SSc features at myositis diagnosis in 20 patients with seronegative scleromyositis
| Patient No. | ACR/EULAR SSc features | Early SSc features | Laboratory | Classification criteria | ||||||
|---|---|---|---|---|---|---|---|---|---|---|
| SSc skin involvement | ILD | Raynaud | Abnormal NFC | First non-Raynaud SSc symptom | Lower oesophageal dysmotility | Nuclear ICAP patterns/titers | Serum CK, IU/L | EULAR/ACR IIM (%probability) | ACR/EULAR SSc | |
| Scleromyositis presenting with definite SSc | ||||||||||
| 1 | Diffuse | Y | Y | Y | Myositis | Y | AC5 1:1280 | 2139 | N (38%) | Y |
| 2 | Diffuse | N | N | N | Puffy fingers | Y | AC4 1:640 | 6680 | Y (79%) | Y |
| 3 | Limited | N | Y | Y | Trig. neuropathy | ND | AC6/7 1:5120 | 2564 | N (38%) | Y |
| 4 | Limited | N | Y | N | Myositis | Y | AC6/7 1:1280 | 3675 | N (13%) | Y |
| 5 | Limited | N | Y | Y | Puffy fingers | Y | N | 385 | N (23%) | Y |
| 6* | Limited | Y | Y | ND | Sclerodactyly | ND | AC8 1:1280 | 656 | N (38%) | Y |
| 7 | Limited | Y | Y | Y | GERD | Y | N | 546 | N (38%) | Y |
| 8 | Limited | N | Y | N | Sclerodactyly | ND | AC6/7 1:1280 | 2974 | N (3%) | Y |
| 9 | Limited | Y | Y | Y | Sclerodactyly | Y | N | 358 | Y (85%) | Y |
| 10 | Limited | Y | Y | Y | Dyspnea | Y | N | 880 | N (5%) | Y |
| 11 | Limited | N | Y | Y | Myositis | ND | AC1 1:1280 | 1743 | N (5%) | Y |
| Scleromyositis with Raynaud phenomenon as the presenting SSc features | ||||||||||
| 12 | Sine | N | Y | Y | Myositis | N | N | 300 | Y (77%) | N |
| 13 | Sine | N | Y | N | Myositis | Y | AC6/7 1:1280 | 1494 | Y (89%) | N |
| 14 | Sine | N | Y | Y | GERD | Y | AC6/7 1:640 | 1738 | Y (67%) | N |
| 15 | Sine | N | Y | Y | Myositis | Y | N | 1765 | Y (89%) | N |
| 16 | Sine | N | Y | ND | Myositis | ND | AC4 or AC5 1:2560 | 1536 | Y (75%) | N |
| Scleromyositis with interstitial lung disease as the presenting SSc features | ||||||||||
| 17 | Sine | Y | N | ND | Myositis | ND | AC6/7 1:1280 | 6000 | N (38%) | N |
| Scleromyositis with isolated muscle involvement as the presenting SSc features | ||||||||||
| 18 | Sine | N | N | N | Myositis | ND | AC4 1:1280 | 9329 | Y (75%) | N |
| 19 | Sine | N | N | Y | Myositis | ND | N | 12 410 | Y (90%) | N |
| 20 | Sine | N | N | ND | Myositis | ND | AC5 ND | 2000 | Y (91%) | N |
| Total | Sine, 45% | 30% | 75% | 73%, n=11/15 | Myositis, 55% | 91%, n=10/11 | ANA+, 65% | Median CK, 1754 | 50% | 55% |
*This patient had an axial myopathy.
ACR, American College of Rheumatology; ANA, antinuclear antibody; CK, creatine kinase; GERD, gastro-oesophageal reflux disease; ICAP, International Consensus on Antinuclear Antibody Patterns; IIM, idiopathic inflammatory myopathy; ILD, interstitial lung disease; N, no; ND, no data; NFC, nailfold capillaroscopy; ; sine, sine scleroderma; SSc, systemic sclerosis; Trig., trigeminal; Y, yes.