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Urticarial vasculitis in pediatric systemic lupus erythematosus
Ahmed Al Kamzari1, Buthaina Al Musalhi2, Safiya Al Abrawi3
1Child Health Department, Oman Medical Specialty Board, Muscat, Oman.
Insights
Urticarial vasculitis (UV) affects 36% of pediatric systemic lupus erythematosus (pSLE) patients in Oman. This cohort showed unique features, including higher male prevalence and conjunctivitis, in patients with UV.
Area of Science:
- Pediatric Rheumatology
- Dermatology
- Immunology
Background:
- Pediatric systemic lupus erythematosus (pSLE) is a chronic autoimmune disease.
- Urticarial vasculitis (UV) is an inflammatory condition that can affect skin and internal organs.
- The prevalence and specific characteristics of UV in pSLE patients of Arab ethnicity from Oman are not well-documented.
Purpose of the Study:
- To determine the prevalence of urticarial vasculitis (UV) in a pediatric systemic lupus erythematosus (pSLE) cohort of Arab ethnicity from Oman.
- To describe the demographic, clinical, and laboratory features of pSLE patients with and without UV.
Main Methods:
- Retrospective multicenter study conducted in Oman over a 10-year period (2008-2018).
- Inclusion criteria: pSLE patients under 13 years of age.
- Statistical analysis: Univariate statistics.
Main Results:
- 36% (n=53) of 148 pSLE patients were diagnosed with UV.
- pSLE patients with UV were more likely to be male (57% vs 15%), diagnosed younger (5.9 vs 8.5 years), have a family history of SLE (53% vs 36%), and conjunctivitis (32% vs 5.3%).
- UV subgroup showed lower CNS involvement (7.6% vs 20%), leukopenia (9.4% vs 24%), thrombocytopenia (5.7% vs 18%), higher low C3 complement (94% vs 66%), and positive cytoplasmic ANCA (11% vs 0%).
Conclusions:
- A high occurrence of UV was observed in this Omani pSLE cohort.
- UV in pSLE is associated with distinct demographic, clinical, and laboratory features in this population.
- Further research is warranted to understand the specific pathophysiology and management of UV in pSLE.
Background/Objectives:
To determine the prevalence of urticarial vasculitis (UV) in a pediatric systemic lupus erythematosus (pSLE) cohort of Arab ethnicity from Oman and to describe their demographic, clinical, and laboratory features.
Methods:
We conducted a retrospective multicenter study among pediatric rheumatology centers in Oman over a 10-year period from 2008 to 2018. Analyses were performed using univariate statistics.
Results:
A total of 148 pSLE under the age of 13 years were included of which 30% (n = 44) were men. The overall mean age at SLE diagnosis was 7.6 ± 3.5 years while the mean disease duration was 10.1 ± 5.4 years. UV was diagnosed in 36% (n = 53) of pSLE patients. Patients with pSLE and UV were more likely to be male (57% vs 15%; P < .001), diagnosed at a younger age (5.9 vs 8.5 years; P < .001), have a family history of SLE (53% vs 36%; P = .044), and have associated conjunctivitis, (32% vs 5.3%; P < .001) but less likely to have CNS involvement (7.6% vs 20%; P = .045) or hematological manifestations such as leukopenia (9.4% vs 24%; P = .028) and thrombocytopenia (5.7% vs 18%; P = .045). This subgroup was also more likely to be associated with low C3 complement count (94% vs 66%; P < .001) and positive cytoplasmic ANCA (11% vs 0%; P = .022).
Conclusion:
We report a high occurrence of UV in a pSLE cohort associated with unique demographic, clinical, and laboratory features.
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