Cancer screening recommendations in autoimmune disease rely on experience in lieu of data
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Cancer screening recommendations for patients with autoimmune diseases largely rely on clinical experience and extrapolation in the absence of abundant data, according to a speaker at the 2021 Congress of Clinical Rheumatology-East meeting.
“Not all patients with a particular rheumatic disease have the same risk for cancer,” Laura C. Cappelli, MD, MHS, MS, assistant professor of medicine in the division of rheumatology at Johns Hopkins University School of Medicine, told attendees at the meeting. “Unfortunately, there is limited evidence on how to use this information clinically in terms of screening because there have been so few perspective studies looking at screening for cancer in these patients.
“There are a couple perspective studies on dermatomyositis, one comparing PET scans versus very intensive cancer screening, but other than that there isn’t much in the literature,” she added. “Recommendations now are based on clinical experience and sort of extrapolating from what is in the literature. When we go through these recommendations, remember these are not set in stone, but based on the available literature and the clinical experience of those of us who take care of these patients.”
Citing her own 2020 review, penned alongside coauthor Ami A. Shah, MD, also of Johns Hopkins University, and published in Best Practice & Research Clinical Rheumatology, Cappelli detailed the cancer risk factors and suggested screening procedures for several autoimmune diseases, including myositis, rheumatoid arthritis and lupus.
Myositis
Primary risk factors include the presence of the anti-TIF1gamma and anti-NXP-2 autoantibodies, as well as recent diagnosis. According to Cappelli, the highest window of risk is within 3 to 5 years of diagnosis.
For screening these patients for cancer, the typical practice at Johns Hopkins is to consider chest/abdomen/pelvis CT at the time of myositis diagnosis. For women, and particularly those with anti-TIF1gamma autoantibodies, pelvic ultrasound can also be used to detect ovarian or other obstetric/gynecologic cancers.
These procedures are less common 3 to 5 years after diagnosis, as the risk for cancer decreases following that time period, Cappelli said.
Rheumatoid arthritis
According to Cappelli, several studies have suggested that high levels of disease activity, or uncontrolled disease, are associated with an increased cancer risk. History of smoking is also linked to an increase for cancer in patients with RA.
Providers should consider chest CT in patients with RA who have an extensive smoking history, Cappelli said. In addition, monitoring the patient’s complete blood count with differential may be helpful given the risk for hematologic malignancy. Providers should also consider LDH levels if the patients demonstrate B symptoms.
Giant cell arteritis/Polymyalgia rheumatica
The only cancer risk factor that has been described in patients with GCA or polymyalgia rheumatica is recent diagnosis, Cappelli said.
As these patients tend to be older, providers should consider updating all age- and sex-appropriate cancer screenings at the time of new diagnosis, she added.
Scleroderma
In patients with scleroderma, the presence of the anti-RNA Pol III and anti-RNPC-3 autoantibodies are associated with an increased risk for cancer, according to Cappelli.
For those with these autoantibodies and new onset disease within the last 3 years, providers should consider chest/abdomen/pelvis CT, mammography and prostate exam where indicated, she said.
Patients with upper ear-nose-throat (ENT) symptoms can also have an increased risk for cancer of the base of the tongue or other ENT malignancies, and may require specific examinations in those areas.
Sjögren’s syndrome
Cancer risk factors in patients with Sjögren’s syndrome include high levels of disease activity, the presence of cryoglobulins and cytopenia, and low complement levels. According to Cappelli, these patients should receive regular complete blood count monitoring with differential, with LDH assessment considered if B symptoms appear. In addition, providers should test for cryoglobulins and complement levels at the time of diagnosis. Ultrasound of the parotid glands may identify a MALT lymphoma if the glands are enlarged, irregular or not improving with treatment, she added.
Systemic lupus erythematosus
Patients with lupus tend to demonstrate malignancies early in their disease course, Cappelli said. Immunosuppression also increases the risk for cervical intraepithelial neoplasia.
For screening, providers should consider regular monitoring of the complete blood count with differential, along with LDH should B symptoms develop. Patients should also complete regular visits with a gynecologist for examination due to the risk for vulvar and cervical cancer. Annual pap smears to monitor for cervical cancer in patients using immunosuppressive drugs should also be considered.
Vasculitis
Published literature suggests that cumulative cyclophosphamide dose is associated with bladder cancer and hematologic malignancy. However, “this is less of an issue now,” Cappelli said.
For older patients who received high levels of cyclophosphamide in the past few decades, Cappelli suggested regular urinalysis to monitor for microscopic hematuria, as well as assessing complete blood count with differential.
Talk to your patients
“So, what do we do with all this information?” Cappelli said.
According to Cappelli, it is important for rheumatologists and other providers to maintain an open line of communication with patients, regarding any past cancers as well as the risk for future malignancies.
“I try to make it relatively simple, in that I tell them that in several kinds of immunosuppression, we know that nonmelanoma skin cancer is increased, and I give them some examples,” she said. “Now, what can you do? Well, the No. 1 thing you can do is sun protection — staying in the shade, wearing sun-protective clothing, using sunblock that is at least SPF 50, having a dermatologist and having yearly skin checks.
“I say also that there is no convincing risk for incident solid tumors, lymphoma or melanoma,” Cappelli added. “We have the most data for TNF inhibitors and rituximab, but so far this is what we know. And when we get into this discussion further, I also point out that, in some kinds of rheumatic disease, actually having increased disease activity or undertreated disease is associated with developing cancer. That can be helpful for a patient who has bad rheumatic disease and is scared, which is very understandable, because they perceive an increased cancer risk with these medications.”
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Cappelli LC. Autoimmune Disease and Cancer: Many Intersections. Presented at: Congress of Clinical Rheumatology-East annual symposium; August 12-15, 2021 (hybrid meeting).
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