Table 1 (abstract P314). See text for description
From: Proceedings of the 25th European Paediatric Rheumatology Congress (PReS 2018)
Clinical preliminary criteria | Presumed IFN mediated AIDs n=12 | Heathy controls n=8 | Autoimmune diseases (SLE or JDM) n=8 |
|---|---|---|---|
1. Skin manifestations (nodular erythema, violaceous plaques in cold-sensitive acral areas) | 12/12 | 0/8 | 0/8 |
2. Vasculopathy (chill-blain like rash, microangiopathic vasculopathy, gangrene/ulcers/infarcts in acral areas) | 6/12 | 0/8 | 1/8 |
3. Lipodystrophy | 2/12 | 0/8 | 0/8 |
4. Joint manifestations (contractures, non-erosive arthritis) | 5/12 | 0/8 | 2/8 |
5. Myositis (patchy) | 2/12 | 0/8 | 0/8 |
6. CNS manifestations (basal ganglia calcifications, leukoencephalopathy, white matter disease, L/P lymphocytic findings) | 8/12 | 0/8 | 0/8 |
7. Pulmonary involvement (interstitial lung disease, pulmonary fibrosis, pulmonary hypertension | 2/12 | 0/8 | 1/8 |
8. Leukopenia/lymphopenia with flares | 4/12 | 0/8 | 4/8 |
Median 6-gene IFN score (minimum-maximum) | 137.87 (0.251-380.09) | -2.54 (-3.57-2.48 | 106.26 (-1.47-381.69) |
Median) 28-gene IFN score (minimum-maximum) | 324.01 (24.66-949.98) | 0.83 (-7.91-16.79) | 299.9 (2.75-475.96) |
CXCL10 z-score (STD) | 186.07 | 46.91 | 57.59 |