Diagnosis and Management of the Overlap Syndromes of Autoimmune Hepatitis

Abstract

The overlap syndromes must be considered in patients with autoimmune hepatitis and cholestatic findings, concurrent inflammatory bowel disease or steroid-refractory disease.

Brazilian society of hepatology recommendations for the diagnosis and management of autoimmune diseases of the liver

“…Associations between PBC and other autoimmune diseases have been consistently reported and reinforce the current classification of PBC as an autoimmune condition. The reported prevalence of an overlap syndrome of PBC and AIH ranges between 2% and 20% (138) . Overlap syndrome PBCAIH is discussed in details in the corresponding topic.…”

Clinical Presentation and Outcomes of Autoimmune Hepatitis in Inflammatory Bowel Disease

“…In one study, 7-13 % of patients with AIH also had features of primary biliary cirrhosis, 6-11 % showed signs of PSC, and 5-11 % showed a cholestatic syndrome without other diagnostic features [14]. The commonalities of these syndromes include a cholestatic component and failure to respond consistently to conventional corticosteroid therapy [14]. With the association of ulcerative colitis with PSC, AIH-PSC is the most common overlap syndrome in the IBD patient.…”

“…With the association of ulcerative colitis with PSC, AIH-PSC is the most common overlap syndrome in the IBD patient. The addition of cholangiographic changes showing focal strictures and dilations of the biliary tree characteristic of PSC to a patient meeting AIHG criteria for AIH warrants the diagnosis of AIH-PSC overlap [14]. Likewise, patients with AIH may develop co-existing IBD, cholestasis, or resistance to immunosuppressive therapy.…”

“…Some argue that they should be considered more as clinical descriptions rather than distinct pathological entities [14]. Overlap syndromes may be variants of a classical autoimmune hepatitis, transitional stages in the evolution of PBC or PSC, or could be two diseases in the same individual [18].…”

“…Floreani et al examined the outcomes in seven patients with AIH/PSC overlap syndrome in relation to ''classic'' PSC patients. Treatment with prednisone and azathioprine in combination with ursodeoxycholic acid was associated with better survival in overlap patients than in adults with classic PSC [14,21]. However, one study showed that concomitant autoimmune disease such as AIH is an independent risk factor for reduced survival in patients with PSC [33].…”