Renal biopsy in children with IgA vasculitis / Biópsia renal em crianças com vasculite por IgA

Abstract Introduction Henoch-Schönlein purpura nephritis (HSN) is defined as Henoch-Schönlein purpura with kidney involvement, including hematuria and/or proteinuria. The aim of this study was to evaluate the data of HSN patients who underwent renal biopsy, and compare the main clinical and laboratory parameters that may affect renal biopsy findings, treatment protocols, and short- and long-term outcome of those patients. Methods Biopsies performed in 72 HSN patients between January 2007 to January 2017 were retrospectively evaluated. They were divided into two groups according to renal biopsy classification of the International Study of Kidney Disease in Children. Renal outcome, clinical and laboratory parameters, treatment protocols, and outcome were compared between groups. Short- and long-term follow-up of patients were evaluated. Results Of 72 patients, 47 were male (65.3%) and 44 (61.1%) were ≤10 years of age. Neutrophil-lymphocyte ratio was found higher in patients with scrotal involvement (p=0.042). Short-term unfavorable outcome was significantly higher in patients with scrotal involvement (p=0.038). Patients with hypertension and decreased creatinine clearance were found to have more unfavorable outcomes in long-term follow-up (p=0.029, p=0.040). Conclusion Cyclosporin-A and cyclophosphamide could be effective in steroid unresponsive HSN patients. Patients with scrotal involvement, decreased creatinine clearance, and hypertension should be closely monitored for sequelae of HSN.

Resumo Introdução A nefrite da púrpura de Henoch-Schönlein (NPHS) é definida como púrpura de Henoch-Schönlein com envolvimento renal, incluindo hematúria e/ou proteinúria. O objetivo deste estudo foi avaliar os dados de pacientes com NPHS que foram submetidos à biópsia renal e comparar os principais parâmetros clínicos e laboratoriais que podem afetar os achados da biópsia renal, os protocolos de tratamento e o desfecho de curto e longo prazo desses pacientes. Métodos Foram avaliadas retrospectivamente biópsias realizadas em 72 pacientes com NPHS entre Janeiro de 2007 e Janeiro de 2017. Eles foram divididos em dois grupos de acordo com a classificação de biópsia renal do Estudo Internacional de Doenças Renais em Crianças. O desfecho renal, parâmetros clínicos e laboratoriais, protocolos de tratamento e desfechos foram comparados entre os grupos. Foi avaliado o acompanhamento de pacientes de curto e longo prazo. Resultados De 72 pacientes, 47 eram homens (65,3%) e 44 (61,1%) tinham ≤10 anos de idade. A razão neutrófilo-linfócito foi encontrada mais alta em pacientes com envolvimento escrotal (p=0,042). O desfecho desfavorável de curto prazo foi significativamente maior em pacientes com envolvimento escrotal (p=0,038). Constatou-se que pacientes com hipertensão e diminuição da depuração de creatinina apresentaram desfechos mais desfavoráveis no acompanhamento de longo prazo (p=0,029, p=0,040). Conclusão A ciclosporina-A e a ciclofosfamida podem ser eficazes em pacientes com NPHS não responsivos a esteroides. Pacientes com envolvimento escrotal, diminuição da depuração de creatinina e hipertensão devem ser monitorados de perto para sequelas de NPHS.

IgA vasculitis (Henoch-Schönlein purpura) nephritis and psoriasis in a child: is there a relationship? / Vasculite por IgA (púrpura de Henoch-Schönlein), nefrite e psoríase em uma criança: existe relação?

Abstract Background Psoriasis is a chronic immune-mediated disorder that primarily affects the skin in both adults and children but can also have systemic involvement, particularly with arthritis and kidney injury. IgA nephropathy is the most frequent kidney disorder associated with psoriasis. Approximately one third of all cases of psoriasis begin in childhood, but association between psoriasis and renal disorders has scarcely been reported in pediatric patients. Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by IgA deposits in the vessel walls of affected organs and in the mesangium of the kidney. HSP nephritis histopathology is identical to IgA nephropathy. Case report A 6-year-old boy with recent onset of psoriasis developed HSP with kidney involvement, clinically manifested by nephrotic-range proteinuria and hematuria. Kidney biopsy revealed fibrocellular glomerular crescents and mesangial IgA deposits compatible with IgA nephropathy. Treatment with systemic corticosteroids led to the control of hematuria, but as nephrotic-range proteinuria persisted, cyclophosphamide was added, leading to a gradual decrease in proteinuria. Conclusions We propose an underlying common mechanism in the pathogenesis of both HSP and psoriasis, involving a dysregulation of the IgA-mediated immune response, which could predispose to both entities as well as to kidney damage and IgA nephropathy in these patients.

Resumo Histórico A psoríase é uma doença crônica imunomediada que afeta principalmente a pele tanto em adultos quanto em crianças, mas também pode ter envolvimento sistêmico, particularmente com artrite e lesão renal. A nefropatia por IgA é o distúrbio renal mais frequentemente associado à psoríase. Aproximadamente um terço de todos os casos de psoríase começam na infância, mas a associação entre psoríase e distúrbios renais tem sido pouco relatada em pacientes pediátricos. A Púrpura de Henoch-Schönlein (PHS) é uma vasculite sistêmica caracterizada por depósitos de IgA nas paredes dos vasos de órgãos afetados e no mesângio do rim. A histopatologia da nefrite da PHS é idêntica à da nefropatia por IgA. Relato de caso Um menino de 6 anos de idade com início recente de psoríase desenvolveu PHS com envolvimento renal, clinicamente manifestado por proteinúria nefrótica e hematúria. A biópsia renal revelou crescentes fibrocelulares glomerulares e depósitos mesangiais de IgA compatíveis com a nefropatia por IgA. O tratamento com corticosteróides sistêmicos levou ao controle da hematúria, mas como a proteinúria nefrótica persistiu, a ciclofosfamida foi adicionada, levando a uma diminuição gradual da proteinúria. Conclusões Propomos um mecanismo comum subjacente na patogênese tanto da PHS quanto da psoríase, envolvendo uma desregulação da resposta imune mediada por IgA, que poderia predispor a ambas as entidades, bem como a danos renais e nefropatia por IgA nesses pacientes.

Vasculitis IgA en el adulto. Informe de caso / IgA vasculitis in adults. Case report

RESUMEN La vasculitis IgA, también conocida como púrpura de Schönlein-Henoch, es una vasculitis leucocitoclástica que involucra pequeños vasos con depósito de inmunocomplejos IgA. Puede abarcar piel, articulaciones, riñones y tracto gastrointestinal. Su presentación en adultos es rara, y las formas clínicas suelen ser más agresivas. Es objetivo del presente trabajo describir el curso y evolución de vasculitis IgA, en un paciente de 59 años, con púrpuras en miembros inferiores y tronco, hematuria macroscópica, y edema de miembros inferiores. Los complementarios mostraron creatininas elevadas, proteinuria de rango nefrótico, elevación de la IgA y anticuerpos contra el citoplasma de los neutrófilos negativos. Se descartaron causas neoplásicas. El estudio anatomo-patológico del riñón concluyó una vasculitis IgA.

ABSTRACT IgA vasculitis, also known as Henoch-Schönlein purpura, is a leukocytoclastic vasculitis that involves small vessels with deposition of IgA immune complexes. It can include skin, joints, kidneys, and gastrointestinal tract. Its presentation in adults is rare, and the clinical forms are usually more aggressive. The objective of this study is to describe the course and evolution of IgA vasculitis, in a 59-years-old patient, with purples in the lower limbs and trunk, macroscopic hematuria, and lower limb edema. The complementary ones showed elevated creatinines, nephrotic range proteinuria, elevated IgA and negative antibodies against the cytoplasm of neutrophils. Neoplastic causes were dismissed. The anatomical-pathological study of the kidney concluded IgA vasculitis.

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.

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.

Bulluos Henoch-Schönlein Purpura in pediatrics. Case report / Púrpura Henoch-Schönlein bullosa en pediatría. Reporte de caso

Abstract Introduction: Henoch Schönlein purpura (HSP) is the most common type of vasculitis in childhood. HSP affects small blood vessels, and it rarely leads to serious complications such as bullous small vessel vasculitis, as it occurred in the case presented here. Case presentation: 5-year-old male who was brought to a primary healthcare center due to having arthralgia and purple skin lesions on his lower limbs. After the patient was diagnosed with HSP, he developed bullous lesions, so he was hospitalized and analgesic and topical management was started. During his hospital stay, the patient's renal function was monitored, and since he did not experience other complications, he was discharged. Conclusion: The available literature on HSP suggests that its cutaneous bullous manifestation rarely occurs in pediatric population and that, unlike normal HSP cases, it is not always associated with renal and/or gastrointestinal involvement. However, regardless of the dermatological severity of this type of vasculitis, the function of the gastrointestinal and renal systems must be always monitored in these patients.

Resumen Introducción. La púrpura Henoch-Schönlein (PHS) es la forma más común de vasculitis en la infancia; esta se da en pequeños vasos sanguíneos y no es frecuente que genere complicaciones graves como la vasculitis bullosa, tal como sucedió en el caso que se presenta a continuación. Presentación del caso. Paciente masculino de cinco años que fue traído a un centro de atención primaria con un cuadro clínico consistente en artralgias y aparición de lesiones purpúricas en miembros inferiores. Luego de ser diagnosticado con PHS, presentó lesiones bullosas, por lo que fue hospitalizado y se inició manejo analgésico y tópico; durante su estadía en el hospital se vigiló su función renal y, ya que no presentó otras complicaciones, se dio de alta. Conclusión. Las publicaciones disponibles sobre PHS sugieren que su presentación cutánea bullosa en pediatría no es frecuente y que no siempre se relaciona con un compromiso renal y/o gastrointestinal como la variante clásica; sin embargo, siempre debe vigilarse la función de estos sistemas sin importar la gravedad dérmica de esta vasculitis.

Púrpura de Shönlein-Henoch en el curso de trombocitopenia inmune primaria crónica. Presentación de un caso / Shönlein-Henoch Purpura in the Course of Chronic Primary Inmmune Thrombocytopenia. Presentation of a Case

Resumen La púrpura de Shönlein-Henoch es una vasculitis de la infancia caracterizada por una púrpura palpable no trombocitopénica y manifestaciones sistémicas, acompañadas de dolor abdominal, sangrado gastrointestinal, glomerulonefritis, artritis y artralgias. Está relacionada con patologías desencadenantes como las infecciones y las picaduras de insectos. Se presenta el caso de un niño de 4 años de edad, que presentaba cuadros alérgicos de rinitis y urticaria. Se le había diagnosticado una trombocitopenia inmune primaria de evolución crónica. A pesar del empleo de diferentes alternativas de tratamiento, recurrió con trombocitopenia muy severa, asociada a cuadro de púrpura y lesiones maculo-papulares y urticarianas. Los complementarios realizados, a excepción del conteo de plaquetas, estuvieron en rangos normales. La evolución fue satisfactoria en un periodo de tres semanas. Se presenta el caso porque es una forma atípica de esta enfermedad.

Abstract Shönlein-Henoch purpura is a common vasculatis in childhood, characterized by a non-trombocytopenic palpable purpura and systemic manifestations, accompanied by abdominal pain, gastro-intestinal bleeding, glomerulonephritis, arthritis and arthralgias. It is the most common of diseases due to disturbances in the vascular component in children. It is related to triggering pathologies as infections and insect bites. A case of a 4 year old boy is presented who presented allergic episodes of rhinitis and urticaria. He had been diagnosed with an chronic primary inmmune thrombocytopenia. In spite of the different treatment approaches, the patient had a severe thrombocytopenia, associated to purpura and macolu-papular lesions and uticaria. Lab tests, with the exception of platelet count, were within the normal limits. The progress was satisfactory in a three week period.

Púrpura Schõnlein Henoch con compromiso renal: reporte de un caso / Henoch Schõnlein purpura with renal involvement: case report

INTRODUCCIÓN: El Púrpura Schõnlein Henoch es una vasculitis sistémica que afecta principalmente a la piel, articulaciones, sistema gastrointestinal y renal. Es una de las vasculitis más comunes en la infancia. El compromiso renal se da en aproximadamente el40 por ciento de los casos, y en la mayoría se presenta con hematuria, sin embargo, también puede manifestarse como proteinuria, síndrome nefrótico y nefrítico. PRESENTACIÓN DEL CASO: Se presenta el caso de una paciente de 6 años que consulta en Servicio de Urgencia por dolor en el tarso del pie izquierdo de 4 días de evolución, sin antecedentes de trauma, acompañándose de dolor en rodilla izquierda con limitación a la movilización, y lesiones purpúricas en extremidades inferiores. Durante la hospitalización, la paciente presentó al inicio presiones arteriales altas y oliguria, manejándose con antihipertensivos, además del inicio del tratamiento con corticoides. Luego de la mejoría del compromiso articular y cutáneo, se evidenció compromiso renal con proteinuria, que al persistir se aumentó las dosis de corticoides, teniendo favorable respuesta al disminuir sus niveles. Al encontrarse en mejores condiciones, se decidió manejo ambulatorio manteniendo tratamiento corticoidal y antihipertensivo, controlándose en Nefrología y Reumatología Infantil. DISCUSIÓN: Se concluye de este trabajo que la corticoterapia y el manejo de la presión arterial fueron pilares fundamentales para el tratamiento en este caso, mejorando su evolución clínica y principalmente la disminución de la proteinuria con el uso de los corticoides, evitando un compromiso mayor a pesar de que no esté comprobado su beneficio en la literatura.

INTRODUCTION: The Henoch Schõnlein Purpura is a systemic vasculitis that mainly affects the skin, joints, gastrointestinal system and renal system. It is one of the most common vasculitis in childhood. Renal involvement occurs in approximately40 percent of cases, and in most cases it occurs with hematuria. However, it may also manifest as proteinuria, nephrotic and nephritic syndrome. CASE REPORT: We report the case of a 6-year old patient who consulting in the emergency service for 4 days with pain in left foot tarsal, without a history of trauma, accompanied by pain in left knee with limited mobilization, and purpuric lesions in lower extremities. During hospitalization, the patient presented at the beginning high blood pressure and oliguria, handling itself with antihypertensive, in addition to the initiation of treatment with corticosteroids. After improvement of cutaneus and articular involvement, renal involvement was evident with proteinuria, at persist was increased doses of corticosteroids, with favorable response to decrease levels. Being in better conditions was decided ambulatory management keeping antihypertensive and corticosteroid treatment, controlling in Nephrology and Child Rheumatology. DISCUSSION: It can be concluded from this study that the steroids and blood pressure management were fundamental pillars for the treatment in this case, improving their clinical course and mainly the proteinuria decreased with the use of corticosteroids, preventing a greater commitment although is not verified in the literature their advantage.

Case for diagnosis / Caso para diagnóstico

Henoch-Schönlein Purpura (HSP) is the most common vasculitis in children. In the absence of significant renal disease it has an excellent prognosis. In the case described, HSP initially presented together with orchitis. This infrequent event required the exclusion of testicular torsion.

A Púrpura de Henoch-Schönlein é a vasculite mais comum na idade pediátrica, tendo um prognóstico excelente na ausência de doença renal significativa. No caso descrito, a apresentação inicial cursou com orquite, o que não é frequente, obrigando a exclusão de torção testicular.

Edema agudo hemorrágico da infância: relato de três casos / Acute hemorrhagic edema of infancy: report of three cases

O Edema Agudo Hemorrágico da Infância é uma vasculite leucocitoclástica pouco frequente, que ocorre, quase exclusivamente, em crianças entre 4 meses e 2 anos de idade. Caracteriza-se, clinicamente, pela tríade febre, lesões purpúricas na face, pavilhões auriculares e extremidades e edema. Embora os achados cutâneos sejam dramáticos e de surgimento rápido, o prognóstico é favorável, com resolução espontânea dentro de 1 a 3 semanas. Descrevem-se três casos cujos achados clínicos e histopatológicos são característicos de edema agudo hemorrágico da infância.

Acute Hemorrhagic Edema of Infancy is an infrequent leukocytoclastic vasculitis which occurs almost exclusively in children between 4 months and 2 years of age. It is clinically characterized by the triad fever, purpuric lesions on the face, auricular pinna and extremities, and edema. Although the cutaneous findings are dramatic and of rapid onset, the prognosis is favorable, with spontaneous resolution within 1 to 3 weeks. Three cases are described in which clinical and histopathological findings are characteristic of acute hemorrhagic edema of infancy.

Long term follow-up of acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome): case report / Síndrome de vasculite retiniana hemorrágica multifocal aguda (síndrome de Blumenkranz) com seguimento a longo prazo: relato de caso

PURPOSE: To report a 16-year long-term follow-up of a patient with acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome). A 21-year old male was seen in 1994 with acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome), first in the left eye, and later in the right eye. He was treated with retinal photocoagulation in areas of retinal ischemia and oral steroids, followed by sequential annual fundus examination and photography for 16 years. Vision improved to 20/25 in both eyes after retinal ischemic areas photocoagulation and oral steroids, and his vision has been maintained for 16 years. Photocoagulation of retinal ischemia and oral steroids are effective for the treatment of acute multifocal hemorrhagic retinal vasculitis (Blumenkranz syndrome).

Relato de caso com acompanhamento por 16 anos de um paciente com a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz). Um paciente de 21 anos de idade foi diagnosticado em 1994 com a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz), primeiro no olho esquerdo e depois no olho direito. Foi tratado com fotocoagulação retiniana nas áreas retinianas isquêmicas e corticosteroide oral e seguido por exames complementares da retina por 16 anos. A visão melhorou para 20/25 em ambos os olhos após a fotocoagulação retiniana nas áreas isquêmicas da retina e corticosteroide oral permanecendo assim até o momento por 16 anos. A fotocoagulação retiniana nas áreas isquêmicas e o uso de corticosteróide oral são tratamentos efetivos para a vasculite hemorrágica multifocal aguda (síndrome de Blumenkranz).

Púrpura de Schönlein-Henoch en Paciente Pediátrico, a Propósito de un Caso / Schönlein-Henoch púrpura in pediatric patients, a case purpose

La púrpura de Schönlein-Henoch es una vasculitis leucocitoclástica, la más común en pediatría, que afecta a pequeños vasos, su etología no es conocida, es una enfermedad multisistémica que afecta piel, articulaciones, tracto gastrointestinal y riñones. Se presenta caso clínico de escolar de 11 años de sexo femenino la cual cursó con púrpuras palpables en miembro inferiores asociado a dolor abdominal al ingreso de su internación como antecedente dos semanas antes presentó infección en tracto respiratorio alto que evoluciónó favorablemente. En el tratamiento de la púrpura se le administró corticoide con lo cual mejoró el cuadro clínico abdominal favorablemente ya que en un momento se pensó que éste dolor abdominal podría complicarse con proceso quirúrgico como invaginación intestinal.

Schonlein-Henoch Purpura is a leukocytoclastic vasculitis, most common in children which affects small vessels; its etiology remains unknown, it is a multisystemical disease that affects skin, joints, gastrointestinal tract and kidneys. The clinical case is presented in a 11 years old female children, with a palpable purpura associated to abdominal pain, in the moment of the hospitalization, having presented two weeks before a history of upper respiratory tract infection that evolved favorably; with a corticosteroid administration, the Purpura this improved clinically and the abdominal pain decreased, instead of increasing, in which case it was thought that it may be complicated with a surgical process as an intestinal instussusception.