Natural History and Prognosis Constitutional symptoms, such as fever, unintentional weight loss, and fatigue, occur at least in one-third of patients. Although spontaneous remission may occur in nearly two-thirds of patients, between 10% and 30% of patients with sarcoidosis experience a chronic or progressive course. Early in the course of the disease, the prognosis for remission is more favorable, with spontaneous remission observed in 50% to 90% within the first two years of diagnosis, depending on disease stage.
Diagnosis According to an international consensus statement, there are three criteria for diagnosing sarcoidosis: (1) a compatible clinical and radiologic presentation, (2) pathologic evidence of noncaseating granulomas and (3) exclusion of other diseases with similar findings see the reference link.
Certain sarcoidosis-specific syndromes, such as Löfgren and Heerfordt syndromes, can be diagnosed based on clinical presentation alone, avoiding the need for tissue biopsy. An asymptomatic patient with stage I sarcoidosis (i.e., bilateral hilar lymphadenopathy on chest radiography) without suspected infection or malignancy does not require invasive tissue biopsy because the results would not affect the recommended management approach (i.e., monitoring). If there would be an indication for treatment with a confirmed diagnosis.
Skin Cutaneous involvement occurs in about 25% of patients, although probably underdiagnosed, and is often an early finding; although usually asymptomatic, pruritus and pain have been reported.1 Skin lesions include macules, papules, plaques, ulcers, pustules, erythroderma, or hypopigmented lesions.
Eye Because asymptomatic inflammation of the eye can result in permanent impairment, patients require yearly examinations and additional monitoring when the disease flares. Ocular involvement occurs in 20% to 50% of patients and is the presenting symptom in 5%.
Heart Although cardiac sarcoidosis is noted in 25% to 70% of autopsies, symptomatic cardiac involvement occurs in only about 5% of patients. Granulomas often infiltrate the conducting system, leading to arrhythmias and heart block, but cardiac involvement can also lead to dilated cardiomyopathy. Progressive heart failure and sudden death are the most serious complications of cardiac sarcoidosis. It is essential to evaluate palpitations and syncope or near syncope and perform baseline electrocardiography. Any suggestion of cardiac involvement requires echocardiography and prompt referral to a cardiologist.
Neurology Neurologic involvement occurs in about 10% of patients with sarcoidosis. Cranial neuropathy, particularly palsy of the seventh cranial nerve, is the most common neurologic complication.
Calcium metabolism may also be dysregulated, resulting in hypercalciuria, hypercalcemia, and nephrolithiasis with possible renal insufficiency.
Treatment of Pulmonary Sarcoidosis Treatment is not indicated for patients with asymptomatic stage I or II sarcoidosis because a spontaneous resolution is common. Treatment with corticosteroids should be considered for patients with significant symptomatic or progressive stage II or III pulmonary disease or serious extrapulmonary disease. Although no sufficient evidence of its long-term effects, corticosteroids remain the mainstay of treatment based on expert opinion and usual practice for patients with significant symptomatic or progressive stage II or III disease, or serious extrapulmonary disease.
Second- and third-line therapies for pulmonary sarcoidosis, including methotrexate, azathioprine (Imuran), leflunomide (Arava), biologic agents, and corticotropin (H.P. Acthar) gel are reserved for patients with a corticosteroid-refractory disease, intolerable adverse effects, or toxicity from corticosteroids, as well as patients who choose not to take corticosteroids.