Doctors Call for Screening of Juvenile Idiopathic Arthritis Patients to Identify Rare Lung Disease

According to a story from, biologic medicines have had a major positive impact on outcomes for patients with the rare disease juvenile idiopathic arthritis (JIA). However, as these medicines have grown more popular, it appears that they may be linked to a rare and serious complication which has been dubbed systemic JIA-associated lung disease (SJIA-LD). The disease appears to occur exclusively in patients with systemic juvenile idiopathic arthritis and may affect as many as five or six percent of patients that are using biologic drugs.

About Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis is a rare form of arthritis that primarily affects children and teens. While it is known that the disease is autoimmune in origin (meaning that the immune system begins to attack healthy tissue by mistake), what triggers the beginning of the autoimmune response is not known. Any disease considered “idiopathic” does not have an identified cause. Some risk factors for juvenile idiopathic arthritis include being female and a family history of the disease. Symptoms include limping, vague flu-like symptoms, fatigue, loss of appetite, swelling of the joints, joint pain and stiffness, growth problems, and eye inflammation. The disease can also lead to complications such as vision problems, osteoporosis, joint deformities, and contractures. Treatment approaches often include physical therapy, NSAIDs, corticosteroids, and certain chemotherapy agents that suppress the immune system. Surgery may be necessary in severe cases. To learn more about juvenile idiopathic arthritis, click here.

Biologic Drugs and SJIA-LD

Biologic drugs first began being used to treat the disease in 2005. Today, this class of therapies is a first-line treatment for systemic juvenile idiopathic arthritis. For the majority of patients, biologics are a highly effective method for treatment and its positive impact as of now far outweighs the risk of rare complications like lung disease. Outcomes have decisively improved since their introduction.

Lung disease symptoms include fatigue, clubbing of the digits, and coughing. These symptoms suggest decreases in lung function. Studies of patients affected by the disease indicates that these patients often did not see disease remission with treatment, were more likely to be diagnosed when they were less than two years old, and had a history of other adverse events and complications such as allergic reactions, liver problems, and macrophage activation syndrome.

Future research will be necessary in order to determine why this complication appears and how it can be prevented.

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