Extrahepatic Autoimmune Diseases in Patients with Autoimmune Liver Diseases: A Phenomenon Neglected by Gastroenterologists

Table 1

Incidence of concomitant EHAIDs in AILDs.

EHAIDs

Sjogren’s syndrome

Autoimmune thyroid disease

Systemic lupus erythematosus

Rheumatoid arthritis

Systemic sclerosis or scleroderma

Inflammatory bowel disease

Dermatomyositis or polymyositis

Raynaud’s phenomenon

Mixed connective tissue disease

Autoimmune thrombocytopenic purpura

Pernicious anemia

References

AIH

29.9–61.8%

1.4–34.5%

10.0–23.0%

0.7–18.8%

1.8–12.9%

1.2–3.5%

2.0–8.0%

3.6%

—

2.0–4.0%

1 case

—

[3–7]

PBC

36.5–67.4%

3.5–47.4%

14.4–23.8%

1.0–5.2%

1.8–17.0%

0.8–12.3%

2.0–7.5%

0.6–3.1%

18.0–24.0%

0.6–0.8%

1.0%

4.0%

[3, 8–11]

PBC-AIH OS

25.0–43.7%

8.5–20.8%

18.3%

2.8%

4.2%

1.4%

—

1 case

—

—

1 case

1.4%

[12–14]

PSC

60.0–80.0%

2 cases

7.6%

2 cases

5.6%

1 case

1.7–70.0%

—

—

—

—

—

[15–18]

EHAID: extrahepatic autoimmune disease; AILD: autoimmune liver disease; AIH: autoimmune hepatitis; PBC: primary biliary cirrhosis; PSC: primary sclerosing cholangitis; OS: overlap syndrome.