Follow the Inflammation
Dr. Hannah Rosenblum was the physician in charge of the team caring for the patient in the hospital. As she entered his room, she was immediately struck by the man’s ears. They were bright red, and everything above the earlobes was hugely swollen. The rest of the patient’s exam was unremarkable. Even though he had what looked on the X-ray like a pretty bad pneumonia, he wasn’t having any problems breathing. Rosenblum was concerned about the wildly elevated inflammatory markers. Maybe it was an infection, but it seemed far more likely to be one of the many systemic inflammatory diseases. They are uncommon but potentially deadly — important not to overlook.
Rosenblum and her intern, Melissa Mariscal, went through everything they knew about the patient: He had chronic sinusitis and a pneumonia but no fevers or chills. The cartilage in his ears was red and swollen. There was blood in his urine. He had muscle pains that worsened with exertion but no evidence of muscle breakdown. And he had these incredibly high inflammatory markers. The fact that so many parts of his body were affected made a strong argument for some kind of rheumatologic disease. But which one?
As they spoke, the results from the patient’s CT scan came back. He had several masses (called granulomas) and some holes (called cavitations) in his lung tissue. Based on that finding, plus his history of sinusitis, the radiologist strongly suggested they consider the diagnosis of something known as Granulomatosis with polyangiitis (GPA). GPA is a disease of the small- and medium-size arteries in the body, which is a reason it can affect so many different parts. It is characterized by the development of granulomas — clumps of white cells and other tissues that form in reaction to infection or inflammation — in the lungs, in the upper respiratory tracts and in the kidneys. Untreated, the disease can kill, destroying the involved tissues.
Dialing Back the Immune System
Rosenblum and Mariscal immediately looked up GPA. Could it account for other parts of this patient’s presentation? The red ears: yes. The blood in the urine: yes. The muscle pains: yes. The chronic sinusitis: absolutely. The intern put in a referral to the rheumatology team to ask for help diagnosing this disease and treating it.
The rheumatology team recommended blood tests to look for evidence of GPA and rule out other possibilities. Treating GPA requires aggressive suppression of the inflammatory white blood cells, often for months at a time. Before starting this kind of treatment, they would have to make sure he didn’t have any hidden diseases, like tuberculosis, that could suddenly flare up if the white blood cells were taken out of action. He didn’t, and so when the test results came back positive, he was started on high-dose prednisone and a second immune-suppressing medication called rituximab. Treatment lasted months, but improvement started right away. As soon as he got his first dose of prednisone, he told me, he felt “like a million bucks.”
He’s still not much of a complainer. When I spoke to him, he told me he was 98 percent better. But his sister reports that he still tells her about his pain and stiffness at times, especially after sitting for a while. And episodes of congestion. “Does your doctor know?” she always asks him, and he promises to make the call. And, she tells me, maybe he does.