Skin and subcutaneous (L00-L99).
- Pruritus (itching) of other origin.
Liver, gallbladder, and biliary tract-pancreas (pancreas) (K70-K77; K80-K87).
- Bacterial cholangitis
- IgG4-associated cholangitis – IgG4 in serum is elevated and IgG4-positive cells are detectable in bile duct cytology; this disease responds to immunosuppressive therapy, such as corticosteroids
- Intra- or extrahepatic (occurring outside and inside the liver) cholestasis (bile stasis).
- Secondary sclerosing cholangitis (SSC) – rare condition; occurs in some autoimmune diseases.
- Primary biliary cholangitis (PBC, synonyms: non-purulent destructive cholangitis; primary biliary cirrhosis) – relatively rare autoimmune disease of the liver (affects women in about 90% of cases); begins primarily biliary, i.e. at the intra- and extrahepatic (“inside and outside the liver”) bile ducts, which are destroyed by inflammation (= chronic non-purulent destructive cholangitis). In the longer course, the inflammation spreads to the entire liver tissue and eventually leads to scarring and even cirrhosis; detection of antimitochondrial antibodies (AMA); PBC is often associated with autoimmune diseases (autoimmune thyroiditis, polymyositis, systemic lupus erythematosus (SLE), progressive systemic sclerosis, rheumatoid arthritis); Associated with ulcerative colitis (inflammatory bowel disease) in 80% of cases; long-term risk of cholangiocellular carcinoma is 7-15% (5% of patients with ulcerative colitis develop PBC)
- Overlap syndrome of PSC with autoimmune hepatitis (AIH; autoimmune hepatitis) – in 6% of cases.
Mouth, esophagus (food pipe), stomach, and intestines (K00-K67; K90-K93).
- Ulcerative colitis – approximately 5% of sufferers have primary sclerosing cholangitis (PSC).
Musculoskeletal system and connective tissue (M00-M99).
- Sjögren’s syndrome – autoimmune disease from the group of collagenoses.
Neoplasms – tumor diseases (C00-D48).
