Severe Complications and Immunosuppressive Treatments in 33 Patients with Relapsing Polychondritis

Toshiki Nakajima¹, Hajime Yoshifuji¹, Chikashi Terao², Kosaku Murakami¹, Nobuo Kuramoto¹, Ran Nakashima¹, Yoshitaka Imura³, Masao Tanaka¹, Koichiro Ohmura¹ and Tsuneyo Mimori¹, ¹Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, ²Genomic Center, Center for Genomic Medicine, Kyoto University Graduate School of Medicine, Kyoto, Japan, ³Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: human leukocyte antigens (HLA), polychondritis and treatment

Session Information

Date: Monday, November 14, 2016

Title: Miscellaneous Rheumatic and Inflammatory Diseases - Poster II

Session Type: ACR Poster Session B

Session Time: 9:00AM-11:00AM

Background/Purpose: Since relapsing polychondritis (RP) is a rare disease as the prevalence rate is 0.0004% in Japan, its severe complications, prognosis and immunosuppressive therapies have not been fully understood or established. We recently reported association of disease onset and 3 HLA haplotypes (DQB1*05:02, B*67:01 and DRB1*16:02 in linkage disequilibrium) in 102 patients with RP (Terao C et al. Rheumatology (Oxford) doi: 10.1093/rheumatology/kew233), however, we did not survey clinical information such as treatment course. In the present study, we investigated detailed clinical information of 33 patients in a single center.

Methods: We examined medical records of 33 patients with RP who satisfied Damiani’s criteria. HLA haplotypes and anti-type II collagen antibodies were assayed in some of these patients.

Results: Tracheobronchial (TB), central nerve system (CNS), and aortic complications were seen in 17 (51.5%), 4 (9.1%), and 2 (6.0%) patients, respectively. One (3.0%) died, and two (6.0%) with CNS lesions failed into bed-bound status. Recurrence was seen in 25 (75.8%). Positivity of HLA-DQB1 05:02 was 17.6% (3/17),　higher than 2.6% in healthy Japanese. Two of the 3 patients with DQB1 05:02 had TB lesions and one needed TCZ. B 67:01 and DRB1 16:02 were positive in 2 patients (11.8%) and linked with DQB1 05:02. Anti-type II collagen antibodies were seen in 29.2% (7/24). Methotrexate (MTX), azathioprine (AZP), intravenous cyclophosphamide (IVCY), infliximab (IFX) and tocilizumab (TCZ) were used along with glucocorticoid in 10 (30.3%), 8 (24.2%), 5 (15.2%), 5 (15.2%) and 4 (12.1%), respectively. Three patients with TB lesions and 2 with CNS lesions were treated with IVCY, and response rates were 100% and 50%, respectively. IFX was used and partially effective in 1 with TB lesion, 1 with CNS lesion, and 3 patients complicated with Behçet disease. TCZ was used in 3 patients with TB lesions, and response rate was 33%.

Conclusion: The linkage with certain haplotypes of HLA and the positivity of autoantibodies (~30%) consolidate that RP is an autoimmune disease. IVCY showed a good response in patients with TB lesions in the present study. The prognosis of patients with CNS lesions was poor. Further collection of cases is required to elucidate pathophysiology and improve treatments.

Disclosure: T. Nakajima, None; H. Yoshifuji, None; C. Terao, None; K. Murakami, None; N. Kuramoto, None; R. Nakashima, None; Y. Imura, None; M. Tanaka, None; K. Ohmura, None; T. Mimori, None.

To cite this abstract in AMA style:

Nakajima T, Yoshifuji H, Terao C, Murakami K, Kuramoto N, Nakashima R, Imura Y, Tanaka M, Ohmura K, Mimori T. Severe Complications and Immunosuppressive Treatments in 33 Patients with Relapsing Polychondritis [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/severe-complications-and-immunosuppressive-treatments-in-33-patients-with-relapsing-polychondritis/. Accessed .

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