From the Guidelines
Sarcoidosis is a chronic inflammatory disease that can cause a flare, which is a period of sudden worsening or new symptoms, and the primary goal of treatment is to reduce morbidity, mortality, and improve quality of life, as stated in the European Respiratory Journal guidelines 1.
Definition and Cause of Sarcoidosis
Sarcoidosis is characterized by the formation of granulomas in various organs, most commonly the lungs, lymph nodes, eyes, and skin. The exact cause remains unknown, though it likely involves an abnormal immune response triggered by environmental factors in genetically susceptible individuals.
Sarcoidosis Flare
A sarcoidosis flare refers to a period when symptoms suddenly worsen or new symptoms appear after a period of stability. During a flare, inflammation increases, causing symptoms like:
- Increased fatigue
- Worsening shortness of breath
- Chest pain
- Fever
- Joint pain
- Skin rashes
- Vision changes depending on which organs are affected. Flares can be triggered by stress, infections, medication changes, or may occur spontaneously.
Treatment of Sarcoidosis Flares
Treatment for flares typically involves corticosteroids like prednisone (starting at 20-40mg daily, then tapering), as recommended by the European Respiratory Journal guidelines 1. Though severe cases may require immunosuppressants such as methotrexate (15-25mg weekly) or hydroxychloroquine (200-400mg daily). It's essential to monitor organ function during flares, particularly lung function tests and eye examinations, as persistent inflammation can lead to permanent organ damage, as noted in the discussion section of the guidelines 1.
Management and Follow-up
Patients should maintain regular follow-ups with their healthcare provider and report any new or worsening symptoms promptly to manage flares effectively. The management of sarcoidosis can be challenging, and the clinician must consider the various manifestations both initially and over time, as stated in the guidelines 1. The outcome of the disease is variable, and treatment decisions require an assessment of organ involvement, risk for significant morbidity, and impact on quality of life (QoL) of the disease and treatment.
From the Research
Definition of Sarcoidosis
- Sarcoidosis is a systemic, granulomatous disease that can affect multiple organs and has a variable clinical course 2, 3, 4, 5.
- It is characterized by the formation of immune granulomas in involved organs 4.
- The lungs and the lymphatic system are predominantly affected, but virtually every organ may be involved 4, 5.
Symptoms of Sarcoidosis
- Symptoms include pulmonary as well as extra-pulmonary manifestations of the disease 6.
- Common symptoms are persistent dry cough, eye or skin manifestations, peripheral lymph nodes, fatigue, weight loss, fever or night sweats, and erythema nodosum 4.
- Abnormal metabolism of vitamin D3 within granulomatous lesions and hypercalcemia are possible 4.
Treatment of Sarcoidosis
- Systemic corticosteroids are the mainstay of treatment of sarcoidosis 2, 3, 6, 4, 5.
- Immunosuppressive drugs, such as methotrexate, azathioprine, and leflunomide, are used as sparing agents for patients who are intolerant or refractory to corticosteroids 2, 3, 6.
- Biological therapies, such as monoclonal anti-TNF agents, have increased the therapeutic armamentarium available to treat sarcoidosis, but their use is still limited by a lack of licensing and costs 5.
Sarcoidosis Flare
- A flare in sarcoidosis refers to a worsening of symptoms or an increase in disease activity 6, 4.
- Flares can occur in patients who are being treated with corticosteroids or other immunosuppressive drugs, and may require an increase in dosage or a change in treatment regimen 6, 4.
- The management of flares must be tailored to the specific organ or organs involved, and may require close monitoring and adjustment of treatment 6, 5.