‘Communication and collaboration’ essential to managing polymyalgia rheumatica, GCA
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Polymyalgia rheumatica and giant cell arteritis can overlap and resemble one another in diagnosis and disease course, and understanding where one ends and the other begins is critical for rheumatologists and primary care providers alike.
“One important point that applies to the care of both patients with PMR and GCA is the importance of multidisciplinary care,” Sebastian E. Sattui, MD, MS, director of the Vasculitis Center at the University of Pittsburgh Medical Center, and assistant professor of medicine in the division of rheumatology and clinical immunology at the University of Pittsburgh, told Healio.
“Communication and collaboration between rheumatologists and primary care physicians does not stop after diagnosis, since co-management is required in order to optimize outcomes and minimize any disease and/or treatment associated complications,” he added.
Sattui highlighted some recent literature on the topic of these two conditions as essential for both rheumatologists and primary care providers to understand the management and therapeutic landscape.
In a paper published in the Annals of the Rheumatic Diseases, Dejaco and colleagues described treat-to-target recommendations in PMR and GCA. They developed five overarching principles and six recommendations, stressing co-management of both conditions in a shared decision-making paradigm between physician and patient. Avoidance of ischemic complications is critical, as is maximizing health-related quality of life, they added.
Regarding treatment targets, achievement and maintenance of remission is the first priority. Clinicians should also consider comorbidities when assessing disease activity and choosing therapies. Again, collaboration between rheumatology and primary care physicians is imperative not only for early recognition and diagnosis, but also for the holistic management of the patient, the authors stated.
Another important paper was published by Donskov and colleagues in Rheumatology. They surveyed 394 general practitioners and 937 rheumatologists about various topics pertaining to PMR and GCA. Results showed that “a large proportion of people with PMR are not referred for diagnosis” in a timely fashion, the researchers wrote. In addition, in the interval between referral and initial assessment, the proportion of treatment-naïve patients declined.
“This large international survey indicates that a large proportion of people with PMR are not referred for diagnosis, and that the proportion of treatment-naive patients declined with increasing time from referral to assessment,” they concluded. “Strategies are needed to change referral and management of people with PMR, to improve clinical practice and facilitate recruitment to clinical trials.”
Healio sat down with Sattui to discuss some of the fundamental differences between PMR and GCA, treatment options for the two conditions and how rheumatology and primary care can best work together to ensure that all patients with either condition are treated quickly and effectively.
Healio: Could you discuss some of the key differences between PMR and GCA?
Sattui: PMR and GCA are part of a disease spectrum. Some patients have purely PMR and some have purely GCA, but you also have patients who have both. Around 10% to 20% of patients with PMR can eventually develop GCA after initial diagnosis. Around 40% to 50% of patients with GCA have PMR, which can be noticeable early on after the diagnosis of GCA. Also, sometimes those symptoms can develop later when treatments wind down. The two conditions affect the same population, which is exclusively people older than 50 years, with a peak around 70 years. PMR is significantly more common than GCA.
Healio: What are the symptoms of these conditions?
Sattui: Patients with PMR tend to have this very characteristic pain and stiffness of their shoulder girdle and pelvic girdle. Besides PMR symptoms, patients with GCA tend to have vascular/ischemic symptoms. These include temporal headaches, scalp tenderness, jaw claudication and visual deficits. Both conditions can also have what we call constitutional symptoms, including fever, chills, weight loss and fatigue.
Healio: When in the context of PMR should you suspect GCA?
Sattui: Patients with PMR can present with GCA that is not necessarily recognized right away. Response to initial treatment with steroids can be useful to distinguish either entity. Patients with PMR usually require 15 mg to 20 mg of prednisone at the beginning. GCA is a bigger fire to put out and requires 40 mg to 60 mg of prednisone daily. When patients with PMR are not responding to this lower dose of steroids, then you have a suspicion for something else. You may conclude that the patient has GCA in the background, or other alternative diagnoses such as malignancy or infection need to be considered.
Healio: Could you talk about under- and over-diagnosis of these two conditions because they are difficult to differentiate?
Sattui: There is definitely both sides of the spectrum, where there is too much or too little diagnosis, and this can prove to be more problematic with PMR. There is no specific testing for PMR. As rheumatologists, we use biopsies, imaging or serological testing, to aid the clinical assessment.
For GCA, there are more specific testing strategies. Biopsy allows you to diagnose abnormalities in the temporal arteries. There is a broader and more frequent use of imaging in GCA, including ultrasound and CT scans. Imaging techniques though, particularly ultrasound, require a certain degree of expertise.
Auxiliary testing for PMR is not as specific and imaging, an emerging tool in diagnosis, is not commonly used in routine practice like that. That is one of the challenges of diagnosis.
Healio: What about laboratory tests for PMR?
Sattui: ESR and CRP are commonly used in practice. However, it is important to remember that these inflammatory markers are very common and may not be specific to one condition. They can be elevated in many conditions. On the other hand, although a normal ESR and CRP make an inflammatory condition like these less likely, rare cases with normal inflammatory markers have been reported.
Healio: What do rheumatologists need to be telling primary care providers who are encountering these conditions?
Sattui: The most important thing for primary care providers to understand about GCA is that it is a medical emergency. These patients need to be seen fast and referred to a rheumatologist immediately. There should be a diagnostic workup, including a biopsy, and treatment decisions should be made immediately.
It is important to acknowledge though that mechanisms need to be in place to better identify and rapidly assess these patients. This is an important aspect that needs to be acknowledged given the difficulties in access to rheumatology care. In Europe, GCA fast-track mechanisms have been shown to minimize delays in evaluation and the adoption of these mechanisms is finally picking up in the United States.
Not all patients with PMR need to be referred to specialty care. However, referral to rheumatology needs to be considered earlier on in patients who are not responding to treatment as expected, as well as those at high risk for glucocorticoid toxicity — like those with a history of diabetes or osteoporosis — and patients with atypical features.
The main message for primary care providers is that not all shoulder pain is necessarily a sign of PMR, and the response to glucocorticoids that can be seen is not exclusive to PMR either. The other important consideration is that if a primary care provider is uncertain about this diagnosis, referral to a rheumatologist is probably helpful. Further management can be coordinated by rheumatology and PCP, as well as the need for periodical follow-up in a specialty clinic.
Healio: You talked about steroid sparing. Could you talk about some of the agents used to that end?
Sattui: In GCA, there has been a good experience with tocilizumab (Actemra, Genentech). This has been the most frequently used steroid-sparing agent. However, some patients cannot get tocilizumab due to contraindications like, for example, a history of gastrointestinal bleed. There are also patients who do not respond to tocilizumab, so there is a need for further agents.
Methotrexate and leflunomide have also been used, but there are no robust data for those medications. Steroid sparing is particularly important in GCA. We are all aware of the toxicity of steroids. Many patients are treated for 2 years or more, so when you have patients who are 70 or 80 years old and receiving these high doses of steroids, comorbidities can be frequent, including cardiovascular disease, osteoporosis and infections.
The data for steroid-sparing agents is not as robust for PMR as GCA. One misconception about this condition is that you can give a patient steroids for a limited amount of time and it will go away. However, we know that about 30% of patients stay on glucocorticoids for several years, and again the toxicity associated with even lower doses cannot be ignored.
There are some data on leflunomide and methotrexate, but we need more studies. We have just had the approval of sarilumab (Kevzara, Sanofi) for refractory PMR, which is a great option to have. However, how it is going to fit into the landscape is something we still need to learn, and whether its effect could go beyond just steroid sparing and potentially into drug-free remission is a question that will be hopefully answered.
References:
Dejaco C, et al. Ann Rheum Dis. 2023;doi:10.1136/ard-2022-223429.
Donskov AO, et al. Rheumatology. 2023;doi:10.1093/rheumatology/keac713.
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Healio interview
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