Vasculitis reumatoidea: una manifestación poco habitual. Presentación de tres casos clínicos y revisión de la literatura

La vasculitis reumatoidea es una complicación sistémica y poco frecuente de la Artritis Reumatoidea. Si bien su incidencia ha descendido en los últimos años con el advenimiento de las nuevas terapias inmunosupresoras y biológicas, continua teniendo una alta morbimortalidad. Predomina en el sexo masculino, en pacientes seropositivos y con un largo período de la enfermedad establecida. Requiere de alta presunción diagnostica, siendo el compromiso cutáneo y nervioso periférico el más frecuente. La biopsia de nervio o piel es requerida habitualmente para su diagnóstico. El tratamiento se basa en corticoides e inmunosupresores. Presentamos tres casos clínicos y realizamos una revisión de la literatura.

Rheumatoid vasculitis is a rare systemic complication of rheumatoid arthritis. Although its incidence has decreased in recent years with the advent of new immunosuppressive and biological therapies, it continues to have a high morbidity and mortality. It predominates in males, in seropositive patients and with a long period of established disease. It requires high diagnostic presumption, with skin and peripheral nervous involvement being the most affected. Nerve or skin biopsy is usually required for diagnosis. Treatment is based on corticosteroids and immunosuppressants. We present three clinical cases and carry out a review of the literature.

A vasculite reumatóide é uma complicação sistêmica rara da artrite reumatóide. Embora sua incidência tenha diminuído nos últimos anos com o advento de novas terapias imunossupressoras e biológicas, continua apresentando elevada morbidade e mortalidade. Predomina no sexo masculino, em pacientes soropositivos e com longo período de doença estabelecida. Exige alta presunção diagnóstica, sendo o envolvimento cutâneo e nervoso periférico os mais afetados. A biópsia de nervo ou pele geralmente é necessária para o diagnóstico. O tratamento é baseado em corticosteroides e imunossupressores. Apresentamos três casos clínicos e realizamos uma revisão da literatura.

Manifestaciones cutáneas comunes de las principales enfermedades hepáticas / Common cutaneous manifestations of major liver diseases

Las enfermedades hepáticas presentan múltiples manifestaciones sistémicas, entre las cuales se destacan los hallazgos en piel, siendo los más comunes el prurito y la ictericia; así mismo, se pueden encontrar angiomas en araña, eritema palmar, xantomas, vasculitis y cambios en anexos. Este artículo tiene como objetivo describir los principales signos y síntomas cutáneos en las enfermedades hepáticas para brindar herramientas semiológicas al clínico en su práctica diaria

Liver disease present multiple systemic manifestations, among which skin findings stand out, being the most common pruritus and jaundice. Other findings can also be manifested like spider angiomas, palmar erythema, xanthomas, vasculitis and changes in skin appendages. The objective of this article is to describe the main skin signs and symptoms of liver diseases to provide semiological tools to the physician in his daily practice.

Huaiqihuang Granules Affect Differentiation of Th17 Cells in IgA Vasculitis Nephritis Mice by Regulating AMPK/ACC Pathway / 中国实验方剂学杂志

ObjectiveTo observe the intervention effect of Huaiqihuang granules （HQH） on immunoglobulin A vasculitis nephritis （IgAVN） mice and explore the underlying therapeutic mechanism. MethodFifty SPF-grade male Kunming mice were randomly divided into a normal group， an IgAVN model group， a dexamethasone group （2.5 mg·kg-1·d-1）， a low-dose HQH group （4 g·kg-1·d-1）， and a high-dose HQH group （8 g·kg-1·d-1）. The mouse model was established using oral administration of gliadin combined with intravenous injection of India ink. After successful modeling， the mice were euthanized after 4 weeks of gastric gavage according to groups. The 24 h urinary total protein （24 h UTP）， urine β2-microglobulin （β2-MG）， serum total protein， albumin， IgA， etc. were detected in each group. Flow cytometry was used to determine the proportion of T helper 17 （Th17） cells in spleen cell suspension. Western blot was employed to detect the expression of adenosine 5'-monophosphate-activated protein kinase α （AMPKα）， phosphorylated AMPKα （p-AMPKα）， acetyl-CoA carboxylase 1 （ACC1）， and phosphorylated ACC1 （p-ACC1） in Th17 cells. Pathological changes in the spleen and kidneys were observed. ResultCompared with the normal group， the IgAVN model group showed significant increases in 24 h UTP， urine β2-MG， total cholesterol （P<0.05）， serum interleukin-17 （IL-17）， IgA， Th17 proportion in the spleen cell suspension， and IL-17 expression in the spleen tissue （P<0.01）， and significantly decreased serum total protein， albumin， p-AMPKα/AMPKα， and p-ACC1/ACC1 expression of Th17 cells （P<0.01）. Compared with the IgAVN model group， in the 4th week， the 24 h UTP， urine β2-MG， serum IL-17， IgA levels， and renal IgA deposition were significantly reduced in each treatment group （P<0.01）， and the Th17 proportion and IL-17 expression in spleen tissue were significantly decreased （P<0.05， P<0.01）. Serum albumin levels significantly increased （P<0.05）. Compared with the IgAVN model group， the dexamethasone group and the high-dose HQH group showed increases in serum total protein （P<0.01）， p-AMPKα/AMPKα， and p-ACC1/ACC1 expression of Th17 cells （P<0.05， P<0.01）. The high-dose HQH group showed a significant decrease in total cholesterol level （P<0.05）. Various treatment groups showed different degrees of improvement in spleen and kidney pathological changes. ConclusionHQH may affect Th17 cell differentiation by regulating the AMPK/ACC pathway， correcting immune inflammatory disorders， and exerting therapeutic effects on IgAVN.

Evaluation of ultrasonography combined with serum Gastrin-17 in differential diagnosis of abdominal IgA vasculitis in children / 国际检验医学杂志

Objective To study the value of ultrasonography combined with serum Gastrin-17 in differenti-al diagnosis of abdominal IgA vasculitis in children.Methods A total of 80 children with IgA vasculitis admit-ted to the hospital from June 2020 to December 2022 were selected,including 45 cases of abdominal IgA vascu-litis(observation group)and 35 cases of other types of IgA vasculitis(without gastrointestinal symptoms,control group).The ultrasonographic characteristics and Gastrin-17 level of abdominal IgA vasculitis were an-alyzed,and the relationship between Gastrin-17 level and purpura symptom score was analyzed.Receiver oper-ating characteristic(ROC)curve was used to analyze the diagnostic value of ultrasonography and Gastrin-17 for abdominal IgA vasculitis in children.Results The symptom score of purpura in the observation group was significantly higher than that in the control group(P<0.001),while the serum Gastrin-17 level in the obser-vation group was significantly lower than that in the control group.Pearson correlation analysis showed that serum Gastrin-17 level was negatively correlated with purpura symptom score(r=-0.758,P<0.001).Ul-trasound images showed"doughnut"-like changes in the intestinal wall,with different degrees of central-thick-ness thickening and reduced echo,mainly submucosal thickening.ROC curve analysis showed that the cut-off value of serum Gastrin-17 in the diagnosis of abdominal IgA vasculitis in children was 2.91 pmol/L,the area under the curve was 0.787(95%CI:0.685-0.888),the sensitivity and the specificity were 75.56%(34/45)and 74.29%(26/35),respectively.The sensitivity of ultrasound combined with Gastrin-17 in the diagnosis of abdominal IgA vasculitis in children was 97.78%(44/45),the negative prediction rate was 95.65%(22/23),and the accuracy rate was 82.50%(66/80),which was significantly higher than those of single diagnosis(P<0.05).Conclusion Serum Gastrin-17 level is low in children with abdominal IgA vasculitis,and ultrasound imaging shows"doughnut"-like changes in the intestinal wall and thickening of the submucosa.Combined de-tection of the two could effectively differentially diagnose abdominal IgA vasculitis in children.

Efficacy of plasma exchange in the treatment of renal injury associated with autoimmune diseases / 中国基层医药

Objective:To investigate the effectiveness and safety of plasma exchange for thrombotic microangiopathy and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, which require renal replacement therapy.Methods:This is a retrospective study. The clinical data of four patients who underwent plasma exchange at the Blood Purification Center, North Branch of the First Affiliated Hospital of Anhui Medical University, from June 2021 to February 2022 were collected and analyzed. The records included a total of 19 treatments performed, including 15 treatments with a single plasma exchange regimen used in 3 patients and 4 treatments with double filtration plasmapheresis used in 1 patient. Changes in condition before and after treatment, as well as corresponding biochemical test results, were recorded.Results:In four patients, clinical symptoms and biochemical indicators improved. Three patients required maintenance hemodialysis, while one patient showed remarkable clinical efficacy. After treatment, hemoglobin level increased from 76 g/L to 83 g/L, blood creatinine level decreased from 703.6 μmol/L to 526.2 μmol/L, anti-SM antibody decreased from > 400 RU/mL to < 2 RU/mL, anti-myeloperoxidase antibody decreased from 255.49 RU/mL to 15.64 RU/mL, and perinuclear anti-neutrophil cytoplasmic antibody turned negative. During treatment, there were two cases of allergic reactions, which were significantly alleviated after treatment.Conclusion:Plasma exchange is safe and effective against thrombotic microangiopathy and anti-neutrophil cytoplasmic antibody-associated vasculitis, which require renal replacement therapy.

Aphasia in Right Hemiparesis and Pain in Left Waist: A Deceiving Case in Vascular Ehlers-Danlos Syndrome / 罕见病研究

The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder. The prevalence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand. The vascular EDS(vEDS) are rare among the subtypes but are the worst in prognosis. The article reports a case of vEDS admitted to the hospital. The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons. The diagnosis was made after the genetic testing. The patient suffered from vEDS. Then, the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient. The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging. This article hopes to report this case and to share the experiences to the better understanding of this disease.

Report of two cases of urticaria vasculitis after vaccination / 上海预防医学

Vaccination is the most economical and effective measure to prevent infectious diseases targeted by vaccines. Despite this， the safety of vaccines has garnered increased attention due to recent vaccine incidents. The tetravalent human papillomavirus vaccine （HPV） is one of the effective means to prevent cervical cancer and in situ adenocarcinoma caused by infection with corresponding serotypes. The inactivated novel coronavirus vaccine is an emergency vaccine developed to prevent novel coronavirus infection after the COVID-19 outbreak， and is also the main measure used to control the spread of COVID-19 at present. The monitoring data show that both vaccines have good safety after inoculation， but due to individual differences and other reasons， rare reactions may occur in a very small number of recipients. This article presents two cases of urticaria vasculitis following inoculation of the tetravalent HPV and the novel corona virus inactivated vaccine.

Identification of inflammatory response genes in ANCA-associated vasculitis by bioinformatics analysis and experimental validation / 安徽医科大学学报

Objective @#To explore the candidate genes and potential molecular mechanisms of anti -neutrophil cyto- plasmic antibodies ( ANCA) -associated vasculitis by bioinformatics and experimental validation , and to provide a scientific theoretical basis for the treatment of potential inflammatory targets for ANCA-associated vasculitis .@*Methods@#The GSE108109 chip data was retrieved from the Gene Expression Omnibus (GEO) database , and the differential genes were processed , analyzed and screened using the R language related program package . Kyoto encyclo- pedia of genes and genomes (KEGG) and gene ontology (GO) enrichment analysis was carried out using DAVID online network cable , and the interaction network of the protein encoded by the selected genes of inflammatory syn- drome was constructed through STRING web site . Further endogenous competitive RNA ( ceRNA) regulatory net- work was predicted and constructed through miRWalk and DIANA-LncBase databases , and key genes were screened from the network to draw ROC curve . The renal biopsy samples of patients with ANCA-associated vasculi- tis confirmed by our hospital were collected as the experimental group , and the renal biopsy samples of IgA ne- phropathy and micro-adaptive nephropathy were collected as the control group . Immunohistochemical staining was performed on the collected renal biopsy samples , and the average optical density was calculated by semi -quantita- tive analysis of immunohistochemical staining to further verify the expression of the key genes screened by the bioin- formatics analysis . Pearson linear correlation analysis was performed between the average optical density results and the clinical inflammatory data of patients . @*Results @#846 differential genes were screened , of which 444 genes were significantly up-regulated and 402 genes were significantly down-regulated . Through KEGG and GO analysis , im- portant differentially expressed genes related to inflammation regulation were obtained . Among them , CSF1R and TNFRSF1B , two differentially expressed genes never reported in ANCA-associated vasculitis , attracted our atten- tion . At the same time , we constructed multiple ceRNA regulatory axes including KCNQ1OT1 -hsa-miR-125 a-5p- TNFRSF1B . There were 15 samples of ANCA-associated vasculitis , 6 samples of IgA nephropathy , and 3 samples of micropathological kidney . Immunohistochemical results of renal biopsy specimens showed that the expression of CSF1R and TNFRSF1B in ANCA-associated vasculitis kidney tissue was higher than that in the control group . Pearson correlation analysis of clinical data of patients in ANCA group showed that the expression of CSF1R was positively correlated with the content of neutrophil count ( r = 0. 587) , and the expression of TNFRSF1B was posi- tively correlated with the content of serum C -reactive protein ( r = 0. 646) . @*Conclusion @#Key genes related to in- flammatory regulation such as CSF1R and TNFRSF1B were investigated by bioinformatics methods , and a rigorous ceRNA regulatory network was constructed . The expression of CSF1R and TNFRSF1B in ANCA vasculitis was high- er than that in the control group through immunohistochemistry . The results provides a scientific theoretical basis for the molecular mechanism of inflammation , and laid a good foundation for new therapeutic targets of ANCA-related vasculitis for inflammation .

Vancomycin-associated hemorrhagic occlusive retinal vasculitis masquerading as central retinal vein occlusion / Vasculite hemorrágica oclusiva da retina associada à vancomicina (HORV) mascarada como oclusão da veia central da retina

ABSTRACT A 69-year-old female was referred with sudden unilateral painless decreased vision that began 2 days after uncomplicated cataract surgery in the left eye. Visual acuity was hand motion and biomicroscopy showed a mild anterior chamber reaction, no hypopyon, and an intraocular lens that had been placed within the capsular bag. A dilated fundus examination revealed optic disk edema, widespread deep and superficial intraretinal hemorrhages, retinal ischemia, and macular edema. A cardiological evaluation was normal and thrombophilia tests were negative. After surgery, prophylactic vancomycin (1mg/0.1ml) had been injected intracamerally. The patient was diagnosed with hemorrhagic occlusive retinal vasculitis likely secondary to vancomycin hypersensitivity. Recognition of this entity is important to ensure early treatment and the use of intracameral vancomycin in the fellow eye should be avoided after cataract surgery.

RESUMO Esse caso se refere a uma paciente de 69 anos, sexo feminino, com relato de baixa acuidade visual súbita e indolor no olho esquerdo, de início 2 dias após cirurgia de catarata sem complicações. A acuidade visual era de movimento de mãos e a biomicroscopia mostrou reação de câmara anterior moderada, sem hipópio, e lente intraocular posicionada dentro do saco capsular. A fundoscopia evidenciou edema de disco óptico, hemorragias difusas intrarretinianas superficiais e profundas, isquemia retiniana e edema macular. A avaliação cardiológica foi normal e os testes para trombofilia foram negativos. Ao final da cirurgia foi injetado antibioticoprofilaxia com vancomicina (1mg/0,1ml) na câmara anterior. A paciente foi diagnosticada com vasculite hemorrágica oclusiva da retina secundária à hipersensibilidade a vancomicina. O reconhecimento dessa entidade é importante para o tratamento precoce e para evitar o uso de vancomicina intracameral em caso de cirurgia de catarata no olho contralateral.

Granulomatosis con poliangeítis. A propósito de un caso

La granulomatosis con poliangeítis es una vasculitis sistémica, necrosante y granulomatosa que afecta el tracto respiratorio superior e inferior y los riñones. Se presenta el caso de una paciente blanca, de 59 años de edad, hipertensa y fumadora inveterada, que ingresó por presentar síntomas constitucionales, orinas turbias y hemáticas, así como creatininemia elevada con valor previo normal. Con impresión diagnóstica inicial de infección del tracto urinario se indica antibioticoterapia. Después del tercer día de iniciado el tratamiento, mejoraron los síntomas constitucionales y, de forma progresiva, comenzó con manifestaciones multiorgánicas (renal, respiratoria, cutánea y musculoesquelética) sugestivas de vasculitis sistémica. Como elementos significativos, en exámenes complementarios se detectaron azoados y reactantes de fase aguda aumentados, sedimento urinario activo, imagen nodular en vértice pulmonar derecho y microhemorragias multifocales en ambos campos pulmonares, detectados mediante tomografía computarizada y c-ANCA altamente positivo. Se indicó tratamiento inmunosupresor y citotóxico potente, obteniéndose control inicial de las manifestaciones graves de la enfermedad. Evolutivamente desarrolló complicaciones propias de la entidad y secundarias al tratamiento médico, que la hicieron tributaria de terapia de sustitución renal, falleciendo a los dos años de recibir hemodiálisis iterada. El cuadro clínico, unido a los estudios imagenológicos e inmunológicos (c-ANCA), fueron elementos claves para realizar el diagnóstico.

Granulomatosis with polyangiitis is a systemic, necrotizing, granulomatous vasculitis that affects the upper and lower respiratory tract and kidneys. The case of a 59-year-old white female patient, who is hypertensive and inveterate smoker, is presented; she was admitted for presenting constitutional symptoms, turbid and bloody urine, as well as elevated creatininemia with a normal previous value. With the initial diagnostic impression of urinary tract infection, antibiotic therapy was indicated. After the third day of starting treatment the constitutional symptoms improved, and progressively began with multiorgan manifestations (renal, respiratory, skin and musculoskeletal) suggestive of systemic vasculitis. As significant elements, complementary examinations detected increased nitrogen and acute phase reactants, active urinary sediment, nodular image in the right lung apex and multifocal microhemorrhages in both lung fields through computed tomography, and highly positive c-ANCA. Potent immunosuppressive and cytotoxic treatment was indicated, obtaining initial control of severe manifestations of the disease. Evolutionarily she developed complications specific to the entity and secondary to medical treatment which made her subject to renal replacement therapy, dying two years after receiving iterated hemodialysis. The clinical characteristics, together with the imaging and immunological studies (c-ANCA) were key elements to make the diagnosis.

A great simulator in clinical practice: mononeuritis multiplex in HIV infection

ABSTRACT HIV infection is a chameleon, mimicking several diseases. Herein, we report a previously healthy 39-year-old woman who, over 2 months, developed arthritis, weight loss, and confluent multiple mononeuropathy. Extensive laboratory investigation showed positive serology for HIV, with a CD4 count of 100 cells, and necrotizing vasculitis on a nerve biopsy not associated with CMV co-infection, allowing the diagnosis of polyarteritis nodosa-like vasculitis in an HIV-infected patient. Apart from the infection, HIV-related autoimmunity can affect any organ and contribute to the complexity of the clinical presentation of HIV infection.

Vasculitis por IgA del adulto. Caso clínico

La vasculitis por IgA, es la vasculitis más frecuente en pediatría. Puede presentarse en adultos, con una clínica y evolución diferente y un pronóstico más grave que en los niños, incluida la progresión a enfermedad renal terminal. La historia natural de la enfermedad y de la nefritis, ha sido poco estudiada en adultos; no se dispone de criterios diagnósticos universalmente aceptados y el tratamiento es controvertido, dada la ausencia de estudios controlados, randomizados que lo avalen. Se reporta el caso de un paciente que presentó un síndrome purpúrico petequial, microhematuria, proteinuria y una evolución rápida a la insuficiencia renal, de cuyo estudio etiológico surge el diagnóstico de vasculitis por IgA del adulto.

The IgA vasculitis is the most common vasculitis in Pediatrics. It can also present in adults but with a different clinical course and a worse prognosis, including the possibility of progression to end stage renal disease. The natural history of the disease and its nephritis have been scarcely studied in adults. There is no universal agreement in diagnostic criteria and the treatment is controversial given the absence of controlled randomized trials. We report the case of a patient who presented clinically with a petechial purpuric rash, microhematuria, proteinuria and rapid progression to renal failure that was diagnosed with IgA vasculitis in adult.

A vasculite por IgA é a vasculite mais comum em pediatria. Pode ocorrer em adultos, com apresentação e evolução clínica diferentes e prognóstico mais grave do que em crianças, incluindo progressão para doença renal terminal. A história natural da doença e da nefrite tem sido pouco estudada em adultos; Não existem critérios diagnósticos universalmente aceitos e o tratamento é controverso, dada a ausência de estudos controlados e randomizados que o apoiem. É relatado o caso de um paciente que apresentou síndrome purpúrica petequial, microhematúria, proteinúria e rápida evolução para insuficiência renal, de cujo estudo etiológico surge o diagnóstico de vasculite por IgA do adulto.

Vasculitis crioglobulinémica de rápida evolución / Rapidly evolving cryoglobulinemic vasculitis

Las vasculitis se manifiestan por la inflamación de los vasos sanguíneos, afectando desde los capilares hasta los vasos de mayor tamaño. El compromiso dermatológico por vasculitis comprende desde casos leves, que generan compromiso superficial, hasta úlceras y necrosis de diferentes tejidos. La crioglobulinemia se caracteriza por la presencia de crioglobulinas séricas, que precipitan a temperaturas menores a 37 ºC. Se pueden asociar a enfermedades infecciosas (destacándose la infección por virus de hepatitis C, VHC), inmunomediadas y linfoproliferativas. La vasculitis en contexto de crioglobulinemia se produce por depósitos de inmunocomplejos en capilares, arteriolas, y arterias de pequeño y mediano calibre. El tratamiento consiste en tratar la causa subyacente y modular la respuesta inflamatoria. Se presenta el caso clínico de un varón de 50 años, con historia de hepatitis B crónica, que manifestó isquemia aguda y crítica de ambas extremidades, documentándose vasculitis crioglobulinémica con mala evolución.

Vasculitis is manifested by inflammation of the blood vessels, affecting from the capillaries to the largest vessels. Dermatological compromise due to vasculitis ranges from mild cases that generate superficial involvement to ulcers and necrosis of different tissues. Cryoglobulinemia is characterized by the presence of serum cryoglobulins, which precipitate at temperatures below 37 degrees Celsius. It is associated with infectious, immune-mediated, and lymphoproliferative diseases, especially infection by the hepatitis C virus. Vasculitis in the context of cryoglobulins is produced by immune complex deposits in capillaries, arterioles, and small and medium-sized arteries. Treatment is based on treating the underlying cause and modulating the inflammatory response. We present the case of a 50-year-old man, with a history of chronic hepatitis B, who presented acute and critical ischemia of both extremities, documenting cryoglobulinemic vasculitis with poor evolution.

Criterios broncoscópicos, laboratoriales y anatomopatológicos de enfermedad de la vía aérea en pacientes diagnosticados con granulomatosis con poliangitis / Bronchoscopic, laboratory and anatomopathological criteria for airway disease in patients diagnosed with granulomatosis with polyangiitis

Introducción: los pacientes con granulomatosis con poliangitis (GPA) pueden presentar compromiso de la vía aérea superior (VAS) o inferior (VAI). Objetivos: describimos las manifestaciones endoscópicas de las vías respiratorias, los hallazgos histológicos y los anticuerpos anticitoplasma de neutrófilos (ANCA) en un grupo de pacientes con GPA. Métodos: estudio retrospectivo de historias clínicas de pacientes con GPA sometidos a broncoscopia entre 2012 y 2019. Se analizaron hallazgos de la vía aérea, biopsias y ANCA. Resultados: se incluyeron 40 pacientes, con una edad media de 46,92±17,61 años, predominantemente del sexo femenino (67,5%). Se observó afectación de la vía aérea en el 90% (n=36). El C-ANCA fue reactivo en el 63,9%, P-ANCA en el 25%, ANCA doblemente reactivo en el 8,33% y no reactivo en el 20%. Los hallazgos comunes en la vía aérea superior (VS) fueron sinusitis crónica (41,7%), destrucción del tabique nasal (16,7%); y en la vía aérea inferior (AI): estenosis traqueobronquial (38,9%), traqueobronquitis (25%). Los hallazgos más frecuentes de las biopsias broncoscópicas fueron proceso inflamatorio polimorfonuclear (61,9%) y necrosis geográfica (47,6%). Conclusión: la vía aérea está comprometida hasta en un 90% de los pacientes con GPA. ANCA no reactivos no descartan esta posibilidad. La sinusitis crónica y los procesos fibroestenóticos traqueobronquiales fueron los hallazgos endoscópicos más comunes. La vasculitis en biopsias se encontró en una minoría de casos.

Introduction: patients with granulomatosis with polyangiitis (GPA) may present upper airway (UA) and lower airway (LA) involvement. Objectives: we describe the endoscopic manifestations of the airways, histological findings from biopsied tissue and antineutrophilic cytoplasm antibody (ANCA) in a group of patients with GPA. Methods: retrospective study of medical records of patients with GPA undergoing bronchoscopy between 2012 and 2019. Airway findings, results of biopsies performed and ANCA results were analyzed. Results: 40 patients were included, with a mean age of 46.92±17.61 years and predominantly female (67.5%). Airway involvement was observed in 90% (n=36). The C-ANCA was reactive in 63.9%, P-ANCA in 25%, doubly reactive ANCA in 8.33% and non-reactive in 20%. The findings in upper airway (UA) were: chronic sinusitis (41.7%), destruction of the nasal septum (16.7%); and in lower airway (LA) were: tracheobronchial stenosis (38.9%) and tracheobronchitis (25%). The pathological findings most common of bronchoscopic biopsies were: polymorphonuclear inflammatory process (61.9%) and geographic necrosis (47.6%). Conclusion: the airway is involved in up to 90% of patients with GPA. Non-reactive ANCA does not rule out this possibility. Chronic sinusitis and tracheobronchial fibrostenotic processes were the most common endoscopic findings. Vasculitis in biopsies was found in a minority of cases.

Reporte de pacientes con espondiloartritis y arteritis de Takayasu en Argentina: ¿asociación causal o casual? / Report of patients with spondyloarthritis and Takayasu's arteritis in Argentina: is it a causal or casual association?

Introducción: tanto las espondiloartritis (EspA) como la arteritis de Takayasu (TAK) son enfermedades infrecuentes y su asociación es aún más rara. Objetivos: presentar una serie de pacientes con diagnóstico concomitante de EspA o con rasgos de EspA y TAK en Argentina, y realizar una revisión de la literatura respecto de esta asociación. Materiales y métodos: se recopilaron las características demográficas, clínicas y terapéuticas de pacientes con diagnóstico concomitante de EspA o con algunos rasgos de EspA y TAK, de distintos centros de salud de la República Argentina. Resultados: se describen 7 pacientes, de los cuales 4 presentaban EspA, uno con compromiso axial (EspAax) juvenil, otro con artritis psoriásica (APs), otro con espondilitis anquilosante (EA) y el último con EspAax pura, y 3 de ellos tuvieron rasgos de EspA (enfermedad Crohn, psoriasis y oligoartritis asimétrica de grandes articulaciones). En la mayoría de los casos, los rasgos de EspA se presentaron con una mediana de 4 años antes de la TAK. Conclusiones: varios reportes y series de casos relatan la superposición entre estas dos enfermedades. Si bien las mismas podrían compartir cierta base genética común, todavía no contamos con evidencia sólida que permita estimar que esta asociación no es casual.

Introduction: both spondyloarthritis (SpA) and Takayasu arteritis (TAK) are rare diseases, and their association is even rarer. Objectives: to present a series of patients with a concomitant diagnosis of SpA or with features of SpA and TAK in Argentina and review the literature regarding this association. Materials and methods: the demographic, clinical and therapeutic characteristics of patients with a concomitant diagnosis of SpA or with some features of SpA and TAK were collected from different health centers in Argentina. Results: 7 patients are described, of which 4 had SpA, one with juvenile axial involvement (axSpA), another with psoriatic arthritis (PsA), another with ankylosing spondylitis (AS) and the last patient with pure axSpA and 3 of them had features of SpA (Crohn's disease, psoriasis and asymmetric oligoarthritis of large joints). In most cases, SpA features presented a median of 4 years before TAK. Conclusions: several case reports and case series reported overlap between these two diseases. Although they could share a certain common genetic basis, we still do not have solid evidence that allows us to estimate that this association is not coincidental.

Um raro caso de vasculite leucocitoclástica induzida por oxacilina / A rare case of oxacillin-induced leukocytoclastic vasculitis

A vasculite leucocitoclástica é uma patologia cujos mecanismos estão associados ao processo de inflamação vascular. Estima-se que até 24% dos casos de vasculite estão relacionados ao uso de fármacos, sendo os antimicrobianos beta-lactâmicos um dos grupos farmacológicos comumente associados a este desfecho adverso. A oxacilina, uma penicilina semissintética, possui um anel beta-lactâmico que confere atividade biológica e está associada com maior frequência a relatos de vasculite leucocitoclástica. No entanto, casos semelhantes relacionados a esse antimicrobiano são raros, sendo identificados apenas três casos na literatura. Diante desse contexto, relatamos um quarto caso de vasculite leucocitoclástica em um homem de 56 anos, em tratamento com oxacilina, que desenvolveu a vasculite no 3º dia de uso do antimicrobiano. Além da suspensão da oxacilina, ele foi tratado com 125 mg/dia de metilprednisolona endovenosa por sete dias, seguido de 20 mg/dia de prednisona oral por quatro dias, resultan-do em remissão satisfatória das lesões cutâneas e ausência de novos desfechos adversos. Este caso corrobora a possível relação causal entre o uso de oxacilina e o desenvolvimento da vasculite leucocitoclástica, apesar de sua ocorrência ser rara. A resposta favorável às intervenções terapêuticas, incluindo a suspensão da oxacilina e o uso de corticosteroides, destaca a eficácia dessas abordagens no tratamento dessa complicação (AU).

Leukocytoclastic vasculitis is a pathology whose mechanisms are associated with the process of vascular inflammation. It is estimated that up to 24% of the cases of vasculitis are drug-related, with beta-lactam antimicrobials be-ing one of the pharmacological groups commonly associated with this adverse outcome. Oxacillin, a semisynthetic penicillin, has a beta-lactam ring that confers biological activity and is most frequently associated with reports of leukocytoclastic vasculitis. However, similar cases related to this antimicrobial are rare, with only three cases identified in the literature. Against this background, we report a fourth case of leukocytoclastic vasculitis in a 56-year-old man, on oxacillin treatment, who developed the vasculitis on the 3rd day of antimicrobial use. In addition to oxacillin suspension, he was treated with 125 mg/day of intravenous methylprednisolone for seven days, followed by 20 mg/day of oral prednisone for four days, resulting in satisfactory remission of the skin lesions and no new adverse outcomes. This case provides further evidence supporting the potential causal relationship between the use of oxacillin and the development of leukocytoclastic vasculitis, albeit a rare occurrence. The positive response to therapeutic interventions, such as oxacillin suspension and corticosteroid treatment, underscores the effectiveness of these approaches in addressing this complication (AU),

Enfermedad de Kawasaki. Presentación atípica en un paciente pediátrico

La enfermedad de Kawasaki es un síndrome mucocutáneo caracterizado por vasculitis, que afecta medianos vasos; su principal manifestación es un síndrome febril agudo de, al menos, cinco días de duración y en muchas ocasiones de etiología desconocida. Se aprecia, fundamentalmente, en niños menores de cinco años de edad. Se considera que es frecuente, pero existen subregistros. Se caracteriza por tener dos formas de presentación: típica o atípica. Se presenta el caso clínico de un niño que fue hospitalizado debido a síndrome febril agudo asociado a malestar general e irritabilidad. Al examen físico se observaron edemas discretos en manos y pies así como erupción cutánea, no hepatomegalia ni alteraciones oculares. Teniendo en cuenta la epidemiología, lo atípico del cuadro clínico y los resultados de estudios hemoquímicos, se concluyó el diagnóstico de enfermedad de Kawasaki atípica. Se impuso tratamiento específico y se logró una evolución satisfactoria y la no aparición de complicaciones inmediatas. Esta entidad nosológica requiere de una adecuada valoración clínica-epidemiológica de los pacientes, así como de un minucioso examen físico y un diagnóstico precoz para lograr la evolución favorable de los pacientes y la no presencia de secuelas cardiacas, que pondrían en peligro la vida del paciente y/o su calidad de vida futura.

Kawasaki disease (KD) is a mucocutaneous syndrome characterized by vasculitis that affects medium vessels; its main manifestation is an acute febrile syndrome lasting at least five days and often of unknown etiology. It appears mainly in children under five years of age, it is considered to be frequent, but there are underreportings. It is characterized by having two presentation forms: typical or atypical. The clinical case of a child who was hospitalized due to acute febrile syndrome associated with malaise and irritability is presented. The physical examination revealed discrete edema in the hands and feet as well as a rash, no hepatomegaly or ocular alterations. Taking into account the epidemiology, the atypical clinical picture and the results of hemochemical studies, the diagnosis of atypical Kawasaki disease was concluded. Specific treatment was imposed and a satisfactory evolution was achieved with no immediate complications. This nosological entity requires an adequate clinical-epidemiological evaluation of the patients, as well as a meticulous physical examination and an early diagnosis to achieve a favorable patients' evolution and the absence of cardiac sequelae, which would endanger the patients' life and/or their future quality of life.

Vasculitis pauciinmune asociada a lupus eritematoso sistémico: a propósito de un caso

Introducción: la poliangeítis microscópica (PAM) hace parte del grupo de vasculitis asociadas a anticuerpos anticitoplasmáticos de neutrófilos (ANCA), cuya presencia de anticuerpos contra mieloperoxidasa (MPO) se observa en la mayoría de los casos (70-95 %), asimismo, el compromiso renal presagia mayores tasas de morbilidad y mortalidad, sin embargo, la coexistencia con lupus eritematoso sistémico (LES) es poco frecuente y con mayor énfasis en la variante de LES del adulto mayor o de inicio tardío. Objetivo: dar a conocer un caso poco común de dos enfermedades autoinmunes, lo cual ha sido reportado como síndrome de superposición y brindar información útil que permita ampliar los diagnósticos diferenciales. Presentación del caso: se presenta el caso de un paciente masculino de 76 años con historia de poliartralgias progresivas en deterioro del estado general, con debut en síndrome confusional agudo, caída de filtrado glomerular, microhematuria y proteinuria casi nefrótica. A la evaluación inicial se encontró anemia y trombocitopenia severa, en perfil inmune ANA 1/320 y complemento consumido, cumpliendo criterios para LES, ANCA reactivo específicamente MPO ANCA (1/320) y sospecha de glomerulonefritis con patrón rápidamente progresivo (GNRP). Dado el contexto clínico, se decidió comenzar con pulsos de metilprednisolona consecutivos, seguidos de prednisolona oral y, como terapia de mantenimiento, se instauró ciclofosfamida. Finalmente, con una biopsia renal se confirmó el diagnóstico de vasculitis sistémica pauciinmune con formación de crescencia celular. La evolución clínica del paciente fue satisfactoria, logrando la estabilización de órganos y la normalización de la función renal, hematopoyética y el estado neurológico. Discusión y conclusión: dado que la presentación clínica de LES es heterogénea, se ha reportado su asociación con vasculitis, las cuales comparten un compromiso común de órganos como articulaciones, piel y riñones. Los hallazgos paraclínicos, como por biopsia, fueron consistentes tanto con PAM como con LES, por lo tanto, se diagnosticó como un caso de superposición. Este caso demuestra el enigma del diagnóstico y la complejidad en el manejo de entidades poco frecuentes, de etiologías inmunológicas superpuestas que ponen en peligro la vida de quien la(s) padece, de no tener un diagnóstico oportuno y un tratamiento temprano.

Introduction: Microscopic polyangiitis (MPA) is part of the group of vasculitis associated with antineutrophil cytoplasmic antibodies (ANCA), whose presence of antibodies against myeloperoxidase (MPO) is observed in most cases (70-95 %), likewise, the renal compromise portends higher rates of morbidity and mortality. However, coexistence with Systemic Lupus Erythematosus (SLE) is infrequent, and with greater emphasis on the older adult or late-onset variant of SLE (1). Purpose: Present a rare case of two autoimmune diseases, which has been reported as overlapping syndrome and provide useful information that allows expanding differential diagnoses. Case report: We present the case of a 76-year-old male patient with a history of progressive polyarthralgias in deteriorating general condition, with debut in acute confusional syndrome, drop in glomerular filtration rate, microhematuria and almost nephrotic proteinuria. At the initial evaluation anemia and severe thrombocytopenia were found, in the immune profile ANA 1/320, complement consumed; fulfilling criteria for SLE, ANCAs reactive specifically MPO ANCA (1/320) and suspected Rapidly Progressive Renal Insufficiency (PRRI). Given the clinical context, it was decided to start consecutive pulses of methylprednisolone followed by oral prednisolone, and cyclophosphamide was established as maintenance therapy. Finally, a renal biopsy confirmed the diagnosis of pauci-immune systemic vasculitis with cell crescent formation. These findings were consistent with both SLE and PAM, thus it was diagnosed as a case of overlap. The clinical evolution of the patient was satisfactory, achieving organ stabilization and normalization of renal function, hematopoietic function, and neurological status. Discussion and conclusion: RPGN associated with ANCA present non-nephrotic proteinuria, glomerular filtration rate drop. The most common is anti-glomerular basement membrane (AMBG) antibodies. Serological detection of antineutrophil cytoplasmic antibodies, PR3 and MPO should be available. Microscopic polyangiitis could be determined by renal biopsy, with the presence of cell growth, absence of immune deposits and MPO ANCA positivity. This case is a diagnostic enigma, and the complexity in the management of rare entities, of immunological etiology, superimposed that endanger the life of those who suffer from it, if they do not have a timely diagnosis and early treatment.

Reporte de caso: vasculitis leucocitoclastica como forma de presentación de hepatitis b crónica / Case report: leukocytoclastic vasculitis as presenting manifestation of chronic hepatitis b virus infection

La vasculitis leucocitoclástica, también denominada angeitis cutánea leucocitoclástica, es la forma más común de vasculitis. Si bien la mayoría de los casos son idiopáticos, entre los agentes etiológicos que podemos nombrar se encuentran los agentes infecciosos, las enfermedades del tejido conectivos, las reacciones de hipersensibilidad a medicamentos y las neoplasias solidas o hematológicas. Si bien los procesos infecciosos son una causa conocida de vasculitis leucocitoclástica, la infección por virus de Virus de hepatitis B (VHB) es muy infrecuente. Presentamos una mujer de 47 años, sin antecedentes patológicos previos, que consultó por artralgias en rodillas y tobillos, mialgias en gemelos y rash purpúrico con leve prurito en ambos miembros inferiores, de un mes de evolución. La biopsia cutánea de las lesiones de miembros inferiores fue compatible con vasculitis leucocitoclástica. La serología de hepatitis B fue positiva por lo que inició tratamiento antiviral con Tenofovir y Prednisona con buena evolución de sus lesiones cutáneas

Leukocytoclastic vasculitis, also called leukocytoclastic cutaneous angiitis, is the most common form of vasculitis. Although most cases are idiopathic, etiologic agents include infectious agents, connective tissue diseases, drug hypersensitivity reactions, and solid or hematologic malignancies. Although infectious processes are a known cause of leukocytoclastic vasculitis, hepatitis B virus (HBV) infection is very rare. We present a 47-year-old woman, with no previous pathologic history, who consulted for arthralgias in the knees and ankles, myalgia's and purpuric rash with mild pruritus in both lower limbs, of one month evolution. Skin biopsy of lower extremity lesions was compatible with leukocytoclastic vasculitis. Hepatitis B serology was positive, so she started antiviral treatment with tenofovir and prednisone with good evolution of her skin lesions

Diagnostic usefulness of triple biopsies (Skin, Muscle and Nerve) in clinically suspected cases of vasculitis in a tertiary care hospital in Gujarat

Introduction: The term vasculitis refers to a heterogeneous group of diseases, all characterized by inflammation and destruction of blood vessel walls leading to ischemic, thrombotic, and hemorrhagic damage to tissues of central and peripheral nervous system. The main indication for triple biopsy (skin, muscle and nerve) is to rule out peripheral vasculitis neuropathy. However, the drawback is that any systemic inflammatory process may show changes in a skin biopsy and hence these changes need to be interpreted with caution. The aim of this study is to establish the diagnostic yield and the usefulness of the triple biopsies in clinically suspected cases of vasculitis. Material and Methods: The present study was conducted in the Department of General Pathology, SBKS MI & RC, Vadodara over a period of one year (1/1/2022 to 31/12/2022). All the clinically suspected cases of vasculitis received at OPD were included in the study. Any case with either muscle, nerve or skin biopsy reported as inadequate were excluded from the study. Results: On evaluation the usefulness of triple biopsies for vasculitis, we found a very low diagnostic yield with only 3.3% of peripheral nerve biopsy and 0.8% of muscle and nerve biopsy showed definite vasculitis. In case of suspected peripheral/systemic vasculitic neuropathy, nerve biopsy was sufficient in the majority of case and has the diagnostic armamentarium for the evaluation of vasculitis. Conclusion: In conclusion, nerve biopsies provide the best yield for the diagnosis of vasculitis, as opposed to muscle and skin biopsies. The role of triple biopsies as a routine protocol for the evaluation of vasculitis is questionable.