dc.contributor.author	Zhang, Ying
dc.contributor.author	Shi, Weiwei
dc.contributor.author	Tang, Sha
dc.contributor.author	Li, Jingyi
dc.contributor.author	Yin, Shiwei
dc.contributor.author	Gao, Xuejing
dc.contributor.author	Wang, Li
dc.contributor.author	Zou, Liyun
dc.contributor.author	Zhao, Jinghong
dc.contributor.author	Huang, Yunjian
dc.contributor.author	Shan, Lianyu
dc.contributor.author	Gounni, Abdelilah S
dc.contributor.author	Wu, Yuzhang
dc.contributor.author	Yuan, Fahuan
dc.contributor.author	Zhang, Jingbo
dc.date.accessioned	2014-04-05T12:29:18Z
dc.date.available	2014-04-05T12:29:18Z
dc.date.issued	2013-10-24
dc.date.updated	2014-04-05T12:29:18Z
dc.description.abstract	Abstract Introduction Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is characterised by the autoinflammation and necrosis of blood vessel walls. The renal involvement is commonly characterised by a pauci-immune crescentic glomerulonephritis (PiCGN) with a very rapid decline in renal function. Cathelicidin LL37, an endogenous antimicrobial peptide, has recently been implicated in the pathogenesis of autoimmune diseases. To assess whether serum LL37 reflects renal crescentic formation, we measured the serum levels of LL37 in AAV patients with and without crescentic glomerulonephritis (crescentic GN) as compared to healthy controls (HCs). We also analysed the correlation of the serum levels of LL37 and interferon-α (IFN-α) with the clinical characteristics of the patients. Methods The study population consisted of 85 AAV patients and 51 HCs. In 40 ANCA-positive patients, a parallel analysis was performed, including the assessment of LL37 and IFN-α levels in the serum and renal biopsies. Of those studied, 15 AAV patients had biopsy-proven crescentic GN, and 25 AAV patients lacked crescent formation. The serum levels of cathelicidin LL37 and IFN-α were both measured by ELISA, and the clinical and serological parameters were assessed according to routine procedures. Immunofluorescence staining was performed on frozen sections of kidney needle biopsies from AAV patients with crescentic GN. Results The serum levels of LL37 and IFN-α were significantly increased in AAV patients with crescentic GN compared to AAV patients without crescentic formation and HCs, and patients with high LL37 and IFN-α levels were more likely to be in the crescentic GN group. The LL37 levels were positively correlated with the IFN-α levels, and both LL37 and IFN-α levels showed a positive correlation with serum creatinine and no correlation with complement C3. The renal tissue of crescentic GN patients showed expression of LL37 and IFN-α at the Bowman’s capsule and extracellular sites, suggesting the active release of LL37 and IFN-α. Conclusions Significantly higher levels of LL-37 and IFN-α were observed in AAV patients, particularly those with crescentic formation, and LL37 and IFN-α were expressed in the renal tissue of patients with crescentic GN. These data suggest that serum levels of LL37 and IFN-α may reflect both local renal inflammation and systemic inflammation.
dc.description.version	Peer Reviewed
dc.identifier.citation	Arthritis Research & Therapy. 2013 Oct 24;15(5):R161
dc.identifier.doi	http://dx.doi.org/10.1186/ar4344
dc.identifier.uri	http://hdl.handle.net/1993/23408
dc.language.rfc3066	en
dc.rights	open access	en_US
dc.rights.holder	Ying Zhang et al.; licensee BioMed Central Ltd.
dc.title	The influence of cathelicidin LL37 in human anti-neutrophils cytoplasmic antibody (ANCA)-associated vasculitis
dc.type	Journal Article

The influence of cathelicidin LL37 in human anti-neutrophils cytoplasmic antibody (ANCA)-associated vasculitis

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