From the Guidelines
Sarcoidosis is a chronic inflammatory disease characterized by the formation of granulomas in various organs, primarily affecting the lungs and lymph nodes, with symptoms varying widely depending on organ involvement, and treatment focusing on reducing morbidity and mortality risk or improving quality of life (QoL) 1.
Definition and Characteristics
Sarcoidosis is a complex disease with unknown exact cause, believed to result from an abnormal immune response to an unidentified trigger in genetically susceptible individuals. The disease can affect multiple organs, including the eyes, skin, heart, and others, leading to a wide range of symptoms such as fatigue, persistent dry cough, shortness of breath, skin rashes, and eye inflammation.
Diagnosis and Treatment
Diagnosis typically involves a combination of clinical evaluation, imaging studies like chest X-rays or CT scans, and often a biopsy to confirm the presence of granulomas. Treatment depends on symptom severity and organ involvement, with corticosteroids like prednisone being the primary medication, and immunosuppressants such as methotrexate or hydroxychloroquine used for those who cannot tolerate steroids or have persistent disease 1.
Management and Prognosis
The management of sarcoidosis can be challenging, with the clinician needing to consider various manifestations and the variable outcome of the disease. While some patients have a good outcome and never require treatment, others may experience spontaneous remission within 2-5 years, and some develop chronic disease requiring long-term management 1. Regular follow-up with healthcare providers is essential to monitor disease progression and medication side effects.
Key Considerations
The decision to treat sarcoidosis depends on two major factors: risk for death or organ failure and impairment of QoL. Treatment indications aim to balance the minimization of risk of disability, loss of life, or loss of QoL, and the risk of comorbidities and loss of QoL due to glucocorticoid and other therapies 1. Interstitial lung disease (ILD) or pulmonary hypertension are the main causes of sarcoidosis-related mortality, and represent risks of lifelong exercise intolerance.
From the Research
Definition and Characteristics of Sarcoidosis
- Sarcoidosis is a multisystemic disorder of unknown cause characterized by the formation of immune granulomas in involved organs 2.
- It is an ubiquitous disease with incidence varying according to age, sex, race, and geographic origin, estimated at around 16.5/100,000 in men and 19/100,000 in women 2.
- The lung and the lymphatic system are predominantly affected, but virtually every organ may be involved 2.
Clinical Manifestations
- Sarcoidosis is revealed by persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum 2.
- Other severe manifestations result from cardiac, neurological, ocular, kidney, or laryngeal localizations 2.
- Abnormal metabolism of vitamin D3 within granulomatous lesions and hypercalcemia are possible 2.
Diagnosis and Staging
- Diagnosis relies on compatible clinical and radiographic manifestations, evidence of non-caseating granulomas obtained by biopsy, and exclusion of all other granulomatous diseases 2.
- Chest radiography is abnormal in about 90% of cases and shows lymphadenopathy and/or pulmonary infiltrates, defining sarcoidosis stages from I to IV 2.
Treatment and Management
- Corticosteroids are the mainstay of treatment for sarcoidosis 2, 3, 4.
- Immunosuppressive drugs, hydroxychloroquine, and infliximab may be useful in some patients 3, 4.
- Treatment duration should be of at least 12 months, and low-dose methotrexate and azathioprine are the most useful immunosuppressive drugs 4.
- Topical corticosteroids can be used for limited skin involvement, anterior uveitis, or cough 4.