Granulomatosis with polyangiitis: A case report of early systemic fulminant presentation

Granulomatosis with polyangiitis is a systemic vasculitis that affects medium and small vessels, with high expression of anti-neutrophil cytoplasmic autoantibody. A case is pre sented on a patient with an initial compromise of the lower airway, who did not respond to management, required intensive care unit management, and died due to severe diffuse alveolar hemorrhage. His definitive diagnosis was established with a clinical autopsy. Gran-ulomatosis with polyangiitis is a disease with different ways of presentation, and can have fatal outcomes if it is not diagnosed early.

La granulomatosis con poliangeítis es un tipo de vasculitis que afecta a vasos de mediano y pequeño calibre de manera sistémica, con una alta expresión de anticuerpos contra el citoplasma del neutrófilo. Se presenta el caso de un paciente con un compromiso inicial de la vía área inferior, que no respondió al tratamiento y requirió manejo en unidad de cuidados intensivos. Finalmente, falleció por una hemorragia alveolar difusa severa. Su diagnóstico definitivo se estableció con una autopsia clínica. La granulomatosis con poliangeítis tiene diferentes formas de presentación y puede tener desenlaces fatales si no se diagnostica a tiempo.

Uso excesivo de la prueba de ANCA / Overuse of the neutrophil cytoplasmic antibody test

La desproporcional y alta frecuencia de órdenes médicas de anticuerpos frente al citoplasma del neutrófilo (ANCA, por sus siglas en inglés) dirigidas a nuestros laboratorios clínicos evidencia el sobreuso de la prueba de ANCA. El uso indiscriminado de esta aumenta los gastos sin beneficio de salud. El laboratorio clínico es el eslabón de la cadena diagnóstica que más siente el uso excesivo de las solicitudes de ANCA, básicamente porque genera resultados falsos positivos que comprometen la utilidad clínica de la prueba, además de recargar innecesariamente el trabajo diario del laboratorio. La prueba de ANCA es una herramienta diagnóstica muy útil para las vasculitis sistémicas primarias, pero su valor en situaciones no vasculíticas así como en otras condiciones inflamatorias y en enfermedades infecciosas o tumorales, no ha sido demostrado.1,2 El descubrimiento de los ANCA cambió...(AU)

Eficácia e segurança de Rituximabe comparado a outras drogas no tratamento de vasculites sistêmicas: revisão rápida de evidências / Efficacy and safety of Rituximab compared to other drugs in the treatment of systemic vasculitides: rapid response review of evidence

Objetivo: descrever as evidências disponíveis na literatura científica sobre eficácia e segurança do rituximabe comparado a diferentes tratamentos. Materiais e métodos: é uma revisão rápida de evidências científicas para tomada de decisão informada por evidências em políticas e práticas de saúde. Conclusão: o Rituximabe tem eficácia e segurança similares à da Ciclofosfamida, para terapia de indução de remissão e para manutenção da remissão e, para pacientes com doença recidivante, o Rituximabe é mais eficaz que a Ciclofosfamida para manter a remissão. Para terapia de manutenção, Rituximabe é mais eficaz que Azatioprina, com perfil de segurança similar. Diferentes regimes de dosagem do Rituximabe tem eficácia e segurança similar para terapia de manutenção. O Infliximabe parece ser superior ao Rituximabe nos desfechos de eficácia (indução e manutenção da remissão).

Objective: to describe the evidence available in the scientific literature on the efficacy and safety of rituximab compared to different treatments. Materials and Methods: is a rapid review of scientific evidence for evidence-informed decision making in health policy and practice. Conclusion: Rituximab has similar efficacy and safety to Cyclophosphamide, for remission induction therapy and for maintenance of remission, and for patients with relapsing disease, Rituximab is more effective than Cyclophosphamide in maintaining remission. For maintenance therapy, Rituximab is more effective than Azathioprine, with a similar safety profile. Different dosing regimens of Rituximab have similar efficacy and safety for maintenance therapy. Infliximab appears to be superior to Rituximab in efficacy outcomes (induction and maintenance of remission).

Síndrome de Behçet ou úlcera de Lipschütz: desafio diagnóstico / Behçet's syndrome or Lipschütz ulcer: diagnostic challenge

A ocorrência de úlceras genitais em adolescentes e mulheres jovens tem impacto emocional para as pacientes e seus familiares, pela frequente associação com uma possível etiologia de transmissão sexual. Porém, úlcera de Lipschütz e síndrome de Behçet não têm etiologia infecciosa e devem ser lembradas como possíveis diagnósticos diferenciais. O diagnóstico dessas duas patologias é clínico e pode ser desafiador. Dessa forma, foi realizada uma revisão na literatura com o objetivo de comparar as duas entidades. A úlcera de Lipschütz é causada por uma vasculite local e caracteriza-se pelo surgimento súbito de úlceras na vulva ou vagina inferior. Já a doença de Behçet é causada por vasculite sistêmica, com episódios de remissão e exacerbação, que pode envolver quase todos os sistemas orgânicos. Em ambos os casos, é essencial o referenciamento para reumatologia. O tratamento objetiva suprimir exacerbações, controlar a dor e prevenir infecção secundária.(AU)

The occurrence of genital ulcers in adolescents and young women have an emotional impact for the patient and their families, due to the frequent association of its etiology with a sexually transmitted disease. However, Lipschütz ulcer and Behçet's syndrome do not have an infectious etiology and should be remembered as a possible differential diagnoses. As the diagnosis of these two pathologies is clinical and can be challenging, a review of literature was carried out. The objective of this review of literature was to compare both diseases. Lipschütz ulcer is caused by local vasculitis and is characterized by the sudden appearance of ulcers in the vulva or lower vagina. Behçet's syndrome is caused by systemic vasculitis, with episodes of remission and exacerbation, which can affect almost all organ systems. In both cases, referral to rheumatology is essential. Treatment aims to suppress exacerbations, control pain and prevent secondary infection.(AU)

Vasculitis with a neuromuscular presentation and associated cancer revealed by autopsy

Peripheral neuropathy is common, but rarely due to vasculitis. This report is the case of a 74-year-old woman with systemic vasculitis who presented with progressive arm and leg weakness associated with numbness. Autopsy revealed a colon cancer, which may have triggered the vasculitis. This case illustrates the association between vasculitis and malignancy. The best treatment of vasculitis in patients with cancer-associated vasculitis is usually treatment of the cancer, which often yields remission of the vasculitis. This case also illustrates the difficulty of suspecting vasculitis since the symptoms and signs are nonspecific and protean. It is important not to miss a diagnosis of vasculitis. It is often life-threatening. It is treatable. The critical step in the diagnosis of vasculitis is to think of it.

Psicosis y alteraciones en la sensopercepción como manifestaciones atípicas de la poliangitis microscópica / Psychosis and alterations in sensory perception as atypical manifestations of microscopic polyangiitis

RESUMEN La poliangitis microscópica es una vasculitis sistémica, asociada a la positividad de anticuerpos anticitoplasma de neutrófilos, caracterizada por el compromiso necrosante de los vasos de pequeño calibre. Las manifestaciones clínicas de la poliangitis microscópica son variadas y las formas más severas se manifiestan con glomerulonefritis rápidamente progresiva y capilaritis pulmonar. El compromiso nervioso afecta principalmente el sistema periférico. La afectación a nivel central no es común, y hallazgos clínicos de psicosis y alteración del comportamiento son bastante infrecuentes en el contexto de estas enfermedades autoinmunes.

A B S T R A C T Microscopic polyangiitis is a systemic anti-neutrophil cytoplasmic antibody-associated vasculitis, and is associated with the necrotising small calibre vessels. Its clinical manifestations are varied, and the most severe forms manifest with rapidly progressive glomerulonephritis and pulmonary capillaritis. In the nervous system, it mainly involves the peripheral system. Involvement of the central nervous system is not common, and clinical findings of psychosis and behaviour alterations are infrequent.

Compromiso vesical en paciente pediátrico con poliarteritis nodosa: primera descripción / Bladder involvement in a paediatric patient with polyarteritis nodosa: First description

RESUMEN La poliarteritis nudosa (PAN) es una vasculitis necrosante, rara en la infancia, caracterizada por el compromiso de vasos pequeños/medianos y de múltiples órganos. Presentamos a una paciente que inició a los 4 arios con síndrome febril prolongado, dolor abdominal crónico, mialgias incapacitantes y compromiso en la piel, quien luego de 2 años de cuadro clínico completa criterios clínicos para PAN. Recibió tratamiento con corticoide sistêmico por vía oral e intravenosa, 6 meses de ciclofosfamida por vía intravenosa y manejo de mantenimiento con inmunosupresores convencionales sin respuesta adecuada, logrando control de la enfermedad únicamente con ciclofosfamida por vía oral y corticoide a largo plazo. Luego de 5 años y de recibir una dosis alta acumulada de ciclofosfamida, inicia con cuadros de hematuria macroscópica. Se evaluaron, entre otras causas, la toxicidad por ciclofosfamida y la actividad de la enfermedad. El estudio incluyó biopsia vesical, con hallazgo de vas-culitis necrosante de paredes vesicales. La vasculitis vesical es raramente reportada en la literatura (3-5 casos en adultos) y en lo consultado no hay reportes en niños. Se describe, en nuestro conocimiento, el primer caso de compromiso vesical asociado a vasculitis sistêmica reportado en la edad pediátrica.

A B S T R A C T Polyarteritis nodosa (PAN) is a necrotising vasculitis, rare in childhood, and characterized by the inflammation of small and medium vessels and multiple organ involvement. The case is presented of a 4 year old girl with prolonged febrile syndrome, chronic abdominal pain, disabling myalgia, and skin involvement. After 2years of symptoms, she met clinical criteria for PAN. She received treatment with oral and intravenous systemic corticosteroids, 6 months of intravenous cyclophosphamide and maintenance with conventional immuno-suppressants without an adequate response. However, she showed clinical improvement with oral cyclophosphamide and long-term corticosteroids. She had several relapses during follow-up visits due to irregular treatment requiring a high cumulative dose of cyclophosphamide. Five years later she presented with macroscopic haematuria, and was assessed for, among other causes, cyclophosphamide toxicity and disease activity. The workup included cystoscopy and bladder biopsy with findingof necrotising vasculitis of bladder wall. Bladder vasculitis is rarely reported in the literature (3-5 cases in adults) and in that consulted there are no reports in children. To our knowledge, this is the first case of bladder involvement associated with systemic vasculitis reported in the paediatric age.

Extraocular myositis as an initial manifestation of polyangiitis granulomatosis / Miositis extraocular como manifestación inicial de granulomatosis con poliangeítis

ABSTRACT Granulomatosis with polyangiitis (GPA) is a systemic vasculitis with multiorgan involvement. Although ocular involvement has frequently been described, there are few reported cases of extraocular myositis in this disease. The case is presented of a Mexican woman with GPA who debuted with extraocular myositis.

RESUMEN La granulomatosis con poliangeítis (GPA) es una vasculitis sistémica con afectación multiorgánica. El compromiso ocular ha sido descrito frecuentemente, sin embargo, existen pocos casos reportados de miositis extraocular en esta enfermedad. Presentamos el caso de una mujer mexicana con GPA que comenzó con miositis extraocular.

Granulomatosis de Wegener mimetizando una lesión tumoral de vértice de pulmón. Reporte de un caso clínico y revisión de la literatura / Wegener's granulomatosis mimicking a tumor of the lung vertex. Report of a clinical case and review of the literature

La granulomatosis con poliangeítis antes denominada con el epónimo de granulomatosis de Wegener, clasifica entre las vasculitis sistémicas, de causa desconocida, que afecta a los vasos de pequeño y mediano calibre. Se caracteriza por la afectación del aparato respiratorio en su tracto superior, pulmones, así como a los riñones, aunque puede afectar a otros órganos y sistemas del organismo humano. Los anticuerpos anti citoplasma de neutrófilos son positivos con una frecuencia que alcanza el 80 por ciento de casos, y la característica histológica más relevante es la presencia de lesiones granulomatosas necrosantes. El diagnóstico se basa en las manifestaciones clínicas, la biopsia con estudio histológico de los tejidos y órganos afectados así como la presencia de anticuerpos anti citoplasma de neutrófilos. Hoy en día se agrupa dentro de las denominadas vasculitis asociadas a anticuerpos anti citoplasma de neutrófilos positivas. Nuestro estudio tuvo como objetivo presentar un caso clínico inusual, en el cual reportamos a una paciente de 55 años de edad la cual fue atendida por presentar manifestaciones clínicas, de laboratorio y radiológicas que permiten confirmar el diagnóstico de una Granulomatosis de Wegener. Su forma clínica de expresión inicial mostró. una serie de signos y síntomas constitucionales como fiebre superior de 38 grados de varios días de evolución en horario vespertino, astenia, pérdida de apetito, artromialgias, manifestaciones respiratorias como dolor torácico e intercostal izquierdo, tos con expectoración mucosa, y disnea a esfuerzos físicos en aumento. Al examen físico se recogieron datos positivos como palidez cutánea mucosa, la presencia de disminución del murmullo vesicular a nivel del vértice del pulmón izquierdo con estertores crepitantes y una imagen a modo de masa tumoral redondeada del lóbulo superior del pulmón que orientaba al diagnóstico de una neoplasia de vértice de pulmón. Se realizaron múltiples estudios considerando el proteiforme cuadro clínico, los estudios inmunológicos realizados, y la histología por biopsia pulmonar, los que demostraron la existencia de lesiones de tipo granulomatosas compatibles con G. Wegener. Resultó efectiva la respuesta terapéutica con drogas esteroideas e inmunosupresoras en forma de bolos de ciclofosfamida acompañados de un fuerte tratamiento antibiótico de amplio espectro. Concluimos que se trata de un raro caso de granulomatosis de Wegener cuya forma de debut hizo plantear el diagnóstico inicial de una lesión tumoral de vértice de pulmón. No conocemos de otro caso similar reportado en nuestro medio(AU)

Granulomatosis with polyangiitis, formerly known as Wegener's Granulomatosis, is classified as systemic vasculitis, of unknown cause, affecting small and medium-sized vessels. It is characterized by the involvement of the respiratory system in its upper tract, lungs, as well as the kidneys, although it can affect other organs and systems. Neutrophil anticithoplasma antibodies are positive with a frequency that reaches over 80 percent of cases, and the most relevant histological feature is the presence of necrotizing granulomatous lesions. The diagnosis is based on clinical manifestations, biopsy with histological study of the affected tissues and organs as well as the presence of Neutrophil anticithoplasma antibodies. Nowadays it is grouped into the so-called Neutrophil anticithoplasma antibodies positive vasculitis. Arthromyalgia, respiratory manifestations such as thoracic and left intercostal pain, cough with mucous expectoration, and dyspnea on physical efforts in increase. The physical examination collected positive data such as mucous skin pallor, the presence of decreased vesicular murmur at the level of the left vertex of the lung with crackling rales and an image as a rounded tumor mass of the upper lobe of the lung that guided the diagnosis of a vertex neoplasm of lung. Multiple studies were carried out considering the proteiform clinical picture, the immunological studies performed, and the lung biopsy histology, which demonstrated the existence of granulomatous lesions compatible with G. Wegener. The therapeutic response was effective with steroidal and immunosuppressive drugs in the form of cyclophosphamide boluses accompanied by a strong broad-spectrum antibiotic treatment. We conclude that this is a rare case of Wegener's granulomatosis, whose debut form raised the initial diagnosis of a tumor of the lung vertex. We do not know of another similar case reported in our country(AU)

Arteritis de Takayasu / Takayasu arteritis

Introducción: La arteritis de Takayasu es una vasculitis sistémica considerada como una enfermedad rara. Su mecanismo etiopatogénico se basa en un proceso inflamatorio que afecta la arteria aorta y sus grandes ramas. La ausencia de pulso, la angiodinia, los trastornos hipertensivos y la presencia de nódulos en miembros inferiores se describen como sus principales manifestaciones clínicas. Objetivo: Dar a conocer las principales manifestaciones clínicas que permiten la sospecha diagnóstica de la arteritis de Takayasu en la atención primaria de salud. Caso clínico: Se presenta el caso de una paciente de 36 años de edad, la cual acude con manifestaciones clínicas que hace que se sospeche y finalmente se realice el diagnóstico de arteritis de Takayasu. Conclusiones: La sospecha clínica de la enfermedad se basa en una adecuada historia clínica, epidemiológica y hallazgos de laboratorio, los cuales son perfectamente detectables en la atención primaria de salud(AU)

Introduction: Takayasu arteritis is a systemic vasculitis considered a rare disease. Its etiopathogenic mechanism is based on an inflammatory process that affects the aorta and its large branches. The absence of pulse, the angiodynia, the hypertensive disorders and the presence of nodules in the lower limbs are described as their main clinical manifestations. Objective: To present the main clinical manifestations that allows the diagnostic suspicion of Takayasu arteritis in primary health care. Clinical case: 36-year-old female patient, which presents clinical manifestations that allow the diagnosis of Takayasu arteritis. Conclusions: The clinical suspicion of the disease is based on adequate clinical, epidemiological and laboratory findings, which are perfectly detectable in primary health care(AU)

Papel del estomatólogo en el abordaje terapéutico de la aftosis en la enfermedad de Behçet / Role of the stomatologist in the therapeutic approach of aphtosis in Behçet's disease

Introducción: Las aftas y ulceraciones bucales constituyen un motivo frecuente de consulta al estomatólogo. El afta es una forma particular de ulceración bucal. La aftosis se define por el carácter recidivante de aftas múltiples que evolucionan por episodios de 3-10 días y se repiten por lo menos dos veces al año. Objetivo: Abordar los principales aspectos de la Enfermedad de Behçet que podrían ser útiles al estomatólogo. Desarrollo: La enfermedad de Behçet es una vasculitis sistémica inmunomediada caracterizada por una triada característica de lesiones oculares, úlceras orales y genitales recurrentes. La aftosis bucal y la enfermedad de Behçet parecen deberse al mismo mecanismo patogénico, pero aún falta por encontrar las razones por las cuáles la primera se limita a la cavidad bucal y la segunda es sistémica. Debido a que las úlceras orales recurrentes se encuentran en de igual forma en esta enfermedad y la EB, debe considerarse como diagnóstico diferencial de EB. Conclusiones: El papel de los estomatólogos es importante para establecer el diagnóstico de Enfermedad de Behçet, ya que podrían ser los primeros en detectarla. Las úlceras orales recurrentes son un síntoma inicial común de EB. Se requiere un enfoque multidisciplinario para diagnosticar y tratar la enfermedad(AU)

Introduction: Aphthae and oral ulcers are a frequent reason for consulting the stomatologist. Aphthous are a particular form of oral ulceration. Aphtosis is defined by the recurrent nature of multiple aphthae that progress through episodes of 3-10 days and are repeated at least twice a year. Objective: To describe the main aspects of the Behçet disease that could be useful to the stomatologist. Development: Behçet's disease is an immune-mediated systemic vasculitis characterized by a characteristic triad of ocular lesions, recurrent oral and genital ulcers. Oral aphtosis and Behçet's disease seem to be due to the same pathogenic mechanism, but we still have to find the reasons why the former is limited to the oral cavity and the latter is systemic. Because recurrent oral ulcers are found in the same way in this disease and EB, it should be considered as a differential diagnosis of EB. Conclusions: The role of stomatologists is important to establish the diagnosis of Behçet's disease, since they could be the first to detect it. Recurrent oral ulcers are a common initial symptom of EB. A multidisciplinary approach is required to diagnose and treat the disease(AU)

Afectación cardiovascular en una paciente con arteritis de Takayasu, a propósito de un caso / Cardiovascular involvement in a patient with Takayasu arteritis, in relation to a case

Introducción: la arteritis de Takayasu es una vasculitis sistémica que provoca alteraciones en distintos sistemas de órganos, las más características y peligrosas ocurren en el sistema cardiovascular. Objetivo: socializar las manifestaciones cardiovasculares que con mayor frecuencia se presentan en el curso de la arteritis de Takayasu. Caso clínico: se presenta el caso de una paciente femenina, de 46 años de edad, con manifestaciones clínicas que permiten llegar al diagnóstico de arteritis de Takayasu; dentro del cuadro clínico se presentan múltiples afectaciones cardiovasculares que ensombrecen el pronóstico de la paciente. Conclusiones: las manifestaciones cardiovasculares en la arteritis de Takayasu no solo forman parte de los criterios diagnósticos y de las manifestaciones clínicas de la enfermedad; sino que también forman parte de las complicaciones de la arteritis y su presencia empeora la evolución clínica de la enfermedad y complica el pronóstico del paciente(AU)

Introduction: Takayasu's arteritis is a systemic vasculitis that causes alterations in different organ systems, the most characteristic and dangerous occur in the cardiovascular system. Objective: to socialize the cardiovascular manifestations that most frequently occur in the course of Takayasu's arteritis. Clinical case: the case of a female patient, 46 years of age, with clinical manifestations that lead to the diagnosis of Takayasu arteritis; Within the clinical picture there are multiple cardiovascular affectations that overshadow the prognosis of the patient. Conclusions: the cardiovascular manifestations in Takayasu's arteritis are not only part of the diagnostic criteria and the clinical manifestations of the disease; they are also part of the complications of arteritis and their presence worsens the clinical evolution of the disease and complicates the patient's prognosis(AU)

Tratamento endovascular da doença de Behçet: relato de caso / Endovascular treatment for Behçet's disease: a case report

A doença de Behçet (DB) é uma vasculite multissistêmica de etiologia desconhecida. O acometimento cardiovascular é bem descrito na literatura, sendo que a principal causa de morte na DB é secundária a complicações aneurismáticas. Relata-se aqui o caso de um paciente portador de DB com aneurisma de aorta abdominal reincidente, o qual foi corrigido por meio de endoprótese customizada. O tratamento ideal para portadores de DB ainda é bastante controverso e desafiador, tendo em vista as dificuldades técnicas e as recidivas frequentes. A intervenção endovascular apresenta-se como uma alternativa viável e consideravelmente menos mórbida do que a cirurgia convencional

Behçet's disease (BD) is a multisystemic vasculitis of unknown etiology. Cardiovascular involvement has been thoroughly described in the literature and the major cause of death in BD is secondary to aneurysm complications. In this case report, a patient with BD presented with a recurrent abdominal aortic aneurysm, which was corrected using a custom-made endoprosthesis. The optimal treatment for patients with BD remains highly controversial and challenging because of technical difficulties and frequent recurrence. Endovascular intervention seems to be a feasible alternative with considerably less morbidity than conventional surgery

Algorithm for diagnosis of primary vasculitides / Algoritmo diagnóstico de vasculites primárias

Primary vasculitides are diseases with a wide variety of anatomical, clinical, radiological, and laboratory presentations. Primary vasculitides are difficult to diagnose because of the complexity of clinical presentation, which may lead to delayed treatment and increased financial costs of workup investigations involving non-essential tests. Our objective in the present study is to create an algorithm that helps diagnosis of Primary vasculitides. The algorithm presented in this article allows fast, simple and cost-effective diagnosis of primary vasculitides using just clinical concepts and a few laboratory tests

As vasculites primárias são doenças que possuem apresentações anatômicas, clínicas, radiológicas e laboratoriais muito distintas. Em virtude da complexidade dos quadros clínicos apresentados, as vasculites primárias são de difícil diagnóstico, o que pode promover um retardo no início da terapêutica, além de aumentar os custos financeiros da investigação propedêutica com exames não essenciais. O objetivo deste estudo foi criar um algoritmo que auxilie o diagnóstico das vasculites primárias. O algoritmo apresentado neste artigo permite a realização de diagnóstico rápido, simples e de baixo custo nas vasculites primárias, com a utilização de alguns conceitos clínicos e poucos exames laboratoriais

Classification of Antineutrophil Cytoplasmic Antibody-associated Vasculitis

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of systemic vasculitides, that are characterized by inflammation in the small vessels, ranging from capillaries to arterioles or venules. AAV is divided into three variants based on the clinical manifestations and histological findings such as microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA). MPA often induces rapid progressive necrotising glomerulonephritis, and occasionally induces diffuse alveolar hemorrhage. In contrast, GPA preferentially affects the respiratory tracts from the bronchus to the nasal cavity. GPA can also involve the kidneys, but the frequency of renal involvement is less than MPA. EGPA is based on allergic components such as asthma, peripheral eosinophilia, migratory eosinophilic pneumonia and eosinophil infiltration. Since 1982, when the association between ANCA and systemic vasculitis was first reported, several classification criteria for AAV have been proposed. This review describes the classification criteria for and nomenclature of AAV from the 1990 American College of Rheumatology (ACR) classification criteria to the 2012 revised Chapel Hill consensus conference (CHCC) nomenclature of Vasculitides. New classification trials for AAV such as AAV based on the ANCA-types (myeloperoxidase-ANCA vasculitis, proteinase 3-ANCA vasculitis and ANCA negative vasculitis) and the ACR/European League Against Rheumatism (EULAR) 2017 provisional classification criteria for GPA were also introduced. In addition, the histopathological classification of ANCA-associated glomerulonephritis and the revised 2017 international consensus on testing of ANCAs in GPA and MPA are also discussed.

A Case of Henoch-Schönlein Purpura with Fulminant Complications and Its Long-term Outcome

Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan (99mTc RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.

A case of gastroduodenal ulcer complicating Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis associated with various clinical manifestations and complications, such as gastrointestinal abnormalities. We report a 3-year-old boy who presented with hematemesis and diffuse gastroduodenal ulcerations complicating KD. He received standard medical therapy for the disease and gastric ulcer, which showed effect after a few days. Although rare, peptic ulcers should be considered a complication of KD to ensure early diagnosis and treatment as it may cause severe morbidity.

The association between asthma and Kawasaki disease

Kawasaki disease (KD) is a systemic vasculitis and the most common cause of acquired heart disease among preschool-aged children. Asthma is one of the most common chronic illness of childhood. There is increasing interest in the potential link between inflammatory diseases and allergic diseases, and there is increasing epidemiologic evidence for an association of KD and allergic disease. In regards to asthma, 9 studies have been reported about its association with KD; 5 of them showed positive associations between these 2 diseases, while 4 of them showed no association. There are some possible underlying mechanisms for this association. The serum IgE levels are found to be increased and there is a Th2-predominant immune response in KD. These are common findings of asthma. Human leukocyte antigen-G/regT cells and SMAD3/transforming growth factor-beta might also be involved in the pathogenesis of both diseases. Asthma may increase susceptibility to KD and tends to lead to immune dysregulation. Clinician's recognition of the increased risks of KD and other asthma-related comorbidities among patients with asthma may enable early identification and intervention. The early identification and management of comorbidities may ultimately reduce the economic and social burden of these chronic conditions.

A pediatric case of eosinophilic granulomatosis with polyangiitis accompanied by heart failure mimicking an asthma attack

Eosinophilic granulomatosis with polyangiitis (EGPA, also known as the Churg-Strauss syndrome) is a disorder characterized by asthma, peripheral eosinophilia and systemic vasculitis. It rarely occurs in children, so that physicians may frequently mistake it for a simple uncontrolled asthma. Since a subsequent cardiac involvement is critical for the prognosis, it is important to suspect EGPA in children with severe, uncontrolled asthma. The cardiac manifestations in EGPA are variable from asymptomatic electrocardiogram abnormalities to pericarditis with pericardial effusion, myocarditis with cardiomyopathy, heart failure, and sudden cardiac death. Although delayed treatment may lead to fatal cardiac complications in EGPA, adequate immune suppression can reverse cardiac impairment. We report a 14-year-old girl with persistent asthma refractory to steroids who was eventually diagnosed with an anti-neutrophil cytoplasmic antibody-negative EGPA.

Understanding the importance of cerebrovascular involvement in Kawasaki disease / 소아과

Kawasaki disease (KD) is a systemic vasculitis in infants and young children. However, its natural history has not been fully elucidated because the first case was reported in the late 1960s and patients who have recovered are just now entering middle age. Nevertheless, much evidence has raised concerns regarding the subclinical vascular changes that occur in post-KD patients. KD research has focused on coronary artery aneurysms because they are directly associated with fatality. However, aneurysms have been reported in other extracardiac muscular arteries and their fate seems to resemble that of coronary artery aneurysms. Arterial strokes in KD cases are rarely reported. Asymptomatic ischemic lesions were observed in a prospective study of brain vascular lesions in KD patients with coronary artery aneurysms. The findings of a study of single-photon emission computed tomography suggested that asymptomatic cerebral vasculitis is more common than we believed. Some authors assumed that the need to consider the possibility of brain vascular lesions in severe cases of KD regardless of presence or absence of neurological symptoms. These findings suggest that KD is related with cerebrovascular lesions in children and young adults. Considering the fatal consequences of cerebral vascular involvement in KD patients, increased attention is required. Here we review our understanding of brain vascular involvement in KD.