Table.

Clinical Features of Patients with MCTD Or Sjögren’s Syndrome Presenting with Rapidly Progressive Glomerulonephritis Due to ANCA-associated Glomerulonephritis.

References	Reported year	Age (years/gender)	MCTD	pSS	Interval (months)	MPO/ PR3-ANCA	Cr(mg/dL)	Proteinuria	Microscopic hematuria	Therapy	Outcome
25	2000	58/F	+	-	192	+/-	1.5	0.77 g/day	+	mPSL, PSL, CYP	imp
26	2006	47/F	+	-	228	+/	2.7	6.5 g/day	+	mPSL, PSL, CYP	imp
27	2006	42/F	+	+, #	168	+/-	6.0	+	+	mPSL, PSL, CYP, PE	dead
28	2011	68/F	+	-	48	*+/	0.58	2+	+	PSL	dead
29	2013	42/F	+	-	36	+/-	0.9	11 g/day	+	PSL, CYP	imp
30	2014	35/F	+	-	8	+/	0.95	4.1 g/day	+	mPSL, PSL, CYP	imp
31	1992	63/F	-	+	7	+/-	2.3	2.07 g/day	+	PSL, mPSL, CYP	imp
32	1996	74/F	-	+	36	+/-	2.6	1.60 g/day	+	mPSL, PSL	imp
33	1999	67/F	-	+	7	+/-	2.8	0.43 g/day	+	mPSL, PSL CYP, PE	imp
34	2000	49/F	-	+	24	+/-	1.3	1.20 g/day	+	mPSL, PSL	imp
35	2009	49/F	-	+	12	+/-	1.2	0.48 g/day	+	mPSL, PSL MMF	imp
36	2011	86/M	-	+	0	+/-	4.2	1.31 g/day	+	PSL, CYP	ESRD
37	2014	66/F	-	+	72	+/-	2.8	2.40 g/day	+	PSL	imp
38	2015	64/M	-	+	528	+/-	2.22	1.97 g/day	+	mPSL, PSL, CYP, AZA	imp
38	2015	71/F	-	+	101	+/-	2.04	1.70 g/day	+	mPSL, PSL, CYP	imp
38	2015	57/M	-	+	12	+/-	3.77	6.50 g/day	+	mPSL, PSL, CYP	death
39	2015	65/F	-	+	94	+/-	1.6	1.6 g/day	+	mPSL, PSL AZA	imp
40	2016	71/F	-	+	1	+/+	2.9	1.68 g/day	+	mPSL, PSL, CYP	imp
Our case		67/F	+	+	228	+/-	0.82	0.58 g/gCr	+	mPS, PSL, RTX	imp

Interval: interval between the first symptoms of Sjögren’s syndrome or MCTD and presentation of RPGN, +: presence or positive, -: absence or negative. #: secondary Sjögren’s syndrome, *: MPO-ANCA related crescentic glomerulonephritis and immune complex glomerulonephritis. imp: improvement, ESRD: end stage renal disease, mPSL: methylprednisolone pulse, PSL: oral prednisolone, CYP: cyclophosphamide, PE: plasma exchange, MMF: mycophenolate mofetil, AZA: azathioprine, RTX: rituximab