9 common myths about IgG4-RD

While awareness about immunoglobulin G4-related disease (IgG4-RD) has improved over time, there is still much that is not understood.

Because many symptoms are similar to those of other diseases, confusion is not uncommon and can even delay getting a correct diagnosis.

But learning the facts about lgG4-RD may help you feel more in control of your health.

Myth 1: IgG4-RD only affects one organ at a time

Fact: IgG4-RD can affect a single organ, though in the majority of cases, the disease has multiorgan involvement, meaning it affects several organs. This may happen at the same time or sequentially. And it is one reason that the disease can be confusing and hard to diagnose.

IgG4-RD can impact organs such as the:

• pancreas
• salivary glands
• lymph nodes
• kidneys
• lungs

Myth 2: IgG4-RD is always painful

Fact: Many people with IgG4-RD may have mild symptoms or none at all, at least until organ function is substantially affected.

Other IgG4-RD symptoms may include:

• swollen lymph nodes
• weight loss
• swelling in the face
• eye issues
• jaundice
• gastrointestinal problems

Some people experience mild pain. But most pain-related symptoms are related to specific organs affected by the disease, such as the kidneys or pancreas.

Myth 3: Only older adults develop IgG4-RD

Fact: It’s true that IgG4-RD is most often diagnosed in middle-aged and older adults, especially men, but it can also affect women and, in rare cases, children.

Men are more likely to have forms of IgG4-RD that affect multiple organ systems, such as the pancreas and kidneys.

Myth 4: IgG4-RD is extremely rare

Fact: While IgG4-RD is still considered a rare disease, there is an increasing awareness and improvement in how it is diagnosed, leading to a greater number of diagnoses.

IgG4-RD is sometimes misdiagnosed as another disease or diseases. This is because it can mimic cancer, infections, and autoimmune or inflammatory diseases.

Some diseases that IgG4-RD may be mistaken for include:

• Sjögren’s disease
• various types of cancer
• blood disorders
• Castleman disease
• sarcoidosis

Still, more and more doctors are recognizing the signs of IgG4-RD earlier, resulting in more accurate diagnoses.

Myth 5: IgG4-RD is easy to diagnose

Fact: Diagnosing IgG4-RD isn’t simple.

Because symptoms overlap with many other diseases, an accurate IgG4-RD diagnosis usually involves:

• an examination of tissues under a microscope (histopathology)
• imaging for lgG4-RD to determine organ involvement
• identification of typical IgG4-RD features through blood tests and clinical exams
• ruling out other diseases

It often takes multiple steps and testing to diagnose IgG4-RD, but doctors are getting better at recognizing IgG4-RD.

Myth 6: IgG4-RD always causes obvious symptoms

Fact: Sometimes individuals with IgG4-RD have obvious symptoms, but many people have no symptoms for months or even years. Because not everyone experiences notable symptoms, early detection can be difficult.

IgG4-RD can lead to organ damage even in those who are feeling well. In these cases, the disease may be discovered due to an unrelated health issue.

It can be frustrating when symptoms are hard to pinpoint, but you’re not alone. Staying in communication with your care team is the best way to address your concerns.

Myth 7: Lifestyle changes alone can prevent or reverse IgG4-RD

Fact: Eating a healthy diet and making other lifestyle adjustments may help you better manage lgG4-RD.

But most important is that you follow your treatment plan, which can include corticosteroids and other immunosuppressants, to slow disease progression and prevent organ damage.

Myth 8: IgG4-RD is a cancer

Fact: IgG4-RD is not a cancer, but it can cause tumor-like masses that may be misdiagnosed as cancer. Your healthcare team will have to rule out cancer when diagnosing IgG4-RD.

Unlike several cancers, IgG4-RD responds quite substantially to treatment with glucocorticoids.

Myth 9: Once treated, IgG4-RD never comes back

Fact: Even if your IgG4-RD treatment was considered successful, you can still have a relapse.

While many patients respond well to their treatment, IgG4-RD requires ongoing monitoring. Recurrences or relapses are common and may require long-term therapy.

While it’s understandable to worry about a relapse, the best way to manage IgG4-RD is by having regular follow-ups with your care team to help catch issues early.

Takeaway

There are many myths about IgG4-RD, especially because it can seem similar to other conditions.

The more you know about IgG4-RD, the better prepared you’ll be to work with your medical team to get the care you deserve.

Frequently asked questions about IgG4-RD

Have more questions about IgG4-RD? Read on to get the facts.

Is IgG4-RD an autoimmune disease?

IgG4-RD is considered an immune-mediated fibroinflammatory condition, meaning it involves immune responses that cause chronic inflammation and tissue scarring.

In autoimmune diseases, self-reactive antibodies attack the body’s tissues or elements. In IgG4-RD, specific immune cells build up in tissues and cause inflammation and scarring.

Can IgG4-RD be cured?

There is currently no cure for IgG4-RD. But most people do respond well to treatment.

In many cases, people with IgG4-RD experience remission with glucocorticoids and immunosuppressant treatment, though it’s necessary to continue to monitor and manage the disease to prevent and address a relapse.

You and your care team will work together to navigate IgG4-RD and determine the best course of action for you.

Are IgG4-RD symptoms the same for everyone?

The symptoms of IgG4-RD can vary widely from person to person. Which symptoms you experience depend on the organs involved and the degree of inflammation or scarring.

Some people may have pain, nausea, or swelling, while others may be asymptomatic.

Staying on top of your treatment and working closely with your care team may help you prevent or better manage flare-ups.

Does IgG4-RD always require lifelong treatment?

IgG4-RD is treatable, and many people go into remission. But some people with IgG4-RD may need to be on long-term therapy to sustain disease remission and avoid relapses and/or irreversible organ damage.

Others can reduce treatment dosing or stop therapeutic treatment after remission, though this depends on whether your individual risk of recurrence is high.

Discuss your treatment options with your care team to figure out the plan that’s best for you.

How is IgG4-RD monitored over time?

Regular follow-ups for IgG4-RD include:

• clinical exams
• lab tests for IgG4 levels
• imaging
• biopsies in some cases

Testing helps monitor disease activity and identify which organs are involved.

Long-term monitoring is key to managing flare-ups and preventing permanent organ damage, so you want a team you trust to support you in your care.