Extrapulmonary manifestations of systemic sclerosis, difficult to treat RA and myositis clinical pearls were among the highlights from Day 1.
Our early career and trainee rheumatologists Dr Sadia Islam, Dr Maxine Isbel and Dr Bonnia Liu share their insights from Day 1 of ACR22.
Extrapulmonary manifestations of systemic sclerosis – by Dr Sadia Islam
Australia’s own Dr Laura Ross opened up the session on Extrapulmonary manifestations of systemic sclerosis with an insightful overview of cardiac involvement.
Dr Ross highlighted that “subclinical” cardiac disease is a near universal finding. Notably, patients who already have abnormal TTE parameters carry a poorer prognosis.
A study by Dr Ross and colleagues in patients with normal resting cardiac function found that all but one patient had diffuse myocardial fibrosis on cardiac MRI, and the majority had myocardial oedema. Approximately half had clinically significant reduced exercise capacity on cardiopulmonary exercise testing; a powerful predictor of mortality. Increased risk of ischaemic heart disease was also highlighted.
The mechanism of ischaemic heart disease is poorly understood, however may be related to microvascular pathology or cardiac Raynaud’s. Nevertheless, the assessment of underlying “subclinical” cardiac disease, management and preventative measures remains an area where more work is required.
Dr Michael Hughes and Dr Rishi Naik continued the session with gastrointestinal manifestations in systemic sclerosis, where up to 90% of patients experience oesophageal dysfunction.
Dr Naik introduced a number of advanced tools used by gastroenterologists to assess for oesophageal involvement. Dr Hughes discussed the central role of proton-pump inhibitors, though acknowledged safety concerns regarding long-term use that may be particularly relevant in patients with systemic sclerosis, such as worsening calcinosis, bone health and bacterial overgrowth.
Though there is little evidence to support empiric use of PPIs, there may be a role for empiric therapy in patients with SSc-ILD due to potential silent microaspiration.
12S146. Extrapulmonary Manifestations of Systemic Sclerosis: The Heart and Esophagus
Dr Sadia Islam is a final year rheumatology advanced trainee at Royal Prince Alfred Hospital in Sydney.
Difficult to treat RA – by Dr Maxine Isbel
Defeatism is not a philosophy that this session subscribes to.
Despite ongoing debate on how to define difficult to treat RA, there is a vibrant energy being harnessed for its resolution.
Dr Iris Navarro-Millan opens this session with a focus on the clinical coal face. Her presentation centres around a quote from her patient, “The mental burden of having RA is extraordinarily larger than the physical”. In her next breath, she took mine away by revealing that this physical burden includes a 43% increased risk of cardiovascular disease in those with difficult to treat RA.
This relationship is not straightforward. University education is protective against treatment-resistant RA. That means that social determinants of health are at play, and the association between cardiovascular disease and socioeconomic status is both complex and well established.
She cites a recent publication in which those with comorbid arthritis and depression had a hazard ratio of 6.66 (2.8-15.85) for increased the risk of mortality. This is staggering, but the original article defines depression as filling a prescription for antidepressant. I suspect the relationship has not yet been explored in enough depth. But it needs to be.
The interplay between difficult to treat rheumatoid arthritis, comorbid diseases, disability, mortality and other social determinants of health is too complex to unravel in one presentation, but her key point remains.
We as clinicians need to adjust our focus beyond the metacarpophalangeal joints, beyond the musculoskeletal system, the cardiovascular system and indeed, beyond the very idea that we are the clinician, and they are the patients. We are fellow human beings, and the mental suffering of our patients deserves validation both formally via additional research, and informally by our capacity to connect as human beings.
Dr Christopher Buckley follows with a 180-degree pivot to the biological paradigm of treatment resistant RA and zooms in to a microscopic focus on the cells involved. He mentions the Human Cells Atlas project, an ambitious movement to map every cell of the human body – presumably in health. He makes a good point. Improved understanding of the biological materials we are working with increases the efficiency with which they can be manipulated. It will be exciting to see what this organisation produces in the coming decades.
Novel research techniques are briefly outlined – including single cell sequencing, minimally invasive synovial biopsy, and high-resolution CT. From these a cellular model of RA is being painstakingly constructed. It’s early days, but vigorous vascular response to disease in RA with prolific NOTCH3 signalling is contrasted with fibrosis and macrophage activity in OA. From these little things, big things might grow.
The vision is that one day we might use cellular analysis to predict treatment response and target personalised medicine from the outset. This seems more instinctively accessible to clinical understanding than GWAS studies, which do not receive a significant mention.
Dr Louis Bridges wraps up the session with a summary of future directions for research and treatment in refractory RA. He continually loops back to the importance of a multidisciplinary research approach. Nurses, psychologists, educators, neurobiologists, occupational therapists, physical therapists, health economists, statisticians, computational biologists… all have a role to play in both the generation of research questions and finding solutions.
After all, solving complex problems must involve assessment from multiple and sometimes conflicting viewpoints, and rheumatologists are handicapped by our own expertise.
It is perhaps telling that each of the above presenters could clearly identify the complexity of this problem and the need for further multidisciplinary collaboration. Perhaps at the next congress there will be more examples of such collaboration in action.
Dr Maxine Isbel is a Perth-based final year rheumatology trainee and is currently doing a master’s in clinical research.
Myositis clinical pearls, statin myopathy and cancer screening – by Dr Bonnia Liu
Staying up past midnight on a Friday night used to be easy, but these days my energy levels are not what they used to be.
Thankfully ACR 2022 has once again utilised the wonders of technology and recorded sessions to be viewed at a less frightful hour! Though, for you, my audience, I shall gladly return to my nightowlish lifestyle for the next few days to bring you some of my favourite topics presented at ACR this year!
I have always appreciated the pre-meeting ACR review course (11F101. Review Course) which offers high-yield summaries and introduces some themes which will pop up during the rest of the conference.
Chosen this year, we have Dr Lisa Christopher-Stine, who took us through a series of clinical pearls to help distinguish between statin-associated autoimmune necrotizing myopathy and toxic (self-limiting) statin myopathies (11F101. Review Course – Statin Myopathy).
A few take-home points from me: statin-associated autoimmune necrotizing myopathies are rare, occur years after statin use, are almost always present with weakness and sometimes dysphagia, are associated with HMGCR antibodies (which is highly specific and sensitive) and do not rapidly improve with statin cessation.
In contrast, self-limiting statin myopathies are much more common, usually occur within days or weeks of statin initiation, are never associated with dysphagia and rapidly improve with statin cessation. Most importantly, self-limiting statin myopathies may be rechallenged with alternative statins.
Another interesting point was regarding symptoms of “statin intolerance”. Dr Christopher-Stine refers to the SAMSON and StatinWISE study which both demonstrated that most perceived muscle symptoms caused by statin tablets were nocebo effects rather than the statin itself.
In the next myositis session, Dr Julie Park (12S111. CARE: Clinical Pearls: Myositis – Inflammatory Muscle Diseases) took us through a series of clinical scenarios to discuss management of dermatomyositis, cancer risk, Jo-1 positive interstitial lung disease and the treatment of systemic sclerosis/myositis overlap syndromes.
What was most striking was the association of TIF-1 antibody with a 40% higher cumulative incidence of malignancy, especially ovarian cancer. In Australia, ovarian cancer is the 8th most common cancer affecting women. Unfortunately, ovarian cancer is also associated with poor prognosis, as approximately 70% of ovarian cancers are diagnosed at later stages 3 and 4.
Furthering emphasising the importance of malignancy screening, is the plenary session by Dr Alexander Oldroyd (12S110. Plenary I – 0002. Cancer Screening Recommendations for Patients with Idiopathic Inflammatory Myopathy) who recommended a general framework to identify low, intermediate, and high-risk factors for malignancy, and suggest a series of screening investigations.
One important issue to consider is that there are currently no standardised guidelines for screening and no cost-effective analysis to guide recommendations. We must consider the cost (mental and financial) to our patients and the healthcare system when selecting for appropriate investigations beyond the routine cancer screening programmes in Australia.
Lastly, I should mention the session by Dr Karen Yanelli (12S111. CARE: Clinical Pearls: Myositis – Physical Therapy for Myositis) emphasising the point of “move it or lose it.” Dr Yanelli clarifies that early exercise therapy is safe during the acute flare phase of autoimmune polymyositis/dermatomyositis. However, exercise regimes should be tailored to the patient’s physical level to reduce post-exercise fatigue and delayed onset muscle soreness, which may be prohibitive for patients.
A wide range of exercises include aerobic training, strength training, flexibility exercises, respiratory and relaxation training and gait training, though the long and short of it is that exercise have long-term influence on health, disability and even disease activity.
Facilitating early rehabilitation and routine exercise regimes will require a multidisciplinary approach so please include your physiotherapists and exercise physiologists!
Sessions include:
- 11F101. Review Course – Statin Myopathy
- 12S111. CARE: Clinical Pearls: Myositis – Inflammatory Muscle Diseases
- 12S111. CARE: Clinical Pearls: Myositis – Physical Therapy for Myositis
- 12S110. Plenary I – 0002. Cancer Screening Recommendations for Patients with Idiopathic Inflammatory Myopathy
Dr Bonnia Liu is a rheumatology fellow and nuclear medicine advanced trainee at Austin Health in Melbourne.
