"The greatest enemy of knowledge is the illusion of knowledge." – Stephen Hawking
In my fellowship, I had a challenging RA case. This woman was in her 50s, and usually she was quite dynamic, personable, and spunky. But the disease was wearing her down. She developed methotrexate pneumonitis. Leflunomide gave her severe diarrhea. She got frequent upper respiratory tract infections from etanercept. We could not get her prednisone dose lower than 15 mg/day. Whenever we saw her in clinic and she had encountered a new glitch in her treatment, my preceptor would say to her "you keep me up at night." Disturbed to hear this, I wondered how insecure I would be as a newly minted rheumatologist if my preceptor, with her decades of practice, still had uncertainties.
I feel like in these, the first 3 years of practice after training, I have an inordinate number of patients who keep me up at night. They come in all shapes and sizes. Even cases that I think are straightforward frequently end up being anything but.
I like treating patients with polymyalgia rheumatica, for example, because the relief they get from a little prednisone is so dramatic and immediate. Seems straightforward, no? But I get anxious about the diagnosis sometimes – what if I’m missing a paraneoplastic syndrome? Not infrequently there are patients who absolutely refuse to go on prednisone, and I worry about them, too. Or I get anxious about tapering, because I’ve had it happen often enough that their symptoms recur at 3 mg of prednisone and I need to put them on a DMARD, none of which have any conclusive evidence of efficacy.
Rheumatoid arthritis is not always easy, either. I have had my share of refractory cases, which, in this age of different varieties of biologics, is even more frustrating. (Although, for the life of me, I cannot imagine what it must have been like to practice before the age of biologics!) It’s even worse for refractory psoriatic arthritis, for which there are fewer drug options available.
And what about scleroderma? I watched a patient’s hands progress from being simply puffy at presentation to being contracted, with severe skin tightening and skin ulcerations over the PIP joints, in just 2 years. We are lucky to be close enough to Boston that I could send the patient to a medical center there to receive an investigational therapy, which seemed to lessen the patient’s symptoms somewhat. Unfortunately, she developed urinary bladder cancer and is now ineligible to receive the experimental anti-TGF-beta in an open label extension.
But perhaps my most insomnia-inducing patient is a lovely 70-year-old man with a new diagnosis of dermatomyositis. His antinuclear antibody was negative, and he had no myositis-specific antibodies. We knew we should be looking for a malignancy, but a CT of the chest, abdomen, and pelvis was negative. A colonoscopy was next, but if that did not turn anything up, what would be next? Do we keep searching? How do we know where to look?
Worse, the pulse of methylprednisolone did not work completely, and he ended up needing a percutaneous endoscopic gastrostomy tube because he failed his swallow evaluation miserably. I was really saddened by all this. It made me feel small and insignificant in the face of such a terrible disease.
In that process of placing a PEG tube, we serendipitously found, on biopsy, adenocarcinoma at the gastroesophageal junction. Though this was, indeed, terrible news, it was a source of comfort for me that we had found the malignancy and would at least have a target for treatment.
I know he will not be my last dermatomyositis patient, and there are no prescribed guidelines for searching for a malignancy. How can we possibly find something if we have no idea what it is or where it might be?
I can think of so many more lupus, spondyloarthritis, temporal arteritis, even mechanical back pain patients who worry me, to say nothing of the not-insignificant number of patients for whom the diagnosis is uncertain. True, my learning curve has been steep in these first 3 years, but the more patients I see, the more striking the large number of phenotypes of our diseases. It’s quite humbling and has kept me up more nights than I expected.
Dr. Chan practices rheumatology in Pawtucket, R.I. E-mail her at rhnews@elsevier.com.