Relapsing polychondritis in childhood: three case reports, comparison with adulthood disease and literature review.
Relapsing polychondritis (RP) is a rare autoimmune systemic disease, especially in childhood. To report three new pediatric RP cases, to provide a literature review and to compare with adulthood disease, retrospective data collection from three childhood RP cases was observed in a Brazilian Pediatric Rheumatology Division. A literature review based on a MEDLINE database search was performed. Arthritis and auricular chondritis were present in our three patients. Two cases presented with early and severe laryngotracheal chondritis, besides initial and symptomatic costochondritis. The other case developed prominent epiphyseal plate involvement. Two patients were refractory to corticosteroids and immunosuppressants and required the use of TNF-alpha inhibitors to improve the symptoms, while corticosteroids plus methotrexate induced remission in the other patient. The literature review showed 44 cases of pediatric-onset disease in English language. Arthritis and ear chondritis are the most common initial and cumulative manifestations of RP in children and adults. Nasal and laryngotracheobronchial chondritis are also common manifestations observed during follow-up in childhood. There is also an early severity of respiratory chondritis in childhood, requiring aggressive treatment with corticosteroids, immunosuppressants and biologic agents. The data presented by those 3 children, considered in conjunction with the data from the 44 published cases, may reflect some distinguishing childhood RP features, such as more severe and frequent respiratory tract involvement, symptomatic costochondritis and the atypical pattern of persistent and destructive arthritis with epiphyseal plate involvement. Response to immunosuppressants and biologic agents is anecdotal, but steroids remain the main drug during the flares.
Pediatric Rheumatology Division, Instituto de Puericultura e Pediatria Martagão Gesteira/Federal University of Rio de Janeiro, Rua Silva Ramos, 166/apto 403-Tijuca, Rio de Janeiro 20270-330, Brazil. adrirfonseca@gmail.com
Adriana R
AR
Fonseca
Sheila K F
SK
de Oliveira
Marta C F
MC
Rodrigues
Ierecê L
IL
Aymoré
Romeu C
RC
Domingues
Flavio R
FR
Sztajnbok
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017668:Age of Onset; D001706:Biopsy; D001938:Brazil; D002648:Child; D002675:Child, Preschool; D004351:Drug Resistance; D057915:Drug Substitution; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011081:Polychondritis, Relapsing / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy; D012074:Remission Induction; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents