Research highlights include studies that have found an initial high-risk antibody profile for antiphospholipid syndrome (APS) tended to remain high in pediatric patients, a correlation between obesity and more severe disease flare symptoms in patients with early rheumatoid arthritis (RA), people with inflammatory arthritis face challenges in maintaining employment, a link between aging and physical activity, and people with vasculitis frequently suffer from frailty, new research findings in antiphospholipid syndrome (APS)  insights on improving communications with diverse patient populations, and and new research on reproductive health.

A High-Risk Antiphospholipid Antibody Profile Matters in Pediatric Patients with Antiphospholipid Syndrome

A new study by HSS investigators including Jheel Pandya, MD, Karen Onel, MD, Doruk Erkan, MD, MPH has found that an initial high-risk antibody profile for APS tended to remain high in pediatric patients.

HSS Research Shows Obesity Is Associated with Worse Flare Symptoms and Quality of Life in People with Early Rheumatoid Arthritis 

A recent study from HSS and others has found a correlation between obesity and more severe disease flare symptoms that negatively affect quality of life in patients with early RA, a systemic, autoimmune, inflammatory disorder affecting multiple joints in the body. 

Study Finds People with Inflammatory Arthritis Face Significant Psychological Challenges in Maintaining Employment

An HSS study presented by Joan Westreich, MSW, LCSW, social work coordinator, Early Arthritis Initiative at HSS reveals a significant psychological impact related to inflammatory arthritis patients’ efforts to maintain employment while coping with the challenges of their illness.

New Study Shows Perception of Aging is Linked to Level of Physical Activity in Arthritis Patients

People with arthritis who report more negative feelings about how they are aging tend to get less physical activity and perceive themselves as less healthy, according to a new study by researchers at HSS and Weill Cornell Medicine. However, self-perception of good health explained the effect of negative thinking – providing an opportunity for clinicians to focus on a patient’s outlook on aging as well as their overall health. 

New Study Identifies Outcomes Associated With Frailty in Patients With Vasculitis

According to a new study from researchers at HSS and colleagues from the University of Pittsburgh and the University of Pennsylvania, people with vasculitis frequently suffer from frailty, a process that can affect our ability to recover after an illness or a fall, which in turn is linked to poor health outcomes.

New Antiphospholipid Syndrome Research Findings Presented at ACR Convergence 2023

Investigators from the Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) presented new research findings in antiphospholipid syndrome (APS) at the American College of Rheumatology (ACR) Convergence 2023, the ACR’s annual meeting.

HSS Shares Strategies to Enhance and Enable Communication with Language-Diverse Patients at Annual ACR Convergence

Bella Elogoodin, vice president, Service Excellence & Language Access at HSS, shared strategies to enhance and enable communication with language-diverse patients at the American College of Rheumatology (ACR) Convergence 2023 in a session titled, “Lost in Translation: Creating Access Pathways for Language-Diverse Patients in Rheumatic Care Settings.”

HSS Presents New Reproductive Health Research at the ACR Convergence 2023  

At this year’s American College of Rheumatology (ACR) annual meeting, HSS presented a number of studies focused on reproductive health for patients with systemic lupus erythematosus, rheumatoid arthritis, and other rheumatic diseases, including issues related to fertility, sexual function, use of contraception and HPV vaccination.

The research focuses on the diagnosis of renal disorders, the risk of venous thromboembolism after total knee replacement (TKR), and the care of pediatric and young adult patients with rheumatologic diseases. There are also studies related to the care of rheumatology patients during the COVID-19 pandemic.

“While there have been significant advances in science over the years, there are areas of unmet needs warranting continued investigation,” said S. Louis Bridges, Jr., MD, PhD, Physician-in-Chief, Chair of the Department of Medicine and Chief of the Division of Rheumatology at HSS. “HSS has a relentless focus on improving patient care through innovation and discovery. By spearheading multi-center studies, clinical trials, and laboratory-based research, we continue to improve the quality of life for patients affected by rheumatic and musculoskeletal conditions.”

What follows are some highlights from the meeting:

Geographical Variations in COVID-19 Perceptions and Patient Management: A National Survey of Rheumatologists

A study led by HSS rheumatologist Bella Mehta, MBBS, MS, looked at the perceptions and behaviors of rheumatologists in the United States regarding the risk of COVID-19 for patients with autoimmune disorders and how those patients were managed with immunosuppressive and anti-inflammatory medications. The 271 respondents were asked whether patients with rheumatic diseases were at higher risk of COVID-19 and whether the pandemic had led them to reduce the use, dosage, or frequency of biologics or steroids. Overall, the survey found geographical variations regarding perceptions of patients’ risk of COVID-19, with responses that were significantly different in the Northeast region of the United States compared with other regions.

Can a Clinical Disease Activity Index Based on Patient-Reported Joint Counts (PT-CDAI) Be Used to Inform Target-Based Care in Telemedicine? An Analysis of 2 Early RA Cohort Studies 

A team that included HSS rheumatologist Vivian P. Bykerk, BSc, MD, FRCPC, looked at data from two cohort studies on patients with early rheumatoid arthritis (RA) that compared patient and physician assessment of tender and swollen joints. This research is important because COVID-19 has forced rheumatologists to shift from in-person clinical visits to telemedicine, which limits their ability to carry out complete joint exams. The study found that patient and physician assessments had good agreement when it came to identifying active versus controlled disease activity. In addition, the researchers reported that predictors of higher discrepancies between patient and physician assessments may help identify patient subsets that could benefit the most from more physician-guided training at joint self-assessments as well as more probing questioning during telehealth visits to confirm active synovitis.

Characterization of Antiphospholipid Antibody-Associated Nephropathy: An International Survey of Renal Pathology Society Members

A survey led by HSS rheumatologist Medha Barbhaiya, MD, MPH, sought to determine whether pathologists worldwide use uniform criteria to distinguish antiphospholipid antibody-associated nephropathy (aPL-N) from other forms of thrombotic microangiopathy. The study was done in parallel with an international effort to develop new classification criteria for antiphospholipid syndrome. The web-based survey included 780 members of an international Renal Pathology Society; 111 renal pathologists responded, representing 33 countries. The participants were asked to determine whether two acute and eight chronic aPL-N features were consistent with acute or chronic aPL-N without and were not provided with serology information. Results from the survey indicated that more than 90% of pathologists worldwide agreed that the renal pathologic features most specific for aPL nephropathy are noninflammatory glomerular or small arterial microthrombi and organized microvascular thrombi with recanalization. In the absence of serologic data, more than 75% of pathologists indicated lack of specificity of chronic glomerular or small arterial changes. These findings indicate the importance of serologic test results in biopsy interpretation and suggest higher specificity for certain acute or chronic features.

The Risk of Venous Thromboembolism After Septic Total Knee Replacement (TKR) Revision Is Double the Risk After Aseptic TKR Revision: An Analysis of Administrative Discharge Data

A team led by HSS rheumatologist  Anne R. Bass, MD, assessed the 90-day risk of postoperative venous thromboembolism (VTE) in people who underwent revision TKR surgery. Although it was already known that infection can increase the risk of thrombosis by triggering coagulation pathways, the impact of infection in this context was not well understood. The study, which included 25,441 of these surgeries performed between 1998 and 2014, used data from the New York Statewide Planning and Research Cooperative System. Overall, 69% of these revisions were for mechanical causes (aseptic), 28% were for infection (septic), and 3% were related to a periprosthetic fracture. The researchers found that the risk of VTE after septic revision surgery was double the risk after aseptic revision surgery, and was 2.6-fold higher after revision surgeries for fracture. These findings point to the importance of taking infection and fracture status into account when planning VTE prophylaxis for patients having revision surgery.

Using a Patient-Engaged Approach to Identify Cross-Cutting Disease Factors Impacting Mental Health in Youth with Rheumatologic Disease

A team including HSS chief of pediatric rheumatology Karen Brandt Onel, MD, examined the mental health experiences of those with juvenile arthritis, juvenile dermatomyositis, or systemic lupus erythematous. It included patients aged 14 to 24 and parents of patients between the ages of 8 and 24. The primary outcome was the presence of any clinical or self-diagnosed mental health problem. The survey included information on cross-cutting disease factors such as disease duration, active disease status, current steroid medication, history of disease flare following remission, and appearance-altering comorbidities (such as psoriasis, stretch marks, alopecia, skin ulceration, and visible scarring). The researchers also examined results by mental health problem (depression, anxiety, and self-harm/suicidal ideation). Overall, they found that certain rheumatologic disease factors, including those that alter appearance, are predictive of mental health problems such as depression or anxiety. The findings will help identify targets for mental health screening in youth with rheumatologic diseases.

New Juvenile Idiopathic Arthritis Quality Measure Set for the Pediatric Rheumatology Care and Outcomes Improvement Network

A team that included HSS pediatric rheumatologist Nancy Pan, MD, reported highlights of new quality measures for the treatment of juvenile idiopathic arthritis. The study also provided information about the performance of these new measures, which were created by the Pediatric Rheumatology Care and Outcomes Improvement Network. The measures take into consideration clinical health importance, scientific validity, and feasibility, as well as best practices. They include both clinical evaluations based in part on the clinical Juvenile Arthritis Disease Activity Score and patient-reported outcomes such as pain, physical function, and overall well-being. An analysis of the new measures found that they are feasible for use on patients with juvenile idiopathic arthritis and can help to maximize quality improvement efforts and optimize the delivery of care.

“The reason we conducted this study is that for patients with CRMO or CNO, there is often an enormous delay between first symptoms to diagnosis, because it is confused with many other things,” said study coauthor Karen Brandt Onel, MD, Chief of Pediatric Rheumatology and Professor of Clinical Pediatrics at Hospital for Special Surgery (HSS) in New York City. “It is not uncommon to have children who have a two, or three-year span, or even as much as a 15-year span between the first symptoms and when they are ultimately diagnosed and treated.”

In the new study, the researchers identified 264 cases of CNO and 145 mimicking conditions from 20 centers in 7 countries on 4 continents, using the REDCap online database. Cases were included if they had at least 12 months of followup. The researchers compared clinical and investigational features of CNO patients with those of patients who had conditions that mimicked CNO.

When compared to mimicker diagnoses, CNO patients were predominantly female, more frequently exhibited intermittent versus continued pain, but less commonly had a fever. Clavicular swelling was more common in CNO than in mimickers. Symmetric patterns of bone lesions were also more common in CNO. CNO frequently involved the thoracic spine, clavicle, sternum/manubrium, pelvic bones, bilateral femur, bilateral tibia, unilateral fibula, and foot bones. Imaging features including cortical bone disruption, disorganized bone formation, mass structure, marrow infiltrate, abscess or geographic appearance were less common in CNO.

The researchers say that clinicians can use the new information to help improve the diagnosis of CNO patients. “We are working slowly and steadily towards classification criteria, so we can make the diagnosis quickly and get treatment instituted quickly. We are trying to work through this disease to identify clear as a bell diagnostic criteria, outcomes measures, and treatment,” said Dr. Onel. “This is a diagnosis that is not in everyone’s brains. Especially if there are typical upper extremity lesions, with the clavicle remaining number one, if there is no fever, and there are symmetric lesions, both tibias, both fibulas, both femurs etc., those are things that should push us in the direction of a diagnosis of CNO or CRMO. If you have symmetric lesions and especially if you have upper extremity lesions, you have to think CRMO or CNO.”

Other authors of the study hail from University of Washington, in Seattle and Bothell, WA; Middlemore Hospital, Auckland, New Zealand; Riley Children's Hospital, Carmel, IN; UNC Chapel Hill, NC; Children's Hospital of the King's Daughters/EVMS, Norfolk, VA; PRINTO, Istituto Giannina Gaslini, Genova, Italy; IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy; Children's Hospital Colorado, Aurora, CO; Children's Mercy Kansas City, Kansas City, MO; IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy; Dr. von Hauner Children's Hospital, Munich, Germany; Hacettepe University, Ankara, Turkey; Stanford University School of Medicine, Palo Alto, CA; University of Florence, Firenze, Italy; Alder Hey Children's NHS Foundation Trust Hospital, Liverpool, England, United Kingdom; CRMO Patient Partner, New York, NY, Stony Brook Children's Hospital, Stony Brook, NY; CRMO Patient Partner, Fort Collins, CO; Universitätsklinikum Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany; Anna Meyer Children's Hospital, Firenze, Italy; University of Utah and Primary Children's Hospital, Salt Lake City, UT; Georgetown University, Derwood, MD; Tufts Medical Center, Boston, MA; University of Iowa Carver College of Medicine, Iowa City, IA; University of Liverpool, Liverpool, United Kingdom, Boston Children's Hospital, Boston, MA; Vivantes Children’s Hospital, Wuerzburg, Germany and The Hospital for Sick Children, Toronto, ON, Canada.

Juvenile idiopathic arthritis (JIA) is a condition involving joint inflammation that lasts more than six weeks in children or adolescents under 16 years of age. It is the most common form of childhood arthritis, affecting about 1 in 1,000 children and teens in the United States. Scientists believe a combination of genetic predisposing factors and the environment causes JIA but the exact cause is unknown.

Polyarticular JIA is a chronic form of the disease with symptoms of pain, swelling, stiffness or warmth in four or more joints. Younger children may develop a limp or have difficulty using their fingers yet not complain about pain. Symptoms are typically worse first thing in the morning or after a period of rest and improve throughout the day as the child becomes more active.

“Polyarticular JIA looks a lot like rheumatoid arthritis in adults. Early diagnosis is essential to help keep the disease in remission, ensure children meet developmental milestones and delay or prevent joint destruction as they age,” says Nayimisha Balmuri, MD, a third-year pediatric rheumatology fellow at HSS and lead author of the study. “Reported morning joint stiffness may be a better indicator of early disease symptoms than pain since reporting pain can be influenced by cultural factors. Our findings underscore the importance of early referral of suspected cases for expert diagnosis and treatment.”

The study abstract is featured today in a poster presentation at ACR Convergence 2020, the American College of Rheumatology’s annual conference, which is being held virtually this year.

Dr. Balmuri and colleagues, including senior author Karen Brandt Onel, MD, chief of pediatric rheumatology at HSS, identified 1,684 American patients diagnosed with polyarticular JIA using the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry from July 2015 through February 2020. The CARRA Registry is a collection of information provided by North American patients and families on childhood-onset rheumatic diseases, including JIA.

The investigators extracted data on the presence and duration of morning joint stiffness. They also examined reported family income, the highest level of guardian-completed education and community poverty level, collectively called social determinants of health, using zip codes and the 2014–2018 American Community Survey. Finally, Dr. Balmuri and colleagues included the rheumatoid factor (RF) and anti-cyclic citrullinated peptide (anti-CCP) antibody status from patients’ diagnostic lab results as indicators of disease severity. The patients were seven years of age, on average.

The researchers found that children with polyarticular JIA from less affluent families, defined as having an income of $25,000 to $49,000, were twice as likely to report more than an hour of morning joint stiffness compared to children with the condition from families with an income of more than $100,000. This association between reported morning joint stiffness held even after the researchers adjusted the data for age at diagnosis, and RF and CCP status.

“Family income as an indicator for insurance status may indicate that more affluent families can navigate the healthcare system better, ensuring their children see pediatricians sooner and therefore obtain earlier referrals to pediatric rheumatology sub-specialists,” says Dr. Balmuri. “We can’t prove that with our present study, but our findings raise important questions for further research.”

“We hope our findings encourage physicians to ask patients about morning joint stiffness, not just pain, which may streamline the referral process,” Dr. Balmuri says. “Time to referral affects how soon we can initiate multidisciplinary care, which may include medications, physical therapy and occupational therapy that are crucial for improving mobility and joint function over these children’s lifetimes.”

Judy Campbell, a volunteer at HSS who herself has lupus, wrote it. The hospital’s Department of Social Work Programs approached her about the project when they learned she had written a children’s book some years back. “Chita Shares a Secret,” geared toward ages 6 to 11, explains lupus in terms a child could understand. The story comes alive with a cast of jungle characters and bright, colorful illustrations.

Ms. Campbell says she aimed to convey a positive message for young patients – and their parents – so they would gain the courage to cope with a difficult illness. “I set out to write a positive, upbeat book that would provide a message that it’s OK to have lupus,” Campbell explained.

Lupus causes the body’s immune system, which is supposed to protect us from illness and infection, to attack healthy cells and organs. It affects people differently and is often unpredictable. Common symptoms include fatigue, headaches, joint pain or swelling, and a rash on the face or elsewhere on the body. In severe cases, it can affect the kidneys, heart and lungs.

The book has been well-received by pediatric rheumatologists at HSS and other hospitals around the country. “I love it. It is bright, cheerful and easy to read, but addresses a really big problem,” said Karen Brandt Onel, MD, chief of the Division of Pediatric Rheumatology at HSS. “Kids are so afraid to share with their friends, as they think they will be rejected. The book just tackles it right on.”

Research shows that children are being diagnosed with lupus at a younger age, and there are limited age-appropriate resources to educate them and their families about the condition, according to Priscilla Toral, manager of LupusLine® at HSS, which provides free telephone counseling for people with lupus and their families. “The book provides physicians and the healthcare team with an age-appropriate tool to discuss a diagnosis of lupus, its symptoms and treatment with young patients,” she said. “Adult lupus patients can also use the book to explain their condition to their children.”

The book helps young lupus patients come to terms with a new and difficult diagnosis, while giving them the courage to broach the subject with their family and friends, says Sarah Faith Taber, MD, pediatric rheumatologist at HSS. “It is written in straight-forward, basic language that even young children can understand and illustrated with eye-catching, engaging drawings,” she explains. “Families have been delighted to receive it."

HSS patient Elizabeth Lopez, who was diagnosed with lupus at age 11, said she showed the book to her friends to help them understand her illness.

Campbell, the author, understands lupus all too well. She was diagnosed in her 40s after years of doctor visits to determine what was causing her symptoms. She found a physician at HSS and firmly resolved to live a full and happy life. She has pushed through flare-ups, pain and several surgeries. Instead of dwelling on her illness, she considers herself fortunate for the many opportunities she has had throughout her life. And happy with the care she received at HSS, she says she wanted to give back.

Hospital for Special Surgery has printed more than 1200 copies of the book over the past year for health professionals and lupus organizations nationwide that have requested hard copies. The costs of production, printing and mailing are paid by HSS. The book is also available for everyone to download https://issuu.com/hospital-for-special-surgery/docs/social-work-chita-shares-a-secret

Comments from recipients around the country show it has made a difference. “This is a wonderful resource; we use them (the books) for our patients and include them in our new patient packets,” wrote Duke Pediatric Rheumatology staff. “The story made me cry,” said a health professional at Seattle Children’s Hospital.

The story takes place in the jungle, where a cheetah named Chita has lupus. Dr. Hippopotamus is the kindhearted physician who explains the disease and gives good advice. Chita is sometimes too tired to play with her jungle friends and is afraid, at first, to tell them about her illness. She finally explains her symptoms to her friends and answers their questions. The animals are all very supportive, providing a message of acceptance and encouragement. In the end, they give Chita a tee shirt that says “Chita the Brave.”

Clever illustrations make the story come alive. “The author wanted to convey a sense of belonging and security,” explains Steven Portera, executive producer in the Creative Services Department at HSS, who illustrated the book. “I know firsthand how hard it is to be a kid with a chronic illness and how difficult it can be most times to blend in with your friends. If this project helps just a few kids feel a little better, then it’s all worthwhile.”

"There are not many children affected by rheumatic diseases in the United States, if you consider it collectively. As a nation of over 300 million people, none of these diseases are exactly common: Juvenile idiopathic arthritis is the most common and estimated to affect about 300,00 children in the U.S.," said Dr. Onel at the Rheumatology Nurses Society Annual Conference.

She warned that children treated by rheumatologists who do not specialize in pediatrics may not receive the same level of appropriate care as those treated by pediatric rheumatologists.

Additionally, Dr. Onel noted that by staying current with recent management guidelines and increasing exposure to pediatric rheumatology in fellowship programs, physicians could help reduce treatment disparity and improve the care of children with rheumatic diseases.

Read the full article at healio.com [subscription required].

"Whether anti-TNF treatment can be stopped in children with JIA is the really big question we'd like to answer in the next 10 years," said Dr. Onel at the Rheumatology Nurses Society annual meeting.

According to the article, sixty percent of the patients involved in the study experienced a flare after their anti-TNF therapy stopped.

"Currently, I view stopping anti-TNF therapy more like a long medicine holiday, and advise patients that there's a chance they will have to go back on the treatment. But if you're talking about children and injections, sometimes they do need a breather," added Dr. Onel.

Read the full article at medpagetoday.com.

HSS pediatric rheumatologist Karen Brandt Onel, MD, who did not participate in the drafting of the guidelines, said "these guidelines are meant to be fluid, but we need to be committed to getting patients into remission as quickly as possible. Anything less than that is not OK."

She added that joint and organ damage resulting from JIA could be permanent.

"For whatever reason, the risk of permanent disability from childhood arthritis is understated," she noted

Read the full article at mdedge.com [registration required].

"When I first started, these were the hardest children to treat. They really didn't respond to any medications… They suffered from tremendous side effects including gaining of weight, changing in their face, they would be severely immunosuppressed and specifically they got a lot of arthritis of their hips and many of them needed to have hip replacements," Dr. Onel explained.

According to the video, Weill Cornell Medicine and HSS developed a pilot clinical trial with a drug that was originally created to treat adult rheumatoid arthritis. While it didn't work for adults, they found that the medication helped pediatric patients.

"You have children in the hospital who are desperately ill with daily fevers, and then you give them the medication and it just stops. Instantaneously. It is one of the most remarkable things I have ever seen," Dr. Onel noted.

Eva Chi, a two-year-old HSS patient, felt her symptoms improve dramatically after receiving the drug therapy.

Watch the full video at news.weill.cornell.edu