The mother of a 15-year-old patient with juvenile idiopathic arthritis/enthesitis-related arthritis (JIA/ERA) called the office in tears. She said she was having an insurance problem. Her son had been a star track athlete when he developed severe back pain. Magnetic resonance imaging showed evidence of severe sacroiliitis. He was started on a tumor necrosis factor…
NEW YORK (Reuters Health)—A new study of multisystem inflammatory syndrome in children (MIS-C) shows that the immune system is profoundly altered during acute illness, but gradually returns back to normal. MIS-C is distinct from both COVID-19 and Kawasaki disease, but is associated with previous SARS-CoV-2 infection, the immunological profiling study reveals. Dr. Manu Shankar-Hari, an…
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease of immune dysregulation characterized by unchecked inflammatory responses leading to end-organ dysfunction. Primary HLH results from inherited mutations that impair the capacity for immune regulation; secondary HLH arises from the inappropriate response to an immune stimulus, such as infection, malignancy or autoimmunity. What is less well known is…
The ACR has released clinical guidance documents for pediatric patients with rheumatic disease in the context of the COVID-19 pandemic, including one for multi-system inflammatory syndrome in children (MIS-C). Jay Mehta, MD, MS, an attending physician in the Division of Rheumatology and director of the Pediatric Rheumatology Fellowship at the Children’s Hospital of Philadelphia (CHOP),…
The recommendations for MIS-C focus on general guidance, diagnostic evaluation and therapy options, as well as comparing and contrasting the features of MIS-C and Kawasaki disease.
A 13-year-old, adopted girl of unknown ancestry with social anxiety, selective mutism and Takayasu arteritis presented for evaluation of severe, painful, gingival hyperplasia, which limited her oral intake and resulted in weight loss. The young patient was diagnosed with Takayasu arteritis at age 8, when she presented with a persistently elevated erythrocyte sedimentation rate (ESR),…
A 17-year-old girl presents to the pediatric rheumatology clinic for follow-up of recently diagnosed systemic lupus erythematosus (SLE) characterized by class IV lupus nephritis, photosensitive rash and antiphospholid antibody positivity. She is currently being treated with prednisone, mycophenolate mofetil, and hydroxychloroquine. She is accompanied by her mother, who has been very involved in the patient’s…
Since it was first described, the spectrum of disease caused by deficiency of adenosine deaminase 2 (DADA2) has been broadening. Features described include systemic vascular and inflammatory features and recurrent stroke, which overlap with childhood-onset polyarteritis nodosa (PAN). Previous data show that DADA2 has extensive genotypic and phenotypic variation.1 Results from a recently published study…
Juvenile idiopathic arthritis (JIA) can affect all joints, including the temporomandibular joints (TMJs). For a long time, the TMJ was a “forgotten” joint in pediatric rheumatology, although Sir Frederick Still did comment on a small mandible in his first case series on juvenile arthritis in 1897. In recent years, more attention has been given to…
ATLANTA—Macrophage activation syndrome (MAS), a subset of hemophagocytic lymphohistiocytosis (HLH) disease, can be a fatal result of rheumatic disease. But there’s good news: New therapeutic options for refractory MAS targeting individual cytokines are emerging. At the 2019 ACR/ARP Annual Meeting, possible therapeutic options were presented during a pediatric-focused clinical and translational research track, Therapeutic Approaches…