Forty-four studies (comprising randomised controlled trials/RCTs, controlled trials and observational studies) were included in the review, together with 93 case reports. The overall quality of trials seemed low; quality of observational studies appeared reasonable. Follow-up ranged from six to 72 months, where reported.
Interstitial lung disease (three RCTs; two controlled trials; 18 observational studies; 478 patients): Results of three observational studies using glucocorticoids as monotherapy were mixed. A high quality RCT showed a trend towards stabilisation of lung function following combination therapy compared with placebo, although the difference was not statistically significant. Other results of co-medication were mixed. Adverse events were reported; those that could be partially attributed to glucocorticoids were infections, mood disturbances, dyspepsia, cushingoid appearance, transient shortness of breath, and cataract. There was one case of scleroderma renal crisis.
Diffuse cutaneous disease (two RCTs; eight observational studies; 238 patients): Glucocorticoids as monotherapy was studied in one low-quality RCT, which showed a small benefit (decrease of total skin score from baseline) in the treatment group, compared with placebo; monotherapy resulted in an improvement of skin disease in one observational study. The remaining observational studies showed positive effects of combination therapy involving glucocorticoids. Adverse events possibly related to glucocorticoids were infections, gastrointestinal complaints, vertigo, and hypertension. Scleroderma renal crisis was reported in 10 cases.
Prevention of serum sickness (one controlled trial; five observational studies; 80 patients): It was not possible to evaluate the efficacy of glucocorticoids, but ten cases of scleroderma renal crisis were reported.
Other results were reported in the paper.