From the Guidelines
Sarcoidosis is a systemic granulomatous disease of unknown etiology, characterized by the formation of granulomas in various organs, most commonly the lungs and lymph nodes, and can significantly impact morbidity, mortality, and quality of life if left untreated or poorly managed. According to the most recent and highest quality study, 1, the primary goal of treating sarcoidosis is to reduce morbidity and mortality risk or improve quality of life. The disease can affect multiple organs, including the skin, eyes, heart, liver, and others, leading to a wide range of symptoms such as fatigue, persistent cough, shortness of breath, skin rashes, eye inflammation, or heart rhythm abnormalities.
Key Characteristics of Sarcoidosis
- The exact cause of sarcoidosis remains unknown, but it is believed to result from an abnormal immune response to an unidentified trigger in genetically susceptible individuals 1.
- The disease can resolve spontaneously in up to one-third of patients without therapy, depending on geography and genetic factors 1.
- Some patients develop chronic or progressive obliterative fibrotic disease, causing significant morbidity and mortality 1.
Treatment Approach
- Glucocorticoids, such as prednisone, are the primary medication for treating symptomatic disease, with the aim of reducing inflammation and preventing organ damage 1.
- For patients who cannot tolerate steroids or have persistent disease, immunosuppressants such as methotrexate, azathioprine, or TNF inhibitors like infliximab may be used as steroid-sparing therapies or to treat steroid-refractory disease 1.
- Regular monitoring is essential due to the unpredictable disease course, with some patients experiencing complete resolution while others develop chronic, progressive disease requiring long-term management 1.
Importance of Early Intervention and Management
- Early diagnosis and treatment are crucial to prevent long-term organ damage and improve quality of life 1.
- A comprehensive treatment plan should be tailored to the individual patient's needs, taking into account the severity of symptoms, organ involvement, and response to treatment 1.
From the Research
Definition and Characteristics of Sarcoidosis
- Sarcoidosis is a systemic disease characterized by the formation of non-caseating epitheloid granulomas, which can affect multiple organs, with the lungs and intrathoracic lymph nodes being the most commonly involved [ 2, 3, 4, 5, 6 ].
- The disease can be asymptomatic or chronic, with a gradual onset, and may improve or clear up spontaneously [ 4, 5 ].
- Sarcoidosis is a multisystemic disease of unknown etiology, with a lifetime incidence rate of 0.85-2.4%, mainly affecting people between 25-40 years old [ 6 ].
Organs Involved
- The lungs are the most commonly involved organ, with more than 90% of cases affecting the lungs and intrathoracic lymph nodes [ 3, 4 ].
- Other organs that can be involved include the skin, eyes, cardiac, hepatic, dermatologic, and central nervous systems [ 2, 3, 4, 5 ].
Diagnosis and Treatment
- Diagnosis is typically made after excluding other conditions, with lymph node biopsy and granulomatous findings increasing confidence in the diagnosis [ 4 ].
- Systemic corticosteroids are the first-line treatment, with second-line treatment options including methotrexate or hydroxychloroquine [ 2, 4 ].
- Refractory disease may require comanagement with a sarcoidosis subspecialist, and alternative therapies such as anti-TNF-alpha are being investigated [ 2, 3, 6 ].