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home - Liver - Autoimmune Conditions - Primary Biliary Cirrhosis Written by Dr Sebastian Zeki
Knowledge


Recognises and appropriately investigates patients with auto-immune
liver diseases

Aware of management and complications of autoimmune liver
disease including extra-hepatic manifestations and associations
including malignant complications in PSC

Skills
Appreciates and understands that this range of liver disease is
frequently under-diagnosed and may have been inappropriately
managed

Selects appropriate immunomodulatory therapy has awareness of
side effects and may well require specialist care

Behaviours
Responds urgently to the management challenge of these severe and
often acute diseases and involves more specialist services where
required

Primary Biliary Cirrhosis

Associations Phases GlomerulonephritisHypertrophic osteoarthropathyArthralgiaSclerodactylyRaynaud’sSLETPVitiligoThyroid DiseaseAddison’s Renal Rheum Derm Endocrine 1) Increase AMA only >12yrs2) Increased AMA and LFTs 8-12yrs3) Increased AMA, LFT’s and Pruritis 5-10 yrs4) Decompensated PBC 3-5 yrs Middle aged womenIcterusXanthelasmaScratch MarksMassive hepatosplenomegalyClubbing Prognosis PrognosisPositive Antinuclear antibodies- esp anticentromere antibodies) may identify a subgroup of patients at increased risk for progressing to liver failure.Cigarette smoking-may be associated with more advanced fibrosis stage.Natural history models-The best validated is the Mayo survival modelncludes age, the total serum bilirubin (most important), albumin, prothrombin time, and the severity of fluid retention.Certain polymorphisms of genes involved in immunity (CTLA-4 and TNF-alpha) are associated with prognosis and response to UDCA therapy. Early stage PBC- normal life expectancyBetter prognosis if asymptomatic at diagnosisThe presence of co-existing related disorders confers a worse prognosis Cancer 31% progression 50% progression 6% Primary Biliary Cirrhosis 1) Biliary duct epithelial damage with lympocyte infiltration +/- granulomas (fluid lesions) 2) Piecemeal necrosis 3) Bridging fibrosis 4) Cirrhosis Written by Dr Sebastian Zeki Histopathology Fatigue (excessive daytime somnolence) (40%)Associated with decreased survival.Has been correlated with muscle mitochondrial dysfunction manifested as excessive acidosis after exercise and also autonomic dysfunction.PruritusItching is worse at night, under constricting, coarse garments, in association with dry skin, and in hot, humid weather.Cause unknown but not PBC related Epidemiology95% are female.Age on onset is 30-65. It is more common and severe in Caucasians but can be seen in other ethnic groups.It shows geographical clustering and the incidence is rising (may be better detection). Clinical Manifestations At PresentationAsymptomatic- occurs in 60%.Fatigue and pruritis- occurs in 40%.Rheumatic symptoms.Dermatologic manifestations. Rheumatic symptoms1. Musculoskeletal complaints, often due to an inflammatory arthropathy- 40 % 2. Classic rheumatoid arthritis- 10 % 3. "Arthritis of PBC" -10 %- is a transient nondeforming rheumatoid-factor negative synovitis involving one or more peripheral joints.4. Sjögren's syndrome- 50%. (PBC rare in primary Sjogren’s)5. Limited cutaneous scleroderma- assoc with anticentromere antibodies and may include the CREST syndrome-10% Dermatologic manifestations50 % have hyperpigmentation of skin due to melanin rather than jaundiceXanthomas are much less common now Also get xerosis, dermatographism fungal infections of the feet or nails.

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