Treatments for pulmonary sarcoidosis

Abstract

Corticosteroids (oral or inhaled) are commonly used to treat pulmonary sarcoidosis; however, there is no consensus about when to start treatment, what dose of steroids to give and for how long. Immunosuppressive and cytotoxic agents (used in immunosuppressive doses) are used in addition to oral corticosteroids to treat multisystem and chronic sarcoidosis, or as steroid-sparing agents. We summarize the findings from two Cochrane systematic reviews that have examined the efficacy of corticosteroids and immunosuppressive and cytotoxic drugs in the treatment of pulmonary sarcoidosis. Studies of corticosteroids differed in outcome measures, dose of drug given and length of treatment. For many outcome measures, data could not be pooled for meta-analysis. Oral corticosteroids improved chest X-ray appearance over 3-24 months, with improvement in global score in one study. Little evidence was found of improvement in lung function or of any long-term disease-modifying effect. Follow-up data could not be analysed. Inhaled corticosteroids improved symptoms in one small study but not lung function or chest X-ray. Side-effects of steroids were not well reported. In the immunosuppressive and cytotoxics review, no data could be combined for meta-analysis. Data on lung function, chest X-ray and dyspnoea were largely inconclusive. Methotrexate had a steroid-sparing effect in one small study. Significant adverse events were associated with cyclosporine A, chloroquine and pentoxifylline. Evidence from randomized-controlled trials (RCTs) supporting the use of immunosuppressive and cytotoxic agents is limited.

The Utility of 18F-FDG PET/CT for Diagnosis and Adjustment of Therapy in Patients with Active Chronic Sarcoidosis

“…Another potential limitation was the relatively short followup. The finding that half our patients were still symptomatic despite changes in therapy can be explained by the fact that it may take up to 2 y to favorably respond to corticosteroid treatment (37,38). Thus, it remains to be seen whether the apparent association between the 18 F-FDG PET/CT findings and the decision to change therapy altered the course of disease or affected quality of life in patients with chronic sarcoidosis.…”

Association of the TNF- G-308A polymorphism with TNF-inhibitor response in sarcoidosis

“…In case of intolerable side-effects or steroid resistance, second-line treatment options should be considered, such as methotrexate (MTX), azathioprine or leflunomide, but these therapies may also be subject to failure or intolerance [8][9][10]. Another option to consider is TNF inhibitors, which inhibit the potent pro-inflammatory TNF-a, often a predominant problem in patients with refractory sarcoidosis, and these have shown promising results [11][12][13][14].…”

A review on guidelines for management and treatment of common variable immunodeficiency

“…High doses of systemic corticosteroids (10 mg a day or 20 mg every other day or twice-daily inhaled beclomethasone) is the first choice for interstitial lung disease with restrictive ventilatory defect due to lymphoproliferative disease such as pulmonary granulomas and LIP, but their longterm use is limited because of the risk of infections [136,137]. For long-term therapy, hydroxychloroquine is prescribed with dosage of 200-400 mg a day (range: 3.5-6.5 mg/kg) [138][139][140].…”

“…For long-term therapy, hydroxychloroquine is prescribed with dosage of 200-400 mg a day (range: 3.5-6.5 mg/kg) [138][139][140]. Steroid-sparing immunosuppressive agents have also been recommended in special situations when inflammation is predominantly pulmonary, including cyclosporine A (125 mg a day) [135], methotrexate [141], azathioprine, mycophenolate mofetil and 6-mercapto purine [136]. All mentioned therapeutic modalities are used in case reports or limited studies and their results for extrapolation to CVID should be tested in clinical trials.…”