Managing panniculitis in alpha-1 antitrypsin deficiency: Systematic review of evidence behind treatment

Table 1 Characteristics of patients studied and their response to treatment

	Total, n (male)	Age, median (range)	Response, n (%)				Ref.
			Complete	Partial	None	Not tolerated
Dapsone	21 (5)	33 (13-65)	13 (62)	-	4 (19)	4 (19)	[9-25]
Single-agent antibiotics	21 (9)	33 (7-62)	4 (19)	4 (19)	13 (62)	-	[9,12,15,20,23,25-31]
Immunosuppressants	21 (8)	33 (13-65)	3 (14)	1 (5)	16 (76)	1 (5)	[9,11,14,15,18-20,25,27,28,31-35]
Augmentation	12 (2)	33 (16-65)	12 (100)	-	-	-	[1,12,14,18,19,29,30,36-39]
Antibiotics and steroids	10 (2)	34 (32-42)	1 (10)	3 (30)	6 (60)	-	[19,20,28,34,38,40]
Combination antibiotics	9 (4)	24 (20-62)	-	-	9 (100)	-	[14,15,23,25,36,37,39,41]
NSAIDs	8 (2)	32 (16-65)	1 (13)	1 (13)	6 (75)	-	[1,12,19,23,27,31,42]
Liver transplant	3 (2)	56 (53-57)	2 (67)	1 (33)	-	-	[23,36,43]
Plasma exchange	3 (1)	33 (23-33)	2 (67)	-	1 (33)	-	[18,27,31]
No treatment	2 (1)	34 (7-62)	-	2 (100)	-	-	[15,29]
Other	13 (4)	42 (26-62)	2 (15)	1 (8)	9 (69)	1 (8)	[17,18,20,26,29,32,33,41,42]

Note that each patient investigated could have multiple treatments attempted to control their AATD-related panniculitis. A complete response was one reported by the authors as clinically successful, requiring no further intervention. This may have taken the form of maintenance or intermittent therapy. A partial response was one of incomplete or temporary resolution of symptoms, requiring further treatments. NSAID: Non-steroidal anti-inflammatory drugs.