IGG4

UPDATE 12-18-2025

I was admitted to the hospital on 12/14/2025 with Jaundice, extreme yellowing of the eyes and skin. As of 12/14/2025 my IgG4-RD has now gone to my liver causing the main duck to my liver to stop working and I was admitted into the ER because of the jaundice that was now being experienced after my examination. I was admitted into the hospital and the first thing the next morning they had to go down through my throat to my liver and put a splint in my liver. I will have to repeat this again in six weeks and possibly again in another six weeks. The IgG4-RD is what’s causing my liver to swell. As of 12/18/2025 I still have elevated liver enzymes and they are being monitored. Will have the splint replaced in six weeks, then possibly repeated procedure in another six weeks.

IgG4-related disease (IgG4-RD) is a rare autoimmune condition where the immune system mistakenly attacks the body’s own tissues, causing chronic inflammation and fibrosis (scarring) in various organs. Here’s how it affects the liver and bile ducts:

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What is IgG4-RD?

  • It’s characterized by high levels of IgG4 antibodies and infiltration of IgG4-positive immune cells into organs.
  • Commonly affects the pancreas, bile ducts, salivary glands, kidneys, and sometimes the liver.

Impact on the Liver and Bile Ducts

  • IgG4-RD can cause IgG4-related sclerosing cholangitis, which means inflammation and narrowing of the bile ducts.
  • This narrowing blocks bile flow, leading to:
    • Jaundice (yellowing of skin and eyes)
    • Elevated liver enzymes (due to bile backup and liver stress)
    • Liver swelling or enlargement
  • If untreated, it can progress to fibrosis and permanent damage.

Treatment Approach

  • Endoscopic stent placement helps relieve obstruction.
  • Immunosuppressive therapy (usually corticosteroids first) is key to controlling inflammation and preventing recurrence.
  • Long-term monitoring with blood tests and imaging is essential.

Here’s a helpful list of questions you can take to your doctor to better understand and manage IgG4-related disease and its impact on your liver:

About Your Condition

  • What exactly is IgG4-related disease, and how is it affecting my liver and bile ducts?
  • How severe is the current inflammation and blockage?
  • Is there any permanent damage to my liver at this point?

Treatment Plan

  • What is the goal of the stent placement, and how long will I need it?
  • Why do I need repeat procedures every six weeks—what determines if more are needed?
  • Will I need medication (like steroids or immunosuppressants) to control IgG4-RD?
  • Are there alternative treatments if the stent or medication doesn’t work?

Monitoring and Recovery

  • How often will my liver enzymes and imaging be checked?
  • What signs should I watch for that indicate complications (e.g., infection, stent blockage)?
  • How will we know if the disease is under control?✅ Questions to Ask Your Doctor About IgG4-Related Disease and Liver Health

    About Your Condition

    • What exactly is IgG4-related disease, and how is it affecting my liver and bile ducts?
    • How severe is the current inflammation and blockage?
    • Is there any permanent damage to my liver at this point?

    Treatment Plan

    • What is the goal of the stent placement, and how long will I need it?
    • Why do I need repeat procedures every six weeks—what determines if more are needed?
    • Will I need medication (like steroids or immunosuppressants) to control IgG4-RD?
    • Are there alternative treatments if the stent or medication doesn’t work?

    Monitoring and Recovery

    • How often will my liver enzymes and imaging be checked?
    • What signs should I watch for that indicate complications (e.g., infection, stent blockage)?
    • How will we know if the disease is under control?

    Long-Term Outlook

    • Can IgG4-RD affect other organs in my body?
    • What is the risk of recurrence after treatment?
    • Are there lifestyle changes or diet recommendations to support liver health?

  • Here are some practical tips for monitoring symptoms at home after IgG4-related disease has affected your liver and bile ducts:

    ✅ Watch for Warning Signs

    • Jaundice changes: If your skin or eyes become more yellow, call your doctor immediately.
    • Dark urine or pale stools: These can indicate bile flow problems.
    • Fever or chills: Could signal infection, especially around the stent.
    • Severe abdominal pain: May mean blockage or complications.
    • Persistent nausea or vomiting: Important to report promptly.

 

Immunoglobulin G4 Related Disease
(IgG4-RD)

IgG4-RD is a chronic inflammatory condition that can affect multiple organs at a time. It is an immune-mediated disease that may lead to fibrosis (scarring of tissues).

April 4th is IgG4-RD Awareness Day! https://igg4ward.org/community/

The IgG4ward! Foundation will host a fireside chat, “Hope is Here,” on Friday, April 4th to celebrate IgG4-RD Awareness Day. The event will feature an interview with the first patient to receive B-cell depletion therapy for IgG4-RD, along with discussions on future advancements in the treatment of the disease.

I have IgG4-rd

I have never been Vaxxed, but I did get a mild case of COVID. The hospital did a COVID-19 test up my nose and mannnn….. did it burn for 3 days. I think that is the reason I started having IGG4 symptoms in the weeks to follow. It is possible that the mrna virus was in my test for COVID-19. It was after that I was tested up my nose that I started getting symptoms 30 days later. It was not until 2024 that my local doctor tested me for IGG4-RD. My biopsy showed the IGG4 in my saliva glands from the lower jaw, swollen lymph nodes, and tear ducts, swollen eye lids with enlarged and swollen lymph nodes in my gut and lungs.

X-ray of my swollen bowels.

IgG4-RD and You: IgG4-RD of the Pancreas

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SYMPTOMS

IgG4-related disease is a protean condition that mimics many malignant, infectious, and inflammatory disorders. This multi-organ immune-mediated condition links many disorders previously regarded as isolated, single-organ diseases without any known underlying systemic condition. It was recognized as a unified entity only 10 years ago.

Histopathology is the key to diagnosis. The three central pathology features of IgG4-related disease are lymphoplasmacytic infiltration, storiform fibrosis, and obliterative phlebitis. The extent of fibrosis is an important determinant of responsiveness to immunosuppressive therapies.

IgG4-related disease generally responds to glucocorticoids in its inflammatory stage, but recurrent or refractory cases are common. Important mechanistic insights have been derived from studies of patients treated by B-cell depletion.

Greater awareness of this disease is needed to ensure earlier diagnoses, which can prevent severe organ damage, disabling tissue fibrosis, and even death. Identification of specific antigens and T-cell clones that drive the disease will be the first steps to elucidate the pathogenesis of IgG4-related disease.

https://pubmed.ncbi.nlm.nih.gov/23666890/

https://www.thelancet.com

Nutrition

 

Is IgG4 disease a disability?

If you are living with IgG4-related disease and it negatively impacts your ability to work full time, you might be considering an application for Social Security Disability.

Can IgG4 affect the heart?
IgG4-RD is an uncommon cause of coronary artery disease but can lead to life-threatening complications including myocardial infarction. Regarding the importance of early treatment, clinicians should be aware of cardiac complications in these patients.

How many people have IgG4-RD?
In a 2023 study, the overall incidence of IgG4-RD was estimated to be 0.78 to 1.39 per 100,000 person-years [7]. The epidemiology of specific forms of IgG4-RD is discussed below. Sep 10, 2024 So, it was explained to m that 1 in every 100,000 people are reported to having IgG4-RD.

Full Video
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Where do you hurt with pancreatitis?
NOTE: Once the pancreatitis is wounded it will not repair itself. 
The main symptom of acute pancreatitis is a severe, dull pain around the top of your stomach that develops suddenly. This aching pain often gets steadily worse and can travel along your back or below your left shoulder blade.

Eating or drinking may also make you feel worse very quickly, especially fatty foods.
Consume NO ALCOHOL at all.

Tissue biopsy

  • Tissue biopsy is the gold standard for diagnosis in most settings. Review of archived pathology samples can confirm the diagnosis of IgG4-related disease on histological findings alone, if large specimens such as submandibular gland resections are available.
Also – check out MY Shingles  Neuropathy story.

 

 

Which are THE most often affected organs in the IgG4-related disease?

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It is not unusual for the IGG4 disease to affect anywhere between two to six organs. The following organs are often involved in IgG4-RD: (1) meninges, (2) orbits, (3) lacrimal glands, (4) major salivary glands, (5) thyroid gland, (6) lungs, (7) aorta, (9) kidneys (10) pancreas, (11) bile ducts.

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glucocorticoids

Most cases are with men. The first case reported was a 66-year-old woman with multiple pseudotumors in the salivary glands, pancreas, and retroperitoneum. The first pathologically proven case presented pituitary expansion with blurred vision and hypogonadism and a history of autoimmune pancreatitis.

 

See also An Overview of Hydronephrosis

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Google IGG4

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IgG4-related disease (IgG4-RD) is a chronic inflammatory condition characterized by the infiltration of IgG4-positive plasma cells into various tissues and organs, leading to fibrosis and organ dysfunction. It can affect nearly any organ system, but common sites include the pancreas, bile ducts, salivary glands, kidneys, lungs, and lymph nodes.
### Key Features of IgG4-Related Disease:
1. **Inflammation and Fibrosis**: The hallmark of IgG4-RD is a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells and storiform fibrosis (a characteristic pattern of fibrosis).
2. **Elevated Serum IgG4**: Many patients with IgG4-RD have elevated levels of IgG4 in their blood, although this is not universal and not required for diagnosis.
3. **Multiorgan Involvement**: IgG4-RD can involve multiple organs simultaneously or sequentially. Commonly affected organs include:
   – **Pancreas**: Leading to autoimmune pancreatitis.
   – **Bile Ducts**: Causing sclerosing cholangitis.
   – **Salivary and Lacrimal Glands**: Resulting in swelling and dysfunction.
   – **Kidneys**: Causing tubulointerstitial nephritis.
   – **Lungs**: Leading to various pulmonary manifestations.
   – **Lymph Nodes**: Causing lymphadenopathy.
4. **Symptoms**: Symptoms vary depending on the organs involved but can include abdominal pain, jaundice, dry mouth and eyes, and generalized symptoms like weight loss and fatigue.
5. **Diagnosis**: The diagnosis of IgG4-RD is based on a combination of clinical, serological, radiological, and histopathological features. Biopsy of the affected tissue showing characteristic features is often necessary.
6. **Treatment**: Corticosteroids are the mainstay of treatment and are typically effective in reducing inflammation and symptoms. Other immunosuppressive agents, such as rituximab, may be used in refractory cases or to maintain remission.
7. **Prognosis**: With appropriate treatment, many patients achieve remission, but the disease can be chronic and relapsing, requiring long-term management.
IgG4-RD is relatively rare and was only recognized as a distinct clinical entity in the early 21st century. Ongoing research continues to improve understanding of its pathogenesis, diagnosis, and treatment.


Wikipedia • A COVID‑19 vaccine is a vaccine intended to provide acquired immunity against severe acute respiratory syndrome coronavirus 2, the virus that causes coronavirus disease 2019.

 

Be sure to check out
IGG4ward.org
for support group.

Please watch: Igg4ward VIDEOS

 

IgG4-related disease is a new systemic disease that has only recently been identified and characterized. However, most clinical practitioners are not yet familiar with it and thus are likely to misdiagnose it.

The purpose of this book is to raise awareness of this disease and its diagnostic pitfalls. A wealth of radiological and histopathological images helps clinicians to understand its characteristics in various organs as well as their clinical and basic medical backgrounds.

All of the authors are experts who have published numerous important papers in the field. This book will be useful to physicians in various disciplines such as gastroenterology, rheumatology, ophthalmology, otolaryngology, urology, hematology, and respiratory medicine, not only as a textbook but as an authoritative and comprehensive reference work as well. Can be found on Amazon

HAND CRAMPS – Diabetic disorders

 

Dr Honea