Recapping the ACR Review Course: What You May Have Missed This Year
I always look forward to the ACR yearly review course for an engaging way to get updates on various topics in the field of rheumatology. It’s also a way to reminisce what it’s like to be a high primary school student again and makes you wonder how you were ever able to sit in the same seat all day long, day after day!
Well, despite my aching back, the ACR review course was a day well spent reviewing multiple subjects in the field of rheumatology in a clear and entertaining way. The review course offered interesting tidbits on multiple topics ranging from inflammatory myopathy to lupus nephritis and fibromyalgia. Let’s dig in!
The first lecture was an overview of inflammatory myopathy by Julie J. Paik, MD, MHS, from Johns Hopkins. She started the lecture by going through the Bohan and Peter criteria for inflammatory myopathy from 1975, which — as you may suspect — did not include myopathy-specific auto-antibodies nor necrotizing myopathy which is a more recent description of a subset of inflammatory myopathy. Paik discussed the 2017 EULAR/ACR criteria for inflammatory myopathy which adds multiple components to the diagnosis of inflammatory myopathy, including: neck flexor greater than neck extensor weakness, dysphagia and, importantly, anti-Jo1 antibody.
It’s frustrating: Anti-Jo1 is the only antibody that is a part of this criteria, but this is because the data were originally gathered in 2004 when not all of the antibodies were available. Another disappointment with the new criteria is necrotizing myopathy is not distinguished from other types of inflammatory myopathy. Remember the two antibodies for necrotizing myopathy are anti-HMG-CoA as well as anti-SRP.
Paik went on to discuss many clinical pearls for inflammatory myopathy. Pictures of ovoid palatal patch lesions were shown in patients with dermatomyositis, which is an exam finding that I was not aware of and consists of an oval (hence, ovoid) erythematous lesion on the roof of a patient’s mouth who has dermatomyositis — yet another reason to always look in patients’ mouths! Another important point brought up in the lecture was the emphasis on the association of anti-TIF1-gamma with malignancy. All patients with TIF1-gamma should have age-appropriate malignancy screening, and if the disease is refractory to treatment, a CT chest, abdomen and pelvis or whole body PET scan should be considered.
Perioperative management of rheumatic patients
The next talk addressed the perioperative management of rheumatic patients given by Susan M. Goodman, MD, from the Hospital for Special Surgery. Goodman presented data showing arthroplasty rates are increasing in non-inflammatory arthritis, but appear to be stable in patients with rheumatoid arthritis, although the rates, particularly in small joints, are decreasing likely thanks to advances in treatment in RA. However, the data aren’t as optimistic in other conditions, such as systemic lupus erythematosus and psoriatic arthritis, where the rates of arthroplasty appear to be increasing.
We have patients on chronic immunosuppression and a portion of them are undergoing more joint replacement surgeries; the question is, what do we do with the immunosuppression before and after surgery? Here are some suggestions from Goodman: Continue the current prednisone dose throughout the surgery, stress doses of prednisone are unlikely to be necessary and may increase risk of infection. As for biologic therapy, plan the surgery at the end of the dosing cycle of the medication. For example, adalimumab is given every 2 weeks, so schedule the surgery at week 3. Now, when do we resume the biologic? The decision to restart therapy should be based on clinician judgment, importantly, including a physician’s evaluation of the surgical site for evidence of infection.
Focus on fibromyalgia
Kristine Phillips, MD, PhD, of Vanderbilt University Medical Center, provided a nice overview of fibromyalgia for the third lecture. She began by showing the historical perspectives of fibromyalgia, as the disease has been called many different things throughout the century, including “wandering uterus,” which I wouldn’t recommend putting in a patient’s chart.
Fibromyalgia is defined as chronic, widespread pain; heightened response to pressure; comorbid fatigue; cognitive difficulties; sleep problems; restless leg syndrome and associated affective disorders such as anxiety and depression. A great clinical tip was mentioned: Goodman recommends having an order set for fibromyalgia whenever the disease is suspected to make sure you don’t miss anything. Her order set sends patients for evaluation of sleep apnea as well as checks labs for: thyroid disease, vitamin D deficiency, HIV, hepatitis C and tuberculosis. She also notes rare causes of mimics such as osteomyelitis and infective endocarditis.
Importantly, management was discussed for fibromyalgia, and Goodman emphasized to not use medications until other treatment modalities have failed. She starts with focusing on validating the patient’s symptoms, which helps gain the patient’s trust, then focuses on patient education about the disease, sleep exercises and mental health awareness. Goodman also discourages disability paperwork for fibromyalgia patients. If these methods fail, then the addition of medications such as pregabalin can be pursued.
The focus of the osteoporosis lecture given by Felicia Cosman, MD, of Columbia University, was to identify and treat high-risk osteoporosis patients more aggressively. High-risk osteoporosis patients can be identified, not surprisingly, as patients who have had a previous fracture.
Cosman highlighted that less than 25% of patients with a new fracture are subsequently treated for osteoporosis. The absolute risk of a second fracture within 1 year is 10%, 2 years is 18% and 5 years is 31%. Cosman presented the data on the use of antiresorptive therapy (ie, bisphosphonates and denosumab), which don’t have a decrease in fracture risk for at least 18 months after initiating treatment! Thus, patients with a previous fracture have an increased risk of a second fracture even when they are on an antiresorptive medication.
The point is, in patients with a previous fracture, anabolic medications should be considered which have a more rapid risk-lowering for subsequent fractures. Importantly, anabolic medications have also showed decreased efficacy when used after antiresorptive agents, so anabolic medications should be considered first-line after fractures.
Neurology for the rheumatologist
“Neurology for the Rheumatologist” was a talk by Julius Birnbaum, MD, who is a part of both the division of rheumatology as well as neurology at Johns Hopkins. One of the overarching goals of this lecture was to allow rheumatologists to recognize upper motor causes — such as a mass lesion compressing the cervical spinal cord — of foot drop, as well as differentiate between neuropathies and radiculopathy.
A descriptive case was presented of a 72-year-old man who presents with unilateral foot drop over an unspecified amount of time. On exam, the patient was noted to have increased tone to his muscles with passive range of motion (spasticity), increased reflexes of ankle, and notable weakness to dorsiflexion of the foot/ankle. Birnbaum refers to this as the “triad of upper neuron dysfunction” and prompted an MRI of the cervical spine showing a mass lesion compressing the patient’s cervical spine requiring emergent neurosurgery. The moral of the story from a rheumatologist’s perspective is, not all foot drop is vasculitis!
For the rheumatologists, Birnbaum also emphasized the difference between a radiculopathy (problem at the nerve root) and a neuropathy (problem in the actual peripheral nerve away from the nerve root). He emphasized a radiculopathy is compression further up the nerve, so it presents with more diffuse sensory and motor abnormalities (although less severe) than a peripheral neuropathy, which is much more localized since the anatomic abnormalities are more distal. Neuropathies usually present more acutely compared with a radiculopathy, but also the motor component is usually more severe (which we can see with a vasculitic neuropathy).
Another important aspect of Birnbaum’s talk was focused on small fiber neuropathies, which can present as a whole-body burning sensation. Whole-body pain may sound slightly reminiscent to another condition (ie, fibromyalgia), which is why we should keep small-fiber neuropathy in mind when evaluating patients with suspected fibromyalgia. If the patient has documented Sjogren’s syndrome, then small-fiber neuropathy should be higher on your differential and you should consider pursuing a skin biopsy (gold standard) for evaluation of small-fiber neuropathy as an explanation for widespread pain.
Lupus nephritis and ‘hippo skin’
Gerald B. Appel, MD, of Columbia University Medical Center, gave an overview on the treatment of lupus nephritis. He started his talk by going through the morbidity and mortality data on lupus and emphasized that minorities, including African Americans and Hispanics, have worse outcomes. He then went through the data comparing mycophenolate mofetil (MMF) to cyclophosphamide in the ALMS trial, which showed MMF was not inferior to cyclophosphamide. This is extremely important, considering the different safety profiles of the medications, especially considering the fertility issues with cyclophosphamide in reproductive-age women.
The ALMS maintenance trial then compared maintenance medication with MMF vs. azathioprine and showed that azathioprine was inferior to MMF for sustaining remission from lupus nephritis. Appel noted that he prefers MMF to azathioprine for maintenance in lupus nephritis because of these data; however, if the woman is young and potential pregnancy is a factor, then he has no reluctance in using azathioprine in these situations, considering the pregnancy data with azathioprine are much better compared with MMF.
If you ask another person who attended the ACR’s review course about the talk on cutaneous manifestations of rheumatic disease, the person will likely respond with, “The talk about the hippo skin?” That’s right, Andrea Kalus, MD, of University of Washington, kept the audience engaged throughout her talk on cutaneous manifestations of rheumatic diseases by sprinkling in interesting tidbits about animal skin, like the fact that a polar bear’s skin is black and the fur is clear! Why doesn’t the polar bear, then, appear black? It has something to do with the visible light that is bounced back on clear fur. She lost me at visible light; sorry, I’m not a physicist. The point is, polar bear skin is black. Fun fact!
Kalus spent the majority of her time discussing different types of cutaneous lupus. She pointed out that in discoid lupus, only 10% to 20% of patients have systemic lupus. She also made a point that providers are often timid regarding high-potency topical steroids in patients with discoid lupus on the face due to the concern of disfiguring pigment change and skin thinning. However, she points out that, without treatment, discoid lupus has a high propensity to scar so the risks of side effects from the high-potency topical steroid outweigh the risk of disease evolution and scarring.
Subacute cutaneous lupus is much more associated with systemic lupus than discoid lupus, and the majority of patients are also SS-A-positive. Subacute cutaneous lupus is often present on the chest and is extremely photosensitive. Subacute cutaneous lupus does not scar, which is reassuring. Importantly, when a patient is diagnosed with subacute cutaneous lupus, their medication list should be investigated as many medications are associated with this condition, including hydrochlorothiazide, calcium channel blockers and ACE inhibitors. I’m having a hard time thinking of patients who aren’t on one of those medications, so this is good to know!
The cutaneous manifestation of lupus that is most associated with SLE is acute cutaneous lupus, which is the prototypic malar rash that occurs between 40% and 50% of patients with SLE. A very important point that was brought up in Kalus’ talk is that a study looking at photosensitivity in lupus patients showed cutaneous lupus lesions may take up to 3 weeks to appear! This is important, because some patients may not think they are photosensitive because the lesions can be delayed up to 3 weeks from initial UV light exposure. This highlights the need for strong sun protection!
Update on PsA
To round up a long day of rheumatology reviewing, we had the privilege to listen to a talk by none other than Iain McInnes, MD, PhD, of University of Glasgow, whose Scottish brogue filled the auditorium with knowledge, as well as raucous laughter from great jokes and anecdotes.
McInnes gave an update on PsA for the year, emphasizing the clinical heterogeneity of PsA and the numerous organs that can be involved with the disease; he even pointed out the data supporting the notion that depression associated with PsA may be at least partly from the inflammatory pathophysiology and not necessarily because of failure to cope with the condition itself.
McInnes discussed the IL-17/23 pathway in the pathogenesis of this condition and outlined the broad mechanisms of action that are being advanced to treat PsA, including TNF inhibitors, co-stimulation inhibitor (abatacept), IL-12/23 inhibitors, IL-17 inhibitors (and IL-17 receptor blocker), IL-23 inhibitors, kinase inhibitors and PDE-4 inhibitors. We have a broad pallet of mechanisms to choose from when targeting this disease. Hopefully our knowledge will continue to expand on PsA and we’ll have better and better treatment options for this difficult disease.
All the lectures were bursting at the seams with useful information, but I simply skimmed the surface on some of what I thought were the most interesting and important aspects of the talk.
For more information:
Adam J. Brown, MD, is a rheumatologist in the department of rheumatologic and immunologic disease at Cleveland Clinic.
Disclosure: Brown reports no relevant financial disclosures.
To hear more insights from the ACR/ARHP 2018 Annual Meeting, click here to visit our Rheuminations podcast where Adam J. Brown, MD, tackles medical mysteries and other ripping yarns of the immune system gone awry.