Table 1.
Peripheral Blood Cytokine Profiles in LS: Elevated cytokines associate with T helper cell linages and correlate with other inflammatory disease indicators, such as activity scores and clinical laboratory tests.
| Patient (n) and LS, SSc | Subtype | Ages median (IQR)mean ± SD | Sex (% female) | Clinical and serological correlations | ||
|---|---|---|---|---|---|---|
| CCL2/MCP-1 | Torok et al. (66)† | 69 Ped LS | 8 generalized 41 linear 8 plaque morphea |
13.0 (10.0–16.0) | 67% | – |
| CCL3/MIP-1a | O'Brien et al. (67)† | 87 LS | 49 generalized 28 linear 8 plaque morphea |
50 ± 20 years (89% adult) | 69% | – |
| CXCL8/IL−8 | Ihn et al. (68) | 48 LS 20 SSc | 16 generalized 22 linear 10 plaque morphea |
– | – | – |
| IL-2 | Ihn et al. (69) | 48 LS 20 SSc | 16 generalized 22 linear 10 plaque morphea |
– | – | RF |
| IL−2R | O'Brien et al. (67)† | 87 LS | 49 generalized 28 linear 8 plaque morphea |
50 ± 20 years (89% adult) | 69% | LoSDI |
| IL−4 | Ihn et al. (69) | 48 LS 20 SSc | 16 generalized 22 linear 10 plaque morphea |
– | – | AHA |
| IL−6 | Ihn et al. (69) | 48 LS 20 SSc | 16 generalized 22 linear 10 plaque morphea |
– | – | AHA |
| IL−13 | Hasegawa et al. (70)† | 45 LS | 12 generalized 22 linear 11 plaque morphea |
27 (range 5–67 years old) | 59% | Number of plaque lesions Number of lesions |
| IL−12 | Torok et al. (66)† | 69 Ped LS | 8 generalized 41 linear 8 plaque morphea |
13.0 (10.0–16.0) | 67% | – |
| O'Brien et al. (67)† | 87 LS | 49 generalized 28 linear 8 plaque morphea |
50 ± 20 years (89% adult) | 69% | LoSDI | |
| TNFα | Hasegawa et al. (70)† | 45 LS | 12 generalized 22 linear 11 plaque morphea |
27 (range 5–67 years old) | 59% | IgM AHA IgG ssDNA Muscle involvement |
| TGFβ1 | Uziel et al. (71)† | 55 Ped LS | 16 generalized 28 linear 10 plaque morphea |
9.2 ± 3.6 years | – | – |
| TGFβ2 | Budzynska-Włodarczyk et al. (72) | 17 LS | 9 generalized 6 other subtypes |
45.3 ± 14.6 years | 100% | – |
| sIL−2r | Uziel et al. (73) | 17 Ped LS | – | 8.1 years | – | – |
| sIL−6r | Nagaoka et al. (74) | 45 LS 20 SSc | 12 generalized 22 linear 11 plaque |
– | – | IgM RF Number of lesions Number of body areas |
| Budzynska-Włodarczyk et al. (72) | 17 LS | 9 generalized 6 other subtypes |
45.3 ± 14.6 years | 100% | ESR | |
| Nagaoka et al, (74) | 45 LS 20 SSc | – | – | – | IgM AHA RF Number of lesions Number of body areas |
|
| IL−23 | Danczak-Pazdrowska et al. (75)* | 41 LS | 14 generalized 7 linear 20 plaque |
43.7 ± 17.5 years | 53% | mLoSSI in plaque patients Disease duration in all subtypes |
| IL−17A | Danczak-Pazdrowska et al. (75)* | 41 LS | 14 generalized 7 linear 20 plaque |
43.7 ± 17.5 years | 53% | mLoSSI in plaque patients Disease duration in all subtypes |
| Torok et al. (66)† | 69 Ped LS | 8 generalized 41 linear 8 plaque morphea |
13.0 (10.0–16.0) | 67% | – | |
| IL−1 | Danczak-Pazdrowska et al. (75) | 41 LS | 14 generalized 7 linear 20 plaque |
44 ± 18 years | 50% | – |
| CXCL9./MIG | O'Brien et al. (67)*† | 87 LS | 49 generalized 28 linear 8 plaque morphea |
50 ± 20 years (89% adult) | 69% | mLoSSI LoSDI |
| Mertens et al. (76) | 80 LS | 16 generalized 30 linear 8 plaque morphea |
−80% adult | 59% | mLoSSI | |
| CXCL10./IP-10 | O'Brien et al. (67) | 87 | 49 generalized 28 linear 8 plaque morphea |
50 ± 20 years (89% adult) | 69% | LoSDI |
| Mertens et al. (76) | 80 LS | 16 generalized 30 linear 8 plaque morphea |
−80% adult | 59% | mLoSSI | |
| Magee et al. (77)*† | 69 Ped LS | 8 generalized 40 linear 5 plaque morphea |
12.5 (10.0–16.0) | 46% | PGA-A mLoSSI |
|
| sgp130 | Nagaoka et al, (74) | 45 LS 20 SSc | – | – | – | IgG Number of lesions Number of body areas |
Also demonstrated in skin
Includes pediatric patients
CCL2/MCP-1, Monocyte chemoattractant protein-1;
CCL3/MIP-1a, macrophage inflammatory protein 1 alpha;
CXCL8/IL-8, interleukin 8;
IL-2, interleukin 2;
IL-2R, interleukin 2 receptor;
IL-4, interleukin 4;
IL-6, interleukin 6;
IL-13, interleukin 13;
IL-12, interleukin 12;
TNFα, Tumor necrosis factor alpha;
TGFβ1, Transforming growth factor beta 1;
TGFβ2, Transforming growth factor beta 2;
sIL-2r, soluble interleukin 2 receptor;
sIL-6r, soluble interleukin 6 receptor;
IL-23, interleukin 23;
IL-17A, interleukin 17A;
IL-1, interleukin 1;
CXCL9/MIG, Chemokine (C-X-C motif) ligand 9/Monokine induced by gamma interferon;
CXCL10/IP-10, C-X-C motif chemokine 10/Interferon gamma-induced protein 10;
Sgp130, soluble gp130;
AHA, anti-histone antibody;
ANA, anti-nuclear antibody;
ESR, Erythrocyte sedimentation rate (sed rate);
IgG, Immunoglobulin G;
IgM, Immunoglobulin M;
LoSDI, Localized Scleroderma Damage Index;
mLoSSI, modified LS Skin Severity Index;
RF, Rheumatoid factor;
ssDNA, single stranded DNA antibody.