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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.
Pediatric Dermatology
|May 7, 2020
Summary
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.
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