Relapsing Polychondritis: An Updated Review

Abstract

Relapsing polychondritis is an immune-mediated systemic disease characterized by recurrent episodes of inflammation of cartilaginous and proteoglycan-rich tissues, resulting in progressive anatomical deformation and functional impairment of the involved structures. Auricular and nasal chondritis and/or polyarthritis represent the most common clinical features, but potentially all types of cartilage may be involved. Because of the pleomorphic nature of the disease, with non-specific symptoms at the onset, the diagnosis of relapsing polychondritis is often delayed. In this review article we provide a comprehensive look into clinical presentation, laboratory and instrumental investigations, diagnostic criteria, and therapeutic options.

Acute flare of relapsing polychondritis following surgical treatment of auricular squamous cell carcinoma

“…Given the rarity of the disease, there are no evidencebased guidelines. Besides the above-mentioned treatment options, less severe cases can be treated with colchicine 0.6 mg PO two to four times a day and nonsteroidal antiinflammatory drugs [3].…”

Reconstructive rhinoplasty with costal cartilage grafting: A case report of relapsing polychondritis

“…Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology that primarily affects cartilaginous tissues [1]. RP is characterized by recurrent episodes of inflammation, that causes progressive anatomical deformation and functional impairment of primarily the auricular cartilage, the nasal cartilage, and the respiratory tract [1,2].…”

“…Relapsing polychondritis (RP) is a rare autoimmune disorder of unknown etiology that primarily affects cartilaginous tissues [1]. RP is characterized by recurrent episodes of inflammation, that causes progressive anatomical deformation and functional impairment of primarily the auricular cartilage, the nasal cartilage, and the respiratory tract [1,2]. McAdam et al [3] proposed that RP can be diagnosed when three or more of these features are present, along with a positive biopsy from the affected tissue: (1) recurrent chondritis of both auricles; (2) nonerosive inflammatory polyarthritis; (3) chondritis of nasal cartilage; (4) inflammation of ocular structures, including conjunctivitis, keratitis, scleritis, episcleritis, and uveitis; (5) chondritis in the respiratory tract involving laryngeal and/or tracheal cartilages; and (6) cochlear and/or vestibular damage manifesting as sensorineural hearing loss, tinnitus, or vertigo.…”

“…Nasal chondritis is present in 24% of patients at the time of diagnosis and develops subsequently in 53% throughout the disease's progress [5]. Progressive nasal chondritis leads to the flattening of the nasal bridge, resulting in the painless irreversible saddle nose deformity [1]. However, the number of patients treated for saddle nose deformity remains limited due to the small number of patients reported and the case reports are heterogeneous with different outcomes [1].…”

Schub einer Polychondritis recidivans et atrophicans nach Exzision eines Plattenepithelkarzinoms an der Ohrmuschel

“…Neben den oben genannten Therapien können in weniger schweren Fällen noch Colchicin 0,6 mg 2-4-mal täglich p.o. und nichtsteroidale Antirheumatika eingesetzt werden [3].…”