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• W134771246 abstract "Relapsing polychondritis (RP) is a rare multisystem disorder characterized by widespread, destructive, inflammatory lesions of the cartilage. The peak incidence is between the fourth and the sixth decades and less than 5% of the published cases concern children. The disease includes cartilage inflammation of ears, nose, and larynx. Recurrent flares may lead to floppy ears, saddle nose, and laryngotracheal stenosis. Ear inflammation involves the cartilaginous portion of the pinna and spares the noncartilaginous lobe. Arthritis is common and non-erosive. Most of the children present with conjunctivitis or episcleritis during the follow-up. Hearing impairment occurs in less than 10% of cases. Histology is not mandatory for the diagnosis and may show granular deposits of immunoglobulins and C3, as well as CD4+ lymphocytes and plasma cells associated to a loss of basophilic staining of the cartilage matrix. Erythrocyte sedimentation rate (ESR) is usually elevated but normality, a normal ESR, does not exclude the diagnosis of RP. Autoantibodies against collagen or matrilin-1 have been reported in some cases but with lack of specificity; therefore, the diagnosis remains mainly clinical and the Michet criteria can be used to diagnose childhood onset RP (> Table 157.1). An association to other autoimmune disorders is less common than in adults (less than 15% of reported pediatric cases); when present, it may include hematologic diseases, inflammatory diseases (Goodpasture, Henloch-Shonlein purpura), and an overlapping syndrome with Behcet disease called MAGIC syndrome (Mouth And Genital ulcers with Inflammed Cartilage syndrome). Differential diagnoses in pediatric practice also include rare inherited degenerative chondropathies, Chronic Infantile Neurologic Cutaneous and Articular (CINCA) syndrome/Neonatal Onset Multisystem Inflammatory Disease (NOMID), Wegener’s granulomatosis, and congenital syphilis. Silverman syndrome can be discussed in the presence of saddle nose and ear destruction, but normal X-rays usually rule out this diagnosis. Indeed, some pediatric RP cases have been first diagnosed" @default.
• W134771246 created "2016-06-24" @default.
• W134771246 creator A5043853010 @default.
• W134771246 date "2012-04-05" @default.
• W134771246 modified "2023-09-24" @default.
• W134771246 title "Miscellaneous Conditions Associated with Arthritis" @default.
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• W134771246 doi "https://doi.org/10.1007/springerreference_301602" @default.
• W134771246 hasPublicationYear "2012" @default.
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