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Adult-onset Still disease: a rare disorder with a potentially fatal outcome

PURPOSE: The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. METHODS: The clinical charts of 41 consecutive Italian patients with AOSD refe...

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Autores principales: Priori, Roberta, Colafrancesco, Serena, Gattamelata, Angelica, Di Franco, Manuela, Di Tondo, Ugo, Valesini, Guido
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer International Publishing 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4389061/
https://www.ncbi.nlm.nih.gov/pubmed/26000108
http://dx.doi.org/10.1007/s13317-010-0009-1
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author Priori, Roberta
Colafrancesco, Serena
Gattamelata, Angelica
Di Franco, Manuela
Di Tondo, Ugo
Valesini, Guido
author_facet Priori, Roberta
Colafrancesco, Serena
Gattamelata, Angelica
Di Franco, Manuela
Di Tondo, Ugo
Valesini, Guido
author_sort Priori, Roberta
collection PubMed
description PURPOSE: The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. METHODS: The clinical charts of 41 consecutive Italian patients with AOSD 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%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP 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 patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). CONCLUSION: The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events.
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spelling pubmed-43890612015-05-21 Adult-onset Still disease: a rare disorder with a potentially fatal outcome Priori, Roberta Colafrancesco, Serena Gattamelata, Angelica Di Franco, Manuela Di Tondo, Ugo Valesini, Guido Auto Immun Highlights Original Article PURPOSE: The aim of this study was to assess the clinical and laboratory features of a cohort of Italian patients with adult-onset Still disease (AOSD) with particular attention on possible life-threatening complications. METHODS: The clinical charts of 41 consecutive Italian patients with AOSD 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%), arthralgias (80.4%), skin rash (75.6%), sore throat (53.6%), arthritis (51.2%), lymphadenopathy (48.7%), hepatosplenomegaly (41.4%), myalgia (21.9%), fatigue (12%), diarrhoea and vomiting (9.7%), pleural effusion (9.7%), pericardial effusion (4.8%) and abdominal pain (2.4%). In two patients whose cases are described in detail; the course of the disease was complicated by disseminated intravascular coagulopathy, in one patient with a fatal outcome. ESR, CRP 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 patients while transaminases were elevated in 42.6% of patients (71% considering only patients in an active phase of disease). CONCLUSION: The results of this study are in line with those reported for other cohorts of patients. Even if the prognosis of AOSD is considered favourable, the present study indicates that the disease is a troubling condition needing prompt intervention. Occasionally, AOSD may rapidly worsen with life-threatening events. Springer International Publishing 2010-07-10 /pmc/articles/PMC4389061/ /pubmed/26000108 http://dx.doi.org/10.1007/s13317-010-0009-1 Text en © Springer-Verlag 2010
spellingShingle Original Article
Priori, Roberta
Colafrancesco, Serena
Gattamelata, Angelica
Di Franco, Manuela
Di Tondo, Ugo
Valesini, Guido
Adult-onset Still disease: a rare disorder with a potentially fatal outcome
title Adult-onset Still disease: a rare disorder with a potentially fatal outcome
title_full Adult-onset Still disease: a rare disorder with a potentially fatal outcome
title_fullStr Adult-onset Still disease: a rare disorder with a potentially fatal outcome
title_full_unstemmed Adult-onset Still disease: a rare disorder with a potentially fatal outcome
title_short Adult-onset Still disease: a rare disorder with a potentially fatal outcome
title_sort adult-onset still disease: a rare disorder with a potentially fatal outcome
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4389061/
https://www.ncbi.nlm.nih.gov/pubmed/26000108
http://dx.doi.org/10.1007/s13317-010-0009-1
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