Table 2.
Clinical features of the different age groups over time
| Items on last follow up | Pre-pubertal disease-onset (n = 43) | Peri-pubertal disease-onset (n = 240) | Adolescence (n = 135) | p value |
|---|---|---|---|---|
| Length of follow up in years | 6 [3–9] | 4 [2–6] | 2 [1–4] | <0.001 |
| Total ACR score | 5 [4–7] | 5 [4–7] | 5 [4–7] | 0.686 |
| SLICC-SDI score | 0 [0–1] | 0 [0–1] | 0 [0–1] | 0.284 |
| SLEDAI-2 K | 8 [5–8] | 8 [6–8] | 7 [5–8] | 0.689 |
| ANA positivity | 42 (97.7%) [93.2%, 102.2%] | 230 (95.8%) [93.3%, 98.3%] | 132 (97.8%) [95.3%, 100.0%] | 0.559 |
| pBILAG2004 defined organ/system domain involvement throughout the disease course | ||||
| Constitutional | 18 (41.9%) [27.2%, 56.6%] | 98 (40.8%) [34.1%, 46.5%] | 56 (41.5%) [33.2%, 49.8%] | 0.988 |
| Mucocutaneous | 33 (76.7%) [64.1%, 89.3%] | 157 (65.4%) [59.4%, 71.4%] | 90 (66.7%) [58.7%, 74.7%] | 0.346 |
| Neuropsychiatric | 11 (25.6%) [12.6%, 38.6%] | 57 (27.9%) [22.2%, 33.6%] | 28 (20.7%) [13.9%, 27.5%] | 0.731 |
| Musculoskeletal | 18 (41.9%) [16.5%, 43.9%] | 121 (50.4%) [44.1%, 56.7%] | 73 (54.1%) [45.7%, 62.5%] | 0.374 |
| Cardiorespiratory | 13 (30.2%) [16.5%, 43.9%] | 46 (19.2%) [14.2%, 24.2%] | 35 (25.9%) [18.5%, 33.3%] | 0.141 |
| Gastrointestinal | 8 (18.6%) [7.0%, 30.2%] | 28 (11.7%) [7.6%, 15.8%] | 10 (7.4%) [3.0%, 11.8%] | 0.107 |
| Ophthalmic | 3 (7.0%) [0%, 14.6%] | 12 (5.0%) [2.2%, 7.8%] | 4 (3.0%) [0.1%, 5.9%] | 0.467 |
| Renal | 28 (65.1%) [50.9%, 79.3%] | 153 (63.8%) [57.7%, 69.9%] | 94 (69.6%) [61.8%, 77.4%] | 0.513 |
| Haematological | 26 (60.5%) [45.9%, 75.1%] | 114 (47.5%) [41.2%, 53.8%] | 55 (40.7%) [32.4%, 49.0%] | 0.072 |
Total ACR, SLICC-SDI, SLEDAI-2 K, pBILAG2004 scores are reported as median values and interquartile ranges. For individual pBILAG2004 domains, the total number of patients with activity involvement (defined as a pBILAG2004 domain score of A or B in a given organ domain/system) are provided along with percentage (in curved brackets), and 95% confidence intervals for the percentage [square brackets]. SLICC-SDI and ACR scores are provided from the last follow-up visit.
ACR, American College of Rheumatology; ANA, antinuclear antibodies; BILAG, British Isles Lupus Assessment Group; SLICC-SDI, Systemic Lupus International Collaborating Clinics-standardised damage index; SLEDAI-2 K, Systemic Lupus Erythematosus Disease Activity Index 2000.