[Clinical patterns and treatment of XXXX in a French department].

@entity1 @entity6794 @entity3403 @entity7250 @entity803 @entity204 @entity288
BACKGROUND: @entity6794 ( @entity6794 ) is a rare inflammatory @entity7250 for which accurate epidemiological data are limited and therapy remains a challenge. The primary study aim was to examine all cases of @entity6794 observed in our regional department over a 15-year period in order to describe the relevant characteristics and outcome under therapy. @entity1 AND METHODS: The medical records of all @entity1 with @entity6794 from 1997 to 2012 in the Marne department of France were studied retrospectively. Clinical and histological characteristics, comorbidities, therapeutic modalities and outcome were analysed. RESULTS: Forty-two @entity1 were included (30 @entity1 , 12 @entity1 ). A classical, ulcerative form was found in 39 cases and @entity6794 was multifocal in 28 cases. The number of lesions did not differ according to age or the presence of comorbidities. The most frequent first-line treatments were @entity3403 (23 cases) and oral @entity288 (15 cases), regardless of age, number of lesions or existence of comorbidities. Complete remission of @entity6794 was obtained in 38 cases (median time to remission: 3 months), with relapse occurring in 17 @entity1 (median time to relapse: 12 months after treatment withdrawal). After a median follow-up of 46 months, 8 @entity1 had died (median time to @entity204 : 26 months after treatment initiation). CONCLUSION: This is the first large French series of @entity1 presenting @entity6794 and enabling determination of the annual incidence within the Marne department at around 4.6 cases/1000,000 inhabitants. Our study illustrates the value of first-line treatment with @entity803 , which merits confirmation by further prospective, controlled studies.
