Table 4.
Manifestations of cases with Aicardi-Goutieres syndrome
| Clinical symptoms | Percentage, % |
|---|---|
| Fever | 30.43 (7/23) |
| Skin | 60.87 (14/23) |
| Chilblain-like rash | 47.83 (11/23) |
| Livedo | 4.35 (1/23) |
| Erythaema nodosa | 4.35 (1/23) |
| Raynaud's phenomenon | 4.35 (1/23) |
| Telangiectasia | 4.35 (1/23) |
| Nervous system | 100 (23/23) |
| Intracranial calcification | 100 (21/21) |
| Dystonia | 60.87 (14/23) |
| Leukodystrophy | 54.17 (13/21) |
| Epilepsy | 34.78 (8/23) |
| Brain atrophy | 38.10 (8/21) |
| Microcephaly | 47.62 (10/21) |
| Short stature | 52.38 (11/21) |
| Mental retardation | 57.14 (12/21) |
| Motor skill impairment | 60.87 (14/23) |
| Language disability | 42.86 (9/21) |
| Interstitial lung disease | 19.05 (4/21) |
| Urinary system | 15.00 (3/20) |
| Chronic renal interstitial lesions | 10.00 (2/20) |
| Membrane hyperplasia and membranous nephropathy | 5.00 (1/20) |
| Arthralgia | 13.04 (3/23) |
| Haematological system | 21.05 (4/19) |
| Anaemia | 5.26 (1/19) |
| Neutropaenia | 5.26 (1/19) |
| Thrombocytopaenia | 5.26 (1/19) |
| Lymphopaenia | 5.26 (1/19) |
| Hypohepatia | 42.11 (8/19) |
| Splenomegaly | 9.52 (2/21) |
| Thyroid dysfunction | 46.15 (6/13) |
| Subclinical hypothyroidism | 30.77 (4/13) |
| Hypothyroidism | 15.38 (2/13) |
| Elevated ESR | 53.85 (7/13) |
| Elevated CRP | 15.38 (2/13) |
| Decreased C3 complement | 36.36 (4/11) |
| Decreased C4 complement | 36.36 (4/11) |
| Positive autoimmune antibodies | 66.67 (8/12) |
| Anti-nuclear antibody | 50.00 (6/12) |
| Anti-double-stranded DNA antibody | 27.27 (3/11) |
| Rheumatoid factor | 27.27 (3/11) |
| Anti-neutrophil cytoplasmic antibody | 20.00 (2/10) |
| Anti-Sjogren syndrome A antibody | 9.09 (1/11) |
| Anti-Sjogren syndrome B antibody | 9.09 (1/11) |
| Mortality | 4.35 (1/23) |
ESR erythrocyte sedimentation rate, CRP C-reactive protein