Table 6.

Selected evidence of SSc-ILD treatment with biologics.

STUDY [REF]	STUDY DESIGN	TREATMENT	N	INCLUSION	F/U	EPs	OUTCOME
Lam et al, 2002 [92]	Retrospective	Etanercept 25 mg twice/wk or 50 mg once/wk	18	Met 1980 ACR criteria or 3 of 5 features of CREST	N/A	%FVC,%DLCO	– %FVC and %DLCO declined
Daoussis et al, 2005 [96]	Open-label, randomized, controlled	Rituximab (four weekly 375 mg/m2 rituximab infusions/cycle × 2) vs placebo	8 and 6	SSc-ILD by HRCT and/or PFT	12 Mo	PFT, HRCT	– Increase of %FVC and %DLCO in Rituximab compared to controls
Daoussis et al, 2006 [97]	Retrospective	Rituximab (four weekly 375 mg/m2 rituximab infusions/cycle × 4) vs placebo	8	SSc-ILD by HRCT and/or PFT	24 Mo	PFT, HRCT	– I ncrease of %FVC and %DLCO in Rituximab compared to baseline
McGonagle et al, 2007 [99]	Case report	Rituximab 1000 mg together with 100 mg mPSL at baseline and day 15	1	An SSc-ILD patient failed to PSL + IVCY	3 wks	6MWD, FVC, DLCO, HRCT	– All parameters improved
Daoussis et al, 2010 [100]	Case report	Rituximab (four weekly 375 mg/m2 rituximab infusions/cycle × 4)	1	An SSc-ILD patient failed to PSL + IVCY	6, 12, and 18 Mo	6MWD, SpO2, FVC, DLCO, NYHA class	– All parameters improved
Elhai et al, 2013 [103]	Case report	Abatacept (10 mg/kg/month)	7	SSc with refractory myopathy	Mean 18 Mo	% of the number patients who were TLCO <70% or FVC <75%	– N o change compared with baseline and last visit
Becker et al, 2006 [118]	Case report	Basiliximab (20 mg/month 6×)	10	dcSSc with rapidly progressive disease and organ involvement (8 with ILD)	44 wk	%FVC, %DLCO	– %FVC, %DLCO improved – 4 patients improved ≥10% of FVC – 2 patients improved ≥10% of DLCO

Abbreviations: EP, endpoint; HRCT, high-resolution computed tomography; PFT, pulmonary function test; FVC, forced vital capacity; FEV₁, forced expiratory volume in 1 second; TLC, total lung capacity; DLCO, diffusing capacity of the lung for carbon monoxide; ILD, interstitial lung disease; PSL, prednisolone; Mo, months; y, years.