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AAP National Conference: How to determine if fever without infection is due to autoimmune disease :

October 22, 2016

Your patient has periodic fevers, and you have ruled out an infectious cause. What now?

Pediatric rheumatologist Kabita Nanda, M.D., FAAP, will help sort through the differential diagnosis during a session titled “Fever Without Infection: Autoimmune and Autoinflammatory Diseases (F1188)” from 5-5:45 p.m. Saturday in Room 302 of Moscone South.

Pediatricians often are concerned about systemic-onset juvenile idiopathic arthritis in patients with fever of unknown origin, said Dr. Nanda, a member of the AAP Section on Rheumatology.

“We often, as rheumatologists, get called in when they (general pediatricians) can’t find an infection,” she said.

The session will cover many things rheumatologists think about when they evaluate patients who have fevers without a cause.

Among the possible diagnoses is juvenile arthritis, which affects about 70,000 to 100,000 children under 16 years.

“What I tell families is that it is as common as juvenile diabetes but unfortunately, it doesn’t get the press,” Dr. Nanda said.

Systemic-onset juvenile arthritis, which accounts for 10%-20% of all cases of juvenile arthritis, is especially concerning because patients can develop macrophage activation syndrome — a life-threatening complication.

Dr. Nanda also will review autoinflammatory diseases that can present with fever, including familial Mediterranean fever, cryopyrinopathies, tumor necrosis factor receptor-associated periodic syndrome (TRAPS) and hyper-IgD syndrome.

“Those are difficult to diagnose, even for a rheumatologist,” Dr. Nanda said. “There is some genetic testing available for some of these illnesses. However, genetic testing does not always give you the answer.”

Attendees will learn to recognize nuances in the presentation of periodic fever syndromes, which can provide clues to diagnosis. Things to consider include how often the fever occurs, age of onset and associated symptoms.

Historically, periodic fever syndromes were thought to occur in certain geographic areas. However, the U.S. is so diverse that “I definitely see some surprises,” said Dr. Nanda, assistant professor, Seattle Children's Hospital/University of Washington.
She also will touch on other non-infectious conditions pediatricians should think about in patients who present with fever, including vasculitis, sarcoidosis, inflammatory bowel disease and malignancies.

“Malignancy is a big differential in systemic-onset juvenile idiopathic arthritis, which is why the heightened sensitivity and worry among providers,” Dr. Nanda said.

She encourages pediatricians to reach out to a pediatric rheumatologist if they have a concerning case.

“If you’re in doubt, if you have a question, just call,” she said.

While there are fewer than 400 pediatric rheumatologists throughout the country, most children’s hospitals have subspecialists available for consultation.

Dr. Nanda goes a step farther by flying twice a year from Seattle Children’s to outreach clinics in Alaska, where she covers two hospitals for five days. Her colleagues also visit outreach clinics in Montana and eastern Washington.

“Something else that I think is important for us is to keep educating our trainees and keep encouraging our residents to go into rheumatology,” said Dr. Nanda, who is credentialed at four hospitals and has three state licenses.

She initially thought she would go into hematology/oncology, but a rotation in rheumatology during residency piqued her interest.

“I liked that you could actually see the disease manifestations. You can see arthritis. You can touch it. You can see rashes. And you can see it get better,” Dr. Nanda said. “So it’s very rewarding not only for our patients but also for you as a physician.”

For more coverage of the AAP National Conference & Exhibition visit http://www.aappublications.org/collection/cme
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