PURPOSE: The aim of this study was to assess the clinical and laboratory features of a cohort of Italian @entity1 with @entity11533 ( @entity11533 ) with particular attention on possible life-threatening complications. METHODS: The clinical charts of 41 consecutive Italian @entity1 with @entity11533 referred to our rheumatological department over the last 10 years were retrospectively examined. Data regarding clinical manifestations, laboratory features and complications were collected and compared with those reported in literature. RESULTS: The most frequent manifestations were: fever (90.2%), @entity4841 (80.4%), @entity1910 (75.6%), sore throat (53.6%), @entity625 (51.2%), @entity3329 (48.7%), @entity828 (41.4%), @entity925 (21.9%), @entity1295 (12%), @entity294 and @entity391 (9.7%), @entity1143 (9.7%), pericardial effusion (4.8%) and @entity490 (2.4%). In two @entity1 whose cases are described in detail; the course of the disease was complicated by disseminated @entity1003 , in one @entity1 with a fatal outcome. ESR, @entity1851 and leucocyte count mean values were 69.41 mm/h, 69.05 mg/l and 18,798.5 cell/mm(3) (neutrophils 84.64%), respectively. Serum ferritin levels were increased in 48.7% of @entity1 while transaminases were elevated in 42.6% of @entity1 (71% considering only @entity1 in an active phase of disease). CONCLUSION: The results of this study are in line with those reported for other cohorts of @entity1 . Even if the prognosis of @entity11533 is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, @entity11533 may rapidly worsen with life-threatening events.

XXXX : a rare disorder with a potentially fatal outcome.

@entity1 :: ('9606', 'Species') :: ['patients', 'patient'] @entity3329 :: ('MESH:D008206', 'Disease') :: ['lymphadenopathy'] @entity1143 :: ('MESH:D010996', 'Disease') :: ['pleural effusion'] @entity11533 :: ('MESH:D016706', 'Disease') :: ['adult-onset Still disease', 'AOSD'] @entity1910 :: ('MESH:D005076', 'Disease') :: ['skin rash'] @entity625 :: ('MESH:D001168', 'Disease') :: ['arthritis'] @entity294 :: ('MESH:D003967', 'Disease') :: ['diarrhoea'] @entity391 :: ('MESH:D014839', 'Disease') :: ['vomiting'] @entity1295 :: ('MESH:D005221', 'Disease') :: ['fatigue'] @entity1003 :: ('MESH:D001778', 'Disease') :: ['intravascular coagulopathy'] @entity828 :: ('MESH:C535727', 'Disease') :: ['hepatosplenomegaly'] @entity490 :: ('MESH:D015746', 'Disease') :: ['abdominal pain'] @entity4841 :: ('MESH:D018771', 'Disease') :: ['arthralgias'] @entity925 :: ('MESH:D063806', 'Disease') :: ['myalgia'] @entity1851 :: ('1401', 'Gene') :: ['CRP']
@entity11533 :: (MESH:D016706,Disease) :: ['Adult-onset Still disease']
