What’s Wrong With Me?
A medical mystery resolved.
THE PATIENT: Jiang, a 30-year-old kindergarten teacher in Toronto THE SYMPTOMS: Back pain and low-grade fever THE DOCTOR: Dr. Khalil Sivjee, medical director for the Cleveland Clinic Canada
IN OCTOBER 2015, Jiang started to develop a sore back, which she at first attributed to long days sitting on the carpet with her small pupils. However, when the unusual pain persisted for two weeks, she went to her family doctor.
Jiang’s physician prescribed overthe-counter pain medication and muscle relaxants. Although her back didn’t improve, it didn’t get worse, either, so Jiang endured the discomfort. But by December, she became concerned again when she came down with a low-grade fever, lost her appetite and was losing weight.
This time, her doctor sent her to Sunnybrook Health Sciences Centre for a CT scan, which showed lesions on her spine and small nodules on the bottom of her lungs.
The physicians suspected she had either contracted tuberculosis (TB)— Jiang had recently visited extended family in China—or had a metastatic cancer of unknown origin. “Whenever you see stuff all over the body, you tend to wonder if the primary cause is cancer,” says Dr. Khalil
Sivjee, who, as the hospital’s head of respirology at the time, was asked to consult on the case.
Sivjee ordered a full CT scan of Jiang’s lungs, which revealed that the nodules were present throughout. It also showed enlarged mediastinal lymph nodes—glands located between the sternum and spinal column. Since all these symptoms were consistent with TB, Jiang was placed
in isolation and put on several drugs that target that disease.
Meanwhile, in order to determine which TB bacteria Jiang had been infected with, Sivjee performed a bronchoscopy and lung biopsy to remove fluid samples for testing. Jiang was allowed to go home while she awaited the results but couldn’t return to work, as she might infect others. After almost four weeks, the lab tests came back negative for TB, but the biopsy revealed granulomas (small collections of inflammatory cells) in her tissue samples.
As granulomas can be a sign of TB, Jiang remained on the medication, but Sivjee wasn’t convinced this was the answer—not only due to the test results but because his patient wasn’t responding to the treatment. He took a biopsy of her spine and discovered that the cells at the centre of her granulomas were alive, which is inconsistent with TB.
Sivjee suspected sarcoidosis, an immune system disorder that often manifests in the lungs and affects about 10 to 20 people out of every 100,000. He checked Jiang’s angiotensin-converting enzymes, which control blood pressure, and found that they were elevated, a nearly definitive sign of the condition.
“What happened next required a leap of faith,” Sivjee says. The only treatment for sarcoidosis is steroids, but if Jiang was actually suffering from TB or another immune disorder, steroids could be fatal. Both doctor and patient agreed that the risk was worth taking.
Thankfully, Sivjee’s conclusion was correct, and Jiang showed immediate improvement. Her fever disappeared and within two weeks her appetite was back to normal. Four weeks after the treatment, the nodules were gone; within three months, she was pain-free and back in her classroom.
A biopsy showed that the cells at the centre of Jiang’s granulomas were alive.
Although she felt normal again, Jiang remained on steroids for a year. “The problem with sarcoidosis is that it’s a systemic disease and we don’t know what causes it,” Sivjee says. “It can be very aggressive and can invade several organs, including the heart, which can be fatal. And it can come back.”
He says there are no known lifestyle changes or long-term medications to prevent a relapse. “Sarcoidosis is an interesting, although little understood, condition, and there aren’t many new therapies being studied for treating it,” Sivjee says. “Fortunately this patient has done very well.”