Background/Purpose:

The pathogenesis of SLE is complex and poorly understood.  Infectious triggers, immunological abnormalities, environmental factors and genetic background play a combined role in disease development. A genetic contribution to SLE has been evident from familial clustering of cases with a monozygotic twin concordance rate of 30-40%. Juvenile-onset SLE doesn’t necessarily present identically to adults as described by some investigators suggesting that disease might be more severe in juvenile-onset patients at presentation. We compared family history of SLE, immunology, severity of organ involvement and differences between medications used in both groups.

Methods:

Clinical and demographic data was collected on 25 juvenile-onset SLE patients and compared with 65 patients with adult-onset disease. Adult data was collected randomly from our SLE clinics. All patients met the American College of Rheumatology (ACR) classification criteria for SLE. Juvenile-onset was defined as those who were diagnosed with SLE before16 years of age. Data collected included ethnicity, family history of SLE/autoimmune disease, autoantibody profile, lupus-related disease manifestations and medications used.

Results:

36% of juvenile-onset SLE patients have a positive family history of SLE as opposed to 12% of adult-onset disease patients (p=0.011). Family history of other autoimmune conditions such as rheumatoid arthritis, hypothyroidism did not differ significantly between the two groups. In juvenile-onset patients 21(84%) were female and 4 (16%) male. Mean age of disease onset was 13 years (range 10-16 years). 13 (52%) were Afro-Caribbean, 7(28%)  Caucasian, 4 (16%) Asian and 1 (4%) was of mixed ethnic origin. In adult-onset disease, 60 (92%) were female and 5 (7%) male. Mean age of disease onset was 29 years (17-50 years).  29 (44%) were Afro-Caribbean, 13 (20%) Asian, 21(32%)  Caucasian and 2 (3%) were of mixed ethnicity. 18(72%) patients of juvenile-onset SLE patients had lupus nephritis, 3(12%) had interstitial lung disease, 4(16%) had APS. Only 1(4%) patient had AIHA. 43(66.1%) patients of adult-onset SLE patients had lupus nephritis, 5(7.69%) were diagnosed with interstitial lung disease. 12(18.4%) patients had APS and 4(6.1%) had either AIHA or thrombocytopenia.

Conclusion:

A family history of SLE was significantly more common in juvenile-onset SLE than in adult-onset disease. Frequencies of lupus nephritis and anti-dsDNA antibody positivity were higher in juvenile-onset SLE which may reflect a more severe clinical phenotype. The majority of juvenile-onset SLE patients in our cohort were of African ancestries, who are known to have worse clinical outcomes. Medications and clinical interventions such as mycophenolate mofetil, cyclophosphamide, rituximab were more frequently used in juvenile-onset SLE patients, which supports the likelihood of more severe and difficult to manage disease in this subset of patients.

Autoantibody Profile	Juvenile-onset SLE  (n=25)	Adult-onset SLE (n=65)
ANA	23 (92%)	64(98%)
Anti-dsDNA	18 (72%)	32(49%)
Anti-Ro (SSA)	9 (36%)	24 (36%)
Anti-La (SSB)	3(13%)	7 (10%)
Anti- Sm	7(31%)	16 (32%)
Anti-RNP	10 (40%)	24(36%)
Anti-cardiolipin	13(52%)	19(29%)
Lupus anticoagulant	8(32%)	16(24%)
Anti-C1q	11/17 (65%)	19/30 (63%)
Medications	Juvenile-onset SLE  (n=25)	Adult-onset SLE (n=65)
Prednisolone	21(84%)	53(81%)
Hydroxychloroquine	18(72%)	54(84%)
Mepacrine	1(4%)	5(7%)
Mycophenolate mofetil	17(68%)	32(49%)
Azathioprine	5(20%)	19(29%)
Methotrexate	1(4%)	(3%)
Cyclophosphamide	5(20%)	10(15%)
Rituximab	6(24%)	7(10%)
Plasmapheresis	2(8%)	0
Intravenous immunoglobulin	1(4%)	0

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/a-comparative-study-for-high-prevalence-of-a-positive-family-history-of-systemic-lupus-erythematosus-in-juvenile-onset-systemic-lupus-erythematosus-versus-adult-onset-disease/