Cancer screening in scleroderma, dermatomyositis: What to do?
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Cancer screening for high-risk patients with scleroderma should involve basic age, sex and risk factor targeted screening, paying particular attention to mammography in women and prostate-specific antigen in men, according to Ami Shah, MD, of John Hopkins University, at the virtual ACR State-of-the-Art Clinical Symposium.
Further, screening for high-risk patients with dermatomyositis should follow a similar basic approach regarding age, sex and risk factor screening, but with a few exceptions. These include possible colonoscopy and prostate-specific antigen testing starting at age 40 years, particularly among African American patients, she said. Screening among high-risk women with dermatomyositis should include a pelvic exam and ultrasound, with mammography starting at age 40 years, or younger if the individual has a lump or a striking family history.
“We are seeing more patients with coexisting cancer and rheumatic disease, and we know that this shortens life expectancy for our patients, but also complicates the therapeutic options both for the rheumatic disease and for the cancer,” Shah told attendees of the virtual symposium. “In particular, a rheumatic disease may be a marker of an underlying cancer, and cancer could drive the development and propagation of the autoimmune disease. This raises an important question of whether cancer therapy is actually the right treatment choice for rheumatic disease in some patients.”
According to Shah, there is an elevated risk for cancer around the time of disease onset in both scleroderma and dermatomyositis, with the highest risks seen among distinct autoantibody subsets. In patients with scleroderma, high-risk cancer subgroups include those who are positive for anti-RNA polymerase III or anti-RNPC3, as well those who are negative for centromere topoisomerase 1 and RNA polymerase III, which Shah referred to as “CTP-negatives.”
Patients with anti-RNA polymerase III may be recognized through an explosive onset of skin thickening, Shah said. Further, these patients may not demonstrate Raynaud’s phenomenon at disease onset, but develop it 1 to 2 years later. They are also at higher risk for renal crisis, myopathy and gastric antral vascular ectasia.
Among those with dermatomyositis, high-risk subgroups include those with anti-TIF1 gamma and anti-NXP2, with evidence suggesting that 83% of patients with cancer-associated forms of the disease can be identified through these two antibodies, according to Shah.
Other “red flags” for cancer among patients with either disease include older age at disease onset, aggressive or atypical disease, lack of response to treatment, profound weight loss or constitutional symptoms that are disproportional to disease severity, and personal or striking family history.
When screening for cancer, Shah stressed that there are two important caveats to remember. First, there are currently no evidence-based recommendations for how to screen patients for cancer. Second, there are still no rigorous data suggesting that early cancer diagnosis will improve outcomes.
However, in her own practice, Shah said she follows a basic approach of performing a comprehensive history and physical exam, including traditional risk factors and family history. This also included a breast exam and a referral to a gynecologist for a pelvic exam, or a testicular exam for men older than 40 years. Other recommendations she had for high-risk patients with scleroderma included an ear-nose-throat exam if the patient has globus sensation or unexplained oropharyngeal dysphagia.
“Further research is underway looking at the role of PET/CT and breast MRI in this patient population, and hopefully we will have more data soon to lay the foundation for evidence-based cancer screening recommendations,” Shah said.
In patients with dermatomyositis, Shah also suggested that CT of the chest, abdomen and pelvis would be reasonable. In addition, a PET/CT could be considered in place of a conventional screening, with the caveat that mammography and colonoscopy may still be required, she added.
“A PET/CT may especially be reasonable [if the patient] has treatment refractory disease,” Shah said. “With respect to tumor markers, we do not retain these routinely in our practice, in part because these are nonspecific and may correlate with features of the rheumatic disease itself. Also, it is reasonable to continue annual routine screening measures, with the question of whether there is a role for CT scanning, again, at year 1 and year 2.” – by Jason Laday
Reference:
Shah, A. Cancer screening in rheumatic diseases. Presented at: American College of Rheumatology State-of-the-Art Clinical Symposium. May 16-17, 2020 (virtual meeting).
Disclosures: Shah reports no relevant financial disclosures.