Frequency of Concurrent Autoimmune Disorders in Patients With Autoimmune Hepatitis

Bittencourt, Paulo Lisboa; Farias, Alberto Queiróz; Porta, Gilda; Cançado, Eduardo Luiz Rachid; Miura, I. K.; Pugliese, Renata; Kalil, Jorge; Goldberg, Anna Carla; Carrilho, Flair José

doi:10.1097/mcg.0b013e31802dbdfc

Cited by 65 publications

(68 citation statements)

References 34 publications

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“…Concurrent extrahepatic autoimmune disorders (CAIDs) including autoimmune thyroiditis, diabetes, rheumatoid arthritis (RA) and ulcerative colitis (UC) are indeed common, ranging from approximately 20-49% of patients with AIH in various reports from different continents of the world [7][8][9][10][11][12][13][14][15][16][17][18][19] . Up to 43% of AIH patients report a family history of other autoimmune diseases in their first-degree relatives, commonly with thyroid disease and type 1 diabetes [9,15] .…”

Section: Frequency Of Concurrent Extrahepatic Diseasesmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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For many patients with autoimmune hepatitis (AIH), the presence of extrahepatic features is well recognised both at the time of presentation and during long-term follow-up. Concomitant ‘autoimmune disorders' have been described in 20-50% of patients with AIH, both in adults and children. Indeed, the presence of these associated phenomena has been incorporated into both the original and revised International AIH group scoring systems as an aid to codifying the diagnosis. In acute index presentations, non-specific joint pains sometimes flitting in nature have been reported in 10-60% of patients, and while joint swelling is uncommon, rheumatoid arthritis and mixed connective tissue disease have been reported in 2-4% of patients with AIH. For a majority of patients, these joint symptoms resolve within days of the introduction of immunosuppressive therapy. Rarer features at index presentation include a maculopapular skin rash and unexplained fever, which are features that tend to resolve quickly with treatment. Interestingly, joint pain and stiffness are also well recognised in the context of steroid withdrawal and cessation in AIH. The occasional co-presentation of AIH with coeliac disease is clinically important (1-6%), since for some patients, there is a risk of immunosuppression malabsorption, thus delaying effective treatment. Similarly, the co-existence of selective IgA deficiency (IgAD) can occur in patients with coeliac disease or in isolation. Selective IgAD as a co-existing extraheaptic feature seems to be more common in paediatric patients with AIH. For these patients, they are at an increased risk of respiratory and sinus infections. Although, typically associated with primary sclerosing cholangitis, the presence of inflammatory bowel disease (IBD; both Crohn's disease and ulcerative colitis) has been described in 2-8% of patients with AIH. Interestingly, for patients with autoimmune sclerosing cholangitis, a distinct pattern of IBD has been recently described. Other conditions have been reported at a lower frequency, including Sjogren's syndrome 1-7%, systemic lupus erythematosus 1-3% and glomerulonephritis 1%. Rarer still and at a frequency of <1% include fibrosing alveolitis, haemolytic anaemia, uveitis, mononeuritis multiplex, polymyositis and multiple sclerosis. In contrast, the reported associations between AIH and thyroiditis 8-23%, diabetes 1-10% and psoriasis 3% are commonly seen and notable in clinical practice.

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Section: Frequency Of Concurrent Extrahepatic Diseasesmentioning

confidence: 99%

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Wong

Heneghan²

2015

Dig Dis

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“…small duct PSC) [11]. Obserwuje się wówczas u chorych z AIH cechy zaostrzenia procesu zapalnego, którym towarzyszy uszkodzenie dróg żółciowych.…”

Section: Różnicowanie Zespołów Nakładania W Chorobach Wątrobyunclassified

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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“…However, shared HLAlinked susceptibility was reported to occur in AIH, PSC and AIHPSC overlap (24,177) . The impact of those overlapping features of more than one ALD in prognosis is not entirely clear, but lower response to treatment, accelerated disease progression, higher risk of treatment failure including death and requirement for Adapted from (2,7,9,10) ; AIH: autoimmune hepatitis; PBC: primary biliary cirrhosis; PSC: primary sclerosing cholangitis; ULN: upper limit of normal; ANA: antinuclear antibody; SMA: anti--smooth muscle antibody; AMA: antimitochondrial antibody; SMA: anti-smooth muscle antibody; AAA: antiactin antibody; ALP: alkaline phosphatase; AST: aspartatoaminotransferase; ALT: alaninoaminotransferase; MRCP: magnetic resonance cholangiopancreatography; ERC: endoscopic retrograde cholangiography LT were reported in subjects with AIH-PSC overlap, when compared to their counterparts with AIH (8,(175)(176)(177)(178) , but its prognosis was shown to be better when compared to pure PSC (176) . Regarding AIH-PBC overlap, several studies have shown lower response to ursodeoxicholic acid (UDCA), higher risk of disease progression and decompensation, when compared to classical PBC (139) .…”

Section: Part Iv: Overlap Syndromes Of Autoimmune Diseases Of the Livermentioning

confidence: 99%

“…The most frequent reported disorders were thyroiditis, either Hashimoto thyroiditis or Graves disease, and rheumatoid arthritis, but type 1 diabetes mellitus, Sjogren Syndrome, polymyositis, IgA deficiency, idiopathic thrombocytopenic purpura, urticaria, vitiligo, Addison's disease, inflammatory bowel diseases, celiac disease have also been associated with AIH. Sero-negative or rheumatoid arthritis and thyroiditis were the most frequent associated autoimmune diseases in Brazilian patients with AIH type I and AIH type II, respectively (1,2,10) .…”

Section: Brazilian Society Of Hepatology Recommendations For the Diagmentioning

confidence: 99%

“…Treatment of AIH is introduced to induce clinical remission, which is easily achieved in the first two months of therapy, as well as biochemical remission with normalisation of aminotransferases, gammaglobulins and IgG and restoration of albumin and bilirrubin leves and INR to normal values. Therapy is guided toward complete treatment response, defined by normalization of either AST and ALT as well as IgG, which requires at least 6 months of immunossupression (10) . Treatment is usually required for at least 24 months.…”

Section: Management and Treatment Of Aihmentioning

confidence: 99%

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

Bittencourt

Cançado

Couto

et al. 2015

Arq. Gastroenterol.

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-In order to draw evidence-based recommendations concerning the management of autoimmune diseases of the liver, the Brazilian Society of Hepatology has sponsored a single-topic meeting in October 18th, 2014 at São Paulo. An organizing committee comprised of seven investigators was previously elected by the Governing Board to organize the scientific agenda as well as to select twenty panelists to make a systematic review of the literature and to present topics related to the diagnosis and treatment of autoimmune hepatitis, primary sclerosing cholangitis, primary biliary cirrhosis and their overlap syndromes. After the meeting, all panelists gathered together for the discussion of the topics and the elaboration of those recommendations. The text was subsequently submitted for suggestions and approval of all members of the Brazilian Society of Hepatology through its homepage. The present paper is the final version of the reviewed manuscript organized in topics, followed by the recommendations of the Brazilian Society of Hepatology.

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Frequency of Concurrent Autoimmune Disorders in Patients With Autoimmune Hepatitis

Cited by 65 publications

References 34 publications

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

Association of Extrahepatic Manifestations with Autoimmune Hepatitis

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

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

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