Medical history (history of illness) is an important component in the diagnosis of primary sclerosing cholangitis (PSC). Family history Does your family have a history of diseases of the biliary tract or gallbladder or liver that are common? Social history. Current medical history/systemic history (somatic and psychological complaints). Have you noticed any pain in the … Primary Sclerosing Cholangitis: Medical History
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 … Primary Sclerosing Cholangitis: Or something else? Differential Diagnosis
The following are the most important diseases or complications that may be contributed to by primary sclerosing cholangitis (PSC): Endocrine, nutritional, and metabolic diseases (E00-E90). Deficiency of fat-soluble vitamins A, D, E, K. Liver, gallbladder and bile ducts – pancreas (pancreas) (K70-K77; K80-K87). Acute bacterial cholangitis (Biliary) cirrhosis (liver disease associated with inflammation and fibrosis … Primary Sclerosing Cholangitis: Complications
A comprehensive clinical examination is the basis for selecting further diagnostic steps: General physical examination – including blood pressure, pulse, body temperature, body weight, body height; further: Inspection (viewing). Skin, mucous membranes, and sclerae (white part of the eye) [due topossible symptoms: icterus (jaundice); pruritus (itching)] Abdomen (abdomen) Shape of the abdomen? Skin color? Skin … Primary Sclerosing Cholangitis: Examination
1st order laboratory parameters – obligatory laboratory tests. Cholestasis parameters (elevated) [Only mild transaminase elevation; elevated AP (alkaline phosphatase) (3- to 10-fold) is often suggestive; GGT (gamma-GT) is often normal or discretely elevated]Note: Wg. fluctuations of the AP in the course, even a normal-value AP can not exclude a primary sclerosing cholangitis (PSC)! Bilirubin may … Primary Sclerosing Cholangitis: Test and Diagnosis
General measures Aim for normal weight! Determination of BMI (body mass index) or body composition by means of electrical impedance analysis and, if necessary, participation in a medically supervised weight loss program or program for the underweight. BMI ≥ 25 → participation in a medically supervised weight loss program. Falling below the BMI lower limit … Primary Sclerosing Cholangitis: Therapy
Causal (cause-related) therapy is not available. Therapeutic target Because primary sclerosing cholangitis (PSC) is associated with an increased risk of developing cholangiocellular carcinoma (CCC; bile duct cancer), progression (progression) of the disease should be delayed as much as possible. Therapy recommendations Ursodeoxycholic acid (UDCA; natural bile acid; for use as a drug, ursodeoxycholic acid is … Primary Sclerosing Cholangitis: Drug Therapy
Obligatory medical device diagnostics. Liver ultrasonography (ultrasound of the liver) – basic diagnostics [often normal findings in initial diagnosis of primary sclerosing cholangitis; bile duct dilatation indicative of cholestasis/bile stasis]. Endoscopic retrograde cholangiopancreatography (ERCP; gold standard) – Diagnostic method in gastroenterology that combines endoscopy and radiology. It involves X-ray imaging of the bile duct system … Primary Sclerosing Cholangitis: Diagnostic Tests
Nearly 60% of PSC patients develop dominant biliary stenosis (biliary stricture) during their disease course. If stenoses and/or strictures (high-grade narrowings) are present, endoscopic dilatation (widening, i.e., bougienage, balloon dilatation) or stent implantation (insertion of a stent; “vascular bridge”) is performed In the final stage of primary sclerosing cholangitis, i.e., when the liver is no … Primary Sclerosing Cholangitis: Surgical Therapy
The following symptoms and complaints may indicate primary sclerosing cholangitis (PSC): Later stage Fever* (chills). Weight loss (after many years of progression). Icterus (jaundice), also recurrent (recurring). Fatigue (tiredness) Upper abdominal discomfort* , right-sided Pruritus (itching) * typical of infectious cholangitis (bile duct inflammation).
Pathogenesis (disease development) The pathogenesis of primary sclerosing cholangitis (PSC) is as yet unknown. Immunologic factors, genetic associations (HLA associations), and a genetic background (familial clustering) are discussed. PSC is a chronic cholestatic disease (biliary obstruction) that leads to progressive destruction of the intra- and/or extrahepatic (outside and inside the liver) biliary system. Etiology (causes) … Primary Sclerosing Cholangitis: Causes
