Myofiber stress-response in myositis: parallel investigations on patients and experimental animal models of muscle regeneration and systemic inflammation

Table 1 Clinical, biochemical, immunological and immunohistological features of myositis patients

Patient	Sex	Disease duration (months)	Muscle weakness	DM skin rash	Serum CPK^a (U/l)	EMG^b	ANA (IF)^b	Specific autoantibodies	Muscle biopsy^c
									Inflammation (site of biopsy)	Immunoreactivity for
										MHC-I	εMy (% of total)
Group I
1	F	4	P	A	5027	+	-	Jo-1, RoSSA	++ (VL)	++	36.60
2	F	15	P	A	8570	+	-	Jo-1	++ (D)	++	35.50
3	F	8	P	A	2762	-	+	Jo-1, RoSSA	++ (VL)	+	15.50
4	F	168	P	A	1100	+	-	-	++ (VL)	++	1.09
5	M	29	P	A	1125	-	+	Mi-2	++ (VL)	+	0.33
6	F	13	P	P	292	+	-	-	++ (VL)	++	0
7	F	3	P	A	6705	+	-	Jo-1	+ (VL)	+	7.79
8	F	25	A	A	1840	+	+	tRNA, RoSSA	+ (VL)	++	2.00
9	M	25	P	P	484	+	-	Mi-2	+ (D)	++	6.00
10	M	79	P	A	535	-	+	U1RNP, RoSSA	+ (VL)	++	36.60
11	F	9	P	A	2762	+	+	-	+ (VL)	+	15.55
12	F	13	P	A	666	+	-	-	+ (TA)	++	3.10
13	M	36	P	A	2580	-	+	tRNA	+ (VL)	+	0.20
14	F	103	P	A	893	+	+	Jo-1	+ (VL)	+	0.06
Group II
15	F	27	P	A	173	-	+	Jo-1	- (VL)	+	0.13
16	M	72	P	A	548	-	-	-	- (VL)	+	0
17	F	79	P	A	< 160	-	+	Ro/SSA	- (D)	++	0
18	M	33	P	P	600	ND	+	-	- (D)	++	0.42
19	F	21	P	A	221	+	+	Scl70, Ku	- (VL)	++	2.60
20	F	180	P	A	1157	+	+	Jo-1	- (VL)	+	0
21	F	67	P	A	533	+	+	Mitotic apparatus	- (D)	+	1.1
Group III
22	F	237	P	A	450	-	-	-	- (VL)	-	0.50
23	F	58	P	A	43	+	-	-	- (VL)	-	0
24	F	237	P	A	742	+	-	-	- (D)	-	0
25	M	24	P	A	250	-	-	-	- (VL)	-	0
26	F	15	P	A	1600	-	-	-	- (VL)	-	1.11
27	F	68	P	A	628	+	-	-	- (VL)	-	0
28	M	33	P	P	9868	+	-	-	- (VL)	-	0
29	F	24	P	P	254	-	+	-	- (VL)	-	0

^aValues correspond to those obtained at the time of muscle biopsy. ^b+ and -, presence and absence of positive response, respectively. ^c+ and ++, degree of inflammation and MHC-I immunofluorescence, evaluated as described in Materials and methods; -, absence. DM, dermatomyositis; CPK, creatine phosphokinase; EMG, electromyography; ANA (IF), antinuclear antibodies (immunofluorescence); MHC-I: class I major histocompatibility complex antigen; εMy (% of total), percentage of myofibers positive for embryonic skeletal myosin on total biopsy fibers; F, female; M, male; P, present; A, absent; VL, vastus lateralis muscle; D, deltoid muscle; TA, tibialis anterior muscle; ND, not done.

ISSN: 1478-6362