Organ affected: salivary & lacrimal glands

Patients describe what IgG4-RD felt like, what helped, and how their experiences were similar – and different.

Patient stories : Living with salivary and lacrimal gland IgG4-RD

When IgG4-related disease (IgG4-RD) affects the salivary glands (glands that make saliva) and lacrimal glands (glands that make tears), people often describe a mix of visible changes and day-to-day discomfort. Some notice painless swelling of the glands in the face or around the eyes, and some struggle more with dry mouth or dry eyes, or both.

One of the hardest parts can be the uncertainty at the start. Swelling in these glands can look like other conditions, and it may take time, careful testing, and sometimes a biopsy to get the right diagnosis. That delay can be frustrating, and it’s one reason patient stories matter.

Listening to patient stories can help you feel less alone, and they show that there isn’t just one “right” way this illness shows up.

Featured stories

Bob Wilson: Eye (lacrimal) swelling for decades before IgG4-RD diagnosis and effective treatment

“Back then, the only thing that would really reduce the swelling of my eyes was the use of steroids.”

Bob’s story started in 1980, when he woke up with major swelling around his eyes, especially the left. For years, he was treated as if it were allergies, and steroids were the only thing that reliably brought the swelling down. Much later, imaging and biopsy helped confirm IgG4-RD, learned his disease involved the lacrimal glands and tissues around the eye, and over time he also developed involvement of major salivary glands, especially the submandibular glands under the jaw.

Bob’s IgG4-RD has been hard to control at times, but for long periods of time his disease has been controlled well with B cell depletion treatments. He’s been through a lot of medications through the years, and participated in a number of clinical trials in the interest of finding a better treatment for himself – and helping others with his diagnosis, too.

In the past, rituximab worked very well for about seven years before it stopped working, and other options (like abatacept and ocrelizumab) helped only briefly. He also did well during a trial of rilzabrutinib until it became unavailable.

“So far, so good with inebilizumab,” said Dr. John Stone, rheumatologist with Mass General Hospital and founder of the IgG4ward! Foundation. “What matters most is that the numbers are moving in the right direction, and with the range of therapies we now have, I’m hopeful that we will always have good options – and even better ones in the future – to treat patients like Bob.”

Bob and Karen Wilson: The power of not going it alone

“It’s really been a long journey... and it’s been very difficult,” said Karen.
“It gives us comfort to know that we’re not isolated and that we’re not on an island.”

Bob shares how his IgG4-RD began in 1980 with severe swelling around his eyes that was treated as allergies for years, with steroids offering only temporary relief. Eventually, specialized evaluation helped confirm what was really going on (IgG4-RD), and later revealed submandibular gland involvement as well. And, as often is the case, further evaluation led to the detection of organ involvement elsewhere: in the pancreas, the lungs, the retroperitoneum, a paraspinal mass along the spine, and lymph nodes.

His wife, Karen, describes what it has taken to walk through this with him: travel, time off work, and the emotional ups and downs of a long illness. Together, they emphasize how education and community support can help patients and caregivers feel less isolated and more hopeful.

William Joy: Chronic sinus flares and eye swelling before the right diagnosis

“Several times the eye got so big, it was like a softball size.”

William says his symptoms started when he was about 19, with chronic sinusitis that kept coming back despite treatments, including procedures to remove inflamed tissue. Later the swelling “shifted” to his left eye and at times became severe enough to require hospital stays for IV steroids.

After many years of being told it might be something else (including a cancer scare and years of being followed as Sjögren’s), he finally got a clear IgG4-RD diagnosis—and treatment that helped him feel like he got his life back. He’s been off prednisone for 17 years now.

“Bob’s story is a powerful reminder that IgG4-related disease can hide in plain sight,” said Dr. Stone. “He spent years treating severe eye swelling as ‘allergies,’ getting only temporary relief from steroids, until a full evaluation finally put the right name on it and showed how widely this illness can reach.”

Some common themes among people with IgG4-RD

Many people who are eventually diagnosed with IgG4-related disease (IgG4-RD) tell stories that sound a lot like these. A common pattern is years of symptoms that get labeled as something more common (allergies, chronic sinus problems, “idiopathic” inflammation, Sjögren’s Disease), followed by short-term relief from steroids, and then the long wait, sometimes decades, until someone connects the dots and th e right testing finally leads to answers.

You’ll often hear people describe:

• A long diagnostic road with many doctors and many “almost” answers.

• Symptoms that move or spread over time (for example: sinuses to eye area; eyes to salivary glands; glands to other organs).

• Steroids as the emergency brake. They help fast, but the problem returns when the course ends.

• A mix of visible and invisible burden: swelling people can see, plus fatigue, worry, and the daily stress of not knowing what’s next.

• Relief when the illness finally has a name, because a name usually brings options, monitoring, and hope.

In these stories, the turning point is often the same: a clinician who says, “Let’s look more closely at this,” and uses the right combination of imaging, lab work, and sometimes biopsy to name the disease and build a real plan.

Summary

If you’re dealing with salivary or lacrimal gland IgG4-RD, it can help to hear stories from others. What these stories share is that real life happens in the middle of appointments and lab tests, and you deserve care that takes your symptoms and your experience seriously.

Support is essential

One of the clearest lessons from patient and caregiver stories is that support is not optional, it’s part of treatment. Support can look like:

• a spouse or family member who helps you show up, track records, and get through hard stretches,

• a clinician who takes you seriously and keeps looking, and

• a community like IgG4ward! (even online) that reminds you: you are not alone. You’re not the only person with this condition.

Find out more about IgG4ward

References

1. NIH Genetic and Rare Diseases Information Center (GARD). IgG4-related dacryoadenitis and sialadenitis (formerly Mikulicz disease). rarediseases.info.nih.gov.

2. Puxeddu I, et al. Salivary Gland Pathology in IgG4-Related Disease. A Comprehensive Review. PubMed Central (PMC). 2018. https://pmc.ncbi.nlm.nih.gov/articles/PMC5901485/ 

3. Johns Hopkins Medicine. Sjögren’s Disease: Making the Correct Diagnosis. 2021.  https://www.hopkinsmedicine.org/news/articles/2021/12/sjogrens-disease-making-the-correct-diagnosis