Autoimmune Overlap Syndromes

Woodward, Jeremy; Neuberger, James

doi:10.1053/jhep.2001.23316

Cited by 108 publications

(86 citation statements)

References 112 publications

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“…It is now well-established that a few patients can exhibit, either simultaneously or sequentially, some of the features of primary biliary cirrhosis (PBC) or of primary sclerosing cholangitis (PSC), two chronic cholestatic conditions of presumed autoimmune origin. 2 The early recognition of these overlap syndromes is of importance because it may lead to appropriate treatment and follow-up. Whereas PBC diagnosis is facilitated by the detection of antimitochondrial antibodies in the serum, that of PSC largely depends on cholangiographic evidence of multiple strictures of the biliary tract.…”

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confidence: 99%

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

et al. 2009

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The development of sclerosing cholangitis (SC) is observed in up to 50% of children followed up for autoimmune hepatitis (AIH). In adults, the prevalence is less known, although a recent study found evidence of SC in 10% of AIH patients using magnetic resonance cholangiopancreatography (MRCP). The aim of this study was to assess prospectively the prevalence of SC in adults with AIH. Fifty-nine consecutive patients with AIH diagnosed according to International Autoimmune Hepatitis Group score (women, 71%; mean age, 48 years; cirrhosis, 23%) underwent both MRCP and percutaneous liver biopsy. Twenty-seven patients with cirrhosis of nonbiliary or non-autoimmune etiology served as controls. Fourteen AIH patients (24%) showed mild MRCP abnormalities of intrahepatic bile ducts (IHBDs). None had abnormal common bile duct or convincing evidence of SC on MRCP or biopsy. A diagnosis of overlapping SC was nevertheless retained in one patient with MRCP abnormalities who subsequently developed symptomatic cholestasis despite corticosteroid therapy. Fibrosis score was the only independent parameter associated with bile duct abnormalities on MRCP (odds ratio 2.4; 95% confidence interval 1.4-4.7) and the percentage of patients with IHBD MRCP abnormalities was not different among F3-F4 AIH patients (n ‫؍‬ 24) and cirrhotic controls (46% versus 59%; NS). Conclusion: In this cohort of adult patients with AIH, the prevalence of SC was much lower than previously reported (1.7%). Mild MRCP abnormalities of IHBD were seen in a quarter of patients, but these abnormalities resulted from hepatic fibrosis and not SC. In the absence of cholestatic presentation, MRCP screening does not seem justified in adult-onset AIH. (HEPATOLOGY 2009;50:528-537.)

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Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

et al. 2009

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“…165 In alternativa è possibile attendere i risultati e considerare successivamente l'opportunità dell'associazione con gli immunosoppressori. La terapia di combinazione è obbligatoria nei pazienti con AIH e valori molto elevati di ALP per ottenere la remissione clinica e ridurre lo sviluppo della cirrosi e il ri- Gli effetti favorevoli della terapia di combinazione sulle alterazioni bioumorali e/o sulla progressione della fibrosi nella OS AIH/PBC sono stati descritti anche da altri Autori in studi retrospettivi o prospettici, 151,153,154 anche se la frequenza complessiva dei non responders e la possibilità di un'evoluzione negativa della malattia è maggiore rispetto a quella osservata nell'AIH isolata. 164,166,167 È dunque non ancora chiara se l'evoluzione clinica della OS AIH/PBC sia differente da quella delle forme isolate di AIH o di PBC.…”

Section: Sindromi Da Overlapunclassified

“…154 La remissione delle OS AIH-PSC con corticosteroidi e azatioprina , talvolta associati all'UDCA, si ottiene in una piccola percentuale di casi. La risposta del trattamento immunosoppressivo sembra essere più favorevole nei bambini che negli adulti, probabilmente perché la alterazioni epatitiche sono meno marcate di quelle dei tratti biliari.…”

Section: Sindromi Da Overlapunclassified

Le malattie infiammatorie immuno-mediate (IMID) di interesse internistico: fisiopatologia, aspetti clinici e prospettive di terapia

Fontanella¹,

Nardi²,

Masina³

et al. 2017

Ital J Med

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<img src="/public/site/images/pgranata/rass.jpg" alt="" /> Le IMID: inquadramento introduttivo A. Fontanella, G. UomoInfiammazione e IMID T. d’Errico, M. LaccettiMalattia IgG4-relata C. Mastrobuoni, G. UomoIMID in ematologia F. Rezzonico, A. MazzoneIMID in reumatologia A. Parisi, R. Buono, R. Russo, G. UomoIMIDs in neurologia G.T. Maniscalco, C. FlorioIMID in diabetologia R. Nicosia, C. RicordiIMIDs in endocrinologia M.R. Poggiano, V. NuzzoMalattie infiammatorie intestinali M. Salice, L. Calandrini, C. Praticò, M. Mazza, A. Calafiore, G. Carini, C. Calabrese, A. Belluzzi, F. Rizzello, P. Gionchetti, M. CampieriLa malattia celiaca nel terzo millennio: nuove prospettive su patogenesi, clinica, diagnosi e terapia G. Caio, F. Giancola, R. De Giorgio, U. VoltaMalattie epato-biliari autoimmuni M. Visconti, L. Fontanella, G. Marino MarsiliaPancreatiti autoimmuni P.G. Rabitti, R. BoniIMID e tumori F. GallucciLa sindrome infiammatoria da ricostruzione immune I. Ronga, G. UomoLe malattie autoinfiammatorie M. Gattorno, A. BrucatoIl rischio cardio-vascolare nelle IMIDs A. Fontanella, P. Gnerre, R. NardiQuale ruolo degli inibitori delle PCSK9 in Medicina Interna nella prevenzione cardio-vascolare in alternativa alle terapie tradizionali? P. Gnerre, P. Zuccheri, M. Campanini, G. Pinna, R. NardiTrombo-embolismo venoso e malattie autoimmuni sistemiche A. Fontanella, P. Gnerre, R. NardiIMID nell’anziano: cenni sul trattamento M. MasinaFarmaci biosimilari e farmaci innovativi in reumatologia: quale futuro? M. Todoerti, C. Montecucco

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“…W tych przypadkach, szczególnie postaci zaawansowanych z towarzyszącym brakiem dróg żółciowych (ductopenia), są duże trudności z rozróżnieniem PBC i PSC zwłaszcza, że histologiczne i immunologiczne kryteria nie są dla nich jednoznacznie różnicujące [19,20]. Tak więc zespół nakładania tych chorób może istnieć, ale jest trudny do zdefiniowania i wyjątkowo rzadko prezentowany w literaturze.…”

Section: Zasady Diagnostyki Zespołów Nakładaniaunclassified

Współczesne poglądy na postępowanie diagnostyczne i terapeutyczne w zespołach nakładania (overlap syndromes) w chorobach wątroby

Serafińska¹,

Simon²

2011

Medical Science Review - Hepatologia

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Autoimmune Overlap Syndromes

Cited by 108 publications

References 112 publications

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

Prevalence of sclerosing cholangitis in adults with autoimmune hepatitis

Le malattie infiammatorie immuno-mediate (IMID) di interesse internistico: fisiopatologia, aspetti clinici e prospettive di terapia

Współczesne poglądy na postępowanie diagnostyczne i terapeutyczne w zespołach nakładania (overlap syndromes) w chorobach wątroby

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