First Myositis Cancer Screening Recommendations Released

PHILADELPHIA – The first standardized screening guidelines for patients with idiopathic inflammatory myopathy (IIM) provide recommendations for individual risk stratification, basic and enhanced screening protocols, and screening frequency.

The recommendations, published by the International Myositis Assessment and Clinical Studies Group (IMACS), divide cancer risk for individual patients into low, moderate or high categories based on IIM disease subtype, autoantibody status and clinical characteristics, said Alexander Oldroyd, PhD. , MSc, MBChB from the University of Manchester, England.

“There is a huge unmet need for cancer research. One in four adults with myositis has cancer, either 3 years before or after the diagnosis of myositis. It’s one of the leading causes of death in these patients, and they’re overwhelmingly diagnosed late, so we need standardized methods for early diagnosis,” he said in an interview at the American College of Rheumatology’s annual meeting.

Sharon Kolasinski, MD, of the University of Pennsylvania in Philadelphia, said in an interview that the guidelines are a welcome development for rheumatologists. Kolasinski moderated the meeting where Oldroyd outlined the guidelines, but she did not participate in its formulation.

“I think we’ve all wondered for a very long time: What is the optimal cancer screening for myositis patients? We are all concerned that the onset of their illnesses is coincidentally related to cancer, or that they will develop it soon,” she said.

Oldroyd emphasized that all patients with myositis are at increased risk of developing cancer compared to the general population, and that the low, moderate, and high cutoff categories are only for patients with IIM.

International consensus

The data on which the recommendations are based come from a systematic review and meta-analysis by Oldroyd and colleagues of 69 studies of cancer risk factors and 9 of IIM-specific cancer screening.

The authors of this paper found that dermatitis subtype, older age, male sex, dysphagia, skin lesions, and transcription factor-1 gamma (anti-TIF1-gamma) positivity were associated with a significantly increased risk of cancer.

In contrast, polymyositis and clinically amyopathic dermatomyositis subtypes, Raynaud’s phenomenon, interstitial lung disease, very high serum levels of creatine kinase or lactate dehydrogenase, and positivity for anti-Jo1 or anti-EJ antibodies were associated with a significantly lower risk of cancer.

The consensus recommendations were developed with anonymous input from 75 expert participants in 22 countries, with additional input from 3 patient partners.

Do this

The guidelines list 18 recommendations, of which 13 are strong and 5 are conditional.

An example of a strong recommendation is number 3, based on moderate evidence:

“All adult IIM patients, regardless of cancer risk, should continue to participate in national/regional age- and sex-appropriate cancer screening programs,” the guidelines recommend.

However, patients with confirmed myositis or childhood-onset IIM do not need routine screening for myositis-related cancers, the guidelines state (recommendations 1 and 2).

It is also recommended that all adults with new-onset IIM be tested for myositis-specific and myositis-related autoantibodies to aid in risk stratification of patients.

The guideline divides screening recommendations into basic and enhanced. The basic screening should include a comprehensive history and physical examination, complete blood count, liver function tests, erythrocyte sedimentation rate/plasma viscosity, serum electrolytic protein, urinalysis, and chest X-ray.

Adults with IIM who are determined to be at low risk for IIM-related cancer should undergo basic cancer screening when IIM is diagnosed. Adults at intermediate risk should undergo both basic screening and enhanced screening at diagnosis of IIM, and those at high risk should undergo enhanced screening at diagnosis of myositis, with basic screening annually for 3 years, the recommendations say.

Consider doing this

Conditional recommendations (“clinicians should consider …”) include the use of PET/CT for adults at high risk for cancer when the underlying cancer has not been detected at the time of diagnosis of IIM. They also include one screening test for anti-TIF1-gamma positive dermatitis patients with disease onset after age 40 and who have at least one additional risk factor.

Also recommended are conditional upper and lower GI tracts for high-risk patients when an underlying cancer is not found at IIM diagnosis, nasoscopy in geographic areas with an increased risk of nasopharyngeal cancer, and screening for all IIM patients with red flag symptoms or clinical signs of cancer, including unexplained weight loss, family history of cancer, smoking, unexplained fever or night sweats.


“I think doctors have a lot of questions like, ‘Well, what should I do, when should I do it?’ These are important clinical questions and we need guidance on this. We need to balance overall efficiency and effectiveness, and we need expert opinion on which steps we should take now and which we should take later,” said Kolasinski.

The guideline development process was supported by the University of Manchester, IMACS, National Institute for Health Research (UK), National Institute of Health, National Health Service Northern Care Alliance, The Myositis Association, Myositis UK, University of Pittsburgh, Versus Arthritis, and Musculoskeletal Research Centre. Oldroyd and Kolasinski declared no relevant conflicts of interest.

This story originally appeared on, part of the Medscape Professional Network.

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