Friday, 23 Aug 2019

You are here

Increasing Lung Disease in Systemic JIA

A single-center cohort analysis shows that lung disease (LD) is increasingly seen in children with systemic Juvenile Idiopathic Arthritis (SJIA), especially those complicated by macrophage activation syndrome (MAS).

Prior to 2013, reports of pulmonary disease in SJIA were rare, but since there have been increasing reports of alveolar hypertension (PAH), interstitial lung disease (ILD) and pulmonary alveolar proteinosis (PAP); often with a high mortality rate.

As such, since 2014, the Cincinnati Children’s Hospital Medical Center (CCHMC) has seen an increase in LD and severe lung disease in SJIA.

Between 2010-2018 there were 74 patients with SJIA seen by rheumatology at CCHMC. They have identified a total of 18 children with SJIA-LD - 13 patients received initial consultations and/or evaluations (second opinions) due to concerns of SJIA-LD, and five patients developed their SJIA-LD and were managed at CCHMC; suggesting a prevalence of SJIA-LD as high as 6.8% (5/74).

Clinically, most patients had subtle respiratory symptoms such as mild resting tachypnea with normal oxygen saturation, but 78% (14/18) did have digital clubbing. One patient had pulmonary hypertension on echocardiogram and required sildenafil therapy, but improved over the course of treatment with resolution of these symptoms. 

Radiographic findings of 18 patients found diffuse ground‐glass opacities, subpleural reticulation, interlobular septal thickening, and lymphadenopathy.

Pathologic findings included patchy but extensive lymphoplasmacytic infiltrates and mixed features of pulmonary alveolar proteinosis (PAP) and endogenous lipoid pneumonia (ELP).

SJIA‐LD tended to be younger at SJIA diagnosis (OR=6.5, P=0.007), have prior episodes of MAS (OR=14.5, P<0.001), have had adverse reactions to biologic therapy (OR 13.6, P<0.001), and have higher serum IL‐18 (27,612 vs. 5,413 pg/mL, P=0.047).

Pulmonary disease is increasingly detected in children with SJIA, particularly in association with MAS. This entity has distinct clinical and immunologic features and represents an uncharacterized inflammatory LD.

 

Disclosures: 
The author has no conflicts of interest to disclose related to this subject

Add new comment

More Like This

A Multidimensional Definition of Remission

A study from Leeds suggests 'multi-dimensional remission' (MDR) can be seen in one-third of RA patients achieving DAS28-remission; such patients have better patient-reported outcome measures, making it an optimal goal, especially from a patient's perspective.

Good Pregnancy Outcomes for DMARD Exposed JIA Patients

A study of pregnancy outcomes in 98 women with juvenile idiopathic arthritis (JIA) who were exposed to DMARDs shows no increased risk of major adverse pregnancy outcomes.

A total of 152 pregnancies in 98 women with JIA and 39 pregnancies involving 21 male patients as partners were reviewed. The majority had a polyarticular JIA (61%). The average age at first pregnancy was 24.1 years, and their mean disease duration was 13.8 years.

Autoantibodies Don't Disappear With Remission in RA

Immunologic remission in rheumatoid arthritis, defined as the disappearance of anti-citrullinated protein antibodies and rheumatoid factor, was seen infrequently among patients achieving sustained clinical remission and did not correlate with the disappearance of symptoms, a long-term Dutch study found.

Updated CDC Recommendation for Serologic Diagnosis of Lyme Disease

Serologic testing is the principal means of laboratory diagnosis of Lyme disease. Current recommendations include using a sensitive enzyme immunoassay (EIA) or immunofluorescence assay, followed by a western immunoblot assay for specimens yielding positive or equivocal results. On July 29, 2019, the Food and Drug Administration cleared several Lyme disease serologic assays with new indications for use, allowing for an EIA rather than western immunoblot assay as the second test in a Lyme disease testing algorithm. Thus, serologic assays that utilize a second EIA in place of western immunoblot assay are acceptable alternatives for the serologic diagnosis of Lyme disease.

Trends in Inflammatory Arthritis Care in Germany

The German National Database (NDB) has reviewed their experience in the care and quality of life of inflammatory rheumatic disease patients snce 1993, showing improved use of metrics, improved outcomes and a changing profile of biologic and anti-rheumatic drug use.