T-Lymphocytes and Disease Mechanisms in Wegener’s Granulomatosis

Abstract

The mechanisms underlying Wegener’s granulomatosis (WG) are not well understood. The role of T-cells in the pathogenesis of WG has only recently come into focus of research. This review presents recent developments regarding the role of T-cells in WG. The occurrence of anti-neutrophil-cytoplasmic antibodies (ANCA) directed against proteinase-3 (PR-3) is a hallmark of WG. ANCA seem to mediate vasculitic damage in WG. Apart from ANCA, T-cells are involved in disease mechanisms. T-cells might participate in ANCA formation. Furthermore, T-cells are observed in affected tissue and granulomatous lesions. T-cells are indispensable for granuloma formation in other diseases and this might apply to WG too. In line with this, several aberrations of T-cell populations and alterations of the T-cell response were recently discovered in patients suffering from WG. Therefore, the impact of T-cell polarization, genotypic alterations modifying T-cell function and specific T-cell subsets on disease pathogenesis is discussed. Moreover, the influence of Staphylococcus aureus on T-cells and self-tolerance in WG is further elucidated. Finally, therapeutic options and implications with regard to T-cell-mediated pathogenesis are highlighted.

Granulomatosis With Polyangiitis, Which Is Pathogenetically Associated With Antineutrophil Cytoplasmic Antibodies: Clinical Features

“…Следует отметить, что, в то время как иммунные на-рушения с участием T-хелперов 1-го типа, секрецией ин-терферона γ и фактора некроза опухоли α, в первую оче-редь, свойственны локальной форме ГПА, при генерализо-ванном полиангиите может преобладать Th2-тип иммун-ного ответа [16,17].…”

Immune Cells Profiling in ANCA-Associated Vasculitis Patients—Relation to Disease Activity

“…Considering the significant role of T, B and natural killer (NK) cells in the pathophysiology of AAV, the assessment of their distribution and disease-specific alterations could be helpful in establishing proper diagnosis [ 9 ]. So far, studies analyzing the AAV-related alterations in T, B and NK lymphocyte subsets distribution are sparse [ 8 , 10 , 11 , 12 ].…”

Differential diagnosis of a local form of granulomatosis with polyangiitis: nasal cavity and paranasal sinuses lesions (part 1)

“…Существует тесная связь гранулематозного воспаления с Т-клеточным ответом [18]. По мнению одних авторов, иммунные нарушения с участием T-хелперов 1-го типа, секрецией интерферона (ИФН) γ и фактора некроза опухоли (ФНО) α свойственны в первую очередь локальной форме ГПА, в отличие от генерализованной, которая проявляется сосудистыми воспалительными изменениями с более сильной экспрессией цитокинов Th2-типа иммунного ответа [19,20] [22]. Таким образом, отрицательный результат биопсии не исключает диагноза АНЦА-васкулита.…”