From the Guidelines
Determining if sarcoidosis is "burnt out" in a patient with pulmonary sarcoidosis requires assessing stability or improvement in lung function parameters, such as forced vital capacity (FVC) and diffusion capacity (DLCO), as well as regression of inflammatory changes on chest imaging, as noted in the ERS clinical practice guidelines on treatment of sarcoidosis 1. To evaluate if sarcoidosis has burnt out, several key factors must be considered:
- Clinical symptoms: Resolution of symptoms like cough, shortness of breath, and fatigue is crucial.
- Pulmonary function tests (PFTs): Serial PFTs should demonstrate stability or improvement in lung function parameters, particularly FVC and DLCO, as these are indicators of disease activity and progression 1.
- Chest imaging: High-resolution CT scans should show regression of previously active inflammatory changes, with possible residual fibrotic changes that remain stable over time.
- Laboratory markers: While not the most reliable indicators, serum angiotensin-converting enzyme (ACE) levels and soluble interleukin-2 receptor (sIL-2R) may normalize in burnt-out disease.
- Treatment response: Stability after reduction or discontinuation of corticosteroids or other immunosuppressive therapy strongly suggests burnt-out disease, as indicated by the guidelines 1. It is essential to note that sarcoidosis can appear inactive but later reactivate, and some patients may develop permanent fibrotic changes representing end-stage disease rather than ongoing inflammation, highlighting the need for a comprehensive and ongoing assessment approach 1.
From the Research
Determining Burnt-Out Sarcoidosis
To determine if sarcoidosis is burnt out in a patient with pulmonary sarcoidosis, several factors need to be considered:
- The Scadding stages for pulmonary sarcoidosis, which associate with clinical outcomes 2
- The presence of fibrocytic disease, bronchiectasis, and hilar retraction on high-resolution computed tomography (HRCT) scans 2, 3
- The degree of pulmonary physiologic impairment and the presence of pre-capillary pulmonary hypertension 2
- The response to treatment, including oral glucocorticoids and immunosuppressive agents 2, 4
Diagnostic Tools
Diagnostic tools that can be used to determine burnt-out sarcoidosis include:
- HRCT scans to assess the extent of lung involvement and detect any changes over time 5, 3, 6
- Pulmonary function tests (PFTs) to evaluate lung function and detect any decline 2, 3, 4
- Visual scoring of chest radiographs to assess disease progression 5
Monitoring Disease Progression
Regular follow-up is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications 4. This can be achieved through: