Rheumatol Int (2010) 30:691–693 DOI 10.1007/s00296-009-0981-9 123 CASE REPORT Relapsing polychondritis: new therapeutic strategies with biological agents Paola Sabrina Buonuomo · Claudia Bracaglia · Andrea Campana · Maya El Hachem · Andrea Diociaiuti · Antonella Insalaco · Fabrizio De Benedetti · Beatrice Chiarini Testa · Elisabetta Cortis · Giovanni Carlo De Vincentiis · Alberto G. Ugazio Received: 24 January 2009 / Accepted: 20 May 2009 / Published online: 15 August 2009  Springer-Verlag 2009 Abstract Relapsing polychondritis (RP) is a rare disease of unknown etiology characterized by recurrent episodes of inXammation resulting in the destruction of cartilaginous tissues. We describe a young girl with RP unresponsive to conventional therapy. Keywords Relapsing polychondritis · Biological agents · Pediatric Introduction Relapsing polychondritis (RP) is a severe chronic disorder, Wrst described by Jaksch-Wartenhorst [1], extremely rare in childhood (less than 30 pediatric cases have been reported) and characterized by recurrent episodes of inXammation of the cartilage and surrounding tissues mostly involving ears, nose, joints, spine, and trachea. The eyes, heart, and blood vessels, which have a biochemical structure similar to that of cartilage, may also be aVected [2]. The etiology of this rare disease is unknown. However, the evidence for an autoimmune etiology includes: patho- logical Wndings of inWltrating T cells, the presence of anti- gen–antibody complexes in the aVected cartilage, cellular and humoral responses against collagen type II and other collagen antigens and Wnally, the observation that immuno- suppressive drugs most often induce remission. RP may occur at any age; however, it is more common in the Wfth decade of life. Although RP has been reported in all races, very few data are available for non-Caucasians. Diagnosis is based on typical clinical manifestations and on the modi- Wed diagnostic criteria proposed by McAdam et al. [3]. We report a young girl presenting with saddle-nose deformity, auricular chondritis and dysphonia because of laryngotracheal involvement. Case report A 14-year-old Romanian girl presented with a 6-month his- tory of intermittent pain and swelling of the nose that appeared suddenly after a mild traumatic injury. She later developed painful redness and swelling of the right ear associated with dysphonia and throat pain. The right ear cartilage was swollen, red, painful and tender with a dusky erythema sparing the lobule. Renal and liver func- tion, rheumatoid factor, antiphospholipid antibodies, anti- cardiolipin, antineutrophil cytoplasmic antibody (ANCA), antinuclear antibody (ANA) and Borrelia burgdorferi anti- bodies were normal or negative. Erythrocyte sedimentation P. S. Buonuomo (&) · C. Bracaglia · A. Campana · A. Insalaco · E. Cortis · A. G. Ugazio Department of Pediatrics, Pediatric Rheumatology, Ospedale Pediatrico “Bambino Gesù” IRCCS, Piazza S. Onofrio, 4, 00135 Rome, Italy e-mail: psabrina.buonuomo@opbg.net; savabi@libero.it M. El Hachem · A. Diociaiuti Department of Pediatrics, Pediatric Dermatology, Ospedale Pediatrico “Bambino Gesù” IRCCS, Rome, Italy B. C. Testa Department of Pediatrics, Pediatric Pneumology, Ospedale Pediatrico “Bambino Gesù” IRCCS, Rome, Italy F. De Benedetti Direzione ScientiWca, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy G. C. De Vincentiis Unit of Otolaryngology, Department of Pediatric Surgery, Palidoro, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy