Table 8.

summary of the evidence for efficacy of systemic steroids in the treatment of auto-immune disease

Auto-immune disease + study	Year	LOE	Study design	Study groups	Conclusion
EGPA Moosig et al.	2013	3	A retrospective cohort study at a vasculitis referral centre	150 fulfilled the inclusion criteria. Of those, 104 had more than one follow-up visit severe organ manifestations: heart (46%), kidney (18%) and lungs (10%). Cyclophosphamide was used in 107 patients (71%). The prednisolone-doses of all patients were within the targeted range (i.e. ≤ 7.5 mg) in 69% of the total follow-up time; the median dose at end of follow-up was 5 mg/day	10-year survival rate was 89%, mortality comparable to the general population (SMR 1.29). Patients with cardiac failure had increased mortality (SMR 3.06)
GPA WGET Research Group	2005	1b	180-patient multicentre, placebo-controlled RCT examining the efficacy of etanercept in WGCT	Severe disease received cyclophosphamide and corticosteroids; limited disease received methotrexate and corticosteroids etanercept (25 mg twice weekly) or placebo was added to conventional therapy	Addition of etanercept did not lead to more sustained remissions; lower levels of disease activity; reduction in time to remission nor the number or relative risk of flares; nor fewer severe or life-threatening adverse events or deaths
Relapsing polychondritis McAdam et al.	1976	3	Review	159 reported cases, 23 those of the authors	Three-fourths of our patients required chronic corticosteroid therapy with an average dose of 25 mg per day of prednisone. Corticosteroids decrease the frequency, duration, and severity of flares, but do not stop disease progression in severe cases. Mortality rate 30 percent in our series and 22 percent in the other 136 reported cases
EGPA Ribi et al.	2008	2	RCT	72 patients with newly diagnosed EGPA (FFS of 0) treated with CS alone. At treatment failure or relapse, patients were randomized to receive 6 months of oral AZA or 6 pulses of CYC	93% achieved remission with CS alone, 35% relapsed, mainly during the first year of treatment. Among the 19 patients randomized to additional immunosuppression, 5 of 10 receiving AZA and 7 of 9 receiving pulse CYC achieved remission, P = NS Survival rates in all patients at 1 and 5 years were 100% and 97%, respectively. At the end of followup, 79% of the patients whose disease was in remission required low-dose CS therapy, mainly to control respiratory disease. CS-related adverse events were observed in 31% of the 72 patients
GPA Hoffman et al.	1992	3	An open-label pilot study of weekly low-dose methotrexate (MTX) plus glucocorticoids (GC) for treatment of patients with WG	Weekly administration of MTX (at a mean stable dosage of 20 mg) and GC in 29 WG patients	Marked improvement in 76%. Remission achieved in 69%. 7% improved but had intermittent smoldering disease that precluded total withdrawal of GC, and 17% had progressive disease within 2–6 months of starting the study treatment. Two patients who initially achieved remission later relapsed after GC discontinued. Of those who remain in remission (mean followup time 14.5 months), 72% have not required GC for a mean period of 10 months
Sarcoidosis Aubart et al.	2006	3	Retrospective single-center study	Twenty patients with histologically proven SNS (men/women, 7/13; mean age, 32 ± 9 year) were compared with control patients with sarcoid but without sinonasal (SN) involvement. Each patient was matched with 2 controls for the date of admittance in our institution	SNS had significantly more frequent and severe involvement of vital organs than controls, had a longer history of sarcoidosis, and required systemic treatment more frequently (100% vs. 57.7%, P < 0.001) and for a longer time (78 ± 42 months vs. 29 ± 18 months, P < 0.0001). Corticosteroids maintenance dosage was high (10.5 ± 6 mg daily) and mainly depended on SN involvement
GPA Guillevin et al.	1997	2	Prospective multi-centre RCT	50 newly diagnosed WG patients every patient received a daily injection of methylprednisolone for 3 days, followed by daily oral prednisone (1 mg/kg/day) and a 0.7-g/m2 pulse of CYC. Patients were then randomly assigned to prednisone plus intravenous pulse CYC (group A), n = 27 or prednisone plus oral CYC (group B) n = 23 as first-line treatment	Pulse CYC was as effective as oral CYC in achieving initial remission of WG with fewer side effects and lower mortality. However, in the long term, treatment with pulse CYC does not maintain remission or prevent relapses as well as oral CYC

EGPA eosinophilic granulomatosis with polyangiitis, GPA granulomatosis with polyangiitis, AZA azithromycin, CYC cyclophosphamide