Federica Maritati

TL;DR: Serum IgG4 levels are markedly elevated in active CSS and correlate with the number of organ manifestations and disease activity and longitudinal analysis in 12 CSS cases showed that both the IgG3 level and IgG 4/IgG ratio dropped during disease remission.

TL;DR: Eosinophilic granulomatosis with polyangiitis (EGPA) is a multisystemic disorder, belonging to the small vessel anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, defined as an eOSinophil-rich and necrotizing granulOMatous inflammation often involving the respiratory tract, and Necrotizing vasculopathy predominantly affecting small to medium-sized vessels.

TL;DR: Idiopathic retroperitoneal fibrosis (RPF) as discussed by the authors is a rare fibro-inflammatory disease that develops around the abdominal aorta and the iliac arteries, and spreads into the adjacent retinalum, where it frequently causes ureteral obstruction and renal failure.

TL;DR: The first-line treatment of IgG4-RD is based on glucocorticoids, which are usually efficacious, but B cell depletion induced by rituximab has also been found to induce remission in steroid-resistant disease or has been used as steroid-sparing agent for relapsing disease.

TL;DR: Rituximab (RTX) is a B cell–depleting antibody of proven efficacy in antineutrophil cytoplasmic antibody–associated vasculitis and the efficacy and safety of RTX were tested in a multicenter cohort of patients with adult‐onset IgAV.