[ XXXX . Clinical aspects, nosologic problems. Review of the literature apropos of 30 cases].

@entity1 @entity8 @entity1148 @entity417 @entity75 @entity12390 @entity1669 @entity177 @entity134 @entity11657 @entity2883 @entity491
Thirty cases of @entity2883 are reported, 16 @entity1 and 14 @entity1 aged 49.3 +/- 14 years at the onset of their illness. The presenting symptoms were oto-rhino-laryngeal in 22 cases and pulmonary in 16 cases. @entity8 was initial in only 1 case. The complete picture comprised oto-rhino-laryngeal (29 cases), pulmonary (26 cases), renal (27 cases), articular (16 cases), muco-cutaneous (19 cases), ocular (15 cases), neurological (13 cases), and muscular (10 cases) involvement. An increased sedimentation rate, @entity491 , @entity417 and @entity1148 were common findings. The treatment comprised @entity134 therapy associated with immunosuppressor drugs (28 cases) and plasma exchanges (8 cases). External radiotherapy was used in 5 @entity1 , including 3 with @entity75 , but was not effective. The probability of survival after the initial symptoms was 84 p. 100 at 1 year, 69 p. 100 at 3 years and 59 p. 100 at 5 and 10 years. Eight @entity1 died; infection and @entity177 were the principal causes. Late relapse occurred in 3 @entity1 justifying long-term follow-up and the necessity of using the term "cure" with reserve. The clinical and therapeutic aspects are described in the light of the authors' experience and a review of the literature. The concept of localised form of the disease and bordeline forms with @entity12390 , @entity11657 , Churg and Strauss syndrome and @entity1669 are also discussed.
