DESTIN, FLA. — Treatment is not necessary in all patients with pulmonary sarcoidosis, Dr. Marc Judson said at the annual Rheumatology on the Beach.
More than two-thirds of patients with this form of sarcoidosis, which accounts for the vast majority of cases, will spontaneously remit. Thus the side effects of corticosteroids, the most common form of treatment, often are not worth the limited benefits, said Dr. Judson, professor of medicine at the Medical University of South Carolina, Charleston.
Furthermore, some retrospective evidence suggests corticosteroid treatment promotes relapse. In one study, more than 70% of treated patients relapsed, versus less than 10% who received no treatment, according to Dr. Judson. In another, the relapse rate was higher in patients who received a mean dose of 17 mg of prednisone daily, versus a mean dose of 10 mg daily.
He recommends using a decision analysis based on prognostic factors and degree of pulmonary function in patients with pulmonary sarcoidosis, whereby asymptomatic patients are untreated, and those with mild pulmonary dysfunction and minimal functional limitation are observed without treatment. These patients are likely to experience spontaneous remission.
Patients with an excellent prognosis (see sidebar) are also observed, but palliative care can be attempted when necessary—such as in those who develop severe sarcoid arthritis. Nonsteroidal anti-inflammatory drugs can help in these patients, he said.
In those with mild to moderate pulmonary dysfunction and mild to moderate functional limitation, treatment and observation for deterioration are both acceptable, but Dr. Judson recommends observation initially, with treatment for deterioration, and a steroid trial if no improvement is seen within 3–6 months.
Prednisone at 20–40 mg per day for 2–6 weeks is recommended in those who do undergo treatment. After the initial 2 to 6-week dosing regimen, the dose is tapered over 1–3 months to a maintenance dose, which is used for 3–9 months. The patient is then tapered off the drug over 1–3 months, followed by an observation period and a second trial in those who relapse.
Other treatment options include methotrexate, pentoxifylline, chloroquine, azathioprine, and infliximab. Methotrexate is the most studied and appears to have some benefit; azathioprine appears to have the least, he said.
Recent data on infliximab are promising. Dr. Judson and his colleagues found the overall change from baseline was 2.5% predicted forced vital capacity at 24 weeks in 93 patients treated with either 3 or 5 mg/kg of infliximab. The change was statistically significant. Furthermore, some patients had only mild disease; patients were also treated with prednisone; and the 2.5% change from baseline was in addition to benefits seen with prednisone.
Dr. Judson received research grants from Centocor, maker of infliximab.
ELSEVIER GLOBAL MEDICAL NEWS
Factors Can Predict Sarcoidosis Outcomes
Data suggest most patients with sarcoidosis will have spontaneous remission with or without treatment, while up to a third will develop chronic disease.
A literature review suggests there are several factors that can predict a good (likelihood of remission) or poor (likelihood of chronic disease development) prognosis.
Stage I vs. stage II-III disease as determined by chest x-ray and the presence of erythema nodosum appears to be linked to a good prognosis, Dr. Judson said.
The following predict poor prognosis: black race, extrathoracic disease, age 40 years or more, splenic involvement, lupus pernio, disease duration over 3 years, forced vital capacity less than 1.5 L, and stage IV disease/aspergilloma on chest x-ray. The latter two factors are also risk factors for death from sarcoidosis.
However, death from the disease occurs rarely (in 3%–5% of patients).