Subjective
Date of visit
Reason for visit (follow-up for PSC, evaluation of new symptoms)
Current symptoms:
Fatigue (a common and often debilitating symptom)
Pruritus (itching, a hallmark symptom of PSC) – can be severe and impact quality of life
Right upper quadrant abdominal pain (may be present)
Fatty food intolerance (may develop)
Bone pain or fractures (potential complications)
Diarrhea or constipation (bowel habit changes)
Darkening of urine or pale stools (suggestive of bile duct obstruction)
Yellowish skin deposits (xanthomas) – can occur in some patients
Loss of appetite or weight loss
Changes in sleep patterns or energy levels
Impact of symptoms on daily life and emotional well-being
Objective
Vital signs (temperature, blood pressure, heart rate) – fever may suggest infection
Physical exam:
General (assessing for signs of liver disease – jaundice, ascites)
Skin (looking for signs of jaundice, excoriations from scratching)
Abdomen (palpating for liver size and tenderness)
Lymphadenopathy (enlarged lymph nodes) – may be present
Laboratory Tests
Liver function tests (LFTs):
Elevated alkaline phosphatase (ALP) – a hallmark finding in PSC
Elevated bilirubin (may be present)
Elevated liver enzymes (AST, ALT)
Antimitochondrial antibody (AMA): negative in PSC (helps differentiate from PBC)
Liver function tests (LFTs) – to monitor disease progression
Cholangiogram (imaging test of the bile ducts):
Magnetic resonance cholangiopancreatography (MRCP) – preferred non-invasive option
Endoscopic retrograde cholangiopancreatography (ERCP) – may be used if MRCP inconclusive
Additional Tests (may be ordered depending on clinical presentation):
Liver ultrasound: to assess liver size and structure
FibroScan (non-invasive test): to assess liver fibrosis
Liver biopsy (definitive test for PSC, but not routinely performed)
Serum markers for autoimmune diseases (ANA, ANCA) – to identify potential overlap syndromes
Assessment
Primary sclerosing cholangitis confirmed or suspected (based on clinical presentation, laboratory findings, and imaging)
Stage of disease progression (based on liver function tests and other investigations)
Presence of complications (e.g., small bowel bacterial overgrowth, osteoporosis, malnutrition, liver cirrhosis)
Evaluation for potential associated conditions (ulcerative colitis)
Plan
Treatment plan based on disease stage and symptoms:
Ursodeoxycholic acid (UDCA) – the mainstay of treatment to improve bile flow and liver function
Cholestyramine or other bile acid sequestrants (to manage pruritus)
Fat-soluble vitamin supplementation (vitamins A, D, E, K) – as these vitamins may be poorly absorbed
Antibiotics (to treat or prevent cholangitis – bile duct infections)
Liver transplant evaluation (considered for end-stage PSC)
Referral to a gastroenterologist or hepatologist for complex cases
Education
Explain primary sclerosing cholangitis, a chronic autoimmune disease affecting the bile ducts.
Discuss the treatment plan, potential side effects, and importance of medication adherence.
Importance of a healthy lifestyle with a balanced diet and regular exercise.
Management strategies for pruritus (itching) and other symptoms.
Importance of regular follow-up to monitor disease progression and potential complications.
Provide information about support groups or patient advocacy organizations.
If applicable, discuss the association of PSC with ulcerative colitis and the need for colonoscopy screening.
Follow-up
Schedule for follow-up appointments:
Monitor response to treatment (symptoms, laboratory tests)
Adjust medication regimen as needed
Address any ongoing challenges or concerns
Screen for complications (bone density scans, colonoscopy)
Consider vaccinations (ensure patient is up-to-date on recommended immunizations)
Disclaimer: This information is for educational purposes only and should not be interpreted as medical advice. Please consult with a qualified healthcare professional for diagnosis and treatment of primary sclerosing cholangitis.