Research & Technology

Study Describes a New Complication in Systemic Juvenile Arthritis

Spectrum Health rheumatologist calls finding “foundational”

GRAND RAPIDS, Mich., Jan. 2, 2020 – A Spectrum Health rheumatologist took part in a multi-center study that is the first to describe a deadly set of complications of systemic juvenile arthritis (SJA).

In the United States, up to 300,000 children have some form of arthritis or pediatric rheumatic disease. The most common type is juvenile idiopathic arthritis (JIA), which often develops by age two. Idiopathic means there is no known cause of the disease. Systemic JIA is a subtype in which patients also develop high fevers and rash, and which can lead to multi-organ failure if not treated.

“The study is foundational in being the first to describe a novel lung disease in a subset of patients with systemic JIA,” said Khalid Abulaban, MD, section chief, Spectrum Health pediatric rheumatology. “Researchers are attributing the lung disease to either the JIA itself or to anaphylactic reactions to the medications used to treat it.”

“This insight will impact patient care because the ability to correlate symptoms and risk-factors will help to identify patients who require close attention,” Abulaban added.

Patients with systemic juvenile arthritis often are treated with a type of medication, interleukin (IL)-1 and IL-6 inhibitors, to prevent a life-threatening condition called macrophage activation syndrome (MAS). MAS can produce persistent fevers, headaches, rashes, low blood counts, abnormal functioning of the liver and host of other symptoms.

While most patients respond well to these medications, a subset of patients identified in this study develop severe reactions—sudden, severe, life-threatening allergic reactions such as swelling, wheezing and difficulty breathing.  The same patients also demonstrate the novel form of life-threatening lung disease described by Dr. Abulaban.

Researchers reviewed 61 cases of SJA to identify risk factors associated with this severe form of lung disease. The study highlights risk factors, observable symptoms and clinical characteristics, such as extensive, atypical rashes, that should raise suspicion of lung disease, which otherwise can remain hidden until severe and life-threatening.

“The symptoms sometimes are sub-clinical,” Abulaban said. “Physician awareness of delayed hypersensitivity to these medications could lead to earlier recognition, cessation of the drug and better potential outcomes for patients.”

The study, “Emergent high fatality lung disease in systemic juvenile arthritis,” was published in the journal, Annals of the Rheumatic Diseases.

Vivian Saper, MD, and Guangbo Chen, PhD, of Stanford University are joint first authors; Purvesh Khatri, PhD, and Elizabeth Mellins, MD, of Stanford University are joint senior authors.

 

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