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RELAPSING POLYCHONDRITIS IN CHILDHOOD: THREE CASE REPORTS, COMPARISON WITH ADULTHOOD DISEASE AND LITERATURE REVIEW
Childhood
Arthritis
Costochondritis
Epiphyseal Cartilage
Respiratory Involvement
Author
Affilliation
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil. / Fundação Oswaldo Cruz. Intituto Fernandes Figueira. Departmento de Pediatria. Rio de Janeiro, RJ, Brasil.
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil.
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil. / Municipal Hospital Jesus. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil.
Laboratório de Patologia Cirúrgica. Rio de Janeiro, RJ, Brasil.
Clínica de Diagnóstico Por Imagem. Clínica Multi-Imagens. Rio de Janeiro, RJ, Brasil.
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil. / Universidade do Estado do Rio de Janeiro. Hospital Universitário Pedro Ernesto. Rio de Janeiro, RJ, Brasil.
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil.
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil. / Municipal Hospital Jesus. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil.
Laboratório de Patologia Cirúrgica. Rio de Janeiro, RJ, Brasil.
Clínica de Diagnóstico Por Imagem. Clínica Multi-Imagens. Rio de Janeiro, RJ, Brasil.
Universidade Federal do Rio de Janeiro. Instituto de Puericultura e Pediatria Martagão Gesteira. Divisão de Reumatologia Pediátrica. Rio de Janeiro, RJ, Brasil. / Universidade do Estado do Rio de Janeiro. Hospital Universitário Pedro Ernesto. Rio de Janeiro, RJ, Brasil.
Abstract
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
reXect 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 Xares.
Keywords
Relapsing PolychondritisChildhood
Arthritis
Costochondritis
Epiphyseal Cartilage
Respiratory Involvement
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