What is the prognosis of sarcoidosis?

From the Guidelines

The prognosis of sarcoidosis is generally favorable, with about 60-70% of patients experiencing spontaneous remission within 2-5 years of diagnosis, although mortality from the disease can be as high as 5% 1.

Overview of Sarcoidosis Prognosis

The prognosis of sarcoidosis varies significantly based on several factors including organ involvement, disease severity, and patient demographics.

• Pulmonary sarcoidosis, the most common form, typically has a better prognosis than cardiac or neurological involvement, which can lead to more serious complications.
• About 10-30% of patients develop chronic disease requiring long-term treatment, typically with corticosteroids like prednisone or steroid-sparing agents such as methotrexate or hydroxychloroquine.
• African Americans and older patients tend to have more severe disease and poorer outcomes.

Mortality and Morbidity

Mortality from sarcoidosis is low, but can be as high as 5% 1, with death usually resulting from pulmonary fibrosis, cardiac complications, or neurological involvement.

• Regular follow-up is essential as relapses can occur, even years after apparent remission, and early intervention can prevent progression to irreversible organ damage.
• The use of anti-inflammatory therapy for less severe but impaired patients may prevent progression to irreversible disease 1.

Treatment Approach

The treatment approach for sarcoidosis depends on the presence of symptomatic disease, and the use of oral glucocorticoids as first-line therapy, with implementation of non-biologic and biologic immunomodulatory therapies used primarily as steroid-sparing therapies and/or to treat steroid-refractory disease 1.

• A proposed treatment algorithm highlights key concepts by sarcoidosis phenotype, and is derived from the resulting Delphi consensus recommendations 1.
• The algorithm assists the clinician in making treatment decisions, and considers the use of several treatment regimens, including corticosteroids, methotrexate, and biologic agents such as infliximab.

From the Research

Prognosis of Sarcoidosis

The prognosis of sarcoidosis varies depending on several factors, including the severity of the disease, the organs involved, and the response to treatment.

• Approximately 10% to 30% of patients with sarcoidosis develop progressive pulmonary disease 2.
• The rate of spontaneous remission without serious sequelae ranges from 10% to 82% among patients with pulmonary sarcoidosis 2.
• Lung disease progression occurs in more than 10% of patients and can result in fibrocystic architectural distortion of the lung, which is associated with a mortality rate of 12% to 18% within 5 years 2.
• The mortality rate for sarcoidosis is approximately 7% within a 5-year follow-up period 2.

Factors Affecting Prognosis

Several factors can affect the prognosis of sarcoidosis, including:

• The presence of rheumatological markers 3
• Pulmonary function 3
• Early respiratory symptoms manifestations 3
• The severity of organ involvement 2
• The response to treatment 2, 4, 5, 6

Treatment and Prognosis

Treatment can significantly impact the prognosis of sarcoidosis.

• Corticosteroids are the first-line treatment for symptomatic patients with abnormal pulmonary function test results and lung infiltrates 2, 6.
• Immunomodulatory agents, such as methotrexate and azathioprine, can be used as second-line therapy for patients who do not respond to corticosteroids or have severe side effects 4, 5.
• Patients with sarcoidosis and precapillary pulmonary hypertension should be treated with therapies such as phosphodiesterase inhibitors and prostacyclin analogues 2.

Predicting Prognosis

A scoring system can be used to predict the likelihood of a favorable or unfavorable outcome in patients with sarcoidosis 3.

• The absence of rheumatological markers, normal pulmonary functions, and the presence of early respiratory symptoms manifestations are associated with a favorable prognosis 3.
• A patient with a score of 5 has an 86% probability of having a favorable prognosis, while those with lower scores have a lower probability of a favorable prognosis 3.