ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2274

TNF Inhibitors Provide Long-Term Clinical Benefits in 6 Patients with Early-Onset Sarcoidosis

Tomokazu Nagakura1, Tsuyoshi Yamatou2, Tomohiro Kubota2, Hiroyuki Wakiguchi2, Yuichi Yamasaki2, Yukiko Nonaka2, Tomoko Takezaki2, Harumi Akaike2, Yasuhito Nerome2, Hiroyuki Imanaka2 and Syuji Takei3, 1Department of Pediatrics, House of Meguminoseibo, Usuki, Japan, 2Department of Pediatrics, Kagoshima University Hospital, Kagoshima, Japan, 3School of Health Sciences, Faculty of Medicine, Kagoshima University, Kagoshima, Japan

Meeting: 2014 ACR/ARHP Annual Meeting

Keywords:

Session Information

Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Systemic Juvenile Idiopathic Arthritis, Spondyloarthropathy and Miscellaneous Pediatric Rheumatic Diseases

Session Type: Abstract Submissions (ACR)

Background/Purpose: Early-onset sarcoidosis (EOS) is a NOD2 gene-associated chronic autoinflammatory disease, characterized by the triad of arthritis, rash, and uveitis, which usually occurs in children younger than 4 years of age. The aim of this study was to evaluate the clinical benefits of TNF inhibitors (TNFi) in EOS.
Patients and Methods: Six EOS patients treated with biologic agents at our hospital were reviewed. To evaluate the benefits of biologic agents, patients were retrospectively assessed at baseline, 3, 6, 12 and 36 months and the last visit after initiating the therapy. Severity of each clinical symptom such as arthritis, camptodactyly, uveitis, and skin rash was assigned by scoring from 0 (none) to 3 (severe). Laboratory findings such as CRP, ESR, or matrix metalloproteinase-3 (MMP-3) were also evaluated. In addition, the accumulated continuation rate of each biologic agent was analyzed by Kaplan-Meier method.
Results: The biologic agents were initiated after 10.9 years from onset (0.8-18.1y). The 1st biologic agents (1st Bio) used were infliximab (IFX) in 2, etanercept (ETA) in 2, adalimumab in 1, and IL-6 inhibitor (IL-6i) tocilizumab (TCZ) in 1. Four patients maintained clinical remission with the 1st biologic agents. The rest of 2 had switched to the 2nd biologic agents due to the lack of efficacy, and one of the 2 had switched to the 3rd one. One of the 2 cases treated with ETA showed refractory arthritis; subsequently required to switch to the 2nd Bio (TCZ) followed by the 3rd Bio (IFX) to achieve a clinical remission. A case treated with TCZ showed no clinical improvements; she finally attained clinical remission after switching to the 2nd Bio, IFX. During the TNFi therapy, clinical improvement was observed in joint pain/swelling (6/7), cystic swelling (2/7), uveitis (3/4), and skin rash (4/7), except in camptodactyly (0/7) (Fig.1). On the other hand, patients treated with IL-6i showed poor clinical response. The accumulated continuation rate after 3 years of each biologic therapy was 85.7% in TNFi and 0% in IL-6i (p=0.022, the log-rank test) (Fig.2). Of 4 patients with uveitis at initiating biologic agents, 3 patients treated with IFX attained substantial improvement of uveitis, while one treated with TCZ did not. The other 2 patients who had started biologic therapy without uveitis kept uveits free by the age of 14.6 or 15.3 years old at their last visit.
Conclusion: TNF inhibitors appear to be more effective than IL-6 inhibitor in the treatment of EOS, and may have a potential to prevent the onset of uveitis.


Disclosure:

T. Nagakura,
None;

T. Yamatou,
None;

T. Kubota,
None;

H. Wakiguchi,
None;

Y. Yamasaki,
None;

Y. Nonaka,
None;

T. Takezaki,
None;

H. Akaike,
None;

Y. Nerome,
None;

H. Imanaka,
None;

S. Takei,

Chugai,

2,

Bristol-Myers Squibb,

2,

Eisai,

2,

Takeda,

2.

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