Glucocorticoids in systemic sclerosis: weighing the benefits and risks - a systematic review

To identify indications for which different dosages of glucocorticoids (GCs) have been prescribed in systemic sclerosis (SSc), and to assess the efficacy and safety of GCs in SSc.

A literature search focusing on experimental studies, observational studies, and case reports describing GC use in SSc was conducted using PubMed, EMBASE and Cochrane databases. Information about the study population, GC therapy and its effects was recorded. Available data have been summarised, and efficacy and safety of GCs have been assessed for different indications and dosages.

Forty-four studies and 93 case reports were included in this review. GCs were applied in the treatment of interstitial lung disease (ILD), diffuse cutaneous disease, myopathy, painful hands and cardiac involvement, or accompanying anti-thymocyte globulin to prevent serum sickness in the context of stem cell transplantation. GCs were used in different dosages, predominantly in combination with other immunosuppressive treatments. Monotherapy with GCs led to inconsistent results. Most adverse events recorded were infections. Twenty-three cases of scleroderma renal crisis (SRC) have been reported, mainly in patients with early diffuse disease (n=10) or with anti-thymocyte treatment (n=10). These patients were treated with low to medium dose GCs (n=10), high-dose GCs (n=11) and pulse therapy (n=2).

Evidence of a beneficial role of GCs in SSc is limited. GCs have been part of the therapeutic strategy in the management of ILD, diffuse cutaneous disease or myositis. Awareness for the risk of SRC should persist, especially in patients with diffuse disease who are also treated with possibly nephrotoxic drugs.