RESUMO
A Vasculite associada à imunoglobulina A (VIgA), também conhecida como púrpura de Henoch-Schonlein, púrpura anafilactóide ou púrpura reumática é uma vasculite de pequenos vasos associada a deposição de imunocomplexos IgA, de etiologia ainda desconhecida e que acomete principalmente crianças. Em grande parte dos casos pediátricos, é uma doença autolimitada com manifestações cutâneas, articulares, gastrintestinais e renais. O diagnóstico diferencial inclui outras vasculites, como lúpus eritematoso sistêmico, meningococcemia, coagulação intravascular disseminada e síndrome hemolítica urêmica. Neste artigo abordam-se os principais aspectos da VIgA nas crianças, salientando-se a importância do diagnóstico diferencial precoce. É apresentado o caso clínico de uma paciente do sexo feminino de 5 anos com lesões purpúricas tratada numa primeira abordagem como infecção bacteriana grave. Após reavaliação médica houve alteração terapêutica com uso de glicocorticóides resultando em melhora expressiva dos sintomas. [au]
Vasculitis associated with immunoglobulin A (VIgA), also known as Henoch-Schonlein purpura, anaphylactoid purpura or rheumatic purpura is a small vessel vasculitis associated with deposition of IgA immune complexes, of unknown etiology and affecting mainly children. In most pediatric cases, it is a self-limited disease with cutaneous, joint, gastrointestinal and renal manifestations. The differential diagnosis includes other vasculitis, such as systemic lupus erythematosus, meningococcemia, disseminated intravascular coagulation and uremic hemolytic syndrome. In this article, the main aspects of HSP in children are addressed, highlighting the importance of early differential diagnosis. The clinical case of a 5-year-old female patient with purpuric lesions treated in a first approach as a severe bacterial infection is presented. After medical re-evaluation, there was a therapeutic change with the use of glucocorticoids resulting in a significant improvement of symptoms. [au]
RESUMO
BACKGROUND: The Henoch-Schönlein Purpura (HSP) orIgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. OBJECTIVES: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. METHODS: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. RESULTS: From the178 patients with IgA vasculitis,nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. CONCLUSION: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.
Assuntos
Antirreumáticos/uso terapêutico , Azatioprina/uso terapêutico , Vasculite por IgA/tratamento farmacológico , Nefrite/tratamento farmacológico , Adolescente , Criança , Pré-Escolar , Ciclofosfamida/uso terapêutico , Feminino , Hematúria/etiologia , Humanos , Vasculite por IgA/complicações , Imunossupressores/uso terapêutico , Masculino , Ácido Micofenólico/uso terapêutico , Nefrite/etiologia , Proteinúria/etiologia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.
Assuntos
Humanos , Vasculite por IgA/fisiopatologia , Azatioprina/uso terapêutico , Vasculite/fisiopatologia , Nefrite/tratamento farmacológico , Perfil de SaúdeRESUMO
A 19-year-old patient who mistakenly received two doses of influenza vaccine 10 days before presentation, was admitted with malaise, weakness, and a purpuric non-blanching rash most prominent on the ankles followed by abdominal pain and hematochezia 72h later. The diagnosis of influenza vaccine-related Henoch-Schonlein vasculitis was made. This complication, although rare, is the most common vasculitis related to immunization.
Assuntos
Vasculite por IgA/etiologia , Vacinas contra Influenza/efeitos adversos , Glucocorticoides/uso terapêutico , Humanos , Vasculite por IgA/tratamento farmacológico , Masculino , Metilprednisolona/uso terapêutico , Prednisona/uso terapêutico , Vacinação/efeitos adversos , Adulto JovemRESUMO
ABSTRACT A 19-year-old patient who mistakenly received two doses of influenza vaccine 10 days before presentation, was admitted with malaise, weakness, and a purpuric non-blanching rash most prominent on the ankles followed by abdominal pain and hematochezia 72 h later. The diagnosis of influenza vaccine-related Henoch-Schonlein vasculitis was made. This complication, although rare, is the most common vasculitis related to immunization.
Assuntos
Humanos , Masculino , Adulto Jovem , Vasculite por IgA/etiologia , Vacinas contra Influenza/efeitos adversos , Vasculite por IgA/tratamento farmacológico , Metilprednisolona/uso terapêutico , Prednisona/uso terapêutico , Vacinação/efeitos adversos , Glucocorticoides/uso terapêuticoRESUMO
OBJECTIVE: To characterize scrotal involvement in children and adolescents with IgA vasculitis. METHODS: A cross-sectional retrospective study included 296 IgA vasculitis (EULAR/PRINTO/PRES criteria) patients, 150/296 (51%) were males and assessed by demographic/clinical/laboratory and treatments. Scrotal involvement was defined by the presence of scrotal edema and/or pain/tenderness in physical examination and/or testicular Doppler ultrasound abnormalities. RESULTS: Scrotal involvement was observed in 28/150 (19%) IgA vasculitis patients. This complication was evidenced at IgA vasculitis diagnosis in 27/28 (96%). Acute recurrent scrotal involvement was observed in 2/150 (1%) and none had chronic subtype. Further analysis of patients with scrotal involvement at first episode (n = 27) compared to those without this complication (n = 122) revealed that the median age at diagnosis [4.0 (2.0-12) vs. 6 (1.3-13) years, p = 0.249] was similar in both groups. The frequency of elevated serum IgA was significantly lower in IgA vasculitis patients with scrotal involvement versus without this manifestation (18% vs. 57%, p = 0.017), whereas glucocorticoid (93% vs. 49%, p < 0.0001) and ranitidine use (63% vs. 30%, p = 0.003) were significantly higher in the former group. CONCLUSIONS: The scrotal involvement occurred in almost one fifth of IgA vasculitis patients and was commonly evidenced as acute subtype at diagnosis. Scrotal signs/symptoms improved after a prompt use of glucocorticoid and was associated with low frequency of elevated IgA serum levels.