Relapsing polychondritis: An autoimmune disease with many faces.
ABSTRACT Relapsing polychondritis (RPC) is a rare immune mediated disease which is associated with inflammation in cartilaginous tissue throughout the body. Especially the cartilaginous structures of ear, nose, joints and respiratory tract are affected. In around 30% of the cases an association with other diseases especially systemic vasculitis or myelodysplatic syndrome can be detected. The relative rarity of RPC has not permitted clinical trials to determine the efficacy and safety of therapy strategies. Often the medication in current use is largely empiric and based on case reports. Therefore different immunosuppressants such as cyclophosphamide, azathioprine, cyclosporine, mycophenolate mofetil and also new approaches like tumor necrosis factor alpha blockers (TNF-alpha antagonists) have been used for the treatment of severe manifestations of RPC with varying degrees of efficacy. This review gives a close look to clinical manifestation, diagnosis and also therapy options of RPC.
Article: Serum level of soluble triggering receptor expressed on myeloid cells-1 as a biomarker of disease activity in relapsing polychondritis.[show abstract] [hide abstract]
ABSTRACT: OBJECTIVES: We aimed to identify a serum biomarker for evaluating the disease activity of relapsing polychondritis (RP). METHODS: We measured and compared serum levels of 28 biomarkers potentially associated with this disease, including soluble triggering receptor expressed on myeloid cells-1 (sTREM-1), high-sensitivity C-reactive protein (hs-CRP), and cartilage oligomeric matrix protein (COMP), in 15 RP patients and 16 healthy donors (HDs). We divided the 15 RP patients into active RP (n = 8) and inactive RP (n = 7) groups, depending on the extent of the disease, and compared candidate markers between groups. The localization of membrane-bound TREM-1 in the affected tissue was examined by immunohistochemistry. RESULTS: Serum levels of sTREM-1, interferon-γ, chemokine (C-C motif) ligand 4, vascular endothelial growth factor, and matrix metalloproteinases-3 were significantly higher in RP patients than HDs. Among these markers, sTREM-1 had the highest sensitivity and specificity (86.7 and 86.7 %, respectively). Furthermore, the serum level of sTREM-1 was significantly higher in active RP patients than inactive RP patients (p = 0.0403), but this was not true for hs-CRP or COMP. TREM-1 was expressed on endothelial cells in RP lesions. CONCLUSIONS: The serum level of sTREM-1 may be a useful marker of disease activity in RP.Modern Rheumatology 02/2013; · 1.58 Impact Factor
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ABSTRACT: There is no standardized therapeutic protocol for relapsing polychondritis (RP). Emergence of biologics holds much hope in the management of this connective tissue disease. To evaluate the efficacy and safety of biologics in patients with active RP. A systematic review of the literature using PubMed was performed through December 2010. MeSH terms and keywords were used relating to RP and biologics. All papers reporting the efficacy and/or safety of biologics in RP were selected. Reference lists of included papers were also searched. All publications relate to case series or isolated case reports. No randomized controlled trial has been performed. Thirty papers that included 62 patients were published. These patients were treated with TNFα blockers (n = 43), rituximab (n = 11), anakinra (n = 5), tocilizumab (n = 2), and abatacept (n = 1). The endpoint of treatment differs from 1 publication to the other and therefore makes the comparison of efficacy among the various biologics difficult. Biologics were effective in 27 patients, partially effective in 5 patients, and not effective in 29 patients. Safety appeared to be good. However, 4 deaths were recorded (2 sepsis, 1 postoperatively after aortic aneurysm surgery, and 1 after accidental dislocation of the tracheostomy device). The experience with biologics in RP is very limited and their real efficacy and indications need to be better defined. Randomized controlled trials, although difficult to perform because of the rarity of RP, are needed to determine the place of biologics in the treatment strategy of this orphan disease.Seminars in arthritis and rheumatism 11/2011; 41(5):712-9. · 4.72 Impact Factor