Table 1.

Characteristics of studies included in the meta-analysis (n = 14 studies)

Author and year	CTD-ILD subtype	Study design	N	RTX Dose	Duration	Concurrent treatment	Outcomes	Duration of follow-up	Adverse events with RTX
Daoussis et al. 2012	SSc-ILD	Case series	8	One cycle: IV, 375 mg/ m2, once weekly*4 weeks, every 6 months	4 cycles	None	FVC; DLCO	2 years	2 Respiratory infections requiring hospitalization, 1 with associated leukopenia, 1 infusion reaction
Keir et al. 2012	CTD-ILD	Case series	6	One cycle: 5-IV, 1000 mg administered on day 0 and day 14 1 patient: 375 mg/ m2, once weekly*4 weeks	1 cycle	None	FVC; DLCO	9–12 months	None
Fitzgerald et al. 2015	CTD-ILD	Case series	10	a. 1–1000 mg monthly b. 2-IV, 375 mg/ m2, monthly c. 7-IV, 1000 mg administered on day 0 and day 14	a, b*4 months; c*6 months	CYC*3	FVC; DLCO	3–27 months	None
Chen et al. 2016	Sjögren’s syndrome-ILD	Case series	10	One cycle: IV, 1000 mg administered on day 0 and day 14	Repeated the same protocol every half a year depending on Individual response	Hydroxychloroquine	FVC; DLCO	6 months	None
Lepri et al. 2016	CTD-ILD	Case series	21	Cumulative mean dose: SYN: 1.91 g; SSc: 1.75 g; MCTD: 1.4 g	2 years	ASS: Azathioprine* 8, MTX*1, IVIG*1, cyclosporine*1, CYC*3; SSc: MTX*9, MMF*1 MCTD: MMF, CYC, MTX	FVC; DLCO	2 years	1 Arrhythmia; 3 fatigue; 8 infections (2 serious with hospitalization)
Sharp et al. 2016	CTD-ILD	Case series	24	One cycle: IV, 1000 mg administered on day 0 and day 14	1–2 cycles	Oral immunosuppression	FVC; DLCO	6–12 months	None
Daoussis et al. 2017	SSc-ILD	Cohort (prospective)	33	One cycle: IV, 375 mg/ m2, once weekly*4 weeks, every 6 months	≥ 2 cycles	MTX*2; Hydroxychloro-quine*1; MMF*10	FVC; DLCO	2 years	2 Infusion reactions
Md Yusof et al. 2017	RA-ILD	Cohort (retrospective)	56	One cycle: IV, 1000 mg administered on day 1 and day 14	≥ 1 cycle	CYC	FVC; DLCO	6-12 months	12 Chest infections (none hospitalized)
Sari et al. 2017	SSc-ILD	Case series	14	One cycle: IV, 1000 or 500 mg, 2 infusions biweekly	4 received 1 cycle; 2 received 2 cycles; 2 received 3 cycles; 4 received 4 cycles; 2 received 5 cycles	None	FVC	15 months	None
Doyle et al. 2018	ASS-ILD	Case series	12	Not mentioned	Mean time to initiation of RTX after ILD identification: 4.4 years	Azathioprine, MMF, CYC, IVIG	FVC; DLCO	2 years	1 Anaphylaxis and 2 serious gastrointestinal complications requiring surgery (not described), but later resumed RTX
Sircar et al. 2018	SSc-ILD	RCT	30	One cycle: IV, 1000 mg administered on day 0 and day 15, every 6 months	2 cycles	None	FVC	6 months	1 Severe pulmonary arterial hypertension, 3 infusion reactions
Duarte et al. 2019	CTD-ILD	Case series	49	One cycle: IV, 1000 mg, 2 infusions biweekly	Median number of cycles was 2	None	FVC; DLCO	1 year	None
Ebata et al. 2019	SSc-ILD	Non-randomized study (retrospective)	9	One cycle: IV, 375 mg/ m.2, once weekly*4 weeks	1 up to 3 cycles	Maintenance therapy with immunosuppressant agents	FVC; DLCO	2 years	None
Fui et al. 2019	RA-ILD	Cohort (retrospective)	14	One cycle: IV, 1000 mg administered on day 0 and day 14	Treated for more than 1 year	None	FVC; DLCO	1 year	Discontinued in 2 patients with refractory severe arthritis