Prevalence of different symptoms in patients with and without clinically diagnosed SLE
| SLE (n=294) | No SLE (n=66) | |
| Age, years (mean±SD) | 43±14 | 46±15 |
| Gender (female, %) | 256 (87.1) | 55 (83.3) |
| ACR 1997 criteria (n, %) | ||
| Malar rash | 129 (43.9) | 9 (13.6) |
| Discoid rash | 52 (17.7) | 7 (10.6) |
| Photosensitivity | 155 (52.7) | 22 (33.3) |
| Oral ulcers | 126 (42.9) | 19 (28.8) |
| Non-erosive arthritis | 183 (62.2) | 11 (16.7) |
| Pleuritis or pericarditis | 76 (25.9) | 2 (3.0) |
| Renal disorder | 88 (29.9) | 3 (4.5) |
| Neurological disorder | 37 (12.6) | 6 (9.1) |
| Haematological disorder | 145 (49.3) | 10 (15.2) |
| Immunological disorder | 221 (75.2) | 20 (30.3) |
| Positive ANA | 283 (96.3) | 50 (75.8) |
| SLICC 2012 criteria (n, %) | ||
| Acute or subacute cutaneous lupus | 148 (50.3) | 12 (18.2) |
| Chronic cutaneous lupus | 52 (17.7) | 7 (10.6) |
| Oral ulcers | 126 (42.9) | 19 (28.8) |
| Non-scarring alopecia | 44 (15.0) | 2 (3.0) |
| Arthritis | 183 (62.2) | 11 (16.7) |
| Serositis | 76 (25.9) | 2 (1.0) |
| Renal | 88 (29.9) | 3 (4.5) |
| Neurological Acute confusional state/delirium | 9 (2.7) | 0 (0.0) |
| Psychosis | 12 (4.1) | 2 (3.0) |
| Seizure | 28 (9.5) | 2 (3.0) |
| Mononeuritis | 5 (1.7) | 1 (1.5) |
| Myelitis | 9 (3.1) | 2 (3.0) |
| Neuropathy | 10 (3.4) | 4 (6.1) |
| Leucopenia | 140 (47.6) | 15 (22.7) |
| Thrombocytopenia | 58 (19.7) | 4 (6.1) |
| Immunological criteria ANA | 283 (96.3) | 50 (75.8) |
| Anti-Sm | 36 (12.2) | 2 (3.0) |
| Antiphospholipid antibody (IgG, IgM, LAC, anti-β2 glycoprotein) | 143 (48.6) | 17 (25.8) |
| Low complement (C3, C4) | 168 (57.1) | 14 (21.2) |
| Direct Coombs test | 21 (7.1) | 0 (0.0) |
| Proposed ACR–EULAR criteria | ||
| Constitutional domain Fever | 71 (24.2) | 11 (16.7) |
| Mucocutaneous domain Non-scarring alopecia | 44 (15.0) | 2 (3.0) |
| Oral ulcers | 126 (42.9) | 19 (28.8) |
| Subacute cutaneous/discoid lupus | 94 (32.0) | 17 (25.8) |
| Acute cutaneous lupus | 148 (50.3) | 12 (18.2) |
| Musculoskeletal domain Arthritis | 183 (62.2) | 11 (16.7) |
| Serositis domain Pleural or pericardial effusion | 58 (19.8) | 1 (1.5) |
| Acute pericarditis | 34 (11.6) | 1 (1.5) |
| Haematological domain Leucopenia | 118 (40.3) | 10 (15.2) |
| Thrombocytopenia | 58 (19.7) | 4 (6.1) |
| Autoimmune haemolysis | 43 (14.6) | 1 (1.5) |
| Renal domain Proteinuria | 66 (22.4) | 1 (1.5) |
| Lupus nephritis class II/V | 31 (10.5) | 2 (3.0) |
| Lupus nephritis class III/IV | 37 (12.6) | 0 (0.0) |
| Complement protein domain Low C3 or low C4 | 168 (57.3) | 14 (21.5) |
| Low C3 and low C4 | 101 (34.5) | 2 (3.1) |
| Highly specific antibodies domain Anti-dsDNA antibody | 169 (57.7) | 15 (22.7) |
| Anti-Smith antibody | 36 (12.2) | 2 (3.0) |
| Antiphospholipid antibodies domain IgG, IgM, β2 glycoproteins, lupus anticoagulant | 143 (48.6) | 17 (25.8) |
| Neurological domain Acute confusional state/delirium | 9 (2.7) | 0 (0.0) |
| Psychosis | 12 (4.1) | 2 (3.0) |
| Seizure | 28 (9.5) | 2 (3.0) |
| 1999 ACR neuropsychiatric syndromes* | ||
| Aseptic meningitis | 0 (0.0) | |
| Cerebrovascular disease | 30 (10.2) | – |
| Demyelinating syndrome | 0 (0.0) | – |
| Headache | 12 (4.1) | – |
| Movement disorder (chorea) | 3 (1.0) | – |
| Myelopathy | 6 (2.0) | – |
| Seizure disorders | 11 (3.7) | – |
| Acute confusional state | 6 (2.0) | – |
| Anxiety disorder | 2 (0.7) | – |
| Cognitive dysfunction | 31 (10.5) | – |
| Mood disorder | 9 (3.1) | – |
| Psychosis | 7 (2.4) | – |
| Acute inflammatory demyelinating polyradiculoneuropathy | 0 (0.0) | – |
| Autonomic disorder | 0 (0.0) | – |
| Mononeuropathy | 0 (0.0) | – |
| Myasthenia gravis | 0 (0.0) | – |
| Neuropathy, cranial | 0 (0.0) | – |
| Plexopathy | 0 (0.0) | – |
| Polyneuropathy | 3 (1.0) | – |
*The 1999 ACR NPSLE syndromes were assigned after the presence of NPSLE was confirmed during multidisciplinary assessment.
ACR, American College of Rheumatology; ANA, antinuclear antibody; NP, neuropsychiatric; SLE, systemic lupus erythematosus; SLICC, Systemic Lupus International Collaborating Clinics.