Document details

Six clinical cases of relapsing polychondritis: review.

Author(s): Pinto, Patrícia cv logo 1 ; Brito, Iva cv logo 2 ; Brito, José cv logo 3 ; Pinto, José cv logo 4 ; Ventura, Francisco cv logo 5

Date: 2006

Origin: Acta Médica Portuguesa


Description
Relapsing Polycondritis is a rare connective tissue disease of unknown aetiology that is manifested by inflammatory changes in cartilaginous tissues but the immune damage can spread and involve other tissues.Six patients with relapsing poycondritis treated in our service, between 1990 and 2003, were reviewed. The clinical features, age of onset, time from onset to diagnosis, disease associations and treatment were focused.The female-to-male ratio in our series was 2:4, The median age onset of symptoms was 52 years. A diagnosis delay varied from 8 months to 4 years, with a median time of 18 months. All the five patients had auricular and nasal condritis, laringotracheal involvement was detected in four patients, arthralgia and arthritis also in four patients, cardiac involvement was present in two patients, myelodysplastic syndrome in one patient and ocular disease in four patients.This is small sample study that differs from other studies in sex distribution. This disease is more frequent in females and in our sample men were more affected. The clinical manifestations and disease associations were similar to other populations. Calling attention is the best way to diminish time to diagnosis of this rare pathology. Relapsing Polycondritis is a rare connective tissue disease of unknown aetiology that is manifested by inflammatory changes in cartilaginous tissues but the immune damage can spread and involve other tissues.Six patients with relapsing poycondritis treated in our service, between 1990 and 2003, were reviewed. The clinical features, age of onset, time from onset to diagnosis, disease associations and treatment were focused.The female-to-male ratio in our series was 2:4, The median age onset of symptoms was 52 years. A diagnosis delay varied from 8 months to 4 years, with a median time of 18 months. All the five patients had auricular and nasal condritis, laringotracheal involvement was detected in four patients, arthralgia and arthritis also in four patients, cardiac involvement was present in two patients, myelodysplastic syndrome in one patient and ocular disease in four patients.This is small sample study that differs from other studies in sex distribution. This disease is more frequent in females and in our sample men were more affected. The clinical manifestations and disease associations were similar to other populations. Calling attention is the best way to diminish time to diagnosis of this rare pathology.
Document Type Article
Language Portuguese
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