Although spontaneous improvement is common, the severity and manifestations of disease are highly variable, and many patients require courses of corticosteroids some time during the course of their disease. Thus, serial monitoring for evidence of relapse is imperative. About 90% of patients who have spontaneous remission do so within the first 2 yr after diagnosis; < 10% of these patients have relapses after 2 yr. Those patients who do not experience remission within 2 yr are likely to have chronic disease.

Sarcoidosis is thought to be chronic in up to 30% of patients, and 10 to 20% experience permanent sequelae. The disease is fatal in 1 to 5% of patients. Pulmonary fibrosis with respiratory failure is the most common cause of death worldwide, followed by pulmonary hemorrhage from aspergilloma. In Japan, however, infiltrative cardiomyopathy causing heart failure and arrhythmias is the most common cause of death.

Prognosis is worse for patients with extrapulmonary sarcoidosis and for blacks. Recovery occurs in 89% of whites and 76% of blacks with no extrathoracic disease and in 70% of whites and 46% of blacks with extrathoracic disease. The presence of erythema nodosum and acute arthritis are good prognostic signs. Uveitis, lupus pernio, chronic hypercalcemia, neurosarcoidosis, nephrocalcinosis, myocardial disease, and extensive pulmonary involvement are all poor prognostic signs. However, little difference is demonstrable in long-term outcome between treated and untreated patients, and relapse is common when treatment ends.

Treatment

Because sarcoidosis often spontaneously resolves, asymptomatic patients and those with mild symptoms do not require treatment, although they should be monitored for signs of deterioration. These patients can be followed with serial x-rays, pulmonary function tests (including diffusing capacity), and markers of extrathoracic involvement (eg, routine renal and liver function testing). Patients who require treatment regardless of stage include those with worsening symptoms; limitation of activity; markedly abnormal or deteriorating lung function; worrisome x-ray changes (cavitation, fibrosis, conglomerate masses, signs of pulmonary hypertension); heart, nervous system, or eye involvement; renal or hepatic insufficiency or failure; or disfiguring skin and joint disease.

Treatment is with corticosteroids. A standard protocol is prednisone Some Trade Names
DELTASONE
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0.3 to 1mg/kg po once/day depending on symptoms and severity of findings. Alternate-day regimens are also used (eg, prednisone Some Trade Names
DELTASONE
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40 to 60 mg po once every other day). It is rare to exceed 40 mg/day; however, higher doses may be needed to reduce complications in patients with ocular, myocardial, or neurologic disease. Response usually occurs within 2 to 4 wk, so symptoms and results of chest x-ray and pulmonary function tests may be reassessed between 4 and 12 wk. Chronic, insidious cases may respond more slowly. The drug is tapered to a maintenance dose (eg, prednisone Some Trade Names
DELTASONE
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≤ 10 mg every other day if possible) after evidence of response and is continued for a minimum of 12 mo if improvement occurs. The optimal duration of treatment is unknown. Premature taper can result in relapse. The drug is slowly stopped if response is absent or equivocal. Corticosteroids can ultimately be stopped in most patients, but because relapse occurs up to 50% of the time, monitoring should be repeated, usually every 3 to 6 mo. Corticosteroid treatment should be resumed for recurrence of symptoms and signs, including dyspnea, arthralgia, fever, hepatic insufficiency, cardiac arrhythmia, CNS involvement, hypercalcemia, ocular disease uncontrolled by local drugs, and disfiguring skin lesions.

Data on use of inhaled corticosteroids for pulmonary sarcoidosis are not definitive, but some evidence suggests that this route of administration can relieve cough in patients with endobronchial involvement. Topical corticosteroids may be useful in some cases of dermatologic and ocular disease.

About 10% of patients requiring therapy are unresponsive to tolerable doses of a corticosteroid and should be given a 6-mo trial of methotrexate Some Trade Names
RHEUMATREX
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starting at 2.5 mg po once/wk and increasing in increments of 2.5 mg/wk to a total of 10 to 15 mg/wk as tolerated to keep WBC > 3000/μL. Initially, methotrexate Some Trade Names
RHEUMATREX
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and corticosteroids are both given; over 8 wk, the corticosteroid dose can be tapered and, in many cases, stopped. The maximal response to methotrexate Some Trade Names
RHEUMATREX
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, however, may take 6 to 12 mo. In such cases, prednisone Some Trade Names
DELTASONE
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must be tapered more slowly. Serial blood counts and liver enzyme tests should be performed every 1 to 2 wk initially and then every 4 to 6 wk once a stable dose is achieved. Folic acid (1 mg po once/day) is recommended for patients treated with methotrexate Some Trade Names
RHEUMATREX
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.

Other drugs reported to be effective in small numbers of patients who are corticosteroid-resistant or who experience complicating adverse effects include azathioprine Some Trade Names
IMURAN
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, cyclophosphamide Some Trade Names
CYTOXAN
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, chlorambucil Some Trade Names
LEUKERAN
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, chloroquine Some Trade Names
ARALEN
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or hydroxychloroquine Some Trade Names
PLAQUENIL
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, thalidomide Some Trade Names
THALOMID
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, pentoxifylline Some Trade Names
TRENTAL
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, and infliximab Some Trade Names
REMICADE
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.

Hydroxychloroquine Some Trade Names
PLAQUENIL
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200 mg po bid to tid can be as effective as corticosteroids for treatment of disfiguring skin sarcoidosis and in treatment of hypercalciuria. Although immunosuppressants are often more effective in refractory cases, relapse is common after cessation.

No available drugs have consistently prevented pulmonary fibrosis.

Lung transplantation is an option for patients with end-stage pulmonary involvement, although disease may recur in the transplanted organ.

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