Corticosteroids are the mainstay of treatment for
sarcoidosis. Although the indications for medical
therapy of
sarcoidosis are controversial, standard
therapy for symptomatic, progressive disease consists of
corticosteroids. The British Thoracic Society concluded, with respect to systemic
corticosteroids for the treatment of
sarcoidosis, that some patients required no treatment, some required
prednisone for control of symptoms, and others, with persistent disease, appeared to benefit from long-term
corticosteroid therapy. Inhaled
budesonide can be an effective treatment for lung
sarcoidosis, with few adverse effects, when used in combination with oral systemic
corticosteroids such as
deflazacort administered in a tapered regimen for 6 months. A randomized controlled trial has also demonstrated the efficacy of 3 months of treatment with oral
prednisolone in a tapered regimen followed by inhaled
budesonide for 15 months in patients with early stage
pulmonary sarcoidosis.Alternative drugs are required in chronic resistant
sarcoidosis and/or in conditions where systemic
corticosteroids are contraindicated.
Immunosuppressive agents (
chlorambucil,
cyclophosphamide,
methotrexate,
cyclosporine,
azathioprine), anticytokine agents (
thalidomide,
pentoxifylline),
antimalarials (
chloroquine,
hydroxychloroquine),
melatonin and
monoclonal antibody (
infliximab) have been used in such situations.
Chlorambucil and
cyclophosphamide have been used in anecdotal cases of
pulmonary sarcoidosis as
corticosteroid-sparing agents. However, their toxicity and neoplastic potential recommend prudence in patient selection. A comparison between combination
therapy with
cyclosporine and
prednisone and
prednisone alone has shown an increased prevalence of serious adverse effects with combined
therapy with no between-group differences in treatment efficacy. The cost and toxicity of
cyclosporine limit its use to patients in whom its efficacy has been proven. In patients with chronic or refractory disease,
methotrexate, usually administered once a week as a single oral dose for at least 2 years, has resulted in a significant improvement in respiratory function, chest radiographs and extrapulmonary manifestations. In most patients, this treatment enabled discontinuation of
corticosteroids.
Azathioprine may be effective as a
corticosteroid-sparing agent in the long-term treatment of
sarcoidosis. The combination of
prednisolone and
azathioprine over a period of 2 years has induced long-lasting remission in patients with resistant
sarcoidosis.
Thalidomide at low doses is effective in selected cases of
sarcoidosis with cutaneous and mild pulmonary involvement.
Pentoxifylline alone or combined with low doses of
corticosteroids has achieved significant improvement in respiratory function in patients with
pulmonary sarcoidosis.
Chloroquine and
hydroxychloroquine have been shown to have a specific effect in cutaneous
manifestations, neurological involvement and
hypercalcemia associated with
sarcoidosis.
Infliximab has yielded good results in patients with chronic resistant pulmonary and extrapulmonary
sarcoidosis resistant to
corticosteroid and cytotoxic
therapy. The effectiveness of
melatonin in cutaneous and
pulmonary sarcoidosis has also been confirmed in a single center.