Abstract
ANCA-associated vasculitides (AAV) are severe diseases, potentially affecting lungs, kidney, and other organs. Nevertheless, risk profiling remains difficult. Aim of the current study was to analyze serological characteristics in AAV. The principal goal was to identify diagnostic markers that potentially allow a more sophisticated risk profiling in AAV. AAV subjects were recruited and evaluated for disease activity, disease stage, medication, and laboratory findings. Serum concentrations of the following parameters were measured: IL-1β, IL-6, IL-17 A, IL-17 F, IL-21, IL-22, IL-23, TNF-α, sCD40L, IL-4, IL-10, IL-25, IL-31, IL-33, and INF-γ. A total number of 62 AAV subjects was included in the study (39 females; 23 males). Forty-five subjects were PR3+, 17 subjects showed ANCA specificity for MPO. The majority of all cytokines fell under the lower detection limit of the assay. Serum IL-10 was higher in both, AAV and SSc as compared to controls; it was also higher in early systemic AAV. Serum IL-33 was elevated in AAV and SSc; in AAV, higher levels were found in non-necrotizing GN and RTX untreated subjects. Serum CD40L was raised in AAV as well; higher concentrations were also found in PR3+ and MPO+ patients and early systemic, generalized, and refractory AAV. IL-10 may potentially serve as a marker of early systemic AAV. IL-33 may help to identify subjects with a higher risk for necrotizing GN in AAV.
Highlights
anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitides (AAV) are the most frequent types of primary small-vessel vasculitides, according to the revised Chapel Hill consensus conference nomenclature from 2012 [1]
We will shortly summarize the clinical characteristics of the subjects; the second part will address serological abnormalities, serum levels of specific pro- and anti-inflammatory cytokines
Four parameters fulfilled the criterium that at least 50% of all individual measurements were above the lower detection limit of the assay: IL-10, IL-33, TNF-α, and Soluble CD40 Ligand (sCD40L)
Summary
ANCA-associated vasculitides (AAV) are the most frequent types of primary small-vessel vasculitides, according to the revised Chapel Hill consensus conference nomenclature from 2012 [1]. The third and least frequent type of AAV is eosinophilic granulomatosis with polyangiitis (EGPA) [4]. The prognosis of AAV patients often depends on rapidly initiated diagnostic steps helpful to identify possible end-organ damage. Before the era of intensified immunosuppressive treatment using steroids and cyclophosphamide combined [6], more than 90% of all patients presenting with kidney failure and lung involvement (pulmonary-renal syndrome) died from the disease(s). This situation has significantly been improved in recent years, last but not least, with the introduction of rituximab as a therapeutic measure in generalized and remitting GPA/MPA [7]. Refractory retroorbital manifestations have been shown to respond less sensitive to drug therapy than resistant glomerulonephritis [8]
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