Table 2 Literature review: characteristics of 17 patients

Refs	Sex	Age(years)	ANCA-V	Status	Chronology of PF-ANCA-V	Follow-up(months)	Clinical	Typeof PF	Possible or proven IAH	Vasculitis on lung biopsy	MPO-ANCAs
12	F	61	MPA	Dead: exacerbation of RI	PF preceded ANCA-V (4 years)	1	L K	UIP	0	–	Yes
	M	63	MPA	Alive	Same time	>12	L K	ND	Yes	–	Yes
	F	68	MPA	Dead: exacerbation of RI	Same time	>84	L K	UIP	Yes	–	Yes
	M	64	MPA	Dead: exacerbation of RI + Pneumocystis jirovecili pneumonia	PF preceded ANCA-V (4 years)	8	L K	UIP	1	No	Yes
	M	79	MPA	Dead: exacerbation of RI	Same time	∼12	L K	UIP	0	–	Yes
	F	78	MPA	Dead: exacerbation of RI	Same time	ND	L K	UIP	Yes	No	Yes

13	F	48	MPA	Alive	PF preceded ANCA-V (1 year)	4	L K	ND	Yes	–	Yes
	M	77	MPA	Dead: fatal bacterial pneumonia	PF preceded ANCA-V (5 years)	19	L K	ND	0	–	Yes
	F	72	MPA	Dead: exacerbation of RI	PF preceded ANCA-V (6 years)	5	L K	ND	0	–	Yes
	M	70	MPA	Dead: lung carcinoma	PF preceded ANCA-V (4 years)	4	L K	ND	Yes	No	Yes

16	M	75	MPA	Dead: exacerbation of RI	PF after ANCA-V (3 months)	4	L K	ND	0	–	Yes

10	M	65	MPA	Alive	PF preceded ANCA-V (1 year)	12	L K S N A	PINS	0	–	P-ANCA

14	F	55	MPA	Alive	PF preceded ANCA-V (1 year)	ND	L K O	ND	0	No	Yes

11	F	77	MPA	Alive	Same time	>12	L M	UIP	Yes	Yes	Yes

15	M	71	ND	Dead: unknown	Same time	3	L K	ND	0	Yes	ND
	F	74	MPA	Dead: perforated diverticulum	Same time	48	L K	ND	0	–	P-ANCA
	F	72	MPA	Dead: unknown	Same time	72	L K	ND	0	Yes	P-ANCA

Total	9F/8M	69*							7 (41%)

*Average age.
A, arthritis; ANCA-V, systemic vasculitis related to antineutrophil cytoplasmic antibodies; IAH, intra-alveolar haemorrhage; K, kidney; L, lung; M, muscles; MPA, microscopic polyangiitis; MPO-ANCAs, anti-myeloperoxidase-ANCAs; N, neuropathy; ND, not determined; O, ophthalmology; P-ANCA, perinuclear-ANCAs; PF, pulmonary fibrosis; S, skin; UIP, usual interstitial pneumonia.