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1.
Rev. cuba. med ; 60(3): e1679, 2021.
Article in Spanish | LILACS, CUMED | ID: biblio-1347521

ABSTRACT

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)


Subject(s)
Humans , Antibodies, Antineutrophil Cytoplasmic , Clinical Laboratory Techniques/methods , Systemic Vasculitis , Fluorescence
2.
Femina ; 49(3): 187-192, 20210331. ilus
Article in Portuguese | LILACS | ID: biblio-1224085

ABSTRACT

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)


Subject(s)
Humans , Female , Adolescent , Adult , Behcet Syndrome/complications , Behcet Syndrome/diagnosis , Behcet Syndrome/drug therapy , Oral Ulcer , Systemic Vasculitis/complications , Systemic Vasculitis/diagnosis , Systemic Vasculitis/drug therapy , Prognosis , Uveitis , Vulvar Diseases , Epstein-Barr Virus Infections
3.
Autops. Case Rep ; 10(2): e2020175, Apr.-June 2020. graf
Article in English | LILACS | ID: biblio-1131805

ABSTRACT

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.


Subject(s)
Humans , Female , Aged , Colonic Neoplasms , Paraneoplastic Polyneuropathy , Systemic Vasculitis , Signs and Symptoms , Autopsy , Fatal Outcome , Diagnosis, Differential , Muscular Diseases
4.
Rev. colomb. reumatol ; 27(2): 135-140, ene.-jun. 2020. tab, graf
Article in Spanish | LILACS | ID: biblio-1251648

ABSTRACT

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.


Subject(s)
Humans , Female , Adult , Psychotic Disorders , Microscopic Polyangiitis , Systemic Vasculitis
5.
Rev. colomb. reumatol ; 27(2): 141-146, ene.-jun. 2020. graf
Article in Spanish | LILACS | ID: biblio-1251649

ABSTRACT

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.


Subject(s)
Humans , Child , Pediatrics , Polyarteritis Nodosa , Therapeutics , Bread , Systemic Vasculitis
6.
Rev. cuba. reumatol ; 21(3): e100, sept.-dic. 2019. graf
Article in Spanish | LILACS, CUMED | ID: biblio-1093842

ABSTRACT

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)


Subject(s)
Humans , Female , Middle Aged , Pharmaceutical Preparations , Granulomatosis with Polyangiitis/diagnosis , Systemic Vasculitis/complications , Respiratory Sounds , Granulomatosis with Polyangiitis/diagnostic imaging
7.
Rev. colomb. reumatol ; 26(4): 268-270, oct.-dic. 2019. graf
Article in English | LILACS | ID: biblio-1138819

ABSTRACT

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.


Subject(s)
Humans , Female , Middle Aged , Granulomatosis with Polyangiitis , Systemic Vasculitis , Myositis , Inflammation
8.
Rev. cuba. reumatol ; 21(1): e44, ene.-abr. 2019. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1093802

ABSTRACT

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)


Subject(s)
Humans , Stomatitis, Aphthous , Eye Injuries , Behcet Syndrome , Oral Ulcer , Diagnosis, Differential , Systemic Vasculitis , Mouth
9.
Article in Korean | WPRIM | ID: wpr-762205

ABSTRACT

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.


Subject(s)
Asthma , Child , Chronic Disease , Comorbidity , Heart Diseases , Humans , Hypersensitivity , Immunoglobulin E , Leukocytes , Mucocutaneous Lymph Node Syndrome , Systemic Vasculitis
10.
Article in Korean | WPRIM | ID: wpr-762199

ABSTRACT

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.


Subject(s)
Adolescent , Asthma , Cardiomyopathies , Child , Churg-Strauss Syndrome , Cytoplasm , Death, Sudden, Cardiac , Electrocardiography , Eosinophilia , Eosinophils , Female , Granulomatosis with Polyangiitis , Heart Failure , Heart , Humans , Myocarditis , Pericardial Effusion , Pericarditis , Prognosis , Steroids , Systemic Vasculitis
11.
Article in Korean | WPRIM | ID: wpr-760852

ABSTRACT

PURPOSE: Kawasaki disease (KD) is a common, acute systemic vasculitis in children. Acute phase reactants (APRs) have been used to assist diagnosis, and to predict outcome in children with KD. However, it remains unknown on levels of APRs depending on duration of fever. We aimed to compare APR levels of children with KD who visited with < 5 days duration of fever and with ≥ 5 days. METHODS: Children (≤ 15 years) with complete KD who visited the emergency department were enrolled from March 2012 through February 2018. The children were divided into the early (fever < 5 days) and late (fever ≥ 5 days) presenters. The baseline characteristics, APR levels, such as platelet count, and outcomes were compared between the 2 groups. RESULTS: A total of 145 children with complete KD were enrolled. Median age was 27.0 (interquartile range [IQR], 12.0–46.5) months, and boys accounted for 60.0%. The early presenters (63 [43.4%]) had a younger age (17.0 [IQR, 7.0–45.0] vs. 32.5 [IQR, 14.0–48.0] months; P = 0.006), shorter duration of fever (3.0 [IQR, 2.0–4.0] vs. 6.0 [IQR, 5.0–7.0] days; P < 0.001), and a lower platelet count (336.7 ± 105.2 [× 10³/µL] vs. 381.6 ± 121.8 [× 10³/µL], P = 0.02) than the late presenters. The other APR levels, and frequency of resistance to intravenous immunoglobulin and coronary artery abnormalities showed no differences between the 2 groups. CONCLUSION: Children with KD who visited with < 5 days duration of fever had a lower platelet count compared to those with ≥ 5 days. No differences were found in the other APR levels and the outcomes. It may be necessary to consider the differences in APR levels depending on duration of fever when treating children with KD.


Subject(s)
Acute-Phase Proteins , Blood Platelets , C-Reactive Protein , Child , Coronary Vessels , Diagnosis , Emergency Service, Hospital , Fever , Humans , Immunoglobulins , Leukocyte Count , Mucocutaneous Lymph Node Syndrome , Platelet Count , Systemic Vasculitis
12.
Article in English | WPRIM | ID: wpr-760238

ABSTRACT

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.


Subject(s)
Aneurysm , Arteries , Brain , Central Nervous System , Child , Coronary Vessels , Humans , Infant , Middle Aged , Mucocutaneous Lymph Node Syndrome , Natural History , Prospective Studies , Stroke , Systemic Vasculitis , Tomography, Emission-Computed , Vasculitis , Vasculitis, Central Nervous System , Young Adult
13.
Article in English | WPRIM | ID: wpr-760197

ABSTRACT

Kawasaki disease (KD) is a systemic vasculitis that mainly affects younger children. Intravenous immunoglobulin (IVIG) resistant cases are at increasing risk for coronary artery complications. The strategy on prediction of potential nonresponders and treatment of IVIG-resistant patients is now controversial. In this review the definition and predictors of IVIG-resistant KD and current evidence to guide management are discussed.


Subject(s)
Child , Coronary Vessels , Humans , Immunoglobulins , Mucocutaneous Lymph Node Syndrome , Systemic Vasculitis
14.
Article in English | WPRIM | ID: wpr-766186

ABSTRACT

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.


Subject(s)
Antibodies, Antineutrophil Cytoplasmic , Arterioles , Asthma , Bronchi , Capillaries , Classification , Consensus , Cytoplasm , Eosinophilia , Eosinophils , Glomerulonephritis , Granulomatosis with Polyangiitis , Hemorrhage , Inflammation , Kidney , Microscopic Polyangiitis , Nasal Cavity , Pulmonary Eosinophilia , Respiratory System , Rheumatic Diseases , Rheumatology , Systemic Vasculitis , Vasculitis , Venules
15.
Article in English | WPRIM | ID: wpr-786518

ABSTRACT

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.


Subject(s)
Child , Child, Preschool , Early Diagnosis , Endoscopy , Hematemesis , Humans , Male , Mucocutaneous Lymph Node Syndrome , Peptic Ulcer , Stomach Ulcer , Systemic Vasculitis
16.
Childhood Kidney Diseases ; : 128-133, 2019.
Article in English | WPRIM | ID: wpr-785571

ABSTRACT

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.


Subject(s)
Abdominal Pain , Adult , Arthritis , Biopsy , Cyclosporine , Diagnosis , Emergencies , Erythrocytes , Exanthema , Follow-Up Studies , Hemorrhage , Humans , Intestinal Perforation , Intestines , Intussusception , Kidney , Laparotomy , Lower Extremity , Nephritis , Purpura , Skin , Systemic Vasculitis
17.
J. vasc. bras ; 18: e20180121, 2019. ilus
Article in Portuguese | LILACS | ID: biblio-1012621

ABSTRACT

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


Subject(s)
Humans , Male , Adult , Behcet Syndrome/diagnosis , Behcet Syndrome/therapy , Endovascular Procedures/methods , Prostheses and Implants , Angiography/methods , Stents , Chronic Disease , Systemic Vasculitis , Aneurysm
18.
Rev. cuba. reumatol ; 21(supl.1): e72, 2019. tab
Article in Spanish | LILACS, CUMED | ID: biblio-1099118

ABSTRACT

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)


Subject(s)
Humans , Female , Middle Aged , Cardiovascular Diseases/complications , Clinical Evolution , Takayasu Arteritis/complications , Systemic Vasculitis/complications
19.
J. vasc. bras ; 18: e20180092, 2019. tab, graf
Article in English | LILACS | ID: biblio-990124

ABSTRACT

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


Subject(s)
Humans , Male , Female , Algorithms , Diagnosis, Differential , Systemic Vasculitis/diagnosis , Vasculitis/diagnosis , Blood Vessels , Classification , Antibodies, Antineutrophil Cytoplasmic , Inflammation/complications
20.
Rev. argent. reumatol ; 29(3): 39-45, set. 2018.
Article in Spanish | LILACS | ID: biblio-980207

ABSTRACT

Las vasculitis sistémicas son un grupo heterogéneo de enfermedades poco frecuentes que cursan con inflamación de los vasos sanguíneos y que pueden afectar potencialmente cualquier órgano y sistema del cuerpo. Generan deterioro u obstrucción al flujo sanguíneo y daño a la integridad de la pared vascular


Subject(s)
Systemic Vasculitis , Lung Diseases
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