Autoantibody Profile of Egyptian Juvenile Systemic Lupus Erythematosus Patients and Its Association with Clinical Characteristics and Disease Activity

Figures & data

Table 1 Demographic Data of JSLE Patients Included in This Study (n=100)

Demographic Data	Total (n=100)
Age (years)
Range	9–16
Mean±SD	12.91±1.94
Sex
Female	97 (97.0%)
Male	3 (3.0%)
Disease duration (years)
Range	1–10.83
Mean±SD	3.57±2.66

Table 2 Distribution of Different Clinical, Laboratory Data and SLEDAI Score Among JSLE Patients (n=100)

Cumulative Clinical Data	Number (%)
Malar rash	54 (54.0)
Oral ulcer	33 (33.0)
Polyarthralagia	71 (71.0)
Photosensitivity	47 (47.0)
Alopecia	29 (29.0)
Constitutional symptoms(fever, fatigue)	10 (10.0)
Nephritis	51 (51)
Hematological manifestations	65 (65)
Leukopenia (<4×10^9/L)	30 (30.0)
Neutropenia	30 (30.0)
Lymphopenia	35 (35.0)
Anemia (<12g/dl)	53 (53.0)
Autoimmune hemolytic anemia (AIHA)	19 (19.0)
Thrombocytopenia (<150×10^9/L)	40 (40.0)
Serositis	12 (12.0)
Pleural effusion	3 (3.0)
Pericardial effusion	9 (9.0)
Neuropsychiatric manifestations	27 (27)
Seizures	8 (8.0)
Hemiparesis	3 (3)
Peripheral neuropathy	2 (2)
Psychosis (excluding corticosteroid psychosis)	14 (14.0)
Thromboembolic Event	23 (23)
Arterial thrombosis	7 (7.0)
Venous thrombosis	15 (15.0)
Recurrent thrombosis	1 (1.0)
Cardiac involvement	26 (26.0)
Heart failure (myocarditis)	3 (3.0)
Valvular affection (endocarditis)	21 (21.0)
Pericardial effusion	9 (9.0)
Raynaud’s phenomena	8 (8.0)
Antiphospholipid syndrome	18 (18.0)
Laboratory Data
Creatinine: Increase (>1.2 mg /dl)	5 (5.0)
Creatinine clearance: Decrease (mL/min) (<90 mL/min)	37 (37.0)
Hypocomplementemia
C3: Consumed (mg/dl)	60 (60.0)
C4: Consumed (mg/dl)	35 (35.0)
ESR: High ESR (>20mm/hr)	74 (74.0)
CRP: High CRP(>6 mg/dl)	11 (11.0)
Urine analysis
Pus cell	30 (30.0)
Red Cell	28 (28.0)
Albuminuria	31 (31.0)
Urinary casts	15 (15.0)
Protein/creatinine ratio: Abnormal (>0.2 gram/mg creatinine)	50 (50.0)
24 h urinary protein (abnormal >200 mg)	50 (50.0)
Lupus anticoagulant (LAC) antibodies	26 (26.0)
Anticardiolipin (ACL) antibodies	33 (33.0)
SLEDAI score
Range	2–22
Mean±SD	9.43±4.52
Mild (2-5)	14 (14)
Moderate (6-11)	53 (53)
Severe (≥12)	33 (33)

Abbreviations: ESR, erythrocyte sedimentation rate; CRP, C-reactive protein; C3, Complement 3; C4, Complement 4; SLEDAI, systemic lupus erythematosus disease activity index.

Figure 1 Distribution of renal biopsy classes among JSLE patients with lupus nephritis (n=51).

Table 3 Distribution of Anti ds DNA and Different Anti-ENA Antibodies Profile in Relation to Different Clinical Manifestations and SLEDAI Score in JSLE Patients (n=100)

Clinical Manifestations	Total (n=100)	Anti-dsDNA (n=83)	Anti-ENA
			Anti-SSB/La (n=20)		Anti-SSA/Ro (n=27)		Anti-Smith (n=31)		Anti-U1-RNP (n=41)
Malar rash	54 (54%)	41 (49.4%)	12 (60.0%)		14 (51.9%)		13 (41.9%)		17 (41.5%)
Oral ulcer	33 (33%)	31 (37.3%)	5 (25.0%)		8 (29.6%)		8 (25.8%)		14 (34.1%)
Photosensitivity	47 (47%)	40 (48.2%)	9 (45.0%)		15 (55.6%)		15 (48.4%)		18 (43.9%)
Alopecia	29 (29%)	23 (27.7%)	7 (35.0%)		11 (40.7%)		9 (29.0%)		12 (29.3%)
Constitutional symptoms	10 (10%)	9 (10.8%)	3 (15.0%)		3 (11.1%)		4 (12.9%)		6 (14.6%)
Raynaud’s phenomena	8 (8%)	5 (6.0%)	1 (5.0%)		3 (11.1%)		4 (12.9%)		4 (9.8%)
Polyarthralagia	71 (71%)	61 (73.5%)	13 (65.0%)		22 (81.5%)		17 (54.8%)		29 (70.7%)
Serositis	12 (12%)	8 (9.6%)	2 (10.0%)		4 (14.8%)		3 (9.7%)		4 (9.8%)
Nephritis (renal involvement)	51 (51%)	41 (49.4%)	12 (60.0%)		15 (55.6%)		18 (58.1%)		20 (48.8%)
Hematological manifestations	65 (65%)	54 (65.1%)	17 (85.0%)		12 (44.4%)		25 (80.6%)		29 (70.7%)
Neuropsychiatric manifestation	27 (27%)	22 (26.5%)	4 (20.0%)		9 (33.3%)		8 (25.8%)		13 (31.7%)
Cardiac involvement	26 (26%)	22 (26.5%)	4 (20.0%)		5 (18.5%)		9 (29.0%)		10 (24.4%)
Thromboembolic event	23 (23%)	18 (21.7%)	5 (25.0%)		6 (22.2%)		7 (22.6%)		9 (22.0%)
SLEDAI score	Total (n=100)	Anti-ds DNA (n=83)		Anti-ENA
				Anti-SSB/ La (n=20)		Anti-SSA/Ro (n=27)		Anti-Smith (n=31)		Anti-U1-RNP (n=41)
Mild (2–5)	14 (14%)	9 (10.8%)		3 (15.0%)		4 (14.8%)		5 (16.1%)		3 (7.3%)
Moderate (6–11)	53 (53%)	45 (54.2%)		7 (35.0%)		14 (51.9%)		17 (54.8%)		22 (53.7%)
Severe (≥12)	33 (33%)	29 (34.9%)		10 (50.0%)		9 (33.3%)		9 (29.0%)		16 (39.0%)

Abbreviations: SLEDAI, systemic lupus erythematosus disease activity index; Anti-dsDNA, anti-double-stranded deoxyribonucleic acid antibody; Anti-ENA, anti-extractable nuclear antigens; Anti-U1-RNP, anti-U1-ribonucleoprotein.

Figure 2 Distribution of anti-ENA and other autoantibodies found among JSLE patients. (n=100).

Table 4 Multivariate Regression Analysis of All Variable as a Predictor of Autoantibodies Profile in JSLE Patients (n=100)

Variable	Anti-dsDNA (n=83)	Anti-ENA
Auto Antibodies		Anti-SSB/La (n=20)	Anti-SSA/Ro (n=27)	Anti-Sm (n=31)	Anti-U1-RNP (n=41)
	Odds Ratio (95% CI)	Odds Ratio (95% CI)	Odds Ratio (95% CII)	Odds Ratio (95% CII)	Odds Ratio (95% CI)
Demographic data
Age (years)	0.93(0.61–1.65), p=0.058	0.18(0.12–0.32), p=0.669	3.94(2.60–7.00), p=0.162	0.36(0.24–0.63), p=0.261	3.31(2.18–5.88), p=0.059
Sex	5.67(3.74–10.08), p=0.063	3.24(2.14–5.77), p=0.544	3.03(2.00–5.39), p=0.124	0.48(0.31–1.61), p=0.514	6.06(4.00–10.79), p=0.483
Disease duration (years)	5.25(3.46–9.34), p=0.011*	2.12(1.40–3.77), p=0.797	1.31(1.13–3.71), p=0.044*	2.83(1.87–5.04), p=0.044*	4.23(2.79–7.53), p=0.748
Laboratory data
Creatinine: Increase (>1.2 mg /dl)	0.99(0.65–1.76), p=0.474	0.86(0.57–1.54), p=0.039*	1.83(1.21–3.26), p=0.412	3.21(2.12–5.71), p<0.001**	1.21(0.93–3.82), p=0.122
Creatinine clearance: Decrease (mL/min) (<90 mL/min)	1.59(1.05–2.82), p=0.768	1.47(0.97–2.61), p=0.671	0.78(0.51–1.38), p=0.010*	1.28(0.85–2.28), p=0.046*	2.06(1.36–3.66), p=0.539
Hypocomplementemia
C3: Consumed (mg/dl)	1.52(1.00–2.71), p=0.044*	4.95(3.27–8.81), p=0.020*	1.71(1.13–3.04), p=0.048*	1.33(0.88–2.36), p<0.001**	2.04(1.34–3.62), p=0.765
C4: Consumed (mg/dl)	0.94(0.62–1.68), p=0.632	4.18(2.76–7.44), p=0.699	5.61(3.70–9.99), p=0.037*	1.63(1.07–2.89), p=0.122	4.52(2.98–8.04), p=0.007*
Urine analysis
Pus cell	0.16(0.10–0.28), p=0.046*	2.40(1.13–3.43), p=0.134	1.61(1.08–3.87), p=0.471	1.20(0.79–2.13), p=0.032*	0.45(0.30–0.78), p=0.131
Red cell	3.95(2.61–7.03), p=0.739	0.28(0.20–0.52), p=0.512	1.66(1.10–2.96), p=0.038*	3.41(2.25–6.07), p=0.034*	0.85(0.56–1.51), p=0.159
Albuminuria	0.10(0.06–0.17), p<0.001**	1.78(1.17–3.16), p=0.049*	1.02(0.67–1.81), p=0.744	1.60(1.05–2.84), p=0.035*	2.13(1.61–4.61), p=0.002*
Urinary casts	0.58(0.19–1.24), p=0.164	3.69(2.44–6.57), p=0.444	3.54(2.34–6.30), p=0.475	3.01(1.99–5.36), p=0.010*	1.69(1.12–3.01), p=0.177
Protein/creatinine ratio: Abnormal (>0.2 gram/mg)	3.79(2.50–6.74), p=0.796	3.09(2.04–5.50), p=0.039*	0.89(0.59–1.58), p=0.049*	6.49(4.28–11.54), p=0.8	4.20(2.77–7.48), p=0.539
24 h urinary protein (abnormal >200 mg)	2.47(1.63–4.40), p<0.001**	3.93(2.59–6.99), p=0.105	0.19(0.12–0.33), p=0.478	1.11(0.83–3.44), p=0.015*	2.89(1.91–5.14), p=0.016*
Lupus anticoagulant (LAC) antibodies	0.80(0.53–1.42), p=0.033*	0.69(0.46–1.23), p<0.001**	1.30(0.85–2.31), p=0.038*	1.41(0.93–2.51), p=0.628	1.06(0.70–1.88), p=0.771
Anticardiolipin (ACL) antibodies	0.42(0.27–0.72), p=0.106	1.58(1.03–3.62), p=0.247	3.67(2.42–6.54), p=0.043*	2.14(1.61–5.10), p=0.048*	1.80(1.19–3.20), p=0.043*
Clinical data
Malar rash	1.39(0.91–2.47), p=0.049*	0.13(0.08–0.23), p=0.043*	1.73(1.14–3.08), p=0.041*	1.36(1.04–4.10), p=0.245	0.64(0.44–1.31), p=0.240
Oral ulcer	0.15(0.10–0.27), p=0.017*	1.32(0.87–2.35), p=0.216	3.23(2.13–5.75), p=0.012*	5.17(3.41–9.21), p=0.046*	1.42(0.94–2.53), p=0.025*
Photosensitivity	0.77(0.51–1.37), p=0.030*	5.53(3.65–9.85), p=0.259	0.92(0.60–1.63), p=0.285	3.19(2.10–5.67), p=0.008*	2.20(1.45–3.92), p=0.839
Alopecia	5.29(3.49–9.42), p=0.042*	0.24(0.16–0.42), p=0.121	3.44(2.27–6.12), p=0.028*	1.72(0.92–3.99), p=0.007*	0.60(0.40–1.72), p=0.332
Constitutional symptoms	1.85(1.22–3.29), p=0.06	0.21(0.14–0.37), p=0.865	1.81(1.19–3.22), p=0.575	1.55(1.02–2.76), p=0.013*	1.24(0.92–3.61), p=0.736
Raynaud’s phenomena	0.12(0.08–0.22), p=0.257	1.96(1.29–3.48), p=0.738	3.68(2.43–6.55), p=0.045*	1.12(0.9–4.16), p=0.634	0.74(0.49–1.31), p=0.027*
Polyarthralagia	1.44(0.95–2.56), p=0.061	0.83(0.55–1.48), p=0.036*	1.68(1.11–2.99), p=0.023*	0.91(0.60–1.61), p=0.547	1.31(0.94–4.56), p=0.105
Serositis	2.17(1.32–5.13), p=0.277	1.08(0.71–1.91), p=0.003*	6.88(4.54–12.24), p=0.747	1.26(0.83–2.24), p=0.019*	0.80(0.53–1.36), p=0.796
Nephritis (renal affection)	0.75(0.49–1.33), p=0.008*	1.92(1.27–3.42), p=0.068	0.79(0.52–1.41), p=0.044*	0.95(0.63–1.69), p=0.446	1.85(1.13–3.74), p=0.481
Hematological manifestations	0.17(0.11–0.30), p=0.043*	1.34(1.12–3.30), p=0.258	3.65(2.41–6.49), p=0.047*	1.90(1.25–3.38), p=0.458	1.35(0.89–2.40), p=0.042*
Neuropsychiatric manifestation	1.51(1.02–3.31), p=0.060	3.45(2.28–6.15), p=0.037*	1.65(1.09–2.94), p=0.798	2.65(1.75–4.71), p=0.026*	3.70(2.44–6.58), p=0.049*
Cardiac involvement	0.30(0.21–0.57), p=0.127	1.17(1.11–2.31), p=0.412	3.22(2.13–5.74), p=0.036*	0.56(0.36–1.05), p=0.209	0.57(0.22–1.41), p=0.574
Thromboembolic event	4.21(2.78–7.49), p=0.574	0.82(0.54–1.46), p=0.045*	1.74(1.15–3.10), p=0.54	1.37(0.90–2.44), p=0.284	1.15(0.76–2.05), p=0.030*
SLEDAI score
Moderate (6-11)	4.83(3.19–8.60), p=0.032*	1.38(0.91–2.46), p=0.002*	5.92(3.91–10.54), p=0.669	1.48(0.97–2.63), p=0.029*	1.77(1.03–4.51), p=0.014*
Severe (≥12)	0.98 (0.65–1.74), p=0.698	0.72(0.47–1.28), p=0.004*	1.23(0.81–2.19), p=0.045*	1.62(1.07–2.87), p=0.018*	1.70(1.10–4.11), p=0.614

Notes: *p-value significant predictive <0.05; **p-value significant p<0.001.

Abbreviations: SLEDAI, systemic lupus erythematosus disease activity index; Anti-dsDNA, anti-double-stranded deoxyribonucleic acid antibody; Anti-ENA, anti-extractable nuclear antigens; Anti-U1-RNP, anti-U1-ribonucleoprotein; C3, Complement 3; C4, Complement 4; LAC, lupus anticoagulant antibodies; ACL, anticardiolipin antibodies.