Organ affected: salivary & lacrimal glands

How rheumatology, ENT, and ophthalmology coordinate scans, treatment, and follow-up for salivary and tear-gland disease.

What “gland disease” means

Glands are organs that secrete substances such as hormones and other substances that contribute vitally to normal body function. Examples of glands include the lacrimal glands and the submandibular and parotid glands – the subjects of this lesson. But IgG4-RD often targets many other types of glands, too: the pituitary, thyroid, and prostate glands, for example, as well as the pancreas, among others. Involvement of those other glands will be discussed in their own lessons, in turn.

Glands are sometimes mistaken for lymph nodes because they are frequently found in the same areas. Lymph nodes, however, are very different organs – being critical parts of the immune system. Lymph nodes will also be discussed in their own lesson.

Here we focus on the lacrimal glands , which produce tears, and the submandibular and parotid glands, which produce salivary juices. The submandibular and parotid glands are sometimes termed the major salivary glands.

The head and neck region is a major site of IgG4-RD organ involvement. More than 50% of IgG4-RD patients have disease in this area, and among the most prominently involved head and neck organs are important sets of glands:

• Major salivary glands (parotid and submandibular)

• Lacrimal glands and tissues around the eye (orbit)

Some patients have IgG4-RD that appears to be isolated to these organs. Other patients have more extensive disease that may require blood tests and imaging to detect.

The collaborative care team

To help ensure you receive the best care for IgG4-RD, a team-based, multidisciplinary approach is the standard of care and usually the safest path. That means one lead clinician coordinates the overall plan but other specialists—ophthalmology, otolaryngology (ear/nose/throat, or ENT), radiology, and pathology—contribute based on which organs are involved and what type of help is required. This kind of shared care can make diagnosis clearer, reduce delays, and help your doctors agree on one treatment and follow-up strategy, so important details don’t get missed.

For head-and-neck gland disease, three specialists often work closely:

Rheumatology

Helps connect symptoms across organs and guide immune-based treatments.

Otolaryngology (ear/nose/ throat, or ENT)

Evaluates salivary glands, sinus issues, ear problems, and throat symptoms. May perform biopsies or other procedures when needed.

Ophthalmology (eye specialists)

Evaluates lacrimal/orbital disease and helps protect vision when inflammation affects tissues around the eye.

In addition to these three clinical specialists, radiologists and pathologists also contribute to the care of IgG4-RD patients, particularly with regard to diagnosis.

A simple way to picture this: one clinician “holds the map,” and the specialists each check their part of the terrain, so the whole plan stays connected.

Biopsy to establish the diagnosis

The lacrimal, submandibular, and parotid glands are common biopsy sites that clinicians use to establish the diagnosis. These glands are logical choices for biopsy sites because the glands are easily accessible by physical examination and do not require imaging support or a major surgical procedure to acquire diagnostic tissue. Sometimes the diagnosis is approached via a needle biopsy, but often a small piece of tissue from either the lacrimal gland or submandibular gland is removed through a minor operation, typically performed on an outpatient basis.

Biopsy of one of these glands is often a crucial step in confirming an IgG4-RD diagnosis because there are a number of other diseases that can mimic IgG4-RD in involving these organs. These other diagnoses include:

• Sjögren’s syndrome

• Granulomatosis with polyangiitis

• Eosinophilic granulomatosis with polyangiitis

• Sarcoidosis

• Lymphoma

• Tuberculosis

Check for involvement beyond the head and neck

When IgG4-RD seems to involve the major salivary glands, experts emphasize that it’s important not to stop the evaluation there. IgG4-RD is often a multi-organ disease, so clinicians commonly look for involvement elsewhere using imaging such as CT scans.

This doesn’t mean something will be found—it means the care team is being careful

Determining what treatment is best

Treatment in IgG4-RD is about choosing the right level of immune control for your situation. Your care team is weighing how active the inflammation is right now, whether there is risk to important organs, and how to limit medication side effects over time.

For salivary and lacrimal gland disease, doctors often think in “tiers.” If the swelling is mild and you feel well, careful monitoring can be reasonable. If symptoms are bothersome or organs are at risk, doctors may use medicines that calm inflammation quickly and then decide how to prevent the disease from coming back.

Common treatment options:

Close observation (watchful waiting)

For mild, isolated gland disease with minimal symptoms, some patients can be monitored closely, especially after clinicians check for hidden disease in other organs.

Glucocorticoids (steroids), such as prednisone

Steroids often shrink swelling quickly and can help people feel better fast, but the tradeoffs are side effects and the common pattern of disease returning after tapering or stopping. (Refer to the Chapter 3, Lesson 2 on Steroids.)

B-cell depletion therapy

Medicines that reduce B cells (such as inebilizumab) can lead to major improvement in gland swelling for some patients and can reduce reliance on repeated steroid courses. (Refer to Chapter 3, Lesson 3 on B-cell depletion).

Other immunosuppressants (“steroid-sparing” medicines)

Some clinicians use conventional immunosuppressants (often called “DMARDs,” such as azathioprine, mycophenolate, or methotrexate) as steroid-sparing therapy, but responses to these medications are variable. B-cell–targeted therapy is often favored for patients who need longer-term control because it can produce strong responses and help reduce repeated steroid exposure.² (Refer to Chapter 3, Lesson 5 on DMARDs).

Maintenance strategy vs treat-at-flare strategy

After you improve, some care teams re-treat only if the disease returns (“reactive”), while others use scheduled maintenance to help prevent relapse. The serum IgG4 concentration is often a very good indicator of when clinicians and patients should be thinking about resuming treatment: ideally when the serum IgG4 level is clearly rising but there is no clinical evidence of active disease. The best plan depends on a patient’s individual situation and follow-up access. Because the field is still evolving, recommendations may change as new studies come out.

Supportive symptom care

Even while the immune treatment plan is being decided, doctors may also recommend symptom support (for example, measures for dry eyes or dry mouth), tailored to what’s bothering you most.

How doctors choose among these options

Doctors usually start by answering two questions:

• How active is the inflammation right now?

• Is any organ at risk of damage if we wait?

If gland swelling is mild and you’re not very bothered, your team may simply recommend close monitoring, but only after checking for signs that IgG4-RD is affecting other organs too.¹

If symptoms are more bothersome, or testing suggests active inflammation, steroids may be used to calm things quickly. The catch is that steroids can cause many serious side effects (like raising blood sugar or thinning bones) if used at doses that are too high or for too long even at low doses. In addition, IgG4-RD often returns when steroids are tapered or stopped, so many teams try to keep steroid exposure as low as they safely can.

When stronger or longer-lasting control is needed, many specialists use B-cell–targeted therapy because gland swelling can improve substantially in some patients and it can reduce repeated steroid cycles.

After you improve, the plan may be to treat again only if the disease returns, or to use scheduled maintenance; doctors are still learning which long-term approach fits which patients best, so follow-up and communication about symptoms really matter.

Treatment options for IgG4-RD

When IgG4-RD affects the salivary or lacrimal glands, the good news is that there are several effective ways to manage it, but the “right” plan depends on your symptoms and what else (if anything) is involved elsewhere in the body.

Treatment for head-and-neck IgG4-RD is often similar to treatment in other organs, but there are important real-life details that shape decisions: like how much swelling is bothering you, whether vision or breathing could be affected, and whether there are signs of IgG4-RD in other organs.

Your treatment team will think through these choices and discuss them with you, step by step, with the goal of reducing swelling, protecting function, and preventing long-term damage.

The treatment options may include:

1. Close observation (watchful waiting)
   If swelling is mild, symptoms are limited, and testing does not suggest urgent organ risk, careful observation may be reasonable, with a clear follow-up plan and vigilance. ⁷

2. Glucocorticoids (steroids)
   Glucocorticoids (steroids) can reduce swelling quickly, but relapse after taper is common, and side effects can be significant, so many experts try to limit total steroid exposure when possible.

3. B cell–targeted therapy
   B cells are immune cells that help coordinate antibody responses; in IgG4-RD, parts of this system can become misdirected. Treatments that reduce certain B cells can be very effective for many patients.⁶

Many people with IgG4-RD experience marked improvement in gland swelling after taking rituximab (a B-cell–depleting therapy). Your care team must tailor decisions about which medications to prescribe for you based on the full organ picture and other health risks (for example, diabetes may push clinicians to avoid steroids when possible).⁷

There is also strong clinical trial support for B cell depletion in IgG4-RD. The Phase 3 MITIGATE trial reports that inebilizumab reduced flare risk by 87% compared with placebo during the controlled period.⁸

Maintenance and monitoring: Making a plan for the months and years ahead

Once swelling improves and things feel steadier, the next question becomes: how do we keep IgG4-RD under control and catch problems early?

In good IgG4-RD care, your team takes a proactive approach. They build a follow-up plan that matches your risk, especially if the disease has involved more than one organ, if it has returned before, or if sensitive areas like the eyes are involved.

In general, clinicians use two main strategies:

1. Reactive monitoring (treat, then watch closely)

With this approach, treatment is given to calm active disease, then the care team monitors you carefully and treats you again if there are signs of return. This can work well when you have reliable follow-up, clear instructions on what symptoms to report, and a plan for repeat labs or imaging when needed.

2. Scheduled maintenance (planned repeat treatment)

With this approach, some patients receive treatment at set intervals to help prevent relapse—especially when prior flares have been frequent, organ risk is higher, or the consequences of a flare would be serious (for example, vision- threatening disease). The interval is individualized, based on your history and how your disease behaves.

A practical takeaway is this: reactive monitoring depends on strong communication and timely check-ins.
If visits are infrequent or symptoms could return quietly, some teams lean toward scheduled maintenance to reduce the chance that inflammation smolders long enough to cause damag e before it’s noticed

Summary

Gland disease in IgG4-RD—especially in the salivary and lacrimal (tear) glands—often responds well to treatment, but it benefits from a coordinated plan. This approach helps the car e team:

• Check beyond the glands for hidden organ involvement

• Choose therapy that controls inflammation while limiting harm

• Set a follow-up plan so disease doesn’t quietly return

References

1. IgG4ward! Foundation. IgG4-RD and You: IgG4-RD of the Head and Neck. YouTube. Accessed Feb 22, 2026. https://www.youtube.com/watch?v=xV8QjAwF0rQ

2. IgG4ward! Foundation. IgG4-RD & You (playlist listing head-and-neck episode). YouTube. Accessed Feb 22, 2026. https://www.youtube.com/playlist?list=PLsjkLF0IOMq9BPPrnfwJ6qBw8ns2Hp_9J

3. Stone JH (IgG4ward “Question of the Week”). IgG4-RD, the salivary glands, other potential organ involvement… IgG4ward.org. Accessed Feb 22, 2026. https://igg4ward.org/personal-igg4-rd-stories/igg4rd-question-of-the-week-igg4rd-salivary-glands/

4. IgG4ward! Foundation. It Takes a Team: The Specialists Who Support IgG4-RD. IgG4ward.org. Accessed Feb 22, 2026. https://igg4ward.org/igg4rd-care-team-specialists/

5. IgG4ward! Foundation. Education and Resources (patient-facing education hub). IgG4ward.org. Accessed Feb 22, 2026. https://igg4ward.org/education-and-resources/

6. Stone JH, Zen Y, Deshpande V. IgG4-Related Disease. N Engl J Med. 2012;366:539–551.

7. Baker MC. IgG4-Related Head and Neck Disease Treatment (IgG4ward PeachJAM clinician education session). Jan 3, 2025.

8. IgG4ward! Foundation. Positive MITIGATE trial results (inebilizumab reduced flare risk by 87%). IgG4ward.org. Jun 5, 2024. https://igg4ward.org/research/positive-mitigate-trial-results-igg4rd/