Non-classical phenotypes of autoimmune hepatitis and advances in diagnosis and treatment

Table 1 Non-classical phenotypes of autoimmune hepatitis

Non-classical phenotype	Salient features
Acute severe disease	Corticosteroids effective in 36%-100%[49]
	Protracted treatment can be complicated by infection[49]
	High mortality if no better within 2 wk of therapy[85]
	MELD score ≥ 12 identifies 97% of treatment failures[58]
Asymptomatic mild hepatitis	Common (25%-34%) but unstable state[202122878889]
	Symptoms develop in 26%-70%[2021]
	Progression possible if untreated[20212287]
	Improves quickly with therapy[22]
Atypical histological features	Centrilobular necrosis is an early acute form[18333435363792]
	Transition to interface hepatitis possible[35]
	Coincidental biliary changes lack cholestatic profile[41]
	Fatty changes may co-exist[5894]
Absent or variant serological markers	Seronegativity possible in 13%[31]
	Other features and treatment outcome similar[3132100]
	Non-standard autoantibodies possible[101102103104]
	Conventional autoantibodies may be expressed later[30]
	Screen for celiac disease[105106107108]
Concurrent cholangiographic changes	Abnormal cholangiograms in 44% with CUC[116]
	Poor outcome if biliary changes and CUC[121122123124]
	MRC abnormalities in 8% adults without CUC[117]
	MRC abnormalities may be associated with fibrosis[119]
Male gender	0.2-0.5 cases/100 000 per year[127128]
	Low frequency of concurrent immune diseases[130131132]
	No diversity of HLA DRB1*04 alleles[130131132]
	Better survival than women[136]
Non-Caucasian	Cholestatic features may be common[45141142143]
	Male predominance possible[47]
	Socioeconomic factors important[137138146147]

MELD: Model of End Stage Liver Disease; MRC: Magnetic resonance cholangiography; CUC: Chronic ulcerative colitis; HLA: Human leukocyte antigen.