Recognizing the Unrecognized: Advancing Research in Childhood Sjögren’s Disease

An Interview with Dr. Akaluck “Ben” Thatayatikom

Sjögren’s disease has been recognized in adults for almost a century, first described by Swedish ophthalmologist Henrik Sjögren in the early 20th century. The autoimmune condition is most commonly associated with dry eyes and dry mouth in middle-aged women. Yet in children, the disease often looks very different—and for decades, it was rarely discussed in pediatric medicine.

According to Dr. Akaluck “Ben” Thatayatikom, pediatric rheumatologist at AdventHealth, this lack of awareness has led to years of missed or delayed diagnoses.

“When I was a fellow around the year 2000, there was no discussion of pediatric Sjögren’s disease in any rheumatolgy meetings,” Dr. Thatayatikom recalls. “There wasn’t even a chapter on it. That means we almost certainly missed many cases.”

Today, his work is helping to change that.

A Disease That Looks Different in Children

One of the biggest challenges in recognizing Sjögren’s disease in children is that the symptoms often differ from those seen in adults.

“Adults typically present with dry eyes and dry mouth,” Dr. Thatayatikom explains. “But in children, those symptoms are often absent or not noticeable early on.”

Instead, children may experience a wide range of symptoms affecting multiple organs. Younger children often develop recurrent swelling of the salivary glands—known as parotitis—while older children and adolescents may present with arthritis, unexplained fatigue, skin rashes, abdominal symptoms, or nerve pain.

Because these symptoms can resemble many other conditions, children may see multiple specialists before the disease is recognized.

“These patients may visit neurologists for pain, dermatologists for rashes, gastroenterologists for abdominal symptoms, or cardiologists for fainting episodes,” he says. “It’s a systemic autoimmune disease that can affect many organs, which makes it difficult to recognize.”

The Case That Sparked a Research Path

Dr. Thatayatikom’s focus on pediatric Sjögren’s disease began with a patient whose symptoms had puzzled multiple physicians.

The child had experienced recurrent salivary gland swelling beginning at age 2 and had been evaluated by several specialists. Even a pediatric rheumatologist initially ruled out a rheumatic disease.

Eventually, the patient was referred to Dr. Thatayatikom at the University of Florida.

Working with an oral medicine specialist, Dr. Thatayatikom evaluated the child’s salivary gland function and found that they were not producing normal saliva. The findings pointed toward Sjögren’s disease.

“That case opened my eyes,” he says. “We realized that many children with recurrent salivary gland swelling might actually have Sjögren’s disease.”

Since then, he has followed that patient for nearly a decade—and identified many more children with similar presentations.

Why Diagnosis Is So Difficult

Several factors make Sjögren’s disease particularly difficult to diagnose in pediatric patients.

First, the disease can present in many different ways depending on the child’s age and the organs involved.

Second, there are currently no established diagnostic criteria specifically for children. Physicians must rely largely on adult criteria, which emphasize symptoms like dry eyes and dry mouth—features that may not appear early in pediatric patients.

“Using adult criteria means we will miss many children,” Dr. Thatayatikom explains.

Laboratory testing presents another challenge. The antibodies commonly used to diagnose Sjögren’s disease in adults are found in fewer than half of pediatric cases.

“If the antibody test is negative, many physicians assume it’s not Sjögren’s,” he says. “But that is not true in children.”

Because of these limitations, diagnosis often requires a combination of clinical evaluation, imaging, and specialized testing. At AdventHealth, Dr. Thatayatikom routinely performs salivary gland ultrasound, a non-invasive technique that can help identify early gland changes.

In some cases, physicians may also perform a minor salivary gland biopsy from the lip to confirm the diagnosis.

The Impact of Delayed Diagnosis

Research published by Dr. Thatayatikom and colleagues shows that many children experience symptoms for one to five years before receiving a diagnosis.

For patients and families, this delay can significantly affect quality of life.

In the short term, symptoms such as gland swelling, arthritis, and fatigue can disrupt daily activities and school attendance.

“It affects the entire family,” Dr. Thatayatikom says. “When children are sick and cannot attend school, parents often miss work as well.”

The long-term consequences may be even more serious. Over time, chronic inflammation can lead to permanent organ damage.

In adults, long-standing Sjögren’s disease can affect multiple organ systems—including the kidneys, pancreas, nerves, and glands throughout the body.

Early recognition and treatment may help prevent some of these complications.

Encouragingly, Dr. Thatayatikom’s research suggests that children may respond particularly well to early intervention.

“In adults, gland damage is often permanent,” he explains. “But in children, we sometimes see improvements after treatment. Changes seen on ultrasound can return toward normal, which suggests that early treatment may reverse some of the damage.”

Building a Global Research Effort

To better understand pediatric Sjögren’s disease, Dr. Thatayatikom participates in an international research collaboration studying children with the condition.

The global registry has grown rapidly in recent years. While the initial study included 70 patients, the cohort has now expanded to approximately 150 cases worldwide.

Remarkably, AdventHealth has contributed a large proportion of those patients.

“We have contributed about 30 percent of the cases in this international study,” Dr. Thatayatikom says.

His team is also collaborating with researchers at the University of Oklahoma to investigate the genetic factors that may contribute to the disease in children.

These studies aim to identify biological pathways that could lead to improved diagnostic tools and targeted therapies in the future.

“When you study something that is not well understood, every discovery can lead to new insights,” he says.

Raising Awareness Across Specialties

Because Sjögren’s disease can affect many organs, Dr. Thatayatikom emphasizes the importance of awareness across multiple medical specialties.

Patients may first present to dentists, oral medicine specialists, ophthalmologists, dermatologists, cardiologists, gastroenterologists, hematologists, nephrlogists or neurologists before reaching a rheumatologist.

“Education is critical,” he says. “As we collect more data and share what we learn, more physicians will recognize the disease earlier.”

Looking Ahead

Dr. Thatayatikom hopes that future research will lead to simpler diagnostic tools—such as biomarkers that could detect the disease earlier.

“If we had a simple test that could identify Sjögren’s disease early, that would change everything,” he says.

His long-term vision also includes establishing a dedicated pediatric Sjögren’s clinic at AdventHealth, where patients could receive specialized care while supporting research and education for future physicians.

Although the disease is still classified as rare, Dr. Thatayatikom believes it may be more common than previously thought.

“It may not truly be rare,” he says. “It may simply be unrecognized.”