From the Guidelines
In burnt out pulmonary sarcoidosis, immunosuppression generally offers no benefit and is not recommended. Burnt out sarcoidosis represents end-stage fibrotic disease where active inflammation has resolved, leaving permanent scarring and fibrosis that cannot be reversed with treatment. According to the European Respiratory Journal guidelines 1, the recommendations for treatment of sarcoidosis focus on patients with active disease, and there is no clear benefit of immunosuppression in burnt out disease. The guidelines suggest the use of glucocorticoids and other immunosuppressive agents in patients with symptomatic pulmonary sarcoidosis who are at higher risk of future mortality or permanent disability, but this does not apply to burnt out disease. Medications like prednisone, methotrexate, azathioprine, or TNF-alpha inhibitors that are effective for active inflammatory sarcoidosis will not improve lung function or symptoms in the burnt out phase. Instead, management should focus on supportive care, including:
- Pulmonary rehabilitation
- Oxygen therapy if hypoxemia is present
- Vaccination against respiratory pathogens
- Treatment of complications like pulmonary hypertension or bronchiectasis Immunosuppression in this setting carries risks including infection, bone loss, diabetes, and other side effects without providing clinical benefit, as noted in the guidelines 1. The distinction between active inflammation and burnt out disease is crucial and may require comprehensive evaluation including pulmonary function tests, high-resolution CT imaging, and sometimes bronchoalveolar lavage to assess inflammatory markers before making treatment decisions.
From the FDA Drug Label
The risk of post-transplant lymphomas may be increased in patients who receive aggressive treatment with immunosuppressive drugs, including AZASAN. Corticosteroids, including prednisone tablets, suppress the immune system and increase the risk of infection with any pathogen, including viral, bacterial, fungal, protozoan, or helminthic pathogens
There is no direct benefit of immunosuppression in a patient with burnt out pulmonary sarcoid mentioned in the provided drug labels.
- The labels discuss the risks associated with immunosuppressive therapy, including increased risk of infection and malignancy.
- They do not provide information on the benefits of immunosuppression in patients with burnt out pulmonary sarcoid. 2 3
From the Research
Benefits of Immunosuppression in Burnt Out Pulmonary Sarcoidosis
- The use of immunosuppressive therapy in sarcoidosis patients, including those with burnt out pulmonary sarcoidosis, is supported by several studies 4, 5, 6.
- Corticosteroids are the first-line treatment for sarcoidosis, but long-term use can lead to toxicity, making it necessary to consider second-line agents such as methotrexate and azathioprine 6, 7.
- Methotrexate has been shown to be effective in reducing prednisone dose and improving lung function in patients with pulmonary sarcoidosis, with a response rate of around 80% 8.
- Azathioprine has also been found to have similar steroid-sparing potency and positive effects on lung function as methotrexate, although it may have a higher infection rate 7.
- The effectiveness of methotrexate has been found to be unrelated to treatment dosage and baseline characteristics, and it has a low relapse rate in complete responders who discontinue therapy 8.
Considerations for Immunosuppressive Therapy
- The choice of immunosuppressive agent should be based on the individual patient's needs and response to treatment, as well as the potential risks and benefits of each agent 5, 6.
- Patients with burnt out pulmonary sarcoidosis may require a multidisciplinary approach to their care, including close monitoring and adjustment of their treatment regimen as needed 4.
- Further research is needed to identify the most effective treatment strategies for sarcoidosis, including the use of biologic agents and other novel therapies 4, 5.