SSc features at last follow-up in 20 patients with seronegative scleromyositis
| Patient No. | ACR/EULAR SSc features | Non-ACR/EULAR SSc features | Classification criteria | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| SSc skin involvement | ILD | Raynaud | Abnormal NFC | Lower oesophageal dysmotility | Isolated DLCO ≤70% | SRC | Non ACR/EULAR SSc autoantibodies | EULAR/ACR IIM | ACR/EULAR SSc | Follow-up duration, years | |
| Scleromyositis presenting with definite SSc | |||||||||||
| 1 | Diffuse | Y | Y | Y | Y | N | N | Anti-U4/U6RNP | N | Y | 1.9 |
| 2 | Diffuse | N | Y | N | Y | Y | N | Anti-RuvBL1/2 | Y | Y | 9.5 |
| 3 | Diffuse | N | Y | Y | ND | N | N | Anti-SMN | N | Y | 6.5 |
| 4 | Limited | N | Y | N | Y | N | N | Anti-SMN | N | Y | 5 |
| 5 | Limited | N | Y | Y | Y | N | Y | N | Y | Y | 0.3 |
| 6 | Limited | Y | Y | ND | ND | N | N | N | N | Y | 0.3 |
| 7 | Limited | Y | Y | Y | Y | N | N | N | N | Y | 9.5 |
| 8 | Limited | N | Y | N | ND | N | N | Anti-SMN | Y | Y | 4.5 |
| 9 | Limited | Y | Y | Y | Y | N | Y | N | Y | Y | 7 |
| 10 | Limited | Y | Y | Y | Y | N | N | N | N | Y | 3 |
| 11 | Limited | N | Y | Y | ND | N | N | N | N | Y | 3.5 |
| Scleromyositis with Raynaud phenomenon as the presenting SSc features | |||||||||||
| 12 | Sine | N | Y | Y | N | ND | N | N | Y | N | 18 |
| 13 | Limited | N | Y | N | Y | N | N | Anti-SMN | Y | Y | 29 |
| 14 | Limited | Y | Y | Y | Y | N | N | Anti-SMN | Y | Y | 22 |
| 15 | Limited | Y | Y | Y | Y | N | N | N | Y | Y | 21 |
| 16 | Sine | N | Y | ND | Y | ND | N | N | Y | N | 8 |
| Scleromyositis with interstitial lung disease as the presenting SSc features | |||||||||||
| 17 | Limited | Y | Y | ND | ND | N | N | N | N | Y | 32 |
| Scleromyositis with isolated muscle involvement as the presenting SSc features | |||||||||||
| 18 | Limited | N | N | N | ND | Y | N | Anti-RuvBL1/2 | Y | N | 3 |
| 19 | Diffuse | Y | Y | Y | Y | N | Y | N | Y | Y | 6 |
| 20 | Sine | N | Y | ND | Y | ND | N | Anti-U5RNP | Y | N | 13.5 |
| Total | Limited, 80% | 45% | 95% | 69%, n=11/16 | 93%, n=13/14 | 12%, n=2/17 | 15% | 45% | 60% | 80% | Mean duration 6.8 |
ACR, American College of Rheumatology; DLCO, diffusing capacity of lung for carbon monoxide; IIM, idiopathic inflammatory myopathy; ILD, interstitial lung disease; NFC, nailfold capillaroscopy; N, no; ND, no data; NFC, nailfold capillaroscopy; sine, sine scleroderma; SMN, survival of motor neuron; SRC, scleroderma renal crisis; SSc, systemic sclerosis; Y, yes.