IgG4-related disease (IgG4-RD) is a rare, systemic condition characterized by inflammation and fibrosis (scarring) in various organs. It can affect nearly any organ, including the pancreas, salivary glands, kidneys, and lymph nodes. Despite increasing recognition, its exact cause remains unknown, though both genetic and environmental factors may contribute.
A hallmark feature of IgG4-RD is the presence of elevated serum IgG4 levels and dense infiltration of IgG4-positive plasma cells in affected tissues. This leads to the formation of tumor-like masses or organ enlargement, often mimicking other conditions, including cancer. Common symptoms vary based on the organs involved and can include swelling, pain, or dysfunction in the affected areas.
Recent advancements have enhanced our understanding and management of IgG4-RD. Notably, the U.S. Food and Drug Administration (FDA) approved Uplizna (inebilizumab), the first targeted therapy for IgG4-RD. This medication works by depleting B cells, which play a central role in the disease’s inflammatory process. Clinical trials demonstrated that Uplizna significantly reduced the risk of disease flare-ups by 87% compared to a placebo, offering a promising alternative to traditional steroids.
Early diagnosis and appropriate treatment are crucial to prevent irreversible organ damage. If you experience unexplained swelling, pain, or organ dysfunction, consult your healthcare provider. With ongoing research and emerging therapies, the outlook for individuals with IgG4-RD continues to improve.
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