From the Guidelines
The workup for sarcoidosis should begin with a thorough history and physical examination, followed by chest X-ray and pulmonary function tests, as recommended by the European Respiratory Society guidelines 1.
Initial Evaluation
The initial evaluation of sarcoidosis typically involves a combination of clinical assessment, imaging studies, and laboratory tests. High-resolution CT scan of the chest is often necessary to evaluate lung involvement, and tissue biopsy showing non-caseating granulomas is the gold standard for diagnosis, commonly obtained from accessible sites like lymph nodes, skin lesions, or via bronchoscopy 1.
Laboratory Tests and Imaging
Laboratory tests should include complete blood count, comprehensive metabolic panel, calcium levels, and angiotensin-converting enzyme (ACE) levels, though ACE has limited specificity 1. Additional testing may include ophthalmologic examination, electrocardiogram, and specialized imaging based on suspected organ involvement.
Prognosis and Treatment
The prognosis for sarcoidosis is generally favorable, with spontaneous remission occurring in 60-70% of patients within 2-5 years 1. Treatment is not always necessary for asymptomatic patients with minimal organ involvement. When treatment is indicated, oral corticosteroids (typically prednisone 20-40mg daily for 6-12 months with gradual taper) remain first-line therapy, as supported by the Delphi consensus recommendations 1.
Second-Line Options
For patients who cannot tolerate or respond to steroids, second-line options include methotrexate (10-25mg weekly), hydroxychloroquine (200-400mg daily), or TNF-alpha inhibitors like infliximab (3-5mg/kg IV at weeks 0,2, and 6, then every 8 weeks) 1.
Monitoring and Prognostic Factors
Patients with pulmonary sarcoidosis should be monitored with regular pulmonary function tests and chest imaging, while those with extrapulmonary involvement require organ-specific monitoring. Poor prognostic factors include Black race, older age at onset, extrapulmonary involvement (especially cardiac, neurologic, or advanced pulmonary disease), and chronic disease course lasting more than two years 1.
From the Research
Work-up for Sarcoidosis
- Diagnosis of sarcoidosis relies on three criteria: a compatible clinical and radiologic presentation, pathologic evidence of noncaseating granulomas, and exclusion of other diseases with similar findings 2
- A multidisciplinary approach is recommended for best outcomes, with the pulmonologist often being the referral physician for diagnosis and management 3
- Computed tomography (CT) is essential for atypical manifestations of the disease to avoid confusion with differential diagnoses and comorbidities 4
- High-resolution computed tomographic (HRCT) scans are superior to chest X-rays in assessing the extent of disease, and some CT features may discriminate an active inflammatory component from fibrosis 5
- MRI may be a viable alternative to HRCT in the assessment of lung parenchyma and disease progression in patients with pulmonary sarcoidosis 6
Prognosis for Sarcoidosis
- Prognosis is variable and depends on epidemiologic factors, mode of onset, initial clinical course, and specific organ involvement 2
- The optimal treatment for sarcoidosis remains unclear, but corticosteroid therapy has been the mainstay of therapy for those with significantly symptomatic or progressive pulmonary disease or serious extrapulmonary disease 2
- 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
- Regular follow-up is necessary to monitor changes in the disease, including extension, progression, remissions, flare-ups, and complications 3