Table 2.

Selected evidence of SSc-ILD treatment with glucocorticoids.

STUDY [REF]	STUDY DESIGN	TREATMENT	N	INCLUSION	F/U	EPs	OUTCOME
Pakas et al, 2002 [60]	Open-label	IV CY (500 mg/m2 initially, followed by 750 mg/m2/month × 5 and bimonthly × 3) in combination with PSL (low dose (<10 mg/day) or high dose (1 mg/kg/day and decrease to 5 mg/day on alternate day))	12 and 16	SSc-ILD with FVC <70%	12 Mo	HRCT, PFT, dyspnea score	(Low dose group) – No improvement (High dose group) – I mprovement of %GGO, %FVC, %DLCO, and severity of dyspnea at 12 months
Campos et al, 2009 [61]	Randomized, single-blinded	IV CY (750–1000 mg/m2/monthly × 6 and bimonthly × 3) in combination with PSL (low dose (<10 mg/day) or high dose (1 mg/kg/day and decrease to 5 mg/day on alternate day))	10 and 13	SSc-ILD with GGO or honeycombing and/or the presence of active alveolitis in BALF with DOE, and decrease of FVC	12 Mo	%FVC and %DLCO and HRCT	(Low dose group) – R adiographic improvement
Domiciano et al, 2009 [62]	Prospective, open label, controlled	IV CY (1000 mg/m2 × 12 with or without PSL (60 mg/day for a month and decrease to 10 mg/day)	9 and 9	SSc-ILD with NISP pattern by lung biopsy	12 and 36 Mo	%FVC, %DLCO, %FEV1, %TLC	– %FVC, %DLCO comparable
Ando et al, 2009 [62]	Retrospective	IV CY (500 mg/m2 × 6) with 0.3–0.5 mg/kg/day of PSL), PSL alone (0.5–1.0 mg/kg/day), without treatment	7 and 14 and 50	SSc-ILD based on PFT and CT findings	Median 9.8 + 3.3 y	Change of FVC,	– Change of FVC: better in IVCY combination or PSL alone compared to without treatment

Abbreviations: EP, endopoint; CY, cyclophosphamide; DD, disease duration; BAL, broncoalveolar lavage; HRCT, high-resolution computed tomography; PFT, pulmonary function test; GGO, ground-glass opacity; FVC, forced vital capacity; DLCO, diffusing capacity of the lung for carbon monoxide; FEV₁, forced expiratory volume in 1 second; ILD, interstitial lung disease; IV, intravenous; NSIP, nonspecific interstitial pneumonia; PSL, prednisolone; Mo, months; y, years.