The Analysis of Tryptase in Serum of Sarcoidosis Patients
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The Analysis of Tryptase in Serum of Sarcoidosis Patients Elena Bargagli,1,4,5 Angela Mazzi,1 Fabrizio Mezzasalma,1 Anna Perrone,1 Carmela Olivieri,1 Antje Prasse,2 Nicola Bianchi,3 Maria G Pieroni,1 and Paola Rottoli1
Abstract—Sarcoidosis is a systemic granulomatous disease of unknown etiology characterized by activation of macrophages and T lymphocytes. Relatively little is known about the role of mast cells and their mediators in the pathogenesis of sarcoidosis. Tryptase is an enzyme produced by activated mast cells, regarded as a marker of mast cell activation. To analyse tryptase concentrations in serum of sarcoidosis patients in an attempt to define the role of tryptase and mast cells in the pathogenesis of sarcoidosis and to evaluate the potential of tryptase as marker of disease severity. Quantitative analysis of tryptase concentrations was performed in serum of patients with stable sarcoidosis (n=12), progressive sarcoidosis (n=23) and controls (n=13). Patients enrolled in the study had been monitored at Siena Regional Referral Centre for Sarcoidosis from onset for at least 12 months. Significantly higher concentrations of tryptase were found in peripheral blood of sarcoidosis patients (6.08±3.98 μg/l) than controls (2.96±1.75μg/l; p=0.012). Patients with progressive disease showed the highest tryptase concentrations in serum. Tryptase and mast cells may be involved in the immunopathogenesis of sarcoidosis and further studies are required to understand if tryptase may represent a marker of sarcoidosis severity. KEY WORDS: sarcoidosis; serum; mast cell; tryptase.
(BAL) is useful for diagnosis of sarcoidosis and can replace biopsies in many cases; it can also provide information on immunopathogenesis [2–3]. Lymphocytic alveolitis with increased CD4/CD8 ratio is the most common finding in sarcoidosis patients [4, 5]. The crucial role of lymphocytes and macrophages in granuloma formation has been widely studied but there is relatively little data on involvement of mast cells (MC) [6]. Increased MC count has been observed in lung tissue of patients with various interstitial lung diseases (ILD) such as sarcoidosis [7–8] and idiopathic pulmonary fibrosis [9]. Heard et al. described these cells as closely apposed to fibroblasts and suggested that mast cells and their mediators are involved in fibroproliferative response [10]. Recently Edwards et al. analysed involvement of mast cells in fibrotic lung damage in usual interstitial pneumonia (UIP), systemic sclerosis (SS), lymphangioleiomyomatosis (LAM) and sarcoidosis patients by focusing on their expression in areas of active lung fibrosis [11]. Kawatani et al. reported increased BAL
INTRODUCTION Sarcoidosis is a multisystemic disorder of unknown etiology characterized by a T-helper 1 response with accumulation of CD4 positive T lymphocytes and activated macrophages in the lung, resulting in alveolitis and granuloma formation [1]. Bronchoalveolar lavage 1
Respiratory Diseases Section, Department of Clinical Medicine and Immunological Sciences, Siena University, Siena, It
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