The importance of early treatment for IgG4-related disease

Immunoglobulin G4-related disease (IgG4-RD) often develops quietly with no symptoms, and when early symptoms occur, they can be mild or nonspecific. If left untreated, it can progress over time and eventually lead to organ damage and a wide range of symptoms, including jaundice (a yellowing of the skin and whites of the eyes), gastrointestinal problems, and pain.

Early treatment of IgG4-RD matters because it helps to manage symptoms and progression, as well as prevent organ damage and other potential complications.

As difficult as the disease can be to recognize, diagnosing and getting it treated as early as possible can benefit your long-term well-being.

Why early treatment matters

IgG4-RD involves chronic inflammation, which in its early stages can cause organ swelling or mild dysfunction. But over time, it can lead to scar tissue formation, known as fibrosis.

Preventing the transition from IgG4-RD inflammation to fibrosis is crucial because inflammation can be addressed through treatment, whereas fibrosis is largely permanent and can lead to irreversible organ dysfunction.

Early treatment may therefore improve IgG4-RD long-term outcomes.

Common early symptoms that shouldn’t be ignored

It can be challenging to recognize the early signs of IgG4-RD because symptoms may not be obvious at first or mimic those of other diseases.

Often, there are no symptoms in the earliest stages of the disease. It is still important to know what symptoms to watch for, as this may bring you a step closer to a correct IgG4-RD diagnosis and treatment.

Symptoms of IgG4-RD that may occur early on in the disease include:

• painless swelling in the face, neck, armpits, or groin
• unexplained weight loss
• fatigue
• abdominal discomfort
• jaundice
• side or back pain
• shortness of breath

Keep in mind that IgG4-RD affects people differently, so symptoms may vary from person to person.

What early treatment typically involves

Your IgG4-RD care team will be able to recommend the best type of treatment for you.

The goal of treatment is twofold: achieving remission, characterized by minimal or no symptoms, and preventing relapses, which involve new or worsening disease activity that could lead to organ damage.

Your care team may include doctors from different specialties, depending on which organs are affected by the disease. Treatment may be bodywide or specific to certain organs.

IgG4-RD treatment options include:

• glucocorticoids, which derive from steroid hormones, to rapidly reduce inflammation
• nonsteroid immunosuppressive medications to manage the disease and avoid long-term steroid use, which is linked to serious side effects
• biologic therapies, or those derived from living organisms, which are more targeted in suppressing abnormal immune activity
• organ-specific treatments, including possible surgery
• supportive care to treat symptoms, depending on the organs involved

Management of the disease also typically involves close IgG4-RD monitoring and follow-up to evaluate your response to treatment and adjust it if needed.

Once your care team has recommended a treatment plan, don’t hesitate to ask questions. You have the right to advocate for yourself and to become an active decision-maker in discussions related to your treatment and well-being.

Benefits of starting treatment early

Starting treatment as soon as possible can help prevent further inflammation and scarring. Early treatment can also help you manage symptoms and prevent long-term IgG4-RD complications.

Benefits of starting IgG4-RD treatment early include:

• preventing organ damage
• inducing potential remission
• preventing IgG4-RD flares
• maintaining or improving your quality of life

Risks of delayed or untreated IgG4-RD

Delaying treatment allows inflammation to linger and may increase the chances of fibrosis and complications.

Potential risks from delays include:

• progressive organ damage
• irreversible fibrosis
• chronic pain or functional impairment
• organ failure and other potential complications
• reduced response to treatment once pronounced fibrosis has occurred

Looking ahead: Living well with IgG4-RD

Living with IgG4-RD can be challenging, but early treatment along with support can make a meaningful difference.

It’s important to be educated about your condition, so you can recognize new symptoms, communicate with your care team, and participate in shared decision-making about your health.

Because IgG4-RD can affect different organs and tissues, you may need to work with a multidisciplinary care team that can address your different symptoms and balance treatments.

Living with IgG4-RD often means having frequent doctor appointments and scans, as well as dealing with symptoms like pain, fatigue, and sudden weight loss. It’s important to have supportive family and friends who can accompany you to doctor visits and also provide support during flares or if the disease progresses.

Having a trusted team of healthcare providers and support from loved ones can help improve your quality of life.