Immunoglobin G4-related disease (IgG4-RD) is a rare fibro-inflammatory disease of unknown etiology that has been recently recognized. It can cause fibro-inflammatory masses in almost every organ of the body and is associated with dense lymphoplasmacytic infiltration of IgG4-postitive plasma cells, storiform fibrosis and elevated levels of serum IgG4.1 IgG4-RD is a systemic disease that may…
What rheumatologists need to know about identifying and diagnosing immunoglobulin G4-related disease (IgG4-RD)
The past five years have been busier than usual for the Churg-Strauss syndrome. It was renamed eosinophilic granulomatosis with polyangiitis (EGPA).1 Longitudinal cohorts totaling 484 patients—approximately as many as all previous series combined—were described.2,3 A proposal was advanced to remove and rename a subset in which vasculitis may not be present.4 And shortly after the…
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome or allergic granulomatosis and angiitis, is a rare small- and medium-vessel vasculitis. This disease was first described by American pathologists Jacob Churg and Lotte Strauss in 1951.1 Although the vasculitis is often not apparent in the initial phases of the disease, EGPA can affect any…