Introduction
Methodology
Results
Clinical and Laboratory Features
Manifestation | No. | % |
---|---|---|
Mucocutaneous | ||
Malar rash | 32 | 41.0 |
Oral/nasopharyngeal ulcers | 23 | 29.4 |
Alopecia | 22 | 28.2 |
Photosensitivity | 19 | 24.0 |
Punctate erythema | 7 | 8.9 |
Discoid rash | 5 | 6.4 |
Epistaxis | 5 | 6.4 |
Gumbleeding | 2 | 2.5 |
Musculoskeletal | ||
Arthralgia | 21 | 26.9 |
Arthritis | 14 | 17.9 |
Haematologic | ||
Pallor | 16 | 20.5 |
Neuropsychiatric | ||
Seizures | 3 | 3.8 |
Psychiatric symptoms | 2 | 2.5 |
Renal | ||
Gross hematuria | 9 | 11.5 |
Gastrointestinal | ||
Abdominal pain | 7 | 8.9 |
Vomiting | 7 | 8.9 |
Constitutional | ||
Fever | 41 | 52.5 |
General fatigue | 22 | 28.2 |
Weight loss | 14 | 17.9 |
Loss of appetite | 7 | 8.9 |
Category | At the time of diagnosis | during the disease course | ||
---|---|---|---|---|
No. | % | No. | % | |
Mucocutaneous
Manifestations
|
71
|
91.0
|
72
|
92.3
|
Malar rash | 51 | 65.3 | 60 | 76.9 |
Discoid rash | 25 | 32.0 | 28 | 35.8 |
Photosensitivity | 43 | 55.1 | 57 | 73.0 |
Oral ulcers | 42 | 53.8 | 53 | 67.9 |
Alopecia | 31 | 39.7 | 41 | 52.5 |
Musculoskeletal
Involvement
|
32
|
41.0
|
42
|
53.8
|
Arthritis | 17 | 21.7 | 31 | 39.7 |
Arthralgia | 24 | 30.7 | 40 | 51.2 |
Pulmonary Involvement
|
11
|
14.1
|
17
|
21.7
|
Pleuritis/Pleural effusion | 11 | 14.1 | 16 | 20.5 |
Cardiac Involvement
|
13
|
16.6
|
17
|
21.7
|
Pericarditis/Pericardial Effusion | 12 | 15.3 | 14 | 20.5 |
Valvular anomaly on Echocardiography | 8 | 10.2 | 8 | 10.2 |
Ventricular/atrial Hypertrophy | 4 | 5.1 | 5 | 6.4 |
Renal involvement
|
49
|
62.8
|
56
|
71.7
|
Hematuria | 19 | 24.3 | 26 | 33.3 |
Proteinuria | 32 | 41.0 | 20 | 25.6 |
CNS involvement
|
24
|
30.7
|
25
|
32.0
|
Seizure | 12 | 15.3 | 15 | 19.2 |
Behavioral changes | 9 | 11.5 | 10 | 12.8 |
Hematologic
Involvement
|
37
|
47.4
|
54
|
69.2
|
Laboratory
| ||||
Cellular casts | 11 | 14.1 | 22 | 28.2 |
Increased creatinine | 6 | 7.6 | 11 | 14.1 |
Hemolytic anemia | 7 | 8.9 | 8 | 10.2 |
Leukocytopenia | 12 | 15.3 | 25 | 32.0 |
Lymphocytopenia | 17 | 21.7 | 32 | 41.0 |
Thrombocytopenia | 11 | 14.1 | 20 | 25.6 |
Low C3 | 7 | 8.9 | 7 | 8.9 |
Item | N | % |
---|---|---|
Infection
|
27
|
34.5
|
Pneumonia | 11 | 14.1 |
Sepsis | 8 | 10.2 |
Tuberculosis (pulmonary, endotracheal, miliary) | 6 | 7.7 |
Cellulitis | 2 | 2.5 |
Neurologic
|
15
|
19.0
|
Seizure requiring therapy for 6 months | 12 | 15.3 |
Cerebral atrophy by imaging (Cranial CT scan) | 2 | 2.5 |
Lateral rectus palsy | 1 | 1.2 |
Musculoskeletal
|
5
|
8.7
|
Deforming or erosive arthritis | 3 | 3.8 |
Osteoporosis with fracture or vertebral collapse | 2 | 2.5 |
Osteomyelitis | 1 | 1.2 |
Muscle atrophy or weakness | 1 | 1.2 |
Skin
|
4
|
5.0
|
Scarring chronic alopecia | 2 | 2.5 |
Extensive scarring or panniculum other than scalp and pulp space | 2 | 2.5 |
Gastrointestinal
|
2
|
2.0
|
Autoimmune hepatitis by biopsy | 2 | 2.0 |
Renal
|
2
|
2.5
|
Estimated or measured glomerular filtration rate < 50% | 2 | 2.5 |
Growth failure
|
4
|
5.1
|
Pubertal delay
|
2
|
2.5
|
Ocular
|
1
|
1.2
|
Ischemic retinopathy | 1 | 1.2 |
Pulmonary
|
1
|
1.2
|
Pulmonary hypertension | 1 | 1.2 |
Therapy
Clinical Outcomes
Discussion
Present Study | Wang, 20035 | Balkaran, 200410 | Bakr, 200511 | Salah, 20099 | Hamijoyo, 200912 | |
---|---|---|---|---|---|---|
No.of
Patients
| 78 | 153 | 33 | 52 | 207 | 147 |
Country
| Philippines | Taiwan | Trinidad | Egypt | Egypt | Philippines |
Follow-up, y
| ||||||
Mean
Range
| 1.7 ± 1.8 [0.1-6.4] | 6.1 ± 9.0 | 1 ± 9.3 | |||
Age at diagnosis, y
Mean+SD
Range
| 14 ± 2.7 8 - 18 | 13.5 ± 5.5 | 5-17 | 11.9 ± 2.6 6-16 | 10 ± 2.7 2 - 16 | 12.29 ± 2.9 4 - 16 |
F:M ratio
| 10:1 | 5.9:1 | 6.6:1 | 12:1 | 2.69:1 | 11.2:1 |
Rash
| ||||||
Malar | 65.3 | 77.1 | 39 | 46.2 | 74.8 | |
Discoid | 32.0 | 2.0 | 37 | 19.0 | ||
Photosensitivity
| 55.1 | 24.8 | 21,2 | 57.8 | ||
Oral ulcers
| 53.8 | 26.1 | 19.6 | 49.7 | ||
Alopecia
| 39.7 | 13.1 | 34.6 | 45.5 | 51.7 | |
Musculoskeletal
| 53.8 | 69.7 | 65.4 | |||
Arthritis | 21.7 | 57.5 | 46.9 | 61.9 | ||
Neuropsychiatric
| 30.7 | 4.6 | 7.7 | 7.2 | 8.2 | |
Serositis
| 26.9 | 15 | 14.3 | |||
Pleural
Effusion
| 14.1 | 6.3 | ||||
Pericardial
Effusion
| 15.3 | 7.7 | ||||
Nephritis
| 62.8 | 58.8 | 63.6 | 80.8 | 20.8 | 48.3 |
Hematologic
| 51.2 | 79.7 | 38.8 | |||
Thrombocytopenia | 14.1 | 19.6 | 29.2 | |||
Hemolytic
Anemia
| 8.9 | 44.4 | 17.0 | |||
Leukocytopenia | 15.3 | 34.6 | 27.5 |
Cumulative Features | Present Study n = 78 (%) | Agcaoili 198613 n = 31(%) | Bahabri, 1997 n = 6014 (%) | Gedalia, 199915 n = 61 (%) | Pattaragarn, 200516 n = 82 (%) | Hiraki, 200817 n = 256 (%) | Salah, 20099 n = 207 (%) | Hoffman200918 n = 56(%) | |
---|---|---|---|---|---|---|---|---|---|
Country
| Philippines | Philippines | Saudi Arabia | African-America | Latin America | Thailand | Canada | Egypt | Europe |
Rash
| 75.6 | 84 | 81 | ||||||
Malar | 71.7 | 90.3 | 40 | 69 | 52 | 53.5 | 66 | 38.2 | 69.6 |
Discoid | 35.8 | 21 | 9 | 2 | 10.1 | 13.2 | |||
Photosensitivity
| 73 | 15 | 20 | 56 | 21.8 | 44 | 44.6 | ||
Oral ulcers
| 67.9 | 45.1 | 16 | 31.7 | 22.2 | 28.6 | |||
Musculoskeletal
Involvement
| 53.8 | 91.6 | 31.7 | 39.6 | |||||
Arthritis | 39.7 | 74.1 | 79 | 75 | 67 | 59.3 | |||
Neuropsychiatric
| 32 | 32.2 psych 25.8 sz | 27 | 31 | 40 | 20.8 | 27 | 24.2 | |
Hematologic involvement
| 69.2 | 66.6 | 73.4 | ||||||
Hemolytic
anemia
| 10.2 | 18 | 12 | 19.3 | 38.5 | ||||
Thromobocyto
penia
| 25.6 | 6.4 | 26 | 22 | 13.9 | 21.7 | 31.5 | ||
Leukocytopenia | 32 | 12.9 | 46 | 55 | 26 | 63.6 | |||
Serositis
| 26.9 | ||||||||
Carditis | 17.9 | 32.26 | 28 | 16 | 16.7 | ||||
Pleuritis | 20.5 | 25.81 | 36 | 24 | |||||
Renal
Involvement
| 71.7 | 65 | 44 | 55 | 86.2 | 55 | 67 | 62.5 |