Growing up, Ana Vieira accepted her myriad health problems as her own personal “normal.”
“I was a fragile child, always feeling unwell,” says the 49-year-old, who lives in Portugal. “I had recurrent nosebleeds and acute parotitis with high fevers, joint and muscle pain and unexplained weight loss.” As if those symptoms weren’t alarming enough, at age 13, her teeth started massively decaying. Although her dentist was able to rebuild her decayed teeth, Vieira still had issues smiling. “I didn’t want people to see my teeth,” she says.
A few years later, she became even more self-conscious when she developed Bell’s palsy, a type of facial paralysis that left her struggling to speak and chew. This was a result of another bout of parotitis with a fever, which kept her in bed for more than two weeks. Although the paralysis improved after six months, she was left with permanent damage. “I still have facial asymmetry—you can see it in my lips when I speak or smile,” she says.
But what no one knew at the time—not even the doctors who examined Vieira—was that these were all signs of Sjögren’s (“SHOW-grins”) disease, an autoimmune condition in which the immune system damages the glands that produce and control moisture in the body.
Vieira was diagnosed with Sjögren’s disease at 32 years old (20 years after her first symptoms appeared), but she wasn’t getting proper treatment because her doctor was unfamiliar with the condition. It was another 10 years before she reached out to a patient advocacy group and was connected with a team who understood Sjögren’s disease. There, Vieira got proper care and adequate treatments to slow down the progression of the disease. “I was able to have a better quality of life,” she says.
Vieira’s delayed diagnosis is far from unusual among people living with Sjögren’s disease. The autoimmune condition affects up to 4 million globally, and yet because some Sjögren’s disease symptoms resemble other illnesses, it can take years for people to get properly diagnosed.
Vieira’s story illustrates the need for better awareness and targeted treatments to help manage symptoms effectively. “There is no cure for Sjögren’s disease,” says Federico Zazzetti, M.D., Global Medical Affairs Lead and Director of Rheumatology, Johnson & Johnson Innovative Medicine. “Doctors are working to gain a better understanding of the disease and its outcomes to advance in the discovery of novel targeted therapies.”
To that end, Johnson & Johnson is working on researching and developing potential treatments to target symptoms of Sjögren’s disease. Here are five things we now know about Sjögren’s disease.
1.
Although the disease itself was identified nearly a century ago, it remains difficult to diagnose. In the U.S., it takes almost three years, on average, for a person to be properly diagnosed.
Sjögren’s disease isn’t straightforward, as its symptoms overlap with other diseases, explains Dr. Zazzetti. “Diagnosis is often based on classic symptoms like dry mouth, dry eyes, fatigue and joint pain. It’s extra challenging because in addition to autoimmune conditions such as rheumatoid arthritis, lupus and fibromyalgia, other diseases like cancer can have similar symptoms.”
Looking back, Vieira can see the through line of inflammation across all her symptoms from childhood to adulthood. But at the time, they seemed disparate. This is not uncommon for people living with Sjögren’s disease, says Dr. Zazzetti. “Often, the process of diagnosing can involve a lot of doctor visits to different specialists and diagnostic tests to rule out other medical conditions.” This may include viral infections, diabetes, other autoimmune diseases and even some types of cancer.
2.
Nine out of 10 people living with Sjögren’s disease are women, the majority of whom are diagnosed around age 40. While scientists aren’t sure why women are at greater risk, some research suggests that the decline in estrogen levels during menopause may play a role in increasing the production of autoantibodies (antibodies that mistakenly attack your body’s proteins, cells, tissues and organs).
Sjögren’s disease can also occur in men and children. Notably, children are less likely to have dry eyes and dry mouth, the symptoms most commonly associated with the disease—a fact that mirrors Vieira’s own experience with symptoms early on.
It’s also possible that those living with Sjögren’s disease will develop other autoantibody-driven diseases such as lupus and rheumatoid arthritis (RA). “While autoimmune diseases aren’t genetic, we do know some genes put a person at high risk of developing autoimmunity,” Dr. Zazzetti says.
Different environmental factors could trigger autoimmunity in a person predisposed to developing an autoimmune disease (this can include viral infections, exposure to ultraviolet light and chronic or acute instances of stress). For example, someone might have autoimmune hepatitis, but another family member might have systemic lupus erythematosus or Sjögren’s disease. “When a patient has one autoimmune disease, you need to look for other potential associated ones,” he adds.
3.
“Sjögren’s disease has glandular manifestations,” says Dr. Zazzetti. The autoimmune system attacks the moisture-secreting glands and mucous membranes, resulting in dryness wherever the body naturally self-lubricates, such as the mouth, eyes and even skin. The lack of moisture can lead to problems like eye infections, tooth decay and recurring nosebleeds, he adds.
In Vieira’s case, her childhood tooth decay was likely a result of decreased saliva (though at the time she says she didn’t have any dry mouth complaints). Likewise, her nosebleeds were likely due to decreased function in the mucous membranes of her nasal passages.
Symptoms of Sjögren’s disease are often different from patient to patient. While dry mouth and dry eyes are the most common symptoms, fatigue and joint pain can also persist. Other symptoms include “brain fog” and neurological problems, interstitial lung disease, interstitial cystitis and difficulty swallowing. “Sjögren’s is a systemic disease,” explains Dr. Zazzetti. “It affects multiple organs and tissues throughout the body, including the joints, lungs, kidneys, nervous system and blood vessels.”
Vieira also experienced symptoms such as migraines and extreme fatigue—the result of “chronic inflammation,” according to Dr. Zazzetti. “By the time I was in college, I had recurrent migraines that were so severe I took anti-inflammatory drugs on a daily basis, which affected my liver,” Vieira says. Eventually, as she took fewer anti-inflammatory drugs, her liver function returned to normal.
4.
Currently, topical treatments—like artificial tears, gels and saliva substitutes—are the first line of therapy for mild cases of Sjögren’s disease. However, these only help alleviate symptoms of dryness and not the disease itself, says Dr. Zazzetti. Eye treatments may include eye drops, ointments or tear plugs to keep the eyes moist. To alleviate dry mouth, doctors may recommend saliva stimulators and antifungal medications to prevent infections such as candidiasis (yeast infection).
For moderate to severe cases like Vieira’s, medications for cancer or for other autoimmune diseases are often used off-label to help manage symptoms and try to avoid disease progression. Corticosteroids are another possible treatment, but if used long-term, they can cause severe side effects, such as infection, osteoporosis and an increased risk of cardiovascular disease and type 2 diabetes. “The goal is to use the lowest dose possible for the shortest amount of time in very specific organic manifestations,” says Dr. Zazzetti.
Treating the underlying causes of the disease is crucial because it addresses the root of the problem and helps control symptoms throughout the body, Dr. Zazzetti says. “Sjögren’s disease can lead to serious long-term complications like lymphoma—and treating the cause may prevent or delay these complications,” he explains.
5.
Scientists are looking into other avenues of research to gain a deeper understanding of the disease and how it moves through the body. “In the next three to five years, we could potentially see different drugs to treat moderate to severe Sjögren’s disease,” Dr. Zazzetti says.
Currently, Johnson & Johnson is researching and developing new molecules that are believed to help target and reduce the pathogenic autoantibodies that cause symptoms in individuals living with Sjögren’s disease, rather than treating symptoms as they appear.
“We aim to intervene in the underlying mechanisms that cause Sjögren’s,” Dr. Zazzetti says. “[Sjögren’s disease] is thought to begin with an unknown environmental trigger. The immune system responds, autoimmunity develops and the body releases autoantibodies, leading to chronic inflammation—which affects salivary glands and lacrimal glands (located in the upper eyelids) and extends to other organs and systems. By targeting pathogenic autoantibodies, we hope to avoid the development of disease symptoms.”
Dr. Zazzetti says researchers are exploring ways to help target immune pathways involved in Sjögren’s disease—for example, creating monoclonal antibodies (lab-created proteins that mimic natural antibodies) to fight white blood cells and cytokines (proteins that help manage inflammation in the body). “There is also a focus on understanding genetic and molecular factors that may lead to more personalized treatment plans,” he says, “plus research on biomarkers that could help predict disease severity in individuals and guide therapeutic choices.”
Vieira, who has become a vocal proponent of patient self-advocacy, is optimistic about the future—for both herself and other people living with Sjögren’s disease. “There’s a growing number of clinical trials, and researchers increasingly want to learn from individual experience with the disease,” she says. “Useful and accessible treatment that can help improve quality of life will be a game changer.”
