and proteome analysis in bronchoalveolar lavage of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis and idiopathic pulmonary fibrosis.

The aim of this study was to analyze the type of immune response (Th1, Th2) and protein composition of bronchoalveolar lavage (BAL) of patients with sarcoidosis, pulmonary fibrosis associated with systemic sclerosis (SSc) and idiopathic pulmonary fibrosis (IPF). Flow cytometry analysis of intracellular cytokines revealed different patterns: in IPF and SSc Th2 profiles were predominant, whereas in sarcoidosis Th1 prevailed. The proteomic analysis of BAL fluid (BALF) showed that there were quantitative differences between the three diseases.

Tryptase concentrations in bronchoalveolar lavage from patients with chronic eosinophilic pneumonia.

In order to characterize BAL (bronchoalveolar lavage) in CEP (chronic eosinophilic pneumonia) and to investigate the possible role of mast cells and tryptase in the pathogenesis of this interstitial disease, cells and tryptase levels were determined in BAL of patients with CEP and in a group of healthy controls. The results show that a statistically significant increase in tryptase concentration was found in patients with CEP compared with the healthy controls. This is the first report that shows an increase in tryptase levels in CEP and could reflect higher mast cell activation as well as larger mast cell populations in the lungs of these patients.

Gardening in greenhouses as a risk factor for silicosis.

Silicosis is a typical occupational disease, although some cases caused by non-industrial exposure have also been reported. We saw a 53-year-old male gardener with recurrent non-productive cough. A routine radiograph of the chest showed bilateral pulmonary nodules and subsequent computed tomography suggested that the infiltrates could be metastases.

Bronchoalveolar lavage fluid protein composition in patients with sarcoidosis and idiopathic pulmonary fibrosis: a two-dimensional electrophoretic study.

We used two-dimensional (2-D) electrophoresis to analyze the protein composition of fluid recovered by bronchoalveolar lavage (BALF) from patients with sarcoidosis and idiopathic pulmonary fibrosis, two forms of interstitial lung disease with different cellular composition and cytokine profile in BALF. They are also characterized by different pathogenesis and clinical evolution, idiopathic pulmonary fibrosis being less favorable than sarcoidosis due to rapidly progressive pulmonary fibrosis. Thirty-eight proteins or protein fragments, never previously assigned in BALF samples, were identified by various methods including mass fingerprinting of tryptic digests.

Clinical, laboratory and radiological findings in pulmonary fibrosis with and without connective tissue disease.

Uncertainty exists over whether to consider "lone" idiopathic pulmonary fibrosis (LIPF) and pulmonary fibrosis associated with connective tissue disorders (PFCTD) as significantly different entities. We retrospectively analysed data collected at the time of first diagnosis in 17 patients with LIPF and in 14 patients with PFCTD and compared survival in the two groups. At first evaluation, the time from onset of respiratory symptoms, spirometric volumes and the diffusing capacity for carbon monoxide were not significantly different between the two groups.

Detection of specific antibodies in immune complexes of farmer's lung patients.

The authors examined 23 precipitin-positive symptomatic patients with Farmer's Lung(FL) and compared them to different groups of exposed asymptomatic precipitin-positive(EAPP) and precipitin- negative(EAPN) farmers. The sera were tested using several techniques (i.e.

Imparied interferon-gamma production by peripheral blood mononuclear cells and effects of calcitriol in pulmonary sarcoidosis.

Pulmonary sarcoidosis (S) is a granulomatous disease of unknown etiology characterized by spontaneous release of cytokines and 1,25-dihydroxyvitamin D3 (calcitriol) at the sites of granulomatous reaction. Stimulated by our previous findings that high levels of interferon-gamma (IFN-gamma) occur in this disease and that calcitriol reduces IFN-gamma production by peripheral blood mononuclear cells (PBMC) from normal subjects, we designed the present study to evaluate IFN-gamma production and the effect of calcitriol on the release of this cytokine by PBMC in S patients. The cells were stimulated with staphylococcal enterotoxin A (SEA) and A23187 calcium ionophore.

Mast cells in bronchoalveolar lavage in sarcoidosis: correlation with alveolar lymphocytes.

Today it is believed that mast cells (MC) are important not only in IgE-mediated reactions, but also in delayed hypersensitivity reactions, and that their functions are mediated by factors released by T lymphocytes. Recent studies have shown their presence in bronchoalveolar lavage (BAL) of patients with asthma and interstitial lung disease. MC have been identified by us in the BAL of patients with sarcoidosis and lung cancer, and in controls.

Alveolar lymphocytes in the follow-up of sarcoidosis.

Bronchoalveolar lavage (BAL) was performed in a group of 25 patients with pulmonary sarcoidosis in order to monitor variations in alveolar lymphocytes (AL) in comparison to the clinical evolution of the disease. Two BAL were performed in each patient, the second 6-12 months after the first (8.9 +/- 3, M +/- SD).