Abstract
BackgroundSystemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. A small number of studies have examined the role of AMPs on autoimmune diseases which has not been studied in scleroderma yet. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc.MethodsForty-two patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) were enrolled. For SSc patients, the following data were recorded: disease subset (limited/diffuse), autoantibodies (antinuclear, anti-centromere (ACA), and anti-SCL-70), blood tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), modified Rodnan skin score, presence and history of digital ulcers, kidney, gastrointestinal disease and lung involvement assessed by computed tomography and pulmonary function tests. Association between serum AMPs and disease characteristics were analysed.ResultsTwenty-nine of the patients had diffuse (69%) and 13 of the patients had limited (31%) systemic sclerosis. Average disease duration was 5.5 years. Pulmonary involvement was detected in 20 patients (47.6%). Serum concentration of alpha defensin was higher than healthy subjects (563 ± 415 vs 377 ± 269 ng/mL, p = 0.02). However, no difference was observed for beta-1 and beta-2 defensins in SSc patients and healthy controls. In sub-group analysis patients with interstitial lung disease had higher levels of alpha defensin than those without lung involvement (684 ± 473 vs 430 ± 299 ng/ml, p = 0.04). There was also correlation between alfa defensin serum concentrations and CRP (r = 0.34).ConclusionsAlpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease.Trial registrationThis study is not a clinical trial study.
Highlights
Systemic sclerosis (SSc) is a chronic multisystemic autoimmune disease manifested by diffuse fibrosis of the skin and internal organs [1]
We found that serum concentrations of alpha defensins are increased in SSc patients
The level of Antimicrobial peptides (AMP) expression varies among individuals, and it has been suggested that this variation is due to genetic differences in the genes encoding defensins
Summary
Systemic sclerosis (SSc) is a chronic multisystemic autoimmune disease manifested by diffuse fibrosis of the skin and internal organs [1]. One of the critical components of innate immune system is antimicrobial peptides (AMPs). Besides having direct antimicrobial effects, AMPs can incite inflammation by activating immune effector cells and chemotaxis of neutrophils [4]. Epithelial AMPs may stimulate production of pro-inflammatory cytokines such as IL-17, IL-22, and TNF alpha [7]. Another important role of AMPs involves the maintenance of the host microbiota besides their antimicrobial functions. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc
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