IgG4-related disease (IgG4-RD) is a rare, systemic fibro-inflammatory condition characterized by tissue infiltration with IgG4-positive plasma cells. It can affect virtually any organ, making diagnosis challenging. Symptoms vary depending on the organs involved, but common manifestations include swelling of the salivary or lacrimal glands, pancreatic involvement, and lymph node enlargement.
What makes IgG4-RD so perplexing? Its presentation is often subtle, mimicking other conditions like cancer or infections. Elevated serum IgG4 levels are a hallmark, but aren't always present, and not all patients with high IgG4 have the disease. Diagnosis requires a combination of clinical findings, imaging, and biopsy confirmation, demonstrating the characteristic IgG4-positive plasma cell infiltration.
While the exact cause remains unknown, IgG4-RD is believed to be an autoimmune disorder. Treatment typically involves corticosteroids or other immunosuppressants to reduce inflammation and prevent organ damage. Early diagnosis and treatment are crucial to improving patient outcomes and minimizing long-term complications. Research continues to shed light on this enigmatic disease, offering hope for better diagnostic tools and targeted therapies.
