What are the clinical aspects of sarcoidosis?

From the Guidelines

Sarcoidosis management should prioritize minimizing risk of disability, loss of life, and loss of quality of life due to pulmonary involvement or other organ involvement, while also considering the risks of comorbidities and loss of quality of life due to glucocorticoid and other therapies. The clinical aspects of sarcoidosis are diverse and require careful assessment and management.

Common Clinical Aspects

• Pulmonary involvement (90% of cases) with symptoms like dyspnea, cough, and chest pain, along with bilateral hilar lymphadenopathy on chest imaging
• Extrapulmonary manifestations include skin lesions (erythema nodosum, lupus pernio), eye involvement (uveitis), cardiac abnormalities (arrhythmias, heart failure), neurological symptoms, and hypercalcemia

Diagnosis and Treatment

Diagnosis typically requires demonstration of non-caseating granulomas in affected tissues, with exclusion of other granulomatous diseases.

• Treatment depends on disease severity, with mild cases often requiring only monitoring
• For symptomatic disease, prednisone (20-40mg daily for 6-12 months with gradual taper) is first-line therapy, as recommended by the European Respiratory Journal 1
• Steroid-sparing agents like methotrexate (10-25mg weekly), azathioprine (50-200mg daily), or hydroxychloroquine (200-400mg daily) may be used for maintenance or in steroid-resistant cases, as suggested by the European Respiratory Review 1
• TNF-alpha inhibitors like infliximab (3-5mg/kg IV at weeks 0,2, and then every 4-8 weeks) are reserved for refractory cases

Monitoring and Disease Course

Regular monitoring includes pulmonary function tests, chest imaging, and organ-specific assessments.

• The disease course varies widely, with spontaneous remission in 60% of patients, while others experience chronic progressive disease requiring long-term immunosuppression, as noted by the American Journal of Respiratory and Critical Care Medicine 1

From the Research

Clinical Aspects of Sarcoidosis

• Sarcoidosis is a systemic disease characterized by noncaseating granulomatous inflammation of multiple organ systems, with pulmonary, cardiac, and neurologic involvements having the worst prognosis 2.
• The disease can affect multiple organs, with a predilection for the lung and thoracic lymph nodes, and is estimated to affect 2-160 people per 100,000 worldwide 3.
• The mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period, with more than 60% of deaths resulting from advanced cardiopulmonary disease 3, 4.

Diagnosis and Treatment

• Scadding stages for pulmonary sarcoidosis are associated with clinical outcomes, with stages I and II having a higher chance of radiographic remission compared to stages III and IV 3.
• First-line treatment for patients with symptomatic pulmonary sarcoidosis is oral glucocorticoids, such as prednisone, with a typical starting dose of 20-40 mg daily for 2 weeks to 2 months 3, 4.
• Second-line treatments include immunosuppressive agents, such as methotrexate and azathioprine, which have been shown to have significant steroid-sparing potency and a positive effect on lung function 5, 6.
• Anti-tumor necrosis factor agents, such as infliximab, are effective for advanced disease and can be used as third-line therapy 2, 5.

Treatment Outcomes

• The rate of spontaneous remission without serious sequelae in patients with pulmonary sarcoidosis ranges from 10% to 82% 4.
• Up to 40% of patients with pulmonary sarcoidosis progress to stage IV disease, which is associated with a higher risk of mortality 3.
• Patients with sarcoidosis and precapillary pulmonary hypertension can respond to targeted pulmonary arterial hypertension medications, such as phosphodiesterase inhibitors and prostacyclin analogues 3, 4.
• Relapse rates for sarcoidosis range from 13% to 75%, depending on the stage of the disease, number of organs involved, socioeconomic status, and geography 3.