What is the treatment for pulmonary sarcoidosis?

From the Guidelines

The treatment for pulmonary sarcoidosis should start with corticosteroids, specifically prednisone at 20-40mg daily for 1-3 months, then gradually tapered to the lowest effective dose, with second-line options including methotrexate, hydroxychloroquine, azathioprine, or mycophenolate mofetil, and TNF-alpha inhibitors like infliximab for refractory cases, as recommended by the European Respiratory Society guidelines 1.

Treatment Approach

The approach to treating pulmonary sarcoidosis depends on the severity of symptoms and organ involvement. For mild cases with minimal symptoms, a watch-and-wait approach may be appropriate, as 30-60% of cases resolve spontaneously within 2-5 years 1.

First-Line Treatment

For patients with significant symptoms or declining lung function, corticosteroids are the first-line treatment. Typically, prednisone is started at 20-40mg daily for 1-3 months, then gradually tapered to the lowest effective dose (often 5-10mg daily) for a total treatment duration of 6-12 months 1.

Second-Line Options

For patients who cannot tolerate or respond inadequately to corticosteroids, second-line options include:

• Methotrexate (10-25mg weekly) 1
• Hydroxychloroquine (200-400mg daily) 1
• Azathioprine (50-200mg daily) 1
• Mycophenolate mofetil (1000-3000mg daily) 1

Refractory Cases

For refractory cases, TNF-alpha inhibitors like infliximab (3-5mg/kg IV at weeks 0,2, and 6, then every 4-8 weeks) may be used 1.

Supportive Measures

Pulmonary rehabilitation, supplemental oxygen for hypoxemia, and management of complications like pulmonary hypertension are important supportive measures. Regular follow-up with pulmonary function tests and chest imaging is essential to monitor disease progression and treatment response 1.

Goal of Therapy

The goal of therapy is to relieve symptoms, improve lung function, and prevent progression to pulmonary fibrosis 1.

From the FDA Drug Label

Symptomatic sarcoidosis The treatment for pulmonary sarcoidosis is prednisone (PO), as it is indicated for symptomatic sarcoidosis 2.

• The drug is used to manage the condition.
• Prednisone is the specific treatment mentioned in the drug label.

From the Research

Treatment Overview

• The primary treatment for pulmonary sarcoidosis is glucocorticosteroids, which can be administered orally or via inhalation 3, 4, 5, 6.
• The initial dose of oral steroids is typically 30-60 mg/day of prednisolone or its equivalent, with the possibility of tapering to alternate day therapy during the maintenance phase 3.
• Inhaled steroids can also be used during the maintenance phase for treating pulmonary sarcoidosis 3, 5.

Second-Line Treatments

• Other drugs that may be effective in treating sarcoidosis and have a steroid-sparing capacity include methotrexate, azathioprine, chlorambucil, and cyclophosphamide 3, 7.
• Anti-tumor necrosis factor agents, such as infliximab or adalimumab, may be used to treat inflammatory disease that persists on combination treatment with glucocorticoids and a second-line agent 4, 7.
• Chloroquine can be used for chronic skin lesions, and potassium para-aminobenzoate may soften fibrotic lesions and keloids 3.

Treatment Duration and Monitoring

• The duration of treatment varies depending on the clinical situation, ranging from 6 to 18 months to lifetime 3.
• Continuing signs of disease activity and functional impairment require ongoing treatment 3.
• Symptomatic patients with stage II-III pulmonary sarcoidosis and extrapulmonary manifestations of the disease should be adequately treated 3, 4.
• Treatment should be tailored to individual patients, taking into account the severity of disease, quality of life, and potential side effects of treatment 4, 7, 6.