Co-existing diseases

Immune-mediated diseases

Even though we do not know the exact cause of PSC, it seems that the immune system plays an important role, especially given that many PSCers also suffer from one or more disease resulting from abnormal activity of the body’s immune system (immune-mediated diseases). A recent study in the Netherlands (Lamberts et al., 2011) found that over seventy per cent of the 241 PSC patients they tested also had an immune-mediated disease, and that nearly twenty per cent had immune diseases outside the liver and gut. Interestingly, 22.7% had more than one additional immune-mediated disease. Some of these diseases are quite rare. In the table below, we reproduce the study’s results, and briefly describe each condition with a weblink to sources of additional information.

Associated immune-mediated diseases in 241 patients with Primary Sclerosing Cholangitis (Lamberts et al., 2011)

Immune-mediated Disease


PSC Patients

Any immune-mediated disease a


Inflammatory Bowel Disease

Group of inflammatory conditions of the colon and small intestine. This includes Ulcerative Colitis and Crohn’s Disease.


Autoimmune Hepatitis

A condition where the immune system attacks liver cells by mistake, causing the liver to become inflamed.





A disease where abnormal collections of chronic inflammatory cells form as nodules in multiple organs such as lungs, skin, eyes and lymph nodes.


Thyroid disease

Impaired function of the thyroid.


Diabetes mellitus type 1

Autoimmune destruction of the insulin-producing beta cells of the pancreas.



Skin disease whereby the immune system acts as if it is fighting an infection or healing a wound, which leads to the rapid growth of skin cells causing psoriatic plaques to form.


Lichen planus

A chronic mucocutaneous disease that affects the skin, tongue, and oral mucosa in the form of papules, lesions and rashes.


Rheumatoid arthritis

A chronic, progressive disease causing swelling and damaging cartilage and bone around the joints.


Erythema nodosum

Inflammation of the fat cells under the skin that results in tender, red nodules, often located on the shins but can appear elsewhere.


Coeliac disease

Disease causing an immune response that damages the lining of the small intestine.


Pyoderma gangrenosum

A disease that causes tissue to become necrotic, causing deep ulcers that usually occur on the legs.



A condition that causes depigmentation of the skin.


Wegener’s disease

A disease that results in inflammation of the tissues, including the blood vessels.


Bechterew’s disease (Ankylosing spondylitis)

A disease that can eventually cause fusion of the joints in the spine and pelvis.



Inflammation of the white wall of the eye (the episclera).


a 39 patients had more than one additional immune-mediated disease including IBD and AIH
b8 patients had more than one additional immune-mediated disease other than IBD

Lamberts et al. Immune-mediated diseases in primary sclerosing cholangitis. Dig Liver Dis (2011). doi:10.1016/j.did.2011.05.009 (in press)

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More associated diseases

Chapman (2011) has also observed the following diseases with primary sclerosing cholangitis:

  • Chronic pancreatitis
  • Retroperitoneal fibrosis
  • Retro-orbital tumours
  • Immunodeficiency states
  • Sjögren’s syndrome
  • Angio-immunoplastic lymphadenopathy
  • Histiocytosis X
  • Autoimmune haemolytic anaemia
Chapman, 2011. Primary sclerosing cholangitis. Medicine,39(10): 588-591

Reviewed 10/05/12

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