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Arthritis Research and Therapy

, 18:92

First Online: 22 April 2016Received: 27 November 2015Accepted: 11 April 2016


BackgroundAnti-neutrophil cytoplasmic antibodies associated vasculitides AAV is a group of autoimmune diseases, characterized by small vessel inflammation. Phagocytes such as neutrophils and monocytes are the main effector cells found around the inflamed vessel wall. Therefore, we wanted to investigate aspects of function and activation of these cells in patients with AAV.

MethodsFlow cytometry was used to evaluate: the expression of activation markers CD11c, CD62L, CD177 and C5aR; the number of recently released neutrophils from bone marrow, defined as CD10D16 cells in peripheral blood; and the capacity of peripheral blood monocytes and polymorphonuclear leukocytes PMN to produce reactive oxygen species and to phagocytose opsonized bacteria.

ResultsAAV patients n = 104 showed an increase of CD10CD16 neutrophils and total PMN in peripheral blood, suggesting a combination of increased bone marrow release and prolonged survival. An increased percentage of AAV PMN expressed CD177 but no other signs of activation were seen. A decreased production of reactive oxygen species was observed in AAV phagocytes, which was associated with disease activity. Moreover, granulocytes from patients with microscopic polyangiitis showed lower oxidative burst capacity compared to patients with granulomatosis with polyangiitis or eosinophilic granulomatosis with polyangiitis. In addition, decreased phagocytosis capacity was seen in PMN and monocytes.

ConclusionOur results indicate that phagocytes from AAV patients have impaired function, are easily mobilized from bone marrow but are not particularly activated. The association between low reactive oxygen species formation in PMN and disease severity is consistent with findings in other autoimmune diseases and might be considered as a risk factor.

KeywordsANCA Vasculitis PMN Monocytes Oxidative burst Phagocytosis AbbreviationsAAVanti-neutrophil cytoplasmic antibodies associated vasculitides

ANCAthe anti-neutrophil cytoplasmic antibodies

BVAS3Birmingham Vasculitis Activity Score version 3

E. coliEscherichia coli

EGPAeosinophilic granulomatosis with polyangiitis

ELISAenzyme-linked immunosorbent assay

GeoMFIgeometric mean fluorescence intensity

GPAgranulomatosis with polyangiitis

IVDin vitro diagnostic

MPAmicroscopic polyangiitis


NETsneutrophil extracellular traps

NAPDHnicotinamide adenine dinucleotide phosphate-oxidase

PMAphorbol 12-myristate 13-acetate

PMNpolymorphonuclear leukocytes

PR3proteinase 3

ROSreactive oxygen species

SLEsystemic lupus erythematosus

SLEDAI-2 KSystemic Lupus Erythematosus Disease Activity Index 2000

SLICC-ACR-DISystemic Lupus International Collaborative Clinics-American College of Rheumatology damage index

Electronic supplementary materialThe online version of this article doi:10.1186-s13075-016-0994-1 contains supplementary material, which is available to authorized users.

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Autor: Åsa  CM Johansson - Sophie Ohlsson - Åsa Pettersson - Anders A. Bengtsson - Daina Selga - Markus Hansson - Thomas Hellm


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