Table 3.

Research supporting the role of combined therapy, with glucocorticoids plus either cyclophosphamide or calcineurin inhibitors or triple therapy with the three of them, for the treatment of anti-MDA5 positive rapidly progressive interstitial lung disease (RPILD).

Reference	Type of study	Patients (n) RPILD	Exitus
16	Retrospective	n=20	9/20 (45%)
		12 (GC+CYC+CNI)	7/12 (58%)
		8 (GC+CYC or CNI) *	2/8 (25%)
40	Retrospective	n=15†ฎ	6/15 (40%)
		5 (GC+CYC+CNI)	Not specified
		10 (GC+CNI)	Not specified
34	Retrospective	n=10	6/10 (60%)
		10 (GC+CYC+CNI)
35	Retrospective	n=7	6/7 (85%)
		7 (GC+CYC+CNI)
22	Retrospective	n=11	0/11 (0%)
		11 (GC+CYC+CNI)
19	Retrospective	n=17	1/6 (16%)
		6 (GC+CYC+CNI)	3/11 (27%)
		11 (GC+CYCor CNI)*
10	Retrospective	n=28	25%
		14 (GC+CYC+CNI)	71%
		14 (Historical cohort)
37	Retrospective	n=12	3/4 (75%)
		4 (GC+CYC+CNI)	3/8 (37%)
		8 (GC+CNI)
39	Retrospective	n=7†ฎ	6/6 (100%)
		6 (GC+CYC+CNI)	1/1 (100%)
		1 (GC+CNI)
28,29,30,31,	Case reports (sum up)	n=16	5/16 (31%)
32,33,36,		10 (GC+CYC+CNI)	5/10 (50%)
38,41,42,		5 (GC+CNI)	0/5 (0%)
43,45,46.		1 (gc+cyc)	0/1 (0%)

GC, glucocorticoids; CYC, cyclophosphamide; CNI, calcineurin inhibitors; RP-ILD, rapidly progressive interstitial lung disease.

^*Number of patients treated with each combination not specified.

^†2patients with chronic NI.