Innumerable Liver Masses in a Patient with Autoimmune Hepatitis and Primary Sclerosing Cholangitis Overlap Syndrome (original) (raw)
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FUKUSHIMA JOURNAL OF MEDICAL SCIENCE, 2013
A 60-yearold woman was admitted to our hospital because of anorexia and vomiting. Hematology revealed elevated levels of hepatobiliary enzymes and positive results for antinuclear antibody (ANA) and antimitochondrial (AMA) M2 antibody. Immunoglobulin (Ig) G and IgM levels were extremely elevated, 3,379 mg/dl and 4,250 mg/dl, respectively. A diagnosis of primary biliary cirrhosis and autoimmune hepatitis (PBC-AIH) overlap syndrome was made. However, IgM levels as high as the level of 4,250 mg/dl in this case has not been reported previously. This case may be of value in studying elevated IgM levels with PBC-AIH overlap syndrome and relationships to the mechanism of onset.
Overlap syndrome of primary sclerosing cholangitis and autoimmune hepatitis
European Journal of Gastroenterology & Hepatology, 2001
We report a 46-year-old patient with the typical biochemical, histological and cholangiographic ®ndings of primary sclerosing cholangitis (PSC) whose clinical and laboratory ®ndings would also qualify her for the diagnosis of de®nite autoimmune hepatitis (AIH), according to the aggregate score of the International Autoimmune Hepatitis Group. We suggest that this patient may represent an example of the overlapping syndrome of PSC and AIH. Eur
Internet Journal of Rheumatology and Clinical Immunology, 2016
Autoimmune cholangitis is the antimitochondrial antibody-negative autoimmune hepatopathy with clinical and histological features similar to that of primary biliary cirrhosis. Autoimmune cholangitis has a predominant cholestatic phase. However, transaminasemia might be dominant in certain patients, indicating associated autoimmune hepatitis. Such an autoimmune hepatopathy has been termed as hepatic overlap syndrome. Due to the autoimmune nature of the disease, associated diseases of other organs have been reported. We report a case of an adult female with a very rare presentation of hepatic overlap syndrome of autoimmune cholangitis and autoimmune hepatitis associated with hypopituitarism.
Hepato-gastroenterology
We have assessed two different overlap syndrome groups in patients with AIH-PBC and AIH-AIC, with respect to therapy response and outcome. In this retrospective, non-randomized study, a total of 22 overlap cases were collected, 12 of those had a simultaneous form of AIH-PBC and 10 of those with AIH-AIC. Two groups were compared in terms of clinical, biochemical, immunological, histological features and response to treatment. The mean follow-up time was 31.7 +/- 11.0 mo in AIH-PBC and 41.1 +/- 29.6 mo in AIH-AIC, respectively. The clinical and laboratory characteristics at presentation were not significantly different between the two groups, except a higher serum IgM level and lower AIH score in AIH-PBC group compared to AIH-AIC group (p < 0.05). First-line treatment was UDCA alone in 3 of AIH-PBC group and combination of UDCA and immunsuppressives in the remaining AIH-PBC (n = 9) and in all of the AIH-AIC (n = 10). During follow-up, only one of 10 patients in IIH-AIC group, but s...
Alimentary Pharmacology & Therapeutics, 2008
Background Primary sclerosing cholangitis/autoimmune hepatitis (PSC/AIH) and primary biliary cirrhosis/AIH (PBC/AIH) overlap syndromes are poorly defined variants of AIH. Few large patient series exist, and there are little data on long-term outcomes.Aim To compare presentation, clinical course and outcome of patients with PSC/AIH and PBC/AIH, with patients with definite AIH.Methods Two hundred and thirty-eight AIH patients were compared with 10 PBC/AIH patients and 16 PSC/AIH patients presenting consecutively between 1971 and 2005 at a single centre.Results Autoimmune hepatitis patients were significantly more likely to present with jaundice (69.4% vs. 25%; P = 0.0145) than PBC/AIH patients. Median serum aspartate aminotransferase activity at presentation was higher in AIH patients compared with PBC/AIH and PSC/AIH patients respectively (620 vs. 94 vs. 224 IU/L; P < 0.05). PBC/AIH patients demonstrated no response to standard AIH therapy more frequently than AIH patients (25% vs. 0.8%; P = 0.0057). Significant reduction in survival was identified between patients with PSC/AIH and those without (hazard ratio: PSC/AIH vs. AIH = 2.08, PSC/AIH vs. PBC/AIH = 2.14; P = 0.039).Conclusions Patients with PSC/AIH have severe disease and significantly worse prognosis than patients with AIH or PBC/AIH. Recognition and close follow-up of this cohort are warranted.
Overlap Syndrome between Primary Biliary Cholangitis and Primary Sclerosing Cholangitis
ACG case reports journal, 2018
Overlap syndrome indicates the coexistence of 2 or more autoimmune liver diseases in the same individual, occurring simultaneously or sequentially. Cases of overlap of autoimmune hepatitis (AIH) with primary biliary cholangitis (PBC) and of AIH with primary sclerosing cholangitis (PSC) are known and have defined criteria for diagnosis. Overlap between PBC and PSC has been reported in only a few case reports. The cause for the rarity of this entity compared to other overlap syndromes is unclear. We present a case of an overlap syndrome of PBC with PSC in a 35-year-old woman.
Journal of …, 2011
Some patients present with overlapping features between disorders within the spectrum of autoimmune liver diseases (i.e. autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC)) and are commonly classified as having an ''overlap syndrome''. Standardized definitions of ''overlap syndromes'' are lacking. The aim of this report by the International Autoimmune Hepatitis Group (IAIHG) is to evaluate if there are important reasons to classify conditions with overlapping features between autoimmune liver diseases as separate diagnostic entities. Definition of diagnostic criteria for overlap conditions can only be arbitrary. The IAIHG scoring system for diagnosis of AIH has been widely used to diagnose ''overlap syndromes'', but was not intended for such use and has not proven to be an efficient tool for this purpose. Some patients with overlapping features between a cholestatic and hepatitic disorder appear to benefit from treatment with a combination of ursodeoxycholic acid and immunosuppressants, but this strategy is not evidence-based, and it seems unjustified to define new diagnostic groups in this regard. The IAIHG suggests that patients with autoimmune liver disease should be categorized according to the predominating feature(s) as AIH, PBC, and PSC/small duct PSC, respectively, and that those with overlapping features are not considered as being distinct diagnostic entities. The IAIHG scoring system should not be used to establish subgroups of patients. Patients with PBC and PSC with features of AIH should be considered for immunosuppressive treatment. Due to the low prevalence of such ''overlap syndromes'', prospective interventional therapeutic trials cannot be expected in the foreseeable future.
Overlap Syndromes of Autoimmune Hepatitis: An Open Question
Digestive Diseases and Sciences, 2012
The headword ''overlap syndromes'' of liver diseases includes the coexistence of autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. These syndromes often represent a diagnostic and therapeutic challenge for hepatologists; it remains unclear whether these overlap syndromes form distinct entities or they are only variants of the major autoimmune liver diseases. The most frequent reported association occurs between autoimmune hepatitis and primary biliary cirrhosis, whereas the overlap between autoimmune hepatitis and primary sclerosing cholangitis is less frequent, typically at young age and often attendant with an inflammatory bowel disease. The choice therapy is based on ursodeoxycholic acid and immunosuppressive drugs, used at the same time or consecutively, according to the course of disease. The diagnostic scores for autoimmune hepatitis can help for diagnosis, even though their definitive soundness is lacking.