Kidney and lung involvement constitute key disease features and largely confer higher disease burden and mortality in patients with ANCA-associated vasculitis (AAV). Lung biopsy is only performed in a minority of cases and diagnosis of lung involvement relies on clinical and imaging findings.
The complement pathway is critically involved in many autoimmune diseases including vasculitides. In a multinational effort, Triaille and colleagues studied the clinical manifestations and in vivo activation of the type 1 interferon pathway in twelve patients with hereditary C1q deficiency (C1QDef).
Using a database of cellular signatures and a combination of gene editing tools, expression assays and advanced sequencing techniques the authors elucidate in their work how a specific genetic locus on chromosome 21 (chr21q22) influences macrophage-mediated inflammation through the ETS2 pathway.
MANDARA ist eine doppelblind, randomisiert-kontrollierte Phase III Studie mit einem aktiven Vergleichsarm ohne Placebo, die die Nicht-Unterlegenheit des IL5-Inhibitors Benralizumab (Benra) gegenüber Mepolizumab (Mepo) bei refraktärer oder “relapsing” EGPA zeigen sollte.
This study corroborates previous findings of high relapse rates after cessation of 12 months of TCZ therapy.
Almost half of the patients experienced disease relapses within two years.
Why some patients get autoimmune diseases, including vasculitis, is still not well understood.
Besides genetic predisposition (in the case of GCA HLA-DR4), infections have been associated with different autoimmune diseases.
The aim of the present study was to investigate the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia (PMR) and to determine the optimal treatment approach.
Polymyalgia rheumatica (PMR) often relapses when the glucocorticoid (GC) dose is reduced.
Traditionally, methotrexate (MTX) is then used as a GC-sparing measure.
Interleukin-6 is often understood as a pro-inflammatory cytokine, similar to TNFa and IL-1b.
This is not correct, as IL-6 also triggers inflammation-limiting mechanisms, such as the expression of the Tissue Inhibitor of Metalloproteinases (TIMP).
Despite a significant improvement in outcome through the use of glucocorticoids and cyclophosphamide, relapses in granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA) remain a major problem.
