Long-term clinical course of anti-glycyl tRNA synthetase (anti-EJ) antibody-related interstitial lung disease pathologically proven by surgical lung biopsy

Table 7 The clinical courses and therapy of the 12 patients

Patient number	Response to initial therapy	Disease onset	Diagnosis	Pathological diagnosis	Initial therapy	Observation period (months)	Period from initial therapy to relapse (months)	Therapy at relapse^a	Prognosis	Disease behavior
1	improved	acute	IIP	c-NSIP	PDN	29	-	-	alive	A
2	improved	acute	IIP	unclassifiable IP	PDN + CyA	27	-	-	alive	A
3	improved	acute	IIP	unclassifiable IP	PDN + TAC	90	-	-	alive	A
4	improved	chronic	PM	f-NSIP	Low-PDN	72	-	-	alive	A
5	improved	chronic	IIP	c-NSIP	PDN + TAC	76	-	-	alive	A
6^SS	improved	chronic	DM	f-NSIP	PDN + TAC	19	-	-	alive	A
7	relapsed	acute	IIP	f-NSIP	PDN	94	29	PDN	alive	B
8	relapsed	acute	PM	f-NSIP	PDN^c	82	61	PDN	alive	B
9	relapsed	acute	DM	c-NSIP	PDN^c	70	8	PDN	alive	B
10	relapsed	acute	IIP	c-NSIP	PDN^c + CyA	115	46	PDN + CyA	alive	B
11	relapsed	chronic	PM	f-NSIP	PDN + AZA	104	78	PDN	alive	B
12	deteriorated	acute	PM^b	unclassifiable IP	PDN + CyA	17	-	-	dead	C

^aPatients have received these therapies until ILD relapsed., SS: Sjögren’s syndrome, IIP: idiopathic interstitial pneumonia, PM: polymyositis, DM: dermatomyositis, ^bsimultaneous onset of PM and interstitial lung disease, c-NSIP: cellular nonspecific interstitial pneumonia, f-NSIP: fibrosing nonspecific interstitial pneumonia, IP: interstitial pneumonia, PDN: 0.5 mg/kg/day of prednisone, CyA: cyclosporine A, TAC: tacrolimus, Low-PDN: 0.2 mg/kg/day of prednisone, ^cstarted with methylprednisone pulse therapy (500 mg/body), AZA: azathioprine, A: progressive, irreversible disease with potential for stabilization, B: reversible disease with risk of progression, C: progressive, irreversible disease despite therapy

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