What are the consequences when IgG4 fails

By SWA

IgG4-related disease is a condition that can affect multiple organs and parts of the body. It's characterized by the infiltration of IgG4-secreting plasma cells and can lead to the formation of tumor-like swellings in affected organs. The failure or dysregulation of IgG4, therefore, refers to situations where there is an abnormal increase in IgG4 levels or function, contributing to disease development.

A high IgG4 level is a crucial marker in the context of diagnosing and understanding IgG4-Related Disease (IgG4-RD). Elevated serum IgG4 concentrations can support the diagnosis of IgG4-RD, especially when considered alongside the appropriate clinical context and symptoms.

What is IgG4-RD?
Immunoglobulin G4-related disease (IgG4-RD) is a chronic immune-mediated fibroinflammatory disorder that often manifests with tumor-like masses and/or painless enlargement of multiple organs. Serum IgG4 level is often but not always elevated.

The consequences of IgG4-related disease can vary widely depending on the organs involved:

  1. Pancreas: Involvement of the pancreas can lead to autoimmune pancreatitis, which might present with jaundice, abdominal pain, and weight loss. It can mimic the symptoms of pancreatic cancer and can lead to pancreatic insufficiency, affecting digestion and absorption of nutrients.

  2. Biliary Tree: IgG4-related disease can affect the bile ducts, leading to sclerosing cholangitis. This condition can cause obstructive jaundice, itching, and potentially liver damage over time.

  3. Salivary Glands and Lacrimal Glands: Involvement of these glands can lead to swelling and dryness, mimicking Sjögren's syndrome. This can result in dry mouth and dry eyes (sicca symptoms).

  4. Kidneys: IgG4-related disease can lead to tubulointerstitial nephritis, which may present with renal insufficiency, proteinuria, and even kidney failure if not properly treated.

  5. Lungs: In the lungs, it can cause interstitial pneumonia, nodules, and thickening of the bronchial walls, potentially leading to respiratory symptoms like cough and shortness of breath.

  6. Retroperitoneal Fibrosis: This can cause the fibrous tissue to encase abdominal organs, potentially leading to hydronephrosis (water on the kidney) due to ureteral obstruction, and it can affect the function of other abdominal organs.

  7. Lymph Nodes: Enlargement of lymph nodes can occur, which is usually benign but can mimic malignant conditions.

  8. Vascular System: There can be involvement of the vascular system, including conditions like vasculitis, which can affect blood flow to various parts of the body.

The broad range of potential symptoms and organ involvement makes IgG4-related disease a challenging condition to diagnose and manage. Treatment usually involves immunosuppressive therapy, such as corticosteroids and rituximab, aimed at reducing inflammation and the levels of IgG4-producing cells. Early diagnosis and treatment are crucial to prevent irreversible organ damage and maintain quality of life.

Reference: Catalytic Antibodies in Norm and Systemic Lupus Erythematosus
https://www.intechopen.com/chapters/54555

Longest recorded IgG4 levels post mRNA shot! - IgG4 part 13
https://www.youtube.com/watch?v=0UFS4EVgf3U&t=18s

read also: Understanding IgG4 https://swaresearch.blogspot.com/2024/03/understanding-igg4.html

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