Updates from the ACR Convergence 2023 Review Course, part 2
SAN DIEGO—In a field as vast as rheumatology how can a one-day review course cover many of the highest yield topics that matter to clinicians and patients? Under the leadership of moderators Noelle Rolle, MBBS, assistant professor in the Division of Rheumatology, associate program director of the Rheumatology Fellowship at the Medical College of Georgia, Augusta University, and Julia Schwartzmann-Morris, MD, associate professor, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, N.Y., accomplishing this goal proved possible at the pre-ACR Convergence 2023 Review Course on Saturday, Nov. 11.
Transitions
Dr. Rebecca Sadun
Rebecca Sadun, MD, PhD, assistant professor in medicine and pediatrics, Duke University School of Medicine, Durham, N.C., focused on transitions from pediatric to adult rheumatology care for patients with juvenile idiopathic arthritis (JIA).
Did you know that about 50% of patients with JIA are lost to follow-up when they are meant to be transitioning from pediatric to adult care?1 In addition, said Dr. Sadun, about 20% of patients with JIA who achieve disease remission will go on to have recurrence, sometimes many years later in life;2 thus, it’s important to make sure that these patients are followed closely over time and not lost to follow-up. Moreover, adult patients with a history of JIA are at risk for significant complications of their disease, including contractures, erosions and vision loss due to uveitis.
Dr. Sadun explained that, when thinking about these transitions of care, two elements are very important:
- Understanding the disease process in JIA, and
- Understanding the psychology and expectations of patients.
The Disease(s)
JIA is not one disease, but rather an umbrella term for a number of specific conditions, said Dr. Sadun.
Oligoarticular JIA is the most common form of JIA and can be painless (e.g., children may have joint effusions but not report any associated pain with this inflammation). There is not a clear adult equivalent for oligoarticular JIA.
In contrast, rheumatoid factor (RF) positive JIA and RF negative JIA can be thought of as the pediatric equivalents of adult seropositive and seronegative rheumatoid arthritis (RA), respectively.
Psoriatic arthritis in children and adults share most of the same features.
For all of these patients, adult rheumatologists should ask about a history of uveitis and of temporomandibular joint (TMJ) involvement, both of which can occur independent of peripheral arthritis.
In patients with a history of enthesitis-related JIA, which is similar to spondyloarthritis in adults, patients should be asked about a history of axial disease.
Dr. Sadun stated clearly that systemic JIA (sJIA) is essentially adult-onset Still’s disease, but with age of onset before 16 years. sJIA can be very aggressive, may have a number of systemic manifestations and may be poorly responsive to tumor necrosis factor-α (TNFα) inhibitor therapy. It is important to ask these patients about a history of lung involvement, macrophage activation syndrome (MAS), fevers and rash.
Finally, undifferentiated JIA is a wastebasket term for patients who either don’t meet criteria for another form of JIA or meet criteria for more than one form of JIA. In these patients, the adult rheumatologist should inquire about associated and extra-articular symptoms and treat on the basis of the type of adult inflammatory arthritis the patient has most closely exhibited to date.
A key point that Dr. Sadun discussed was that patients with JIA may uniquely have TMJ joint involvement. This can be an erosive, destructive process, with loss of joint space and development of micrognathia. On exam, it is important for pediatric and adult rheumatologists to auscultate the TMJ joints with their stethoscopes and listen for crepitus. If there is crepitus that worsens over time, this is worrisome for increasing disease activity in this joint. Patients should also be evaluated for maximum mouth opening capacity, which is defined as the maximal interincisal distance on unassisted active mouth opening. If this opening distance decreases over time, MRI with TMJ protocol may be needed to assess for disease activity and damage in this joint.
Differing Goals
Dr. Sadun noted a number of differences in management goals and medication uses between pediatric and adult rheumatologic care. She explained that the pursuit of remission can look quite different in adults than in children.
In a child, the risks associated with mild disease activity that persists for many decades may be greater than that seen in an older adult with fewer years to experience their disease. It is also important to recognize that children typically have much lower risks of infections than older adults. For both of these reasons, the goal in pediatric rheumatology is to achieve true disease remission, meaning absolutely no evidence of ongoing disease activity.
Because children tend to tolerate immunosuppression well overall and because of their more efficient drug metabolism, pediatric rheumatologists often feel more comfortable using higher doses of DMARDs and biologics than what is used in adult patients. For example, many patients with JIA may be started on methotrexate at a dose of 25 mg per week instead of working up to this dose.
This means that during the patient’s transition to adult care, the adult rheumatologist should have a conversation with the pediatric rheumatologist to understand why certain doses of medication were used. This will also demonstrate to the patient that the adult rheumatologist values the opinion of their pediatric counterpart, a physician with whom the patient has a strong relationship.
Dr. Sadun presented two mnemonics to help adult rheumatologists remember key topics in the transition for patients. The first is TRANSFER: 1) understand the Treatment history; 2) be aware of Recent complications or Recent medication changes; 3) think about Adherence challenges and root causes for these issues; 4) consider Needs (e.g., referrals to other specialists); 5) be cognizant of their Social history, including transportation needs; 6) consider Financial challenges; 7) be aware of Emotional and intellectual challenges that patients may face; and 8) take into account Reasons that this is a good—or not a good—time for transfer to adult care.
The second mnemonic is WELCOME: 1) Welcome the patient and congratulate them on the transfer; 2) Explain similarities and differences in adult care (e.g., adult clinics may have a stricter policy if a patient is late for appointments); 3) Let patients and parents know respective roles; 4) keep the Channel of communication open with the pediatric rheumatologist; 5) provide an Opportunity for questions from patients and parents; 6) conduct a thorough Medical visit and Minimize medication changes at the first visit; and 7) End the visit by supportively communicating expectations. It is also important to include sexual and psychological history taking in the visit, and parents should be asked to leave during the interview to ensure the patient can be open and honest.
Jason Liebowitz, MD, is an assistant professor of medicine in the Division of Rheumatology at Columbia University Vagelos College of Physicians and Surgeons, New York.
References
- Hazel E, Zhang X, Duffy CM, Campillo S. High rates of unsuccessful transfer to adult care among young adults with juvenile idiopathic arthritis. Pediatr Rheumatol Online J. 2010 Jan 11;8:2. doi: 10.1186/1546-0096-8-2. PMID: 20148143; PMCID: PMC2820032.
- Packham JC, Hall MA. Long-term follow-up of 246 adults with juvenile idiopathic arthritis: functional outcome. Rheumatology (Oxford). 2002;41(12):1428–1435.
