Atypical Wegener's Granulomatosis Accompanied with Rheumatoid Arthritis: A Case Report

Wegener's granulomatosis (WG) is highly correlated with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA). Patients with rheumatoid arthritis (RA) rarely contract WG. Although several concurrent cases have been reported overseas, there are no known cases in Korea. Here we report a unique case of RA with atypical WG testing positive for perinuclear ANCA (p-ANCA) and negative for anti-myeloperoxidase (MPO) antibodies. The 62-yr-old female patient presented with multiple joint pain and showed typical blood test results for RA, i.e., an elevated erythrocyte sedimentation rate and C-reactive protein concentration, and positive for rheumatoid factor and anti-cyclic citrullinated peptide antibodies. RA was clear based on a total score of 10 when applying the classification criteria developed by the American College of Rheumatology/European League Against Rheumatism (2010). In an autoimmune target test, speckled and skeleton patterns were observed. In an ANCA test, p-ANCA was observed (titer, 1:2,560), and tests for anti-proteinase 3 (PR3) and anti-MPO antibodies were negative. After admission, multiple nodules were detected on a chest X-ray and a computed tomography scan. We suspected that she had rheumatic nodules or vasculitis and performed an open lung biopsy. We detected necrotic granulomatous vasculitis, classified as WG, thus leading to WG diagnosis. In conclusion, WG was diagnosed in an RA patient who was negative for c-ANCA (negative PR3) and positive for p-ANCA (negative anti-MPO), and this peculiar finding is likely to improve diagnosis in cases of RA with atypical WG.

Mujer con artritis reumatoidea, nódulos pulmonares cavitarios y úlcera de piel / A woman with rheumatoid arthritis,cavitary pulmonary nodules and skin ulcer

Rheumatoid arthritis (RA) more often affect women, but extra-articular manifestations ­ cardiac, cutaneous, gastrointestinal, nervous, ocular, pulmonary and renal, are predominant in men [1­7]. These changes are found in approximately 40-50% of active chronic disease [1, 5, 6, 8]. Skin nodules occur in up to 20% of cases, mainly if rheumatoid factor is positive [1-3, 5], and are related to small vessel vasculitis, similarly to digital changes and ulcers of extremities. Pulmonary nodules are associated with positive rheumatoid factor and anti-cyclic citrulinated peptide antibody; and can evolve with necrosis and central cavitations [1-3, 5-7]. The woman herein reported had longstanding seropositive RA with cavitary pulmonary nodules and indolent cutaneous ulcer in the left elbow

La artritis reumatoide (AR) afecta con mayor frecuencia a las mujeres, pero es extraarticular. manifestaciones cardíacas, cutáneas, gastrointestinales, nerviosas, oculares, Pulmonar y renal, son predominantes en los hombres [1­7]. Estos cambios se encuentran en aproximadamente el 40-50% de las enfermedades crónicas activas [1, 5, 6, 8]. Los nódulos cutáneos se producen en hasta el 20% de los casos, principalmente si el factor reumatoide es positivo [1-3, 5], y están relacionados con vasculitis de pequeños vasos, de forma similar a la digital Cambios y úlceras de extremidades. Los nódulos pulmonares están asociados. con factor reumatoide positivo y péptido citrulinado anticíclico anticuerpo; y puede evolucionar con necrosis y cavitaciones centrales [1-3, 5-7]. La mujer aquí reportada tenía una AR seropositiva de larga data con Nódulos pulmonares cavitarios y úlcera cutánea indolente en el codo izquierdo.

Febre reumática: um modelo animal para uma vacina humana / Rheumatic fever: an animal model for a human disease

Avaliamos o papel da proteína M na indução de lesões similares às observadas na febre reumática (FR), utilizando o rato Lewis como modelo experimental. Identificamos a cepa M1 do S. pyogenes como a mais freqüentemente isolada em amostras locais. Produzimos a proteína M1 recombinante e, mostramos que a imunização usando esta proteína produziu lesões similares às lesões patognomônicas da FR em 50 por cento dos animais. Demonstramos resposta humoral e celular específica contra a proteína M1 e contra as regiões M1AB e M1C. Não observamos, entretanto, reações cruzadas com a miosina cardíaca. Células mononucleares infiltrantes do miocárdio de animais imunizados foram cultivadas, em presença de IL-2, mostrando que estas células foram sensibilizadas. Em conclusão, o modelo proposto ajudará a esclarecer a imunopatologia da FR...

We have evaluated M protein role in eliciting rheumatic fever-like lesions, using the Lewis rat as an animal model. We have identified S. pyogenes M1 strain as the most frequently isolated from local samples. We have produced M1 recombinant protein and shown that immunization using this protein led to rheumatic fever characteristic lesions in 50 per cent of the animals. We demonstrated a humoral and cellular specific response targeting M1 protein and M1AB and M1C regions. We did not observe, however, cross reactions with cardiac myosin. Mononuclear cells infiltrating myocardium of immunized animals were grown in presence of IL-2, showing that these cells were sensitized. In conclusion, the proposed model will help clarifying on the...

Rheumatic fever: epidemiological study from Iraq

Rheumatic fever and rheumatic valvular diseases are considered major health problems in the developing communities. Accordingly, this study was attempted in order to assess the behavior of the various clinical manifestations of acute rheumatic fever [i.e., polyarthritis, carditis, Sydenham's chorea, subcutaneous nodules and erythema marginatum] with regard with age, gender and weather in our region. The study involved 110 patients proved to have acute rheumatic fever according to modified Jones criteria. The patients were studied according to gender, age groups [below and above 15 years], and according to cold and hot weather. Females were affected more frequently than males [68 vs. 42; 1.6:1]. The mean age was 15.9 +/- 4.5 years. Ages ranged from 8-35 years. Polyarthritis dominated clinical presentation; it constitutes 74.55% [P<0.001] in all aspects of the study, followed by Sydenham's chorea [16.36%], and then carditis [9.09%]. Subcutaneous nodules and erythema marginatum were not encountered. Acute rheumatic fever is still a major health problem in our area. Polyarthritis predominates followed by Sydenham's chorea, and then carditis. Age and weather had no effect on the disease parameters studied, but female gender significantly predominated over male gender

Avaliação laríngea em pacientes reumatológicos / Laryngeal assessment in reumatic disease patients

As doenças reumáticas produzem alterações sistêmicas e podem, por isso, comprometer os vasos sangüíneos, as serosas e as mucosas de todo o trato aerodigestivo. Casos esporádicos de acometimento laríngeo por doenças reumáticas têm sido descritos. Esse estudo tem por objetivo avaliar e descrever as alterações laríngeas encontradas em pacientes reumatológicos. FORMA DE ESTUDO: coorte transversal. MATERIAL E MÉTODO: Estudo transversal com pacientes portadores de lúpus eritematoso sistêmico, esclerodermia e doença mista do tecido conjuntivo. Os pacientes submeteram-se a exame clínico otorrinolaringológico e à videolaringoestroboscopia. RESULTADOS: Foram incluídos no estudo 27 pacientes sendo que 26 conseguiram realizar a videolaringoestroboscopia. Alterações laríngeas foram observadas em 11 dos 12 pacientes portadores de lúpus, nos 11 pacientes portadores de esclerodermia e nos 3 pacientes portadores de doença mista do tecido conjuntivo. Lesões sugestivas de nódulo em bambu foram identificados em 5 pacientes e 92,3 por cento dos pacientes apresentaram sinais laríngeos de síndrome faringolaríngea do refluxo. CONCLUSÃO: Neste estudo identificamos 5 lesões sugestivas de nódulos em bambu e sinais laríngeos de refluxo em quase todos os pacientes.

Aspectos anatomopatológicos da febre reumática / Pathological aspects of the heart in rheumatic fever

A febre reumática é uma doença inflamatória, sistêmica e recorrente, que se manifesta cerca de uma a seis semanas após a infecção aguda da orofaringe por estreptococos beta-hemolíticos do grupo A. Presume-se que seja uma resposta imunológica a antígenos estreptocócicos ou uma reação auto-imune induzida pelos estreptococos, em indivíduos geneticamente suscetíveis. Acomete crianças de 5 a 15 anos, mas 20 por cento dos surtos iniciais ocorrem em indivíduos de meia idade ou idosos. No coração há comprometimento dos tecidos conjuntivos pericárdico, miocárdico e endocárdico. A pericardite é exuberante e predominantemente fibrinosa, com aspecto macroscópico de "pão com manteiga". A miocardite causa aumento cardíaco, principalmente por dilatação ventricular. No tecido conjuntivo cardíaco encontram-se nódulos de Aschoff, que retratam histologicamente as fases evolutivas da doença: exsudativa, proliferativa e cicatricial. O nódulo de Aschoff, na fase proliferativa, é patognomônico de atividade reumática, apresentando degeneração fibrinóide do colágeno e acúmulo de células inflamatórias, dentre elas as células de Anitsch-kow. Pequenas vegetações são encontradas nas linhas de fechamento valvar, mais comumente mitro-aórticas. Histologicamente são elevações da superfície valvar, decorrentes da deposição de fibrina e plaquetas ou da degeneração fibrinóide do colágeno local. O dano cardíaco é cumulativo, aumentando a cada episódio de atividade. As alterações reumáticas crônicas mais importantes resultam da cicatrização das lesões agudas valvares e podem ocorrer em todas as valvas. A estenose mitral é a seqüela reumática mais freqüente, seguida pela estenose mitro-aórtica e pela estenose aórtica isolada. Pode haver associação de estenose e insuficiência valvar, que é mais comum nas lesões aórticas.

Fiebre reumática: revisión bibliográfica / Rheumatic fever: bibliographical revision

La fiebre reumática (FR) continúa siendo en las zonas rurales un problema de salud, especialmente en niños. Con frecuencia se consulta en los servicios de emergencia por un cuadro febril agudo con artritis concomitante.Saber reconocer y tratar una fiebre reumática en sus estados iniciales evita las secuelas cardiacas posteriores. Aunque la incidencia y la prevalencia de la FR han disminuido gracias a la profilaxis que se realiza con los antibióticos disponibles, hay factores de riesgo que condicionan su desarrollo.Como médicos familiares tenemos la oportunidad de detectar dichos riesgos y actuar antes de que se presente el problema. Ya que es una enfermedad que deja secuelas incapacitantes que deterioran la calidad de vida de los pacientes.El artículo que a continuación usted leerá, es una revisión bibliográfica donde podrá encontrar lo más importante y reciente acerca de la FR, desde su concepto pasando por una adecuada revisión de la sintomatología y etiología, lo que permitirá al lector si hace un correcto uso de este, poder llegar a diagnóstico precoz y adecuado tratamiento, por consiguiente, a disminuir la tasa de morbimortalidad por FR, sin dejar de tener en cuenta y hacer énfasis en que la profilaxis es la medida más importante y efectiva para reducir la morbimortalidad.

The rheumatic fever (F.R.) continues to be a health problem in children in rural areas. Consulted in the emergency room for having symptoms such as fever and concomitant arthritis.The Objective of this study is to know how to recognize and to treat a rheumatic fever in its initial states so when can avoid the later heart problems. Although the incidence and the prevalencia of the RF has diminished thanks to the prevention that is carried out with the available antibiotics, there are factors of risk that condition its development.As doctors we have the opportunity to detect these risks and to act before the problem is arises. Since it is an illness that will leave sequels that deteriorate the quality of life of the patient. In this article you can find the most important and recent information about F.R. It will explain the etiology, symptoms that the reader will be able to use to make a diagnosis and give the appropriate treatment. This will lead to a decrease of the mortality rate of R.F.

Nódulo y artritis erosiva: reporte de un caso / Nodules and erosive arthritis: a report of a case

Los síndromes histiocíticos son afecciones de difícil diagnóstico, que conciernen a los dermatólogos, a los médicos internistas, a los reumatólogos y a los patólogos. Las decisiones terapéuticas pueden ser de agresividad variable, produciendo una gran morbilidad. La reticulohistiocitosis multicéntrica es un padecimiento infrecuente cuya dificultad diagnóstica, así como los diagnósticos diferenciales, deben ser ampliamente conocidos.

Diagnosis of rheumatic fever: current status of Jones Criteria and role of echocardiography.

The original Jones Criteria as proposed by Dr. T. Duckett Jones have been modified four times and the updated revised criteria were published in 1992. According to this latest publication major manifestations are carditis, polyarthritis, chorea, erythema marginatum and subcutaneous nodules. Minor manifestations include fever, arthralgia and laboratory findings of elevated erythrocyte sedimentation rate, C-reactive protein and prolonged PR interval on ECG. For making a diagnosis of acute rheumatic fever, two major, or one major and two minor manifestations must be accompanied by supporting evidence of antecedent group A streptococcal infection in the form of positive throat culture or elevated or rising anti-streptolysin titre. The updated guidelines also highlighted a subgroup of "exceptions to Jones Criteria" for patients with chorea, indolent carditis and previous history of rheumatic fever or "rheumatic heart disease". Role of echocardiography has not been defined in these modifications but may be important, as clinical detection of soft murmurs may be difficult due to tachycardia. Doppler and color flow mapping is more sensitive in picking up minor digress of valvular regurgitation. Several studies have confirmed that the yield of carditis with valvular regurgitation increased with use of echocardiography in patients with acute rheumatic fever. Also echocardiography is of great help in mixed valve lesions to determine the severity of each lesion. Other abnormalities detected on echocardiography in acute carditis include prolapse of the valve, focal nodular thickening of leaflets and pericardial effusion. Jones Criteria are guidelines to assist the physician and should not be substituted for clinical judgement as strictly following them may result in underdiagnosis of this disease in our country.

Cardiopatia reumática e endocardite infecciosa em paciente com síndrome de imunodeficiência adquirida / Rheumatic Heart Disease and Infective Endocarditis in a Patient with Acquired Immunodeficiency Syndrome

A 36-old-woman was admitted with an infectious syndrome, respiratory insufficiency and vasculitis. There was a history of chronic intravenous drug abuse, sexual promiscuity and rheumatic heart disease. She had HIV positive tests. The vasculitis and heart failure worsened and the patient died of stroke. At autopsy it was found histologic evidence of AIDS, rheumatic heart disease with Aschoff nodes, infective endocarditis with cerebral abscesses and thalamic infarction

Paciente do sexo feminino com 36 anos de idade, vida sexual promíscua e usuária de drogas endovenosas, com passado de cardite reumática, foi internada com síndrome infecciosa, insuficiência respiratória, insuficiência cardíaca congestiva e vasculite. A pesquisa para anticorpo anti-HIV foi positiva em teste realizado. Houve agravamento da vasculite e da insuficiência cardíaca e evolução para o óbito com acidente vascular cerebral. À necropsia constatou-se quadro anatomopatológico de síndrome de imunodeficiência adquirida (SIDA), cardiopatia reumática com nódulos de Aschoff e, ainda, endocardite infecciosa com abcessos cerebrais e infarto hemorrágico no tálamo.

Nódulos de aschoff: un enigma histológico. (Revision bibliográfica: 1ª parte) / Aschoff nodule: a histologic enigma. (Bibliographic reviewing 1ª part)

Desde la descripción original de los cuerpos o nódulos de Aschoff hasta nuestros días, los aspectos histopatogénicos han sido motivo de serias discusiones; uno de los temas álgidos lo constituye precisamente la naturaleza celular de los elementos que forman parte de los citados cuerpos, en particular porque se han atribuido diversos orígenes, ya sea elementos linfocitoides, células epitelioides, miocitos, endotelio vascular, leucocitos endoteliales, histiocitos y elementos mesenquimatosos indiferenciados. La presente revisión bibliográfica procura establecer, en un principio, un perfil histórico de todos aquellos aportes científicos de trascendencia

Histopathology of auricular appendages in rheumatic heart disease.

Forty-four left auricular appendages removed from patients presenting clinically as rheumatic heart disease (RHD) were subjected to histopathologic examination. The aim was to identify the changes so far reported in RHD. It was found that Aschoff's nodules, though specific for RHD, are much less frequent than the other changes reported. Viewing the histopathologic alterations in the perspective of clinical data is important in identifying the rheumatic process.

Nodulosis gotosa: una entidad nodulosa o patológica?

Los nódulos subcutáneos están presentes en múltiples enfermedades sistémicas incluyendo lupus eritematoso sistémico, fiebre reumática, artritis reumatoidea y gota. Llamamos la atención sobre aquellos pacientes con tofos subcutáneos múltiples por depósito de urato monosódico sin historia de artritis gotosa. Presentamos un caso de un hombre con presencia de nódulos subcutáneos indoloros de seis años de evolución en carpos, metacarpofalángicas, interfalángicas proximales, codos y rodillas, sin antecedentes de artritis. Planteamos la existencia de un subgrupo clínico de pacientes con gota, cuyo curso es más benigno con respecto al compromiso articular y se proponen sus criterios clínicos

Granuloma anular subcutâneo: relato de um caso / Subcutaneous granuloma annulare: case report

Um caso de uma paciente do sexo feminino de seis anos de idade com lesäo de granuloma anular subcutâneo na regiäo plantar é relatado. Após dois meses ela desenvolveu uma lesäo típica de granuloma anular na perna. Uma revisäo da literatura também é apresentada

Immunohistochemical characterization of Aschoff nodules and endomyocardial inflammatory infiltrates in resected left atrial appendages.

Presence of Aschoff nodules and other chronic inflammatory cells in the left atrial appendage even in the absence of rheumatic activity has been reported in a high percentage of patients with chronic rheumatic valvular heart disease. This study was conducted on 37 left atrial appendages resected at the time of closed mitral valvotomy. Aschoff nodules were present in 61.2 percent of resected appendages, positivity being 71.4 percent in the age group of 20 years of less and 53.3 percent in patients more than 20 years of age. The histological findings did not change significantly in the presence of activity. The frequency of Aschoff nodules and chronic inflammatory cells and their subtypes also did not show any correlation with age, sex, duration of symptoms or severity of mitral stenosis.