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Autologous Stem Cell Transplantation in Relapsing Polychondritis
Published in Richard K. Burt, Alberto M. Marmont, Stem Cell Therapy for Autoimmune Disease, 2019
Falk Hiepe, Andreas Thiel, Oliver Rosen, Gero Massenkeil, Gerd-Rüdiger Burmester, Andreas Radbruch, Renate Arnold
Although relapsing polychondritis is a separate clinical entity, up to 30% of patients also suffer from another inflammatory disease (Table 1). The most common associated disease is vasculitis, which can affect vessels of all sizes. Five to 14% of all patients with relapsing polychondritis have biopsy-confirmed leukocytoclastic vasculitis, ranging from isolated cutaneous leukocytoclastic vasculitis to aortitis. Although vasculitis most commonly leads to dilatation of the aortic ring and ascending aorta, aneurysm of the descending thoracic or abdominal aorta can also occur. Aortic rupture has also been reported. Dermatological and renal manifestations, neuropathies, audiovestibular abnormalities, and episcleritis are most likely due to microscopic angiitis. Vasculitis seems to worsen the prognosis of relapsing polychondritis.17 Relapsing polychondritis is infrequently accompanied by defined vasculitides like Wegener’s granulomatosis and polyarteritis nodosa.18 The co-occurrence of relapsing polychondritis and Behcet’s disease has also been reported; this constellation was named “MAGIC syndrome” (mouth and genital ulcers with inflamed cartilage syndrome).19 Roughly 25% of patients with relapsing polychondritis have associated autoimmune diseases such as systemic lupus erythematosus, rheumatoid arthritis, mixed connective tissue disease, Sjogren’s syndrome, and hypothyroidism.
Connective Tissue Diseases: ENT Complications
Published in John C Watkinson, Raymond W Clarke, Louise Jayne Clark, Adam J Donne, R James A England, Hisham M Mehanna, Gerald William McGarry, Sean Carrie, Basic Sciences Endocrine Surgery Rhinology, 2018
There is an association of autoimmune disorders such as Behçet’s disease with relapsing polychondritis and inflammation of the auricular cartilages seen as part of the MAGIC syndrome (mouth and genital ulcers with inflamed cartilage).
Relapsing polychondritis: state-of-the-art review with three case presentations
Published in Postgraduate Medicine, 2021
Bogna Grygiel-Górniak, Hamza Tariq, Jacob Mitchell, Azad Mohammed, Włodzimierz Samborski
Approximately 30% of RPC patients have an autoimmune disease or other systemic disorder, which may occur before, during, or after the diagnosis[36]. The most common associated disease is vasculitis, present in 12–18% of patients (mainly GPA)[6]. In some RPC patients, Behcet’s disease can develop (MAGIC syndrome – mouth and genital ulcers with inflamed cartilage)[41]. Other connective tissue disorders co-existed in RPC include SLE, Sjogren syndrome, scleroderma rheumatoid arthritis, and spondyloarthritis [66,67]. In some cases, myelodysplastic syndrome is diagnosed [63,68].