Table 3. Acute exacerbation of interstitial lung disease in patients with systemic autoimmune rheumatic diseases: review of the literature.

Year	Author	Ref	Number of cases	Type of study	Radiological or histological pattern	Outcome	Incidence
2003	Rice AJ	4	8 (2 DM, 2 SSc, 2 SLE, 1 RA, 1 Still)	Autopsy Study; retrospective	3 NSIP, 5 none§	8/8 patients died*	NA
2006	Parambil JG	5	9 (5 AR, 2 PM, 1 SSc, 1 MCTD)	Biopsy study; retrospective	2 UIP, 1 NSIP, 3 OP, 3 none§	5/9 patients died	NA
2007	Park IN	6	4 (3 RA, 1 SSc)	Cohort study; retrospective	3 UIP, 1 NSIP	4/4 patients died	1-year frequency, 3.3%
2009	Suda T	7	6 (5 RA, 1 pSS)	Biopsy study; retrospective	3 UIP, 2 NSIP, 1 unclassifiable	5/6 patients died	1-year incidence, 1.25%
2016	Toyoda Y	8	10 (6 RA, 2 PM/DM, 1 SLE, 1 pSS)	Cohort study; retrospective	8 UIP, 2 NSIP	1/10 patients died	overall incidence, 6.5%
2007	Silva CI	9	8 (3 RA, 4 DM, 1 pSS)	Biopsy study; retrospective	5 UIP, 3 NSIP	6/8 patients died	NA
2013	Ando M	24	3 (2 UCTD, 1 AAV)	Cohort study; retrospective	3 UIP	3 patients died	NA
Present series	Manfredi A	–	9 (2 RA, 2 UCTD, 2 SSc, 1 MCTD, 1 AAV, 1 pSS)	Cohort study; prospective	6 UIP, 2 NSIP, 1 OP	5/9 patients died	1-year incidence, 5.77%

^§, in 8 patients there was no evidence of interstitial pneumonia before acute exacerbation; *, the study reviewed only deceased patients; DM, dermatomyositis; PM, polymyositis; SSc, systemic sclerosis; SLE, systemic lupus erythematosus; RA, rheumatoid arthritis; Still, adult Still disease; Pss, primary Sjögren’s syndrome; UCTD, undifferentiated connective tissue disease; MCTD, mixed connective tissue disease; AAV, associated vasculitis; NA, not available.