Expert consensus versus classification systems for selected case profiles*
| No. | Skin thickening of the fingers | Finger-tips | Telangiectasia | Abnormal nailfold capillaries | Puffy fingers | Raynaud's phenomenon | ILD/pulmonary fibrosis | PAH | SSc-related autoantibodies | Classified as SSc | Score | 1980 ACR criteria fulfilled | No. of experts agreeing (n=16) |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Whole finger | Pitting scars | – | Yes | Yes | Yes | – | Yes | Yes | Yes | 17 | Yes | 16 |
| 2 | Whole finger | Pitting scars | – | – | Yes | Yes | Yes | – | Yes | Yes | 15 | Yes | 16 |
| 3 | Whole finger | Pitting scars | – | – | Yes | Yes | – | – | Yes | Yes | 13 | Yes | 16 |
| 4 | Whole finger | Pitting scars | Yes | Yes | – | Yes | – | – | – | Yes | 12 | Yes | 15 |
| 5 | Whole finger | – | – | Yes | Yes | Yes | – | – | Yes | Yes | 12 | No | 15 |
| 6 | Whole finger | – | – | Yes | – | Yes | – | – | Yes | 11 | No | 10 | |
| 7 | – | Pitting scars | – | – | Yes | Yes | Yes | – | Yes | 10 | ? | NA | |
| 8 | – | Ulcers | – | – | – | Yes | Yes | – | Yes | Yes | 10 | No | 14 |
| 9 | – | – | – | Yes | – | Yes | Yes | – | Yes | Yes | 10 | No | NA |
| 10 | – | – | – | Yes | Yes | Yes | – | – | Yes | Yes | 10 | No | NA |
| 11 | – | Ulcers | – | – | Yes | Yes | – | – | Yes | Yes | 10 | No | NA |
| 12 | – | – | – | Yes | Yes | Yes | – | – | Yes | Yes | 10 | No | NA |
| 13 | Whole finger | – | – | – | Yes | Yes | – | – | Yes | Yes | 10 | No | 15 |
| 14 | Whole finger | – | – | – | – | Yes | – | – | Yes | Yes | 10 | No | NA |
| 15 | Whole finger | – | – | Yes | – | Yes | – | – | – | Yes | 9 | No | NA |
| 16 | Whole finger | – | Yes | – | Yes | Yes | – | – | – | Yes | 9 | No | NA |
| 17 | Whole finger | Ulcers | – | – | Yes | Yes | – | – | – | Yes | 9 | No | NA |
| 18 | Whole finger | – | – | Yes | – | Yes | – | – | – | Yes | 9 | No | NA |
| 19 | Whole finger | – | – | Yes | Yes | Yes | – | – | – | Yes | 9 | No | 9 |
| 20 | Whole finger | – | – | Yes | – | Yes | – | – | – | Yes | 9 | No | NA |
| 21 | – | Pitting scars | – | – | – | Yes | Yes | – | – | No | 8 | ? | NA |
| 22 | – | – | – | – | Yes | Yes | – | – | Yes | No | 8 | No | NA |
| 23 | – | Pitting scars | – | Yes | – | Yes | – | – | – | No | 8 | No | 4 |
| 24 | – | Pitting scars | Yes | – | – | Yes | – | – | – | No | 8 | No | 2 |
| 25 | – | – | – | Yes | – | Yes | – | – | Yes | No | 8 | No | NA |
| 26 | – | – | – | – | Yes | Yes | Yes | – | – | No | 7 | No | NA |
| 27 | – | – | – | Yes | Yes | Yes | – | – | – | No | 7 | No | NA |
| 28 | Distal to PIP joints | – | – | Yes | Yes | Yes | – | – | – | No | 7 | No | 5 |
| 29 | – | – | – | Yes | – | Yes | Yes | – | – | No | 7 | No | NA |
| 30 | Whole finger | – | – | – | – | Yes | – | – | – | No | 7 | No | NA |
| 31 | Whole finger | – | Yes | – | Yes | – | – | – | – | No | 6 | No | NA |
| 32 | Distal to PIP joints | – | – | – | – | Yes | – | – | Yes | No | 6 | No | 8 |
| 33 | – | – | – | – | – | Yes | – | – | Yes | No | 6 | No | 0 |
| 34 | – | – | – | – | – | – | Yes | – | Yes | No | 5 | No | NA |
| 35 | – | – | – | – | – | Yes | – | Yes | – | No | 5 | No | 0 |
| 36 | – | – | – | Yes | – | Yes | – | – | – | No | 5 | No | 1 |
| 37 | – | – | – | Yes | – | – | – | – | Yes | No | 5 | No | 0 |
| 38 | – | – | – | Yes | – | – | – | – | – | No | 2 | No | 0 |
*Profiles of 38 cases with likely systemic sclerosis (SSc) or an SSc-like disorder, with presence or absence of the manifestations in the newly developed American College of Rheumatology (ACR)/European League Against Rheumatism SSc classification criteria, are shown The cases are listed in order of the points awarded using the classification system (Score column) and are classified as SSc if the score is ≥9. For 18 of the cases, the number of experts agreeing that the case had SSc is shown. None of the cases had skin thickening of the fingers of both hands extending proximal to the metacarpophalangeal joints (the single criteria that, if present, immediately classifies the individual as having SSc). Whole finger is sclerodactyly of the entire finger but not proximal to the metacarpophalangeal joints. ILD, interstitial lung disease; PAH, pulmonary arterial hypertension; ?, inconclusive; NA, not applicable (experts were not asked); PIP, proximal interphalangeal.
Author: In the last column head you say the number of experts was 16, but I don't see this anywhere in the text. Please correct, clarify, or add to the text.
Comp.: Please insert an N-dash in the PAH column for patients 6 and 7. Thanks.