Ana Paula Sakamoto1, Clovis Artur Silva2, Marco Felipe Castro da Silva3, Anandreia Simões Lopes1, Gleice Clemente Souza Russo1, Adriana Maluf Elias Sallum3, Katia Kozu3, Eloisa Bonfá4, Claudia Saad-Magalhães5, Rosa Maria Rodrigues Pereira4, Claudio Arnaldo Len1, Maria Teresa Terreri6. 1. Universidade Federal de São Paulo (UNIFESP), Unidade de Reumatologia Pediátrica, São Paulo, SP, Brazil. 2. Universidade de São Paulo (USP), Faculdade de Medicina, Unidade de Reumatologia Pediátrica, São Paulo, SP, Brazil; Universidade de São Paulo (USP), Faculdade de Medicina, Divisão de Reumatologia, São Paulo, SP, Brazil. 3. Universidade de São Paulo (USP), Faculdade de Medicina, Unidade de Reumatologia Pediátrica, São Paulo, SP, Brazil. 4. Universidade de São Paulo (USP), Faculdade de Medicina, Divisão de Reumatologia, São Paulo, SP, Brazil. 5. Universidade Estadual Paulista (UNESP), Faculdade de Medicina de Botucatu, Hospital das Clínicas de Botucatu, Botucatu, SP, Brazil. 6. Universidade Federal de São Paulo (UNIFESP), Unidade de Reumatologia Pediátrica, São Paulo, SP, Brazil. Electronic address: teterreri@terra.com.br.
Abstract
OBJECTIVES: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. METHODS: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. RESULTS: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p=0.008), discoid rash (16% vs. 4%, p=0.017), photosensitivity (76% vs. 45%, p=0.002) and other cutaneous vasculitides (80% vs. 19%, p<0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p=0.003), fever (32% vs. 56%, p=0.020) and hepatomegaly (4% vs. 23%, p=0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p>0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p=0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p=0.014) was significantly lower in the DV group. CONCLUSION: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.
OBJECTIVES: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. METHODS: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. RESULTS:DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%). A poor outcome, with digital resorption, occurred in 5 (20%). Comparison of patients with and without DV revealed higher frequency of malar rash (80% vs. 53%, p=0.008), discoid rash (16% vs. 4%, p=0.017), photosensitivity (76% vs. 45%, p=0.002) and other cutaneous vasculitides (80% vs. 19%, p<0.0001), whereas the frequency of overall constitutional features (32% vs. 61%, p=0.003), fever (32% vs. 56%, p=0.020) and hepatomegaly (4% vs. 23%, p=0.026) were lower in these patients. Frequency of female gender, severe multi-organ involvement, autoantibodies profile and low complement were alike in both groups (p>0.05). SLEDAI-2K median, DV descriptor excluded, was significantly lower in patients with DV compared to those without this manifestation [10 (0-28) vs. 14 (0-58), p=0.004]. Visceral vasculitis or death were not observed in this cSLE cohort. The frequency of cyclophosphamide use (0% vs. 18%, p=0.014) was significantly lower in the DV group. CONCLUSION: Our large multicenter study identified clinical DV as one of the rare initial manifestation of active cSLE associated with a mild multisystemic disease, in spite of digital resorption in some of these patients.
Keywords:
Childhood-onset systemic lupus erythematosus; Digital vasculitis; Lúpus eritematoso sistêmico de início na infância; Sledai-2K; Vasculite; Vasculite digital; Vasculitis
Authors: Juliana C O A Ferreira; Vitor C Trindade; Graciela Espada; Zoilo Morel; Eloisa Bonfá; Claudia S Magalhães; Clovis Artur Silva Journal: Clin Rheumatol Date: 2018-08-09 Impact factor: 2.980