What is the treatment for vocal cord hyperkeratosis associated with sarcoidosis?

Treatment for Vocal Cord Hyperkeratosis in Sarcoidosis

For vocal cord hyperkeratosis associated with sarcoidosis, oral glucocorticoids (prednisone) at an initial dose of 20 mg once daily is the recommended first-line therapy, with methotrexate (10-15 mg weekly) as an equally effective alternative first-line option if steroids are contraindicated or poorly tolerated. 1

First-Line Treatment Options

Corticosteroid Therapy

• Initial prednisone dose: 20 mg once daily for 3-6 months 2, 1
• After initial treatment period, taper to lowest effective maintenance dose (5-10 mg daily or every other day) 1
• Monitoring requirements:
  • Bone density
  • Blood pressure
  • Serum glucose 1
• Common side effects: diabetes, hypertension, weight gain, osteoporosis, cataracts, glaucoma, mood changes 1

Methotrexate (Alternative First-Line)

• Dosage: 10-15 mg once weekly 1
• Monitoring: Complete blood count (CBC), hepatic and renal function tests 2
• Side effects: Nausea, leukopenia, hepatotoxicity 2
• Particularly useful when:
  • Corticosteroids are contraindicated
  • Patient experiences significant steroid toxicity
  • Disease persists despite adequate corticosteroid therapy 2

Treatment Algorithm Based on Disease Phenotype

The treatment approach should follow a structured algorithm based on disease phenotype:

1. Acute phenotype:

   • Start with prednisone 20-40 mg daily for 3-6 months
   • If disease progresses or toxicity develops, add methotrexate 2

2. Chronic phenotype:

   • Taper corticosteroids to lowest effective dose
   • Add methotrexate if unable to wean prednisone to acceptable level (≤10 mg daily) 2

3. Advanced/refractory phenotype:

   • Consider biologics (infliximab preferred) if disease progresses despite methotrexate
   • Infliximab dosing: 3-5 mg/kg initially, 2 weeks later, then every 4-6 weeks 2, 1
   • For persistent disease, consider repository corticotrophin injection or CLEAR therapy (concomitant levofloxacin, ethambutol, azithromycin, rifampin) 2

Other Treatment Considerations

Alternative Second-Line Options

If methotrexate is not tolerated, consider:

• Leflunomide: 10-20 mg once daily
• Azathioprine: 50-250 mg once daily
• Mycophenolate mofetil: 500-1500 mg twice daily 2, 1

Biologic Therapies

For refractory cases not responding to first and second-line therapies:

• Infliximab: 3-5 mg/kg (initially, 2 weeks later, then every 4-6 weeks)
• Adalimumab: 40 mg every 1-2 weeks
• Rituximab: 500-1000 mg every 1-6 months 2, 1

Important Clinical Considerations

1. Treatment duration: Initial treatment typically lasts 3-6 months with subsequent assessment of response and tapering to maintenance dose 1

2. Monitoring: Regular follow-up to assess:

   • Symptom improvement
   • Vocal cord appearance via laryngoscopy
   • Side effects of medications

3. Treatment pitfalls to avoid:

   • Prolonged high-dose corticosteroid use without steroid-sparing agents
   • Failure to monitor for medication toxicities
   • Abrupt discontinuation of corticosteroids
   • Inadequate duration of therapy before declaring treatment failure

4. Pneumocystis prophylaxis: Consider in patients on high-dose immunosuppression (e.g., ≥20 mg prednisone with a cytotoxic agent for >6 months) 1

The European Respiratory Society strongly recommends glucocorticoids as first-line treatment for sarcoidosis affecting various organ systems, with methotrexate as the preferred second-line agent for patients with inadequate response or steroid intolerance 1. This approach balances efficacy with minimizing long-term steroid toxicity, which has been increasingly recognized as a significant concern in sarcoidosis management 2.