Medical mystery: Her flaming ears foretold a diagnosis that terrified her

In 2012 Grace Brenner, then a second-year law student at American University, was in a physical therapy session trying to ease long-standing neck pain when she felt a sudden, crushing sensation in her chest. The right side where her ribs and sternum met were intensely painful, red and swollen.

“It hurt so much I wondered, ‘Is this a heart attack?’” she recalled.

The therapist summoned the physical medicine specialist who had been treating Brenner, now 33. After taking her vital signs and ruling out a heart attack, he told her he suspected costochondritis, a common, benign condition that causes an inflammation of cartilage and mimics the symptoms of a heart attack. A similar condition, Tietze syndrome, involves swelling in the affected area.

For several years, Brenner said, her rib cage felt so tender that she was unable to wear a bra. Prescription strength anti-inflammatory drugs enabled her to function. “I had to be very careful about lifting anything,” she said.

Each time she cut back on the medication, the pain would flare.

In 2014, after Brenner developed painful swelling in her right hip, the physical medicine specialist told her he suspected lupus, an autoimmune disease that causes inflammation and can affect multiple organ systems, including the skin, joints and heart. He referred her to a rheumatologist.

Autoimmune diseases, in which the body attacks its own tissues and organs, can run in families. Brenner’s was no exception: Both her grandfathers had type 1 diabetes.

On the basis of her history and the results of blood tests, the rheumatologist concluded that she did have lupus, which can wax and wane unpredictably. .

Brenner began taking hydroxychloroquine, an anti-malaria drug that is a mainstay treatment for autoimmune diseases. Within months, her health improved.

By 2018, Brenner said, “I was feeling pretty good.” The rib pain had finally disappeared and her lupus seemed to be under control. Brenner, who was seeing a new rheumatologist, stopped taking the hydroxychloroquine and prescription anti-inflammatory drugs.

“I thought, ‘Maybe I’m getting my life back,’” she recalled.

The respite lasted about a year.

One morning in April 2019, Brenner woke up with a slight nosebleed and a red, swollen nose. “It was a little hard to breathe,” she said. She saw an otolaryngologist, who was puzzled that her nose didn’t appear to be broken and that whatever was wrong seemed to be affecting only cartilage. The doctor advised her to return if her nose looked crooked after the swelling diminished. Two weeks later, her nose looked and felt normal.

Two months later, Brenner pulled out her phone and began hunting for images that resembled her latest weird symptom: beet red ears that were hot to the touch.

What she found left her feeling badly shaken.

Brenner tried to tamp down the rising panic she felt about what popped up on her phone: pictures of people with relapsing polychondritis (RP), a serious, progressive disease in which the body attacks its own cartilage.

The disease, also known as polychondritis, is believed to be autoimmune; it typically targets the ears, which can become floppy, and the nose, which can result in collapsed cartilage requiring reconstructive surgery. In severe cases, the heart, blood vessels or airways can be affected, causing damage that may be fatal.

Polychondritis strikes more women than men, typically between ages 20 and 60; it is estimated to affect about 3 in a million people. There is no cure, nor is there a specific diagnostic test; diagnosis is made on the basis of symptoms. Treatment is focused on alleviating attacks, preventing flares, and preserving cartilage. As is the case with lupus, RP patients often see multiple specialists to manage various manifestations of their disease.

When Brenner saw Maryland rheumatologist David Wolfe, she recounted the swollen nose episode and showed him pictures of her inflamed ears. Wolfe subsequently confirmed what she feared: She had the hallmark symptoms of RP, along with lupus. It is not uncommon for people with polychondritis to have more than one autoimmune disease, he said.

“It’s sort of like a small fire,” Wolfe said of the initial signs of polychondritis. “It could progress to a bigger fire if you don’t put it out.”

Wolfe recommended that Brenner begin taking methotrexate, a drug that suppresses the immune system and is used to treat some cancers and autoimmune diseases.

Brenner balked. “I did the exact opposite of what you should do, which is nothing. I was scared of the side effects [of the drug]. Basically, it was denial."

But after a month of fevers, Brenner agreed to take a medication she thought might have fewer side effects. She also joined an online support group, which she found to be both terrifying and helpful.

Weeks later, she wound up in a Northern Virginia hospital in the throes of a hypertensive crisis: Her blood pressure had skyrocketed to 180/120 mg and her heart rate was 200, double the upper limit of normal. She was diagnosed with hypertension and sinus tachycardia, an elevated heart rate.

She began seeing a cardiologist and was prescribed medicines to lower her heart rate and blood pressure. She also began taking methotrexate, which has worked well in controlling her lupus and RP.

Wolfe said that her hypertension, which first surfaced in law school, as well as her elevated heart rate, appear to be unconnected to her autoimmune diseases and are not typically associated with them.

So far, she shows no sign of the potentially serious lung or eye problems that can result from polychondritis, Wolfe said. “Assuming methotrexate continues to work, I assume she’ll have an excellent prognosis,” he noted.

It is unclear when Brenner developed RP. An inflamed nose or ears are usually the first indicators. It is possible but unlikely, Wolfe said, that costochondritis was a harbinger.

“RP can manifest in subtle ways,” he said. “I can’t rule it out.”

Brenner said she tries not to worry about what might happen if her disease becomes more severe or if methotrexate stops working, although there are other medications she can try.

She now lives with her parents, who moved to the Washington area several years ago. They are in their 70s, and Brenner helps to manage their health care. But having to level with them about the uncertainties surrounding her own health has been difficult.

“They’ve handled it as well as someone can,” she said.