What is the first line of treatment for a 55-year-old female with sarcoidosis, fatigue, weight loss, elevated Serum Angiotensin-Converting Enzyme (ACE) levels, finger clubbing, early Interstitial Lung Disease (ILD) on High-Resolution Computed Tomography (HRCT), and chronic liver disease changes on Ultrasound (USG) abdomen?

First-Line Treatment for Sarcoidosis with Multi-Organ Involvement

For a 55-year-old female with sarcoidosis affecting multiple organs (lungs, liver), the recommended first-line treatment is oral corticosteroids (prednisone 20-40 mg daily) to improve symptoms and prevent organ damage. 1

Disease Assessment

This patient presents with:

• Systemic symptoms: fatigue and weight loss 1
• Pulmonary involvement: early ILD on HRCT with sarcoidosis changes 2
• Hepatic involvement: chronic liver disease changes on USG 3, 4
• Laboratory abnormalities: elevated serum ACE (130) 1
• Physical findings: finger clubbing 5

Treatment Approach

Initial Therapy

• Start oral prednisone at 20-40 mg daily as first-line treatment for symptomatic multi-organ sarcoidosis 1, 5
• This dosage is effective for controlling both pulmonary and hepatic manifestations of sarcoidosis 2, 4
• Treatment is indicated due to:
  • Symptomatic disease (fatigue, weight loss) 1
  • Evidence of pulmonary involvement (ILD) 2
  • Liver involvement with chronic changes 3

Monitoring Response

• Follow-up at 3-6 months after treatment initiation to assess:
  • Symptom improvement (fatigue, weight loss) 1
  • Pulmonary function tests (PFTs) 5
  • Liver function tests 4
  • Repeat imaging as needed 6

Dose Adjustment

• If improvement occurs: gradually taper to lowest effective dose that controls symptoms 1
• If stable disease: continue tapering to minimize long-term side effects 1
• If worsening disease: consider adding steroid-sparing agents 2

Steroid-Sparing Considerations

Consider early addition of steroid-sparing agents if:

• High risk for steroid toxicity 1
• Inadequate response to initial steroid therapy 2
• Expectation of prolonged treatment course 1

Preferred Steroid-Sparing Options:

• Methotrexate is the preferred first-line steroid-sparing agent 2, 7
  • Recent evidence shows methotrexate may be as effective as prednisone with a different side effect profile 7
• Other options include:
  • Azathioprine for hepatic and pulmonary involvement 2
  • Mycophenolate for ILD 2

Special Considerations for This Patient

Hepatic Involvement

• Liver involvement occurs in up to 70% of sarcoidosis patients 4
• Corticosteroids are first-line therapy for symptomatic hepatic sarcoidosis 3, 4
• Consider ursodeoxycholic acid as adjunctive therapy for cholestatic features 4
• Monitor liver function tests regularly during treatment 2

Pulmonary ILD

• Early ILD requires treatment to prevent progression to fibrosis 6
• For SARD-ILD (including sarcoidosis-related ILD), glucocorticoids are conditionally recommended as first-line treatment 2
• Consider mycophenolate, azathioprine, or rituximab if inadequate response to steroids 2

Treatment Duration and Monitoring

• Relapse rates of 20-80% occur upon glucocorticoid withdrawal after 2 years 1
• At least 25% of patients require treatment for more than 2 years 1
• Long-term monitoring is essential due to risk of:
  • Disease progression 5
  • Treatment-related complications 8
  • Development of portal hypertension or cirrhosis in hepatic sarcoidosis 3

Common Pitfalls to Avoid

• Undertreating multi-organ disease: Both pulmonary and hepatic involvement require adequate treatment 3, 5
• Premature discontinuation: Treatment withdrawal too soon leads to high relapse rates 1
• Inadequate monitoring: Regular follow-up of both lung and liver function is essential 6, 4
• Overlooking steroid toxicity: Consider early steroid-sparing strategies to minimize long-term complications 1, 8