Vasculite associada à IgA em criança: relato de caso / IgA vasculitis in a child: case report

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]

Presentación inusual de infección por SARS-CoV-2 simulando púrpura de Schönlein Henoch (vasculitis por inmunoglobulina A) / Unusual presentation of SARS-CoV-2 infection simulating Schönlein Henoch purpura (immunoglobulin A vasculitis)

La vasculitis por inmunoglobulina A, anteriormente llamada púrpura Schönlein Henoch (VIgA/PSH), es la vasculitis sistémica más frecuente en la infancia. El desencadenante más común es una infección previa del tracto respiratorio superior. Se caracteriza por púrpura palpable no trombocitopénica con artralgias y/o artritis, afectación gastrointestinal y compromiso renal. SARS-CoV-2 es un virus ARN que causa la enfermedad COVID-19. Afecta frecuentemente el sistema respiratorio con presentaciones que varían desde una rinitis hasta condiciones severas como síndrome de distress respiratorio, shock séptico o síndrome de inflamación multisistémica (multi-system inflammation syndrome, MIS). Se describe el caso de un niño de 5 años de edad con clínica de VIgA/PSH como forma inicial de presentación y diagnóstico posterior de infección por SARS-CoV-2, derivado al hospital de mayor complejidad, con encefalopatía hipertensiva que presentó evolución favorable y restitución completa del cuadro clínico

Immunoglobulin A vasculitis, previously called Henoch Schonlein purpura (IgAV/ HSP), is the most common systemic vasculitis in childhood. The most common trigger is a previous upper respiratory infection. It is characterized by palpable non-thrombocytopenic purpura with arthralgia and/or arthritis, gastrointestinal and kidney involvement. SARS-CoV-2 is an RNA virus that causes COVID-19 disease. It frequently affects the respiratory system with presentations ranging from rhinitis to severe conditions such as respiratory distress syndrome, septic shock, or multi-system inflammation syndrome (MIS). We describe the case of a 5-year-old boy with symptoms of IgAV/HSP as the initial form of presentation and subsequent diagnosis of SARS-CoV-2 infection, being referred to a more complex hospital with hypertensive encephalopathy, presenting a favorable evolution and complete restoration of the clinical picture.

Risk factors for renal involvement in Henoch-Schönlein purpura

Abstract Objective: Henoch-Schönlein purpura is a systemic vasculitis that mainly occurs in children. Renal impairment is a major complication of Henoch-Schönlein purpura, but there is no established predictive marker for renal involvement. Thus, in this study, we investigated the risk factors for renal involvement in children with Henoch-Schönlein purpura. Method: The medical records of children newly diagnosed as having Henoch-Schönlein purpura between 2005 and 2020 were reviewed retrospectively. Selected laboratory data were recorded before treatment initiation. The date and the age at diagnosis; sex; and the presence of arthralgia, gastrointestinal and renal involvement were obtained retrospectively. Results: This study included a total of 186 patients with Henoch-Schönlein purpura. Among them, 36.0% had renal involvement; 28.4% had only microscopic hematuria, 53.7% had nonnephrotic range proteinuria, and 17.9% had nephrotic-range proteinuria during follow-up. The mean age was higher (p = 0.016) and female sex was predominant (p = 0.001) in patients with renal involvement than in those without renal involvement. Blood neutrophil/lymphocyte ratio (p = 0.002) and platelet/lymphocyte ratio (p = 0.002) were significantly higher than that of the patients without renal involvement. No statistically significant differences were observed in the hemoglobin concentration, platelet count, presence of arthralgia, and gastrointestinal involvement between patients with and without renal involvement. Logistic regression analysis revealed female sex (odd ratio = 3.213) and neutrophil/lymphocyte ratio (odd ratio = 1.329) as risk factors for renal involvement. Conclusions: Female sex and high neutrophil/lymphocyte ratio were risk factors for renal involvement in Henoch-Schönlein purpura.

Risk factors for renal involvement in Henoch-Schönlein purpura.

OBJECTIVE: Henoch-Schönlein purpura is a systemic vasculitis that mainly occurs in children. Renal impairment is a major complication of Henoch-Schönlein purpura, but there is no established predictive marker for renal involvement. Thus, in this study, we investigated the risk factors for renal involvement in children with Henoch-Schönlein purpura. METHOD: The medical records of children newly diagnosed as having Henoch-Schönlein purpura between 2005 and 2020 were reviewed retrospectively. Selected laboratory data were recorded before treatment initiation. The date and the age at diagnosis; sex; and the presence of arthralgia, gastrointestinal and renal involvement were obtained retrospectively. RESULTS: This study included a total of 186 patients with Henoch-Schönlein purpura. Among them, 36.0% had renal involvement; 28.4% had only microscopic hematuria, 53.7% had non-nephrotic range proteinuria, and 17.9% had nephrotic-range proteinuria during follow-up. The mean age was higher (p = 0.016) and female sex was predominant (p = 0.001) in patients with renal involvement than in those without renal involvement. Blood neutrophil/lymphocyte ratio (p = 0.002) and platelet/lymphocyte ratio (p = 0.002) were significantly higher than that of the patients without renal involvement. No statistically significant differences were observed in the hemoglobin concentration, platelet count, presence of arthralgia, and gastrointestinal involvement between patients with and without renal involvement. Logistic regression analysis revealed female sex (odd ratio = 3.213) and neutrophil/lymphocyte ratio (odd ratio = 1.329) as risk factors for renal involvement. CONCLUSIONS: Female sex and high neutrophil/lymphocyte ratio were risk factors for renal involvement in Henoch-Schönlein purpura.

Vasculitis por IGA (púrpura de henoch schönlein) en un paciente pediátrico con COVID-19 y estrongiloidiasis. Reporte de caso

La vasculitis es una enfermedad rara en los niños, siendo la Vasculitis por IgA su presentación más frecuente. Una condición aún poco investigada, es la probable asociación de los procesos tipo vasculitis por IgA con la infección por SARS-CoV-2. Se presenta el caso de una paciente de cuatro años que cursó con lesiones purpúricas palpables a predominio de miembros inferiores, dolor abdominal agudo, y episodios de hemorragia digestiva alta. Inicialmente catalogado como un posible dengue grave y leptospirosis, pero que clínica y laboratorialmente se asoció a un cuadro de vasculitis por IgA. Fue SARS-CoV-2 IgM e IgG: Reactivo. Y tuvo coproparasitológico en el que se identificó al Strongyloides stercoralis. La sintomatología remitió tras la administración de corticoterapia y la evolución fue favorable. Como conclusión, se expuso un caso infrecuente en la población pediátrica, probablemente asociado a los efectos y daños aún desconocidos de la COVID-19 en la actual pandemia.

Vasculitis is a rare disease in children, with IgA Vasculitis being its most common presentation. One condition that is not yet under-researched is the likely association of IgA vasculitis-like processes with SARS-CoV-2 infection. It is presented the case of a four-year-old patient who healed with palpable purplish lesions to lower limb predominance, acute abdominal pain, and episodes of high digestive hemorrhage. Initially listed as a possible severe dengue and leptospirosis, but clinically and laboratorially associated with IgA vasculitis. It was SARS-CoV-2 IgM and IgG: Reactive. And in parasitological study was identified Strongyloides stercoralis. Symptomatology subsided after administration of corticotherapy and the evolution was favorable. In conclusion, it was presented a rare case in the pediatric population, probably associated with the still unknown effects and damage of COVID-19 in the current pandemic.

Vasculitis por IgA (Púrpura de Henoch Schönlein) en un paciente pediátrico con COVID-19 y Estrongiloidiasis / IgA (Henoch Schönlein Purpura) Vasculitis In A Pediatric Patient With COVID-19 And Strongyloidiasis

La vasculitis es una enfermedad rara en los niños, siendo la Vasculitis por IgA su presentación más frecuente. Una condición que se ha asociado al desarrollo de vasculitis es la invasión del endotelio vascular por el Strongyloides stercoralis en casos de hiperinfestación. Otra condición aún poco investigada, es la probable asociación de los procesos tipo vasculitis por IgA con la infección por SARS-CoV-2 y el COVID-19 propiamente. Presentamos el caso de una paciente de cuatro años que cursó con lesiones purpúricas palpables a predominio de miembros inferiores, dolor abdominal agudo, y episodios de hemorragia digestiva alta. Inicialmente catalogado como un posible dengue grave y leptospirosis, pero que clínica y laboratorialmente se asoció a un cuadro de vasculitis por IgA. Fue SARS-CoV-2 IgM e IgG: Reactivo. Y tuvo coproparasitológico en el que se identificó al Strongyloides stercoralis. La sintomatología remitió tras la administración de corticoterapia y la evolución fue favorable.

Vasculitis is a rare disease in children, with IgA Vasculitis being its most common presentation. One condition that has been associated with the development of vasculitis is the invasion of the vascular endothelium by Strongyloides stercoralis in cases of hyperinfestation. Another condition that is not yet under-researched is the likely association of IgA vasculitis-like processes with SARS-CoV-2 and COVID-19 infection itself. It is presented the case of a four-year-old patient who healed with palpable purplish lesions to lower limb predominance, acute abdominal pain, and episodes of high digestive hemorrhage. Initially listed as a possible severe dengue and leptospirosis, but clinically and laboratorially associated with IgA vasculitis. It was SARS-CoV-2 IgM and IgG: Reactive. And in parasitological study was identified Strongyloides stercoralis. Symptomatology subsided after administration of corticotherapy and the evolution was favorable.

Aspectos clínicos y factores asociados a compromiso renal en pacientes pediátricos con púrpura de Schönlein Henoch / Clinical aspects and factors related with renal involvement in pediatrics patients with Schönlein Henoch purpura

INTRODUCCIÓN: La púrpura de Schönlein Henoch (PSH) es la vasculitis más frecuente de la infancia. Es generalmente autolimitada, con morbilidad renal a largo plazo. OBJETIVO: Determinar la frecuencia de las manifestaciones clínicas de la PSH y las variables asociadas al compromiso renal en pacientes con PSH del Servicio de Reumatología pediátrica de la Clínica Universitaria Reina Fabiola durante el periodo 2015-2020. MATERIALES Y METODOS: Estudio observacional, retrospectivo, transversal, analítico. Se incluyeron pacientes menores de 15 años con diagnóstico de PSH. Variables: edad, sexo, mes de diagnóstico, compromiso dermatológico, renal, gastrointestinal, articular y recurrencia y/o persistencia y otras manifestaciones. Análisis estadístico: test T de Student, test chi cuadrado y regresión logística multivariada. RESULTADOS: Se analizaron 107 pacientes, 61 (57%) de sexo femenino, con una media (desviación estándar, DE) de edad de 6,49 (3,48) años. El 100% presentó compromiso dérmico, 19 (18%) púrpura persistente o recurrente, 21 (19%) síntomas gastrointestinales, 38 (36%) compromiso articular y 21 (20%) manifestaciones renales. Se asoció con compromiso renal a pacientes con edad mayor a 7 años (p=0.0064), púrpura persistente o recurrente (p=0.0001), compromiso articular (p=0,0135) y dolor abdominal (p=0,0136). En el análisis multivariado, la púrpura persistente o recurrente se asoció con compromiso renal (OR=7,16; IC95%: 1,81-28,25); p=0.005). CONCLUSIONES: La púrpura persistente o recurrente fue considerada factor de riesgo para presentar compromiso renal y además se evidenció una asociación entre pacientes mayores a 7 años, compromiso articular y dolor abdominal con compromiso renal.

INTRODUCTION: Schönlein Henoch purpura (SHP) is the most frequent vasculitis in childhood. Its course is generally self-limited but can lead to long-term renal morbidity OBJECTIVE: To determine the frequency of the clinical manifestations of PSH. To determine the variables associated with renal involvement in patients with SHP, treated by the Pediatric Rheumatology Service of the Reina Fabiola University Clinic during the period 2015-2020. MATERIALS AND METHODS: Observational, retrospective, transversal, analytical study. Patients under 15 years of age with a diagnosis of HSP, were included. The variables were: age, sex, month of diagnosis, dermatological, renal, gastrointestinal, joint involvement, recurrence and/or prevalence and other manifestations. Statistical analysis: Student's t test, chi square test and multivariate logistic regression. RESULTS: 107 patients were analyzed, 57% were female (n = 61), with a mean age of 6.49 SD of 3.48 years. 100% presented dermal involvement, 19 (18%) persistent or recurrent purpura, 21 (19%) gastrointestinal symptoms, 38 (36%) joint involvement and 21 (20%) kidney manifestations. An association with kidney involvement was found in patients older than 7 years (p = 0.0064), persistent or recurrent purpura (p = 0.0001), joint involvement (p = 0.0135) and abdominal pain (p = 0.0136), but only persistent or recurrent purpura was the only risk factor associated with statistically significant renal involvement (OR = 71.17; 95% CI = 1.81 - 28.25; P = 0.005). CONCLUSION: Persistent or recurrent purpura was considered a risk factor for the fact of presenting kidney involvement and also an association was evidenced between patients older than 7 years, joint involvement and abdominal pain with kidney involvement.

Glomerulonefritis rápidamente progresiva mediada por inmunocomplejos (tipo II). Revisión de la literatura / Immune complex-mediated rapidly progressive glomerulonephritis (type II). Literature review

La glomerulonefritis rápidamente progresiva mediada por complejos inmunes (GMNRP II) es un síndrome clínico caracterizado por el rápido deterioro de la función renal asociado a hematuria, edemas y oliguria. Histológicamente se manifiesta como una glomerulonefritis crescéntica, con la presencia de depósitos granulares en la inmunofluorescencia. Aunque es una enfermedad rara, es grave y puede evolucionar a una enfermedad renal crónica, por lo cual es fundamental su identificación temprana. A continuación, se presenta una revisión sobre este tipo de glomerulonefritis, con énfasis en su etiología y en las opciones terapéuticas existentes en la actualidad

Rapidly progressive immune complex-mediated glomerulonephritis (RPGNMN II) is a clinical syndrome characterized by severe deterioration of renal function associated with hematuria, edema, and oliguria. It is histologically characterized as a crescentic glomerulonephritis, with the presence of granular deposits on immunofluorescence. Although it is a rare condition, it is a potentially serious disease that may progress to chronic renal disease, therefore its early identification is essential. Here we present a review of this form of glomerulonephritis, with emphasis on its etiology and the currently available therapeutic options

Función predictiva del índice de neutrófilos/linfocitos y del volumen plaquetario medio en la púrpura de Henoch-Schönlein relacionada con compromiso renal y gastrointestinal / Predictive role of neutrophil to lymphocyte ratio and mean platelet volume in Henoch-Schönlein purpura related gastrointestinal and renal involvement

Antecedentes: La púrpura de Henoch-Schönlein (PHS) es una vasculitis sistémica de vasos pequeños. El objetivo fue evaluar el índice de neutrófilos/linfocitos (INL) en sangre y el volumen plaquetario medio (VPM) en la PHS e investigar la relación con el compromiso renal y gastrointestinal.Métodos: Se incluyeron niños con PHS y controles sanos. Se evaluaron concentración de hemoglobina, recuento de leucocitos, recuento de trombocitos, INL, VPM, velocidad de sedimentación globular y proteína C-reactiva.Resultados: El INL fue significativamente mayor en los pacientes con PHS con hemorragia gastrointestinal (p < 0,001). El valor ideal de corte del INL para predecir la hemorragia gastrointestinal fue 2,05, con 93 % de sensibilidad y 62 % de especificidad. El VPM fue significativamente mayor en los pacientes con PHS con compromiso renal (p = 0,027).Conclusiones: El INL en sangre y el VPM podrían ser útiles para identificar el compromiso renal y gastrointestinal en la PHS

Background: Henoch-Schönlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. The aim was to evaluate the blood neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) in patients with HSP and to investigate the relationship with gastrointestinal and renal involvement.Methods: Children with HSP and healthy individuals as controls were included. Hemoglobin level, white blood cell count, platelet count, NLR, MPV erythrocyte sedimentation rate and C-reactive protein were evaluated.Results: There were 71 HSP children and 74 controls. NLR was significantly higher in HSP patients with gastrointestinal bleeding than without gastrointestinal bleeding (p < 0,001). The optimal cutoff value of NLR for predicting gastrointestinal bleeding was 2.05, with 93 % sensitivity and 62 % specificity. MPV was significantly higher in HSP patients with renal involvement than without renal involvement (p = 0,027).Conclusions:Blood NLR and MPV may be useful markers to identify gastrointestinal and renal involvement in HSP patients.

Predictive role of neutrophil to lymphocyte ratio and mean platelet volume in Henoch-Schönlein purpura related gastrointestinal and renal involvement. / Función predictiva del índice de neutrófilos/linfocitos y del volumen plaquetario medio en la púrpura de Henoch-Schönlein relacionada con compromiso renal y gastrointestinal.

BACKGROUND: Henoch-Schönlein purpura (HSP) is a systemic small-vessel vasculitis that occurs mainly in children. The aim was to evaluate the blood neutrophil-to-lymphocyte ratio (NLR) and mean platelet volume (MPV) in patients with HSP and to investigate the relationship with gastrointestinal and renal involvement. METHODS: Children with HSP and healthy individuals as controls were included. Hemoglobin level, white blood cell count, platelet count, NLR, MPV erythrocyte sedimentation rate and C-reactive protein were evaluated. RESULTS: There were 71 HSP children and 74 controls. NLR was significantly higher in HSP patients with gastrointestinal bleeding than without gastrointestinal bleeding (p < 0,001). The optimal cutoff value of NLR for predicting gastrointestinal bleeding was 2.05, with 93 % sensitivity and 62 % specificity. MPV was significantly higher in HSP patients with renal involvement than without renal involvement (p = 0,027). CONCLUSIONS: Blood NLR and MPV may be useful markers to identify gastrointestinal and renal involvement in HSP patients.

Antecedentes: La púrpura de Henoch-Schönlein (PHS) es una vasculitis sistémica de vasos pequeños. El objetivo fue evaluar elíndicedeneutrófilos/linfocitos (INL) ensangreyelvolumen plaquetario medio (VPM) en la PHS e investigar la relación con el compromiso renal y gastrointestinal. Métodos: Se incluyeron niños con PHS y controles sanos. Se evaluaron concentración de hemoglobina, recuento de leucocitos, recuento de trombocitos, INL, VPM, velocidad de sedimentación globular y proteína C-reactiva. Resultados: El INL fue significativamente mayor en los pacientes con PHS con hemorragia gastrointestinal (p < 0,001). El valor ideal de corte del INL para predecir la hemorragia gastrointestinal fue 2,05, con 93 % de sensibilidad y 62 % de especificidad. El VPM fue significativamente mayor en los pacientes con PHS con compromiso renal (p = 0,027). Conclusiones: El INL en sangre y el VPM podrían ser útiles para identificar el compromiso renal y gastrointestinal en la PHS.

Púrpura de Schönlein-Henoch en pediatría: Diez años de experiencia en un consultorio de moderado riesgo en un hospital general / Henoch-Schönlein purpura in pediatrics: Ten years of experience at a moderate risk office of a general hospital

Introducción. La púrpura de Schönlein-Henoch (PSH) es la vasculitis leucocitoclástica de pequeños vasos más común en la infancia. Se caracteriza por púrpura palpable y/o compromiso articular, abdominal o renal. Objetivo: Describir las características epidemiológicas, clínicas, de laboratorio y evolutivas en pacientes con PSH. Población y métodos. Estudio observacional, descriptivo, retrospectivo. Se incluyeron pacientes menores de 15 años durante el período 1/1/2008 al 31/12/2017 que cumplían con los criterios de Ankara para el diagnóstico de PSH. Resultados. Se incluyeron 339 pacientes con PSH; la distribución fue similar en ambos sexos; edad promedio de presentación: 7,02 años (± 3,02). El 78 % presentó forma típica y el 22 %, atípica, con compromiso articular previo a la aparición de la púrpura. Manifestaciones articulares: el 82 %; compromiso abdominal: el 54 %; manifestaciones renales: el 19 %. El 68 % de los pacientes que tuvieron compromiso renal lo manifestaron dentro del primer mes desde el debut. El síntoma más frecuente fue la hematuria aislada y el 22 % de los pacientes con compromiso renal presentó formas moderadas-graves de nefritis. El 15 % de los pacientes presentó recurrencia/s. Conclusión. Las manifestaciones articulares fueron más frecuentes de lo descrito en otras series y el compromiso renal fue menor. Si bien este compromiso suele manifestarse en los primeros meses de evolución, se destaca la necesidad del seguimiento de estos pacientes con controles urinarios y de tensión arterial en la evaluación pediátrica.

Introduction. Henoch-Schönlein purpura (HSP) is the most common small vessel leukocytoclastic vasculitis during childhood. It is characterized by palpable purpura and/or joint, abdominal or renal involvement. Objective. To describe the epidemiological, clinical, laboratory, and evolutionary characteristics of patients with HSP. Population and methods. Observational, descriptive, and retrospective study. Patients younger than 15 years who met the Ankara criteria for HSP diagnosis were included in the period between 1/1/2008 and 12/31/2017.Results. A total of 339 patients with HSP were included; the male/female distribution was similar; their average age at onset was 7.02 years (± 3.02). The typical form was observed in 78 % and the atypical form, in 22 %, with joint involvement prior to purpura onset. Joint manifestations: 82 %; abdominal involvement: 54 %; renal manifestations: 19 %. Among patients with renal involvement, 68 % experienced it in the first month after onset. The most common symptom was isolated hematuria, and 22 % of patients with renal involvement had moderate to severe nephritis. Recurrences were observed in 15 % of patients. Conclusion. Joint manifestations were more common than what has been described in other series, whereas renal involvement was less common. Although it usually occurs in the first months of disease onset, it is worth noting that these patients need follow-up with urinary and blood pressure controls as part of their pediatric assessment.

Henoch-Schonlein purpura in pediatrics: Ten years of experience at a moderate risk office of a general hospital. / Púrpura de Schonlein-Henoch en pediatría: Diez años de experiencia en un consultorio de moderado riesgo en un hospital general.

INTRODUCTION: Henoch-Schonlein purpura (HSP) is the most common small vessel leukocytoclastic vasculitis during childhood. It is characterized by palpable purpura and/or joint, abdominal or renal involvement. OBJECTIVE: To describe the epidemiological, clinical, laboratory, and evolutionary characteristics of patients with HSP. POPULATION AND METHODS: Observational, descriptive, and retrospective study. Patients younger than 15 years who met the Ankara criteria for HSP diagnosis were included in the period between 1/1/2008 and 12/31/2017. RESULTS: A total of 339 patients with HSP were included; the male/female distribution was similar; their average age at onset was 7.02 years (± 3.02). The typical form was observed in 78 % and the atypical form, in 22 %, with joint involvement prior to purpura onset. Joint manifestations: 82 %; abdominal involvement: 54 %; renal manifestations: 19 %. Among patients with renal involvement, 68 % experienced it in the first month after onset. The most common symptom was isolated hematuria, and 22 % of patients with renal involvement had moderate to severe nephritis. Recurrences were observed in 15 % of patients. CONLUSION: Joint manifestations were more common than what has been described in other series, whereas renal involvement was less common. Although it usually occurs in the first months of disease onset, it is worth noting that these patients need follow-up with urinary and blood pressure controls as part of their pediatric assessment.

Introducción: La púrpura de Schonlein-Henoch (PSH) es la vasculitis leucocitoclástica de pequeños vasos más común en la infancia. Se caracteriza por púrpura palpable y/o compromiso articular, abdominal o renal. Objectivo: Describir las características epidemiológicas, clínicas, de laboratorio y evolutivas en pacientes con PSH. Población y métodos: Estudio observacional, descriptivo, retrospectivo. Se incluyeron pacientes menores de 15 años durante el período 1/1/2008 al 31/12/2017 que cumplían con los criterios de Ankara para el diagnóstico de PSH. Resultados: Se incluyeron 339pacientes con PSH; la distribución fue similar en ambos sexos; edad promedio de presentación: 7,02 años (± 3,02). El 78 % presentó forma típica y el 22 %, atípica, con compromiso articular previo a la aparición de la púrpura. Manifestaciones articulares: el 82 %; compromiso abdominal: el 54 %; manifestaciones renales: el 19 %. El 68 % de los pacientes que tuvieron compromiso renal lo manifestaron dentro del primer mes desde el debut. El síntoma más frecuente fue la hematuria aislada y el 22 % de los pacientes con compromiso renal presentó formas moderadas-graves de nefritis. El 15 % de los pacientes presentó recurrencia/s. Conclusión: Las manifestaciones articulares fueron más frecuentes de lo descrito en otras series y el compromiso renal fue menor. Si bien este compromiso suele manifestarse en los primeros meses de evolución, se destaca la necesidad del seguimiento de estos pacientes con controles urinarios y de tensión arterial en la evaluación pediátrica.

The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

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.

Henoch-Schönlein purpura nephritis associated with monoclonal gammopathy of renal significance: a case report

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.

The profile and clinical outcomes of patients with renal involvement due to IgA vasculitis: is azathioprine a good option for treatment?

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.

A 19-year old man with IgA vasculitis after vaccination.

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.

A 19-year old man with IgA vasculitis after vaccination

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.