Pulmonary complications often fatal in systemic JIA

Pulmonary complications often fatal in systemic JIA
Pulmonary complications are under-recognized in systemic juvenile idiopathic arthritis, and are often fatal, according to a study published online Nov. 8 in Arthritis Care & Research.

(HealthDay)—Pulmonary complications are under-recognized in systemic juvenile idiopathic arthritis (sJIA), and are often fatal, according to a study published online Nov. 8 in Arthritis Care & Research.

Yukiko Kimura, M.D., from the Hackensack University Medical Center in New Jersey, and colleagues utilized an electronic listserv to identify sJIA patients who developed pulmonary artery hypertension (PAH), interstitial lung disease (ILD), and/or alveolar proteinosis (AP). The 25 patients were compared to a sJIA cohort of patients from the Childhood and Rheumatology Research Alliance (CARRA; 389 participants).

The researchers found that, compared with the CARRA cohort, patients were significantly more likely to be female; have more systemic features; and to have been exposed to an interleukin-1 (IL-1) inhibitor, tocilizumab, infliximab, corticosteroids, intravenous immunoglobulin, cyclosporine, and cyclophosphamide. During the course of their disease, 80 percent of the patients had macrophage activation syndrome (MAS), and 60 percent had MAS at pulmonary diagnosis. PAH was seen in 16 patients, ILD in seven, and AP in five patients. At pulmonary symptom onset, 68 percent of patients were taking or recently (one month or less) discontinued a biologic agent and 48 percent were taking anti-IL-1 therapy. At a mean of 8.8 months following pulmonary diagnosis, 68 percent of died.

"PAH, AP and ILD are under-recognized of sJIA which are frequently fatal," the authors write. "These may be the result of severe uncontrolled systemic disease activity, and may be influenced by medication exposure."

Several authors disclosed financial ties to the pharmaceutical industry.

More information: Abstract
Full Text (subscription or payment may be required)

add to favorites email to friend print save as pdf

Related Stories

Not all juvenile arthritis is the same

Oct 22, 2012

Systemic juvenile idiopathic arthritis (SJIA) is currently classified as a subtype of juvenile idiopathic arthritis but with the addition of systemic inflammation often resulting in fever, rash and serositis. New research ...

Standardized treatment plans developed for new-onset JIA

Jul 03, 2012

(HealthDay) -- Four standardized consensus treatment plans (CTPs) have been developed for patients with new-onset systemic juvenile idiopathic arthritis (JIA), according to research published in the July issue ...

Recommended for you

Mummy remains refute antiquity of ankylosing spondylitis

18 hours ago

Ankylosing spondylitis is a systemic disease that causes inflammation in the spinal joints and was thought to have affected members of the ancient Egyptian royal families. Now a new study published in Arthritis & Rheumatology, a jour ...

Arthritis sufferers excluded from everyday life

Oct 13, 2014

Arthritis is the second leading cause of disability in Australia with many sufferers so severely disabled they cannot engage in basic everyday activities, new UNSW research has found.

User comments