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Updated: Jan 31, 2026

Human Serum Anti-aquaporin-4 Immunoglobulin G Detection by Cell-based Assay
Published on: April 5, 2019
Immunoglobulin (Ig)G-4 related myositis - A new entity?
Vincent Casteleyn1, Helena Radbruch2, Torsten Diekhoff3
1Department of Rheumatology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Abstract:
Immunoglobulin (Ig)G4-related disease is an uncommon systemic autoimmune disorder characterized by infiltration of IgG4+ plasma cells in different organs and elevated levels of IgG4 in peripheral blood. So far, only one case of myositis with abundant IgG4+ plasma cells has been reported and classified as 'polymyositis'. We present an unusual case of chronic inflammatory myopathy in a context of rheumatoid arthritis. Severe granulomatous myositis, featuring abundant IgG4+ plasma cells was identified in two skeletal muscle biopsies within a five-year-interval. We suggest this entity to be a new subtype of immunoglobulin G4-related disease: IgG4-related myositis, while there were no diagnostic criteria fulfilled for the known idiopathic inflammatory myopathies.
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