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.

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

Schõnlein-Henoch purpura in adults: Report of four cases

Schoenlein-Henoch purpura is a systemic small vessel vasculitis mediated by IgA-1 deposition in organs such as the skin, kidney, and gastrointestinal tract; it has been mainly described in children where it has a favourable prognosis. Although much rarer in adulthood it is associated with an increased risk of severe kidney involvement, gastrointestinal com-plications, and prolonged hospital stay. The therapeutic options are wide and vary according to the degree of involvement of the patient and the organ mainly affected.

La púrpura de Schönlein-Henoch es una vasculitis sistêmica de pequeno vaso mediada por depósito de IgA en órganos como la piel, el riñón y el tracto gastrointestinal. Se ha descrito principalmente en niños, grupo de población en el que tiene un pronóstico favorable. Si bien en la edad adulta es mucho menos frecuente, se asocia con un mayor riesgo de compromiso renal severo, complicaciones gastrointestinales y estancia hospitalaria prolongada. Las opciones terapêuticas son amplias y varían según el grado de compromiso del paciente y el órgano más afectado.

Therapeutic effect of mycophenolate mofetil or cyclophosphamide in children with Henoch-Schönlein purpura nephritis of different age groups / 中国当代儿科杂志

OBJECTIVES@#To investigate the difference in the therapeutic effect of mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in children with Henoch-Schönlein purpura nephritis (HSPN) of different age groups.@*METHODS@#A retrospective analysis was conducted on the clinical data of 135 children with HSPN who were treated with MMF or CTX in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from October 2018 to October 2020. According to the immunosuppressant used, they were divided into two groups: MMF group and CTX group, and according to the age, each group was further divided into two subgroups: ≤12 years and >12 years, producing four groups, i.e, the ≤12 years MMF subgroup (n=30), the >12 years MMF subgroup (n=15), the ≤12 years CTX subgroup (n=71), and the >12 years CTX subgroup (n=19). All children were followed up for at least 12 months, and the above groups were compared in terms of clinical outcomes and the incidence rate of adverse reactions.@*RESULTS@#There was no significant difference in the complete response rate between the MMF group and the CTX group after 3, 6, and 12 months of treatment (P>0.05). There were no significant difference in the complete response rate and the incidence rate of adverse reactions between the >12 years MMF subgroup and the ≤12 years MMF subgroup at 3, 6, and 12 months of treatment (P>0.05). The >12 years CTX subgroup had a significantly lower complete response rate than the ≤12 years CTX subgroup at 6 and 12 months of treatment (P<0.05). The >12 years CTX subgroup had a significantly higher incidence rate of adverse reactions than the >12 years MMF subgroup (P<0.05).@*CONCLUSIONS@#The efficacy and adverse reactions of MMF are not associated with age, but the efficacy of CTX is affected by age, with a higher incidence rate of adverse reactions. CTX should be selected with caution for children with HSPN aged >12 years.

Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023) / 中国当代儿科杂志

Kawasaki disease (KD) is an acute self-limiting vasculitis, and it is the most common cause of acquired heart disease in children under 5 years old. One of the improvement goals in pediatric quality control work for the year 2023, as announced by the National Health Commission, is to reduce the incidence of cardiac events and KD-related mortality in children with KD. In order to standardize the diagnosis, treatment, and long-term management practices of KD in China, and effectively prevent and reduce the incidence of coronary artery lesions and long-term adverse effects, the guideline working group followed the principles and methods outlined by the World Health Organization and referenced existing evidence and experiences to develop the "Evidence-based guidelines for the diagnosis and treatment of Kawasaki disease in children in China (2023)". The guidelines address the clinical questions regarding the classification and definition of KD, diagnosis of different types of KD, treatment during the acute phase of KD, application of echocardiography in identifying complications of KD, and management of KD combined with macrophage activation syndrome. Based on the best evidence and expert consensus, 20 recommendations were formulated, aiming to provide guidance and decision-making basis for healthcare professionals in the diagnosis and treatment of KD in children.

Diagnostic yield of bronchoscopic rapid on-site evaluation in severe invasive bronchopulmonary aspergillosis / 中华危重病急救医学

OBJECTIVE@#To explore the diagnostic yield of bronchoscopic rapid on-site evaluation (B-ROSE) in patients with severe invasive bronchopulmonary aspergillosis (IBPA) and provide evidence for starting antifungal treatment before microbiological results were available.@*METHODS@#A prospective cohort study was conducted to select patients with severe pneumonia suspected of IBPA admitted to the respiratory intensive care unit (RICU) in the First Affiliated Hospital of Xinjiang Medical University from June 2014 to June 2022, and those who were primarily infected with other pathogens (such as bacteria, Mycobacterium tuberculosis) at admission were excluded. Whether the antifungal treatment was initiated or not on the basis of the bedside B-ROSE, the B-ROSE was administered as soon as possible within 24 hours after admission to RICU. The current international definition of invasive aspergillosis was used as the gold diagnostic standard, the diagnostic accordance rate, the sensitivity and specificity of B-ROSE were calculated respectively, and the receiver operator characteristic curve (ROC curve) was also plotted, to evaluate the predictive value in diagnosing IBPA.@*RESULTS@#A total of 176 patients with severe pneumonia suspected of IBPA were included in the study. According to international diagnostic standards, there were 81 cases of IBPA and 95 cases of non-IBPA. According to the early diagnosis of B-ROSE, there were 89 cases of IBPA and 87 cases of non-IBPA. The diagnostic accordance rate of B-ROSE was 84.09% (148/176), the area under the ROC curve for B-ROSE in diagnosing severe IBPA was 0.844, the 95% confidence interval (95%CI) was 0.782-0.905, the sensitivity was 87.65%, the specificity was 81.05%, the positive predictive value was 79.78%, the negative predictive value was 88.51%, the rate of underdiagnosis was 12.35% (10/81), and the rate of misdiagnosis was 18.95% (18/95). Compared with the true negative group, the proportion of long-term (≥ 14 days) use of glucocorticoid [70.0% (7/10) vs. 9.1% (7/77), P < 0.01] and the proportion of cases with diabetes [40.0% (4/10) vs. 10.4% (8/77), P < 0.05] were significantly higher in the false negative group (underdiagnosis group). However, B-ROSE of both groups showed mucosal bleeding, congestion and edema [100.0% (10/10) vs. 94.8% (73/77), P > 0.05], indicating that acute mucosal inflammation was non-characteristic. Compared with the true positive group, the proportion of long-term (≥ 14 days) use of glucocorticoid in the false positive group (misdiagnosis group) was significantly reduced [33.3% (6/18) vs. 60.6% (43/71), P < 0.05]. The B-ROSE results showed the proportion of cases with mucosal white spots, black plaques and pseudomembrane was significantly reduced [16.7% (3/18) vs. 52.1% (37/71), P < 0.01] in the misdiagnosed group, which suggest that cases of long-term use of glucocorticoid and cases with B-ROSE showing mucosal white spots, black plaques and pseudomembrane were less likely to be misdiagnosed. The main diseases that were easily misdiagnosed as IBPA included pulmonary tuberculosis (38.9%, 7/18), inflammatory lung adenocarcinoma (27.8%, 5/18) and pulmonary vasculitis (16.7%, 3/18).@*CONCLUSIONS@#Before obtaining microbiological evidence, B-ROSE can assist in decision-making of early anti-aspergillus treatment for severe IBPA. This method is prompt, simple, and has high accuracy and reliability. If B-ROSE lacks characteristic manifestations, especially for severe pneumonia in patients with long-term use of glucocorticoid or diabetes, attention should be paid to the underdiagnosis of IBPA. Diseases such as lung tuberculosis, inflammatory lung adenocarcinoma and lung vasculitis should be vigilant against misdiagnosis as IBPA.

Enfermedad de Buerger o el endotelio como laberinto: a propósito de un caso clínico / Buerger's disease or the endothelium as a labyrinth: case report

La enfermedad de Buerger, también llamada tromboangeítis obliterante, es una enfermedad vascular inflamatoria transmural no aterosclerótica, segmentaria, que afecta pequeños y medianos vasos de las extremidades, de especial asociación etiopatogénica con el tabaquismo. Ocurre comúnmente en hombres menores de 50 años, siendo un diagnóstico diferencial en el escenario de pacientes con isquemia crónica. Se presenta el caso de un hombre de 43 años, con antecedente de tabaquismo pesado, quien cursaba con claudicación intermitente llegando hasta el dolor en reposo, asociado a úlceras de aspecto vasculopático en miembros inferiores, en ausencia de pulsos distales (pedio, tibial posterior); la pletismografía reportó mayor compromiso infrapoplíteo, con circulación colateral segmentaria por arteriografía, sin posibilidad de revascularización endovascular. Se consideró un caso compatible con enfermedad de Buerger, planteándose un manejo híbrido: médico (con agentes antiagregantes) y quirúrgico (con puente femoropoplíteo directo). Se realiza una breve revisión de tema de esta enfermedad de rara aparición

Abstract. Buerger's disease, also called thromboangiitis obliterans, is a non-atherosclerotic, segmental, transmural inflammatory vascular disease affecting small and medium-sized vessels of the extremities with a special etiopathogenic association with smoking; it commonly occurs in men under 50 years of age, and is a differential diagnosis in the setting of patients with chronic ischemia. We describe the case of a 43-year-old man, with a history of heavy smoking, who presented with intermittent claudication reaching pain at rest, associated with ulcers of vasculopathic appearance in the lower limbs, in the absence of distal pulses (pedius, posterior tibial); plethysmography reported greater infrapopliteal involvement, with segmental collateral circulation by arteriography, without the possibility of endovascular revascularization. The case was considered compatible with Buerger's disease, and hybrid management was weighed: medical (with antiplatelet agents) and surgical (with direct femoropopliteal bridging). A brief review of this rare disease is presented

Case report: Takayasu arteritis in a newborn, 5-years follow-up

Takayasu Arteritis (TA) is classified as a large vessel vasculitis, it primarily affects aorta and principal branches. The clinical presentation in pediatric patients is odd and there a few literature about it. The case is about a feminine patient hospitalized after birth with diagnosis of patent ductus arterious (PDA), abnormal widening of the descending aorta, aneurysm of the aortic arch. It is kept under close surveillance for 5 years, with imaging improvement in treatment with methotrexate and immunoglobulin, however she presented relapses on two occasions where the caliber decreased in descending aorta and left iliac artery. We can conclude that Identifying AT findings is important for early diagnosis, medical management, and proper monitoring specifically in pediatric patients where little literature is available.

La arteritis de Takayasu (AT) se clasifica como una vasculitis de grandes vasos desarrollándose sobre todo en aorta y ramas principales. Su presentación en pacientes pediátricos es rara y existe muy poca información en la literatura. Se trata de una paciente femenina hospitalizada al nacimiento, a quien se diagnostica ductus arterioso persistente (DAP), dilatación de aorta descendente y aneurisma de arco aórtico. Se mantiene bajo vigilancia estrecha durante cinco arios, con mejoría imagenológica en tratamiento a base metrotexato e inmunoglobulina, sin embargo, presenta recaídas en dos ocasiones, donde se observa disminución del calibre de aorta descendente y arteria iliaca izquierda. Se puede concluir que el identificar los hallazgos de la AT es importante para realizar un diagnóstico temprano, manejo médico oportuno y vigilancia adecuada, en específico en pacientes pediátricos, de quienes se tiene poca información en la literatura.

Cutaneous polyarteritis nodosa: An atypical simulator. Report of 3 cases and a narrative review of the literature

Objectives: To report the clinical manifestations and therapeutic schemes established in three patients with cutaneous polyarteritis nodosa, as well as to describe the pathophysiology, clinical features, diagnostic criteria, and therapeutic options. Methods: A literature review was performed using Google scholar and PubMed and MeSH terms. There was no limit on the publication date or language for the selection of the articles. Results: Cutaneous polyarteritis nodosa is an uncommon small and medium-sized vessel vasculitis, and is rarely reported in Colombia. Although its pathophysiology is unknown, it is considered to be mediated by the deposition of immunocomplexes in the walls of blood vessels. It can be triggered by infectious agents and be associated with autoimmune diseases. The cutaneous manifestations mainly include subcutaneous painful nodules, livedo reticularis, and ulcers. Myalgia, arthralgia, peripheral neuropathy, and fever can also be present. It represents a diagnostic challenge. Treatment is not standardized and is guided according to the severity. Conclusions: Cutaneous polyarteritis nodosa is a rare entity, with a wide variety of cutaneous manifestations. There is still no specific diagnostic test. Its diagnosis represents a challenge for the dermatologist, and requires multidisciplinary management, in which the rheumatologist plays a fundamental role.

Objetivos: Reportar las diferentes manifestaciones clínicas y los esquemas terapéuticos instaurados en 3 pacientes con poliarteritis nudosa cutánea. Así mismo, describir la fisiopatología, el cuadro clínico, los criterios diagnósticos y las opciones terapéuticas. Métodos: Se realizó una búsqueda de la literatura en Google Académico y PubMed utilizando términos MeSH. En la selección de la bibliografía, la búsqueda no se limitó por fecha de publicación ni por idioma, debido al escaso número de reportes. Resultados: La poliarteritis nudosa cutánea es una vasculitis de pequeño y mediano vaso, infrecuente, poco reportada en Colombia. Su fisiopatología no es del todo conocida, se considera mediada por el depósito de inmunocomplejos en las paredes de vasos sanguíneos; puede ser desencadenada por agentes infecciosos y asociarse con enfermedades autoinmunes. Las manifestaciones cutáneas incluyen principalmente nódulos subcutáneos dolorosos, livedo reticular y úlceras. A nivel extracutáneo podrían encontrarse mialgias, artralgias, neuropatía periférica y fiebre. El diagnóstico representa un desafío. El tratamiento no está estandarizado y es guiado según la severidad de la patología. Conclusión: La poliarteritis nudosa cutánea es una entidad poco frecuente, con una amplia variedad de manifestaciones cutáneas. Hasta el momento, no contamos con una prueba diagnóstica específica. Su diagnóstico representa un reto para el dermatólogo y requiere un manejo multidisciplinario, en el cual el reumatólogo desempeña un papel fundamental.

Tongue Necrosis due to Giant Cell Arteritis / Necrose da língua por arterite de células gigantes

Giant Cell Arteritis is a vasculitis that mainly affects women over 50. The most common manifestations are headache, jaw claudication, and amaurosis. If not diagnosed early, it can lead to rare irreversible ischemic consequences, with tongue necrosis being one of these. We report a case of a previously undiagnosed patient with lateral tongue necrosis who responded well to oral corticosteroid treatment. The diagnosis is clinical, laboratory and histological and may be aided by imaging exams. Initial treatment is with oral corticosteroids, with methotrexate and tocilizumab as alternatives. Diagnostic suspicion and quick start of treatment favorably influence the prognosis of the disease (AU)

Apresentamos um caso de arterite de células gigantes com amaurose, seguida de necrose da língua, diagnosticado clinicamente com boa resposta terapêutica ao corticoide oral. Salientamos que os casos de necrose da língua são extremamente raros. A suspeita diagnóstica de arterite de células gigantes é de fundamental importância e mesmo na impossibilidade de realizar a biópsia de artéria temporal, não se deve retardar o início do tratamento sob pena de acarretar sequelas definitivas ou manifestações mais graves da doença (AU)

Recomendaciones de la Sociedad Argentina de Reumatología en el manejo de la arteritis de células gigantes / Recommendations of the Argentine Society of Rheumatology in the management of giant cell arteritis

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Giant cell arteritis (GCA) is a systemic vasculitis affecting adult patients and involving large and medium vessels. Potential serious complications as blindness may occur and it is considered a medical emergency. The objective of elaborating this guideline was to develop first Argentinian GCA treatment recommendations using GRADE methodology. An expert panel generated clinically meaningful questions addressing aspects of the treatment of GCA in the Population, Intervention, Comparator and Outcome (PICO) format and then a group of methodology experts reviewed and extracted data from literature summarizing available evidence. A patient's focus group discussion took place gathering information on their preferences and experiences. Finally, the vasculitis expert panel, with all the information obtained, voted recommendations here presented.

Guías Argentinas de Vasculitis Suplemento. Tablas arteritis de células gigantes / Argentine Vasculitis Guidelines Supplement. Giant cell arteritis tables

La arteritis de células gigantes (ACG) es una vasculitis sistémica que afecta a personas adultas; compromete vasos arteriales de mediano y gran calibre, con potenciales complicaciones de gravedad, como la ceguera, y es considerada una emergencia médica. El objetivo de estas guías fue desarrollar las primeras recomendaciones argentinas para su tratamiento, basadas en la revisión de la literatura mediante metodología GRADE. Un panel de expertos en vasculitis elaboró las preguntas en formato PICO (población, intervención, comparador y outcomes), y luego un panel de expertos en metodología efectuó la revisión de la bibliografía con la extracción de la evidencia para cada una de las preguntas. Se realizó un focus group de pacientes para conocer sus preferencias y experiencias. Finalmente, con la información recabada, el panel de expertos en vasculitis procedió a la votación de las recomendaciones que a continuación se presentan.

Small vessel vasculitis, different renal outcomes in pediatric patients. Case reports

Vasculitis mainly affects the walls of the blood vessels, and is an uncommon disease in the pediatric population. In general, they are classified according to the EULAR / PreS consensus in children and in adults according to the Chapel-Hill consensus conference. ANCA-associated vasculitis (AAV) is part of small-vessel disease and is represented by granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), microscopic polyangiitis (MPA), and others. The representative renal histopathological findings are focal necrotizing glomerulonephritis with crescents, variable interstitial inflammation, absence of immune complexes, or small deposits of immunoglobulins. Clinically, AAV can manifest with hematuria, proteinuria, high blood pressure, and/or rapidly progressive glomerulonephritis. GPA can severely affect the kidney in 75% of cases. In MPA, renal involvement (75-90%) can be rapid and severe with the possibility of requiring renal replacement therapy in more than half of the patients. Furthermore, up to 25% of patients may have high blood pressure, and the mortality at one year can be up to 85%. In EGPA the renal involvement is usually mild. Three pediatric cases of AAV with different renal outcomes are presented, including the need for renal replacement therapy with the recovery of renal function, kidney transplantation, and death, followed in a fourth level of care institution in Colombia.

Las vasculitis, patologías cuyo hallazgo principal es la afectación de las paredes de los vasos sanguíneos, se presentan de forma infrecuente en la población pediátrica. En general, en niños se clasifican de acuerdo con el consenso de la EULAR/PReS, y en adultos, según la Conferencia de Consenso de Chapel-Hill. Las vasculitis asociadas con ANCA (VAA) hacen parte de las vasculitis de pequeños vasos y están representadas por la granulomatosis con poliangeítis (GPA), la granulomatosis eosinofílica con poliangeítis (EGPA) y la poliangeítis microscópica (PAM), entre otras. A nivel renal, los hallazgos histopatológicos representativos son la glomerulonefritis focal necrotizante con media luna, inflamación intersticial variable, ausencia de complejos inmunes o pequeños depósitos de inmunoglobulinas. Clínicamente, las VAA pueden manifestarse con hematuria, proteinuria, hipertensión arterial o glomerulonefritis rápidamente progresiva. La GPA puede afectar de forma severa el riñón en el 75% de los casos, mientras que, en la PAM, el compromiso renal (75-90%) puede ser rápido y severo con posibilidad de requerir terapia de reemplazo renal en más de la mitad de los pacientes. Además, hasta el 25% de los casos puede tener hipertensión arterial, con una mortalidad a un ario de 85%. En la EGPA, el compromiso renal suele ser leve. Se presentan 3 casos pediátricos de VAA con diferentes desenlaces renales, que incluyen necesidad de terapia de reemplazo renal con recuperación de función renal, trasplante renal y muerte, seguidas en una institución de IV nivel del suroccidente colombiano.

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

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

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

Vasculitis crioglobulinémica por hiperviscosidad y formación de criogel como debut de mieloma múltiple. Caso clínico / Cryoglobulinemic vasculitis due to hyperviscosity and cryogel formation as the onset of multiple myeloma. Report of one case

Multiple Myeloma (MM) is characterized by a clonal expansion of plasma cells in the bone marrow. These cells typically produce a monoclonal immunoglobulin, and its symptoms arise either from plasma cell infiltration in several organs, or secondary to the presence of a monoclonal protein peak. Symptoms can be summarized by the acronym CRAB (hypercalcemia, renal failure, anemia and bone lesions). Sometimes, in the setting of a protein secreting monoclonal gammopathy, formation of cryoglobulins develops. Cryoglobulins are plasma proteins that precipitate at low temperatures, forming a cold - induced precipitate at small vessels, causing a wide range of clinical manifestations. We report a female consulting for ulcers lasting 2 months in the left foot associated with purpuric lesions in both lower limbs. Protein electrophoresis showed a monoclonal peak in the gamma region. Bone marrow aspirate showed 27% of plasma cells with kappa chain restriction by cytometry. The presence of cryoglobulins was confirmed. The patient was treated with dexamethasone and bortezomib, with a progressive healing of lower limb lesions and disappearance of cryoglobulins. She was discharged in good conditions.

Vasculitis reumatoidea como manifestación extraarticular en dos casos / Rheumatoid vasculitis as an extra articular manifestation in two cases

La vasculitis reumatoidea es un proceso inflamatorio poco frecuente que confiere una morbilidad y mortalidad significativa en pacientes con artritis reumatoidea (AR). Presenta una incidencia de 0,7 a 5,4% de los casos de AR y hasta un 40% de mortalidad a 5 años, siendo la manifestación extraarticular de mayor gravedad1,2. Se caracteriza por el desarrollo de vasculitis necrotizante; ocurre típicamente en pacientes masculinos con AR seropositiva de larga data, nodular y erosiva, tabaquistas y, en general, tiene mal pronóstico. Se presenta una serie de casos, paciente masculino y femenino con AR y manifestaciones extraarticulares, necrosis digital y compromiso multiorgánico, interpretados como cuadros de vasculitis reumatoidea, realizando tratamiento de referencia con inmunosupresores.

Rheumatoid vasculitis is a rare inflammatory process that confers significant morbidity and mortality in patients with rheumatoid arthritis (RA); it has an incidence of 0.7 to 5.4% of RA cases and up to 40% mortality at five years, making it the most serious of all the extra-articular manifestations of RA1,2. It is characterized by the development of necrotizing vasculitis, it typically occurs in male patients with long-standing seropositive RA, erosive nodular, smokers and generally has a poor prognosis. A series of cases is presented, male and female with RA and extra-articular manifestations, digital necrosis and multiorgan system involvement, interpreted as rheumatoid vasculitis, undergoing treatment with immunosuppressants.

Miositis por Bartonella henselae: caso clínico en una adolescente / Bartonella henselae myositis: case report in an adolescent

La miositis de origen vírico o bacteriano es frecuente en la edad pediátrica. Causa dolor muscular y debilidad, con fiebre y malestar general. Una causa es la infección por Bartonella henselae, bacteria implicada en la enfermedad por arañazo de gato que, a veces, causa afectación multisistémica. Se presenta el caso de una adolescente que acudió al servicio de urgencias por mialgia intensa, malestar, adelgazamiento y esplenomegalia. En el labortorio se observaron parámetros inflamatorios elevados. Refería contacto con un gato. Entre los estudios realizados, la resonancia magnética (RM) de miembros inferiores mostró una imagen compatible con miositis inflamatoria bilateral. En la RM abdominal, se observaron tres lesiones esplénicas no detectadas previamente y el fondo de ojo mostraba una lesión compatible con oclusión arterial retiniana o vasculitis. Se indicó tratamiento antibiótico por vía intravenosa durante 21 días con cefotaxima y cloxacilina, tras los cuales desaparecieron los signos y síntomas, aunque los reactantes inflamatorios persistieron elevados. Con base en el cuadro clínico (miositis + coriorretinitis + absceso esplénico) se pensó en una posible infección por B. henselae y se inició tratamiento oral con azitromicina y rifampicina durante 14 días. Luego del tratamiento, los valores de laboratorio fueron normales, así como la RM de control, y se constató una IgG positiva para la bacteria

Infectious myositis, whether viral or bacterial, is frequent in pediatric age. It causes muscle pain and weakness, associated with fever and general malaise. One cause is Bartonella henselae, responsible for cat scratch disease, which sometimes causes systemic symptoms. We report the case of an adolescent who came to the emergency room with intense myalgia, malaise, weight loss and splenomegaly. Blood tests showed high inflammatory markers. She had been in touch with a cat. Studies were carried out including: lower limbs MRI suggestive of bilateral inflammatory myositis, abdominal MRI with three previously undetected splenic lesions and dilated fundus examination that showed possible retinal arterial occlusion or vasculitis. After 21 days of intravenous antibiotic therapy (cefotaxime + cloxaciline), she became asymptomatic, but inflammatory markers remained high. Suspecting Bartonella henselaeinfection (myositis + chorioretinitis + splenic abscess), oral azithromycin and rifampicin were prescribed for 14 days. Blood tests and control MRI became normal, and IgG was positive.

Rol de las imágenes en la patología arterial no ateroesclerótica poco frecuente en el abdomen / Role of images in rare vascular pathology in the abdomen

Resumen La patología arterial no ateromatosa constituye un grupo de patologías poco frecuentes, pero de gran importancia debido a su morbilidad y mortalidad asociadas. La presentación clínica de estas entidades es inespecífica, por lo que el estudio inicial con imágenes es fundamental. Debido a esto, es muy importante reconocer los signos radiológicos característicos de cada una en las diversas modalidades imagenológicas para poder sospechar y orientar el diagnóstico.

Abstract Non-atheromatous arterial pathology constitutes a group of infrequent pathologies but of great importance due to their associated morbidity and mortality. In general, the clinical presentation of these pathologies is quite nonspecific, so the clinician decides to perform imaging studies thinking of more common entities. It is for this reason that it is very important to recognize characteristic radiological signs in the various imaging modalities to be able to suspect and orient a pathology of this kind.

Síndrome pulmón-riñón en pacientes con hallazgos incidentales de agentes infecciosos / Pulmonary-renal syndrome in patients with incidental findings of infectious agents

El síndrome pulmón-riñón (SPR) o síndrome reno-pulmonar es la combinación de glomerulonefritis aguda rápidamente progresiva (GNARP) y hemorragia alveolar difusa (HAD) de causa autoinmune. El SPR fue inicialmente descrito por Goodpasture en el contexto del síndrome anti-membrana basal glomerular (MBG). Actualmente, las vasculitis asociadas a ANCA (VAA) explican el 60% (rango 56­77.5%) de casos, el síndrome de Goodpasture el 15% (12.5­17.5%), y un 10% de casos se deben a otras causas. El SPR presenta un gran espectro clínico, desde la capilaritis pulmonar fulminante con HAD y falla respiratoria aguda, hasta formas más sutiles de enfermedad sólo detectables mediante lavado bronquio-alveolar (LBA). El objetivo de este estudio es presentar la primera serie peruana de SPR asociada a agentes infecciosos. Reportamos 3 casos, dos correspondieron a lupus eritematoso sistémico y uno a vasculitis asociada a poliangeítis microscópica. El primer caso se asoció a sobreinfección por C. tropicalis; el segundo caso a A. fumigatus y C. albicans; y el tercero a infestación por A. lumbricoides. Todos los casos se presentaron en mujeres, requirieron soporte ventilatorio invasivo y hemodiálisis, y dos resultaron letales. Concluimos que, el SPR es una condición clínica grave comúnmente asociada a sobreinfecciones o infestaciones, y que conlleva una elevada morbilidad y mortalidad. Puesto que no existen características clínicas específicas, resulta crucial tener un alto índice de sospecha. Las investigaciones pertinentes­pruebas inmunológicas, imagenológicas, y biopsia cutánea, renal y/o pulmonar­para precisar la etiología deben realizarse sin demora ya que el tratamiento precoz puede cambiar el pronóstico de estos pacientes(AU)

Pulmonary-renal syndrome (PRS) or reno-pulmonary syndrome is the combination of acute rapidly progressive glomerulonephritis (RPGNARP) and autoimmune diffuse alveolar hemorrhage (DAH). RPS was initially described by Goodpasture in the context of anti-glomerular basement membrane (GBM) syndrome. Currently, ANCA-associated vasculitides (AAV) explain 60% (range 56­77.5%) of cases, Goodpasture syndrome 15% (12.5­17.5%), and 10% of cases are due to other causes. PRS presents a wide clinical spectrum, from fulminant pulmonary capillaritis with ADH and acute respiratory failure, to subtler forms of disease that can only be detected by bronchoalveolar lavage (BAL). The objective of this study is to present the first Peruvian series of SPR associated with infectious agents. We report 3 cases, two corresponded to systemic lupus erythematosus and one to vasculitis associated with microscopic polyangiitis. The first case was associated with superinfection by C. tropicalis; the second case to A. fumigatus and C. albicans; and the third to infestation by A. lumbricoides. All cases occurred in women, required invasive ventilatory support and haemodialysis, and two were fatal. We conclude that SPR is a serious clinical condition commonly associated with superinfections or infestations, and that it carries high morbidity and mortality. Since there are no specific clinical features, a high index of suspicion is crucial. Relevant investigations­immunological tests, imaging tests, and skin, kidney, and/or lung biopsies­to specify the etiology should be carried out without delay, since early treatment can change the prognosis of these patients(AU)