From the Guidelines
Sarcoidosis is a multisystem inflammatory disorder that requires individualized treatment based on disease severity, organ involvement, and impact on quality of life, with corticosteroids like prednisone remaining first-line therapy for symptomatic patients 1. The exact cause of sarcoidosis remains unknown, though it likely results from an abnormal immune response to an unidentified antigen in genetically susceptible individuals. Sarcoidosis typically affects adults between 20-40 years of age, with higher prevalence in African Americans and Northern Europeans. The clinical presentation varies widely depending on organ involvement, ranging from asymptomatic disease discovered incidentally to severe manifestations including pulmonary fibrosis, cardiac arrhythmias, or neurological complications. Common symptoms include:
- Persistent dry cough
- Shortness of breath
- Fatigue
- Night sweats
- Weight loss Diagnosis requires compatible clinical and radiological findings, histological evidence of non-caseating granulomas, and exclusion of other granulomatous diseases. Treatment depends on disease severity and organ involvement, with many patients requiring no treatment as the disease may resolve spontaneously. For symptomatic patients, corticosteroids like prednisone (starting at 20-40mg daily with gradual tapering over months) remain first-line therapy. Steroid-sparing agents such as methotrexate (10-25mg weekly), azathioprine, or TNF-alpha inhibitors may be used for maintenance therapy or in steroid-resistant cases, as recommended by the European Respiratory Journal 1. Regular monitoring of pulmonary function, cardiac status, and affected organ systems is essential for managing this chronic condition effectively, with the goal of reducing morbidity and mortality risk or improving quality of life (QoL) 1.
From the Research
Definition and Characteristics of Sarcoidosis
- Sarcoidosis is a systemic granulomatous disease of unknown cause affecting multiple organs, most commonly the lungs and intrathoracic lymph nodes 2, 3.
- It is characterized by the presence of noncaseating granulomas in any organ 2, 3.
- The disease can affect various organs, including the heart, liver, spleen, salivary glands, muscles, bones, kidneys, and central nervous system 3.
Diagnosis of Sarcoidosis
- Diagnosis relies on three criteria: a compatible clinical and radiologic presentation, pathologic evidence of noncaseating granulomas, and exclusion of other diseases with similar findings 2.
- An early and accurate diagnosis of sarcoidosis remains challenging due to variable initial presentations, asymptomatic patients, and the lack of a single reliable diagnostic test 2.
Treatment of Sarcoidosis
- Corticosteroid therapy is the mainstay of treatment for patients with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease 4, 5, 2, 3.
- Refractory or complex cases may require immunosuppressive therapy, including methotrexate, azathioprine, leflunomide, and hydroxychloroquine 4, 6.
- Biologic therapy, such as tumor necrosis factor-alpha inhibitors, may be used for patients who are refractory to standard therapy 4, 5.
- The optimal treatment for sarcoidosis remains unclear, and there is a need for further research to determine the best approach to management 4, 5, 2, 3.
Management and Prognosis
- Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement 2, 3.
- Despite aggressive treatment, some patients may develop life-threatening pulmonary, cardiac, or neurologic complications from severe, progressive disease 2.
- End-stage disease may ultimately require lung or heart transplantation for eligible patients 2, 3.