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.

Chronic urticaria with thyroid auto-antibodies linked to urticarial vasculitis

Chronic urticaria is a common disorder of unknown origin, which is often associated with normal routine laboratory values and no evidence of systemic disease. Cutaneous vasculitis is one of the rare presentation of chronic urticaria. We reported a 13-year-old female child, who presented to us with tender urticarial wheals all over the body, who on further evaluation was found to have positive anti-thyroid peroxidase antibodies and was diagnosed to have urticarial vasculitis.

Rare case of immune mediated cutaneous leucoclastic vasculitis with pancytopenia in a rheumatoid arthritis patient with chronic cytomegalovirus infection

Acute febrile illnesses with systemic involvement can present significant diagnostic and therapeutic challenges, particularly in patients with multiple comorbidities. This case report highlights a complex presentation of acute febrile illness with pancytopenia and immune-mediated cutaneous vasculitis in a patient with type II diabetes mellitus. A 52-year-old male with a known history of type II diabetes mellitus presented with fever, decreased oral intake, loose stools, oral ulcers, dysphagia, and rashes over the trunk and abdomen for four days. Upon admission to the ICU, extensive diagnostic evaluations were performed, revealing significant hematological, biochemical, and serological abnormalities. Imaging studies and histopathological examinations were conducted to further investigate the underlying etiology. Laboratory findings indicated pancytopenia, acute kidney injury, and hyperkalemia. Infectious disease workup was largely negative, except for a positive CMV IgG. Imaging studies revealed medical renal disease, and a skin punch biopsy confirmed cutaneous leukocytoclastic vasculitis. Bone marrow biopsy suggested bone marrow suppression. Multidisciplinary management, including dialysis, IV fluids, antibiotics, blood transfusions, steroids, and Ganciclovir, led to the patient抯 gradual improvement and stabilization. The comprehensive diagnostic and therapeutic approach in this case underscores the importance of considering immune-mediated etiologies in patients with atypical presentations. Multidisciplinary collaboration was crucial in managing the multifaceted clinical condition of the patient. Early recognition and prompt multidisciplinary management are essential in similar cases. Extensive diagnostic evaluations should be performed to identify the underlying causes, and immune-mediated etiologies should be considered in complex presentations. Further research is recommended to explore optimal management strategies for such multifaceted conditions.

Anti-neutrophil cytoplasmic antibodies associated vasculitis with interstitial lung disease: a case report

Anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitis is a necrotizing vasculitis that primarily affect small blood vessels of the airway and kidneys. The major clinicopathologic feature include microscopic polyangiitis and granulomatosis with polyangiitis. There are several studies done that have shown the association of interstitial lung disease (ILD) and ANCA vasculitis. Majority of the studies showed that the prognosis was bad in patients with AAV and ILD, then those without it, and patients with pulmonary fibrosis and ANCA had as low a prognosis as patients with IPF without ANCA. We hereby report a case of a female patient in her 70s who was been treated as a case of infection at different hospital, only to be diagnosed later as ANCA associated vasculitis with interstitial lung disease. Methylenetetrahydrofolate reductase (MTHFR) enzyme is one of the key enzymes involved in the metabolism of folate.

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.

Vasculitis cerebelosa por lupus eritematoso sistémico y descompresión suboccipital: reporte de caso / Cerebellar vasculitis due to systemic lupus erythematosus and suboccipital decompression: case report

RESUMEN Las vasculitis de las arterias o vasculitis cerebelosas en el lupus eritematoso sistémico (LES) son extremadamente infrecuentes y podrían estar inmersas o formar parte de los casos de LES neuropsiquiátrico (LESNP), stroke cerebeloso en el LES y grupo de vasculitis del sistema nervioso central (SNC ) por LES. La adición del manejo quirúrgico a esta entidad, mediante descompresión suboccipital, ha sido reportada en muy pocas publicaciones. Se presenta el caso de una paciente mujer de 18 años con LES, LESNP, stroke cerebeloso, con alta sospecha de vasculitis inflamatoria de las arterias cerebelosas a quien, a pesar del manejo médico, se le brindó descompresión suboccipital. La vasculitis inflamatoria de las arterias cerebelosas en el LES es una causa infrecuente de stroke cerebeloso de elevada mortalidad. Existe limitada evidencia científica en la realización de la descompresión suboccipital de esta entidad.

ABSTRACT Arterial vasculitis or cerebellar vasculitis in systemic lupus erythematosus (SLE) are extremely infrequent, and these might be included within cases of neuropsychiatric SLE (NPSLE), cerebellar stroke in SLE, and within the group of central nervous system vasculitis due to SLE. The addition of surgical management to this condition has been reported in few publications. We present the case of a 18- year old female patient with SLE, NPSLE, and cerebellar stroke, highly suspicious of inflammatory vasculitis affecting cerebellar arteries, in whom, in spite of medical management, suboccipital decompression was performed. Inflammatory vasculitis of cerebellar arteries in SLE is an infrequent cause of cerebellar stroke, and it has elevated mortality rates. There is limited scientific evidence with respect to suboccipital decompression for the management of this condition.

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.

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.

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.

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 .

Manifestaciones oftalmológicas en vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos: análisis retrospectivo de 104 pacientes en un centro de referencia en Argentina / Ophthalmological manifestations in anti-neutrophil cytoplasmic antibodies associated vasculitis: retrospective analysis of 104 patients in a reference center in Argentina

Resumen Introducción: el compromiso oftalmológico (CO) en las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (VAA) varía ampliamente según el subtipo de vasculitis. Objetivos: describir la frecuencia, las formas clínicas y los factores asociados del CO en pacientes con VAA al inicio de la enfermedad. Materiales y métodos: estudio retrospectivo, observacional y analítico, de centro único, que incluyó pacientes con VAA a los cuales se los dividió en dos grupos según la presencia o ausencia de CO al inicio de la enfermedad. Se realizó estadística descriptiva, análisis bivariado y multivariado para evaluar los factores asociados al CO. Resultados: se incluyeron 104 pacientes, 64 (61,5%) eran mujeres. El 49% pertenecía al grupo de granulomatosis con poliangitis (GPA), el 34,6% a poliangitis microscópica (PAM) y el 16,3% a granulomatosis eosinofílica con poliangitis (GEPA). Del total, 30 (28,8%) presentaron CO al inicio de la vasculitis (47% en GPA, 17,6% en GEPA y 8,33% en PAM). El compromiso orbitario se observó exclusivamente en los pacientes con GPA. En el análisis multivariado, las variables que se mantuvieron asociadas de manera independiente al CO en el debut de la enfermedad fueron la elevación de la eritrosedimentación y la trombocitosis. Se observaron secuelas en 9 (30%) de los pacientes con CO. Conclusiones: la frecuencia y la forma clínica del CO dependen del tipo de VAA. Debido a su alta frecuencia y también a la posibilidad de secuelas posteriores, resulta importante su rápida identificación y tratamiento adecuado.

Abstract Introduction: ophthalmological involvement (OI) in vasculitis associated with anti-neutrophil cytoplasmic antibodies (AAV) varies widely depending on the subtype of vasculitis. Objectives: to describe the frequency, clinical manifestations, and ophthalmological sequelae as well as factors associated with ophthalmological involvement in patients with ANCA-associated vasculitis (AAV). Materials and methods: a retrospective and analytical study in patients with AAV was performed. OI was recorded at the beginning of the disease, and patients were divided into two groups according to the presence or absence of OI. Descriptive statistics, bivariate and multivariate analysis were performed to evaluate the factors associated with OI. Results: one hundred and four patients with AAV were included, and 61.5% were women. Forty-nine percent of patients presented granulomatosis with polyangiitis (GPA), 34.6% microscopic polyangiitis (MPA), and 16.3% eosinophilic granulomatosis with polyangiitis (EGPA). Of the total, 30 patients (28.8%) presented OI at the beginning of the disease (47% GPA, 17.6% EGPA and 8.33% MPA). The orbital commitment was observed exclusively in patients with GPA. In the multivariate analysis, erythrosedimentation and thrombocytosis showed an association with OI. Ophthalmological sequelae were observed in 9 (30%) patients with OI. Conclusions: the frequency and clinical manifestations of OI depend on the type of AAV. Because the frequency and the possibility of subsequent sequelae are high, prompt identification and appropriate treatment are necessary.

Transcranial Doppler ultrasonography to evaluate cerebral hemodynamic changes in neurocysticercosis / Ultrasonografía Doppler transcraneal para evaluar cambios hemodinámicos cerebrales en neurocisticercosis

Abstract Background Arteritis is a complication of neurocysticercosis (NCC), which is not well known and could trigger strokes. The transcranial Doppler ultrasound (TCD) is a noninvasive method for detecting, staging, and monitoring cerebrovascular diseases. Nonetheless, the utility of TCD to evaluate cerebral hemodynamic changes, suggesting vasculitis associated with NCC remains uncertain. Objective To evaluate cerebral hemodynamic changes using TCD in patients with subarachnoid and parenchymal NCC. Methods There were 53 patients with NCC evaluated at a reference hospital for neurological diseases included (29 with subarachnoid and 24 with parenchymal). Participants underwent a clinical interview and serology for cysticercosis and underwent TCD performed within 2 weeks of enrollment. Mean flow velocity, peak systolic velocity, end diastolic velocity, and pulsatility index were recorded. Results Among the participants, there were 23 (43.4%) women, with a median age of 37 years (IQR: 29-48). Cerebral hemodynamic changes suggesting vasculitis were detected in 12 patients (22.64%); the most compromised vessel was the middle cerebral artery in 11 (91.67%) patients. There were more females in the group with sonographic signs of vasculitis (10/12, 83.33% vs. 13/41, 31.71%; p = 0.002), and this was more frequent in the subarachnoid NCC group (9/29, 31.03% vs. 3/24, 12.5%; p = 0.187), although this difference did not reach statistical significance. Conclusion Cerebral hemodynamic changes suggestive of vasculitis are frequent in patients with NCC and can be evaluated using TCD.

Resumen Antecedentes La arteritis es una complicación de la neurocisticercosis (NCC), que no siempre se conoce y podría desencadenar enfermedad cerebrovascular. La ultrasonografía Doppler transcraneal (DTC) es un método no invasivo que sirve para detectar y monitorizar enfermedades cerebrovasculares. No obstante, la utilidad de la DTC para evaluar los cambios hemodinámicos cerebrales que sugieren vasculitis asociada a NCC sigue siendo incierta. Objetivo Evaluar los cambios hemodinámicos cerebrales utilizando DTC en pacientes con NCC subaracnoidea y parenquimal. Métodos Se incluyeron 53 pacientes con NCC (29 con subaracnoidea y 24 con parenquimal) evaluados en un hospital de referencia para enfermedades neurológicas. Los participantes se sometieron a una entrevista clínica y serología para cisticercosis y a una DTC realizada dentro de las 2 semanas posteriores a la inscripción. Se registraron la velocidad media del flujo, la velocidad sistólica máxima, la velocidad diastólica final y el índice de pulsatilidad. Resultados Los participantes incluyeron 23 (43,4%) mujeres con una mediana de edad de 37 años (rango intercuartílico [RIC]: 29-48). Se detectaron cambios hemodinámicos cerebrales sugestivos de vasculitis en 12 pacientes (22,64%); el vaso más comprometido fue la arteria cerebral media, en 11 (91,67%) pacientes. Hubo más mujeres en el grupo con signos ecográficos de vasculitis (10/12, 83,33% versus 13/41, 31,71%; p = 0,002), y esto fue más frecuente en el grupo de NCC subaracnoidea (9/29, 31,03% versus 3/24, 12,5%; p = 0,187), aunque esta diferencia no alcanzó significancia estadística. Conclusión Los cambios hemodinámicos cerebrales sugestivos de vasculitis son frecuentes en pacientes con NCC y pueden evaluarse mediante DTC.

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 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.