Laryngotracheal stenosis requiring emergency tracheostomy as the first manifestation of childhood-relapsing polychondritis

Author(s)

Buscatti, Izabel M. ^[1] ; Giacomin, Maria Fernanda A. ^[1] ; Silva, Marco Felipe C. ^[1] ; Campos, Lucia M. A. ^[1] ; Sallum, Adriana M. E. ^[1] ; Silva, Clovis A. ^{[2, 1]}

Affiliation

^[1] Univ Sao Paulo, Fac Med, Childrens Hosp, Pediat Rheumatol Unit, Sao Paulo - Brazil

^[2] Univ Sao Paulo, Fac Med, Div Rheumatol, Sao Paulo - Brazil

• Document type: Journal article
• Source: ACTA REUMATOLOGICA PORTUGUESA; v. 38, n. 3, p. 208-211, JUL-SEP 2013.
• Web of Science Citations: 1

Abstract

Relapsing polychondritis is a rare childhood disorder of unknown etiology, characterized by inflammatory; recurrent and destructive cartilage lesions. The chondritis could be widespread and involves generally laryngeal and auricular hyaline cartilages. We described a 9 years and 4 months old girl, who presented recurrent acute laryngotracheitis and laryngotracheal stenosis, which were the first manifestations of relapsing polychondritis, and was submitted to emergency tracheostomy. She also had ear condritis and arthritis, being treated with prednisolone and methotrexate. In conclusion, we reported a rare case of relapsing polychondritis that presented a life-threatening laryngo-tracheo-bronchial disorder requiring tracheostomy. We suggest that the diagnosis of relapsing polychondritis should be considered for patients who present recurrent acute laryngotracheitis with other types of condritis, as well as musculoskeletal manifestations. (AU)

• FAPESP's process: 08/58238-4 - Autoimmunity in children: investigation of the molecular and cellular bases of early onset of autoimmunity
• Grantee: Magda Maria Sales Carneiro-Sampaio
• Support Opportunities: Research Projects - Thematic Grants
• FAPESP's process: 11/12471-2 - Ovarian reserve, antibody anti-corpus luteum and lower genital tract infection in adult women with systemic lupus erythematosus, Behçet's syndrome and Takayasu's arteritis
• Grantee: Clovis Artur Almeida da Silva
• Support Opportunities: Regular Research Grants