Association of autoimmune hepatitis and systemic lupus erythematodes: A case series and review of the literature

Table 1 Review of literature

Author	Year of publication	Patients (n)	Sex	Age(in year)	Clinical presentation	Liver enzymes	Outcome
Chattopadhyay et al[15]	2011	1	Female	20	Fever, polyarthritis, jaundice	Elevated	Remission
Choi et al[16]	2008	1	Female	Unknown	Butterfly-type facial erythema	Elevated	Remission
Chowdhary et al[8]	2008	6	Unknown	Unknown	Unknown	Unknown	Unknown
Deen et al[17]	2009	4	3 female 1 male	Median 11 Range 11-13	Jaundice, splenomegaly, cutaneous involvement, articular involvement, proteinuria (> 0.5 g/d), cardiopulmonary involvement	Elevated	Remission in all cases
Efe et al[18]	2011	4	Female	Median 35	Unknown	Elevated	Unknown
				Range 27-40
Iwai et al[19]	2003	1	Female	60	Elevated liver enzymes	Elevated	Remission
Kaw et al[20]	2006	1	Female	34	Arthralgia, myalgias, skin rash, nausea	Elevated	Remission
Kooy et al[21]	1996	1	Unknown	Unknown	Unknown	Elevated	Remission
Koshy et al[22]	2012	1	Female	30	Jaundice, abdominal distention	Elevated	Exitus letalis1
Mackay et al[11]	1999	1	Female	16	Failure to thrive, jaundice, non-erosive arthritis, oral aftous lesions	Elevated	Progress
Moriwaki et al[23]	1987	1	Unknown	Unknown	Unknown	Unknown	Unknown
Satoh et al[24]	1997	2	Unknown	Unknown	Unknown	Unknown	Remission
Suzuki et al[25]	1993	1	Female	33	Intermittend fever, polyarthralgia, cutaneous involvement, pericardial effusion	Elevated	Remission
Takahashi et al[26]	2007	1	Female	69	Fever, cough, pleural and pericardial effusion	Elevated	Remission
Tojo et al[27]	2004	5	Female	Median 43	Raynaud’s phenomenon, arthralgia, butterfly-type facial erythema, dry mouth, jaundice, edema, ascites, pleural effusion, struma, skin ulcer, fatigue, fever	Elevated in 4 of 5 cases	Remission in all cases
				Range 21-56
Usta et al[28]	2007	1	Female	12	Jaundice, hepatosplenomegaly, polyarthralgia, malaise, butterfly-type facial erythema, arthritis	Elevated	Stable
Yamasaki et al[29]	2004	1	Female	48	Ascites	Elevated	Remission
Yoon et al[30]	2003	2	Female	Unknown	Unknown	Elevated	Unknown

¹Patient died due to acute liver failure.

Table 2 Patient characteristics

	Case 1	Case 2	Case 3	Case 4	Case 5	Case 6
Demographic data
Age at time of diagnosis (yr)	43	59	40	60	27	45
Sex	Female	Female	Female	Female	Female	Female
Year of diagnosis	2012	2003	2006	2001	1994 (SLE)	1991
					2005 (AIH)
Laboratory tests
Hemoglobin (g/dL)	10.7	NA	NA	14.7	13	13.4
Leukocytes (/μL)	0.7	NA	NA	8.9	4.84	8.7
Platelets (/μL)	21	NA	NA	291	232	181
AST (U/L)	316	170	59	307	125	323
ALT (U/L)	99	164	53	298	185	413
Total bilirubin (mg/dL)	8.9	1	1.8	0.83	0.9	4.1
IgG (g/L)	44	19.3	6.77	10.13	29	29
C3 (mg/dL)	4.1	Normal	Low	NA	0.14	NA
C4 (mg/dL)	4.1	Normal	Low	NA	0.14	NA
ANA, homogeneous pattern (titer)	1:2560	1:320	1:640	1:1280	1:160	1:1280
SMA (titer)	1:2380	1:320	Negative	1:60	1:160	Negative
Anti-dsDNA (units)	39	941	Negative	Negative	37	> 200
RNP-Sm (immunoblot)	NA	Positive	NA	NA	Positive	NA
Liver biopsy (Grading/staging1)	Cirrhosis (G2/F4)	Cirrhosis (G1/F4)	Cirrhosis (G3/F4)	Fibrosis (G1/F3)	Fibrosis (G2/F1)	Fibrosis (G3/F3)
	Chronic hepatitis	Chronic hepatitis	Chronic hepatitis	Chronic hepatitis	Chronic hepatitis	Chronic hepatitis
		NASH			PSC
Clinical symptoms of SLE	Arthritis	Arthritis	Arthritis	Arthritis	Arthritis	Arthtitis
	Photosensitivity	Malar rash		Malar rash		Malar rash
	Oral ulcers
	Serositis
Therapy regime	Prednisolone	Prednisolone	Prednisolone	Prednisolone	Prednisolone	Prednisolone
	Azathioprine	MMF	Azathioprine	Azathioprine	Azathioprine	Azathioprine
	Hydroxychloroquine		Hydroxychloroquine	Hydroxychloroquine		Cyclophosphamide
				Cyclophosphamide
Course of disease	Remission	Remission	Remission	Remission	Remission	Remission
			Diagnosis of HCC in 2012

¹Scheuer classification for grading and staging of chronic hepatitis. AIH: Autoimmune hepatitis; ALT: Alanine aminotransferase; ANA: Anti-nuclear antibodies; anti-dsDNA: Anti-double stranded DNA; anti-KLM-1: Anti-liver-kidney microsome type 1; anti-SMA: Anti-smooth muscle; AST: Aspartate transaminase; HCC: Hepatocellular carcinoma; MMF: Mycophenolate mofetil; NASH: Non-alcoholic steatohepatitis; NA: Not applicable; PSC: Primary sclerosing cholangitis; SLE: Systemic lupus erythematodes.