IgG4-related disease—rare but you should not forget it

Abstract

Abstract Immunoglobulin G4-related disease is a systemic immune-mediated disease with insidious evolution characterized by fibroinflammatory lesions over virtually any organ system. Despite the remarkable progression of knowledge, its etiology remains undefined. Due to its relapse-remitting pattern, it could accumulate irreversible damage, increasing comorbidities and mortality. This paper emphasizes key concepts for diagnosing and treating patients with this condition.

Keywords

• Immunoglobulin G4-related disease
• IgG4-RD
• Fibroinflammatory disease
• Review
• Rare diseases
• Chronic relapsing-remitting inflammatory condition