Navigating the diagnostic maze: A case presentation of C1q vasculitis mimicking hypocomplementemic urticarial vasculitis in a patient with systemic lupus erythematosus.

In rare instances, patients with SLE may exhibit atypical clinical manifestations, such as Hypocomplementemic Urticarial Vasculitis, which can pose diagnostic challenges. Here, we present a case report of a 28-year-old female with a history of SLE with lupus nephritis clase IV who developed HUV-like symptoms, ultimately leading to a diagnosis of C1q Vasculitis. This case underscores the importance of considering C1q Vasculitis in SLE patients presenting with HUV-like features and highlights Rituximab as a promising therapeutic option for managing this rare condition.

Clinical, radiological and laboratory characteristics of central nervous system histoplasmosis: A systematic review of a severe disease.

BACKGROUND: The knowledge of central nervous system (CNS) histoplasmosis is limited to case reports and series. OBJECTIVES: Our objective was to synthesise clinical, radiological and laboratory characteristics of CNS histoplasmosis to improve our understanding of this rare disease. METHODS: We performed a systematic review using Pubmed/MEDLINE, Embase and LILACS databases accessed on March 2023 without publication date restrictions. Inclusion criteria comprised: (1) histopathological, microbiological, antigen or serological evidence of histoplasmosis; (2) CNS involvement based on cerebrospinal fluid pleocytosis or neuroimaging abnormalities. We classified the certainty of the diagnosis in proven (CNS microbiological and histopathological confirmation), probable (CNS serological and antigen confirmation) or possible (non-CNS evidence of histoplasmosis). Metaproportion was used to provide a summary measure with 95% confidence intervals for the clinical, radiological and laboratory characteristics. Chi-squared test was used to compare mortality between pairs of antifungal drugs. RESULTS: We included 108 studies with 298 patients. The median age was 31 years, predominantly male, and only 23% were immunocompromised (134/276, 95%CI: 3-71), mainly due to HIV infection. The most common CNS symptom was headache (130/236, 55%, 95%CI: 49-61), with a duration predominantly of weeks or months. Radiological presentation included histoplasmoma (79/185, 34%, 95%CI: 14-61), meningitis (29/185, 14%, 95%CI: 7-25), hydrocephalus (41/185, 37%, 95%CI: 7-83) and vasculitis (18/185, 6%, 95%CI: 1-22). There were 124 proven cases, 112 probable cases and 40 possible cases. The majority of patients presented positive results in CNS pathology (90%), serology (CSF: 72%; serum: 70%) or CSF antigen (74%). Mortality was high (28%, 56/198), but lower in patients who used liposomal amphotericin B and itraconazole. Relapse occurred in 13% (23/179), particularly in HIV patients, but less frequently in patients who used itraconazole. CONCLUSION: Central nervous system histoplasmosis usually presents subacute-to-chronic symptoms in young adults. Neuroimaging patterns included not only focal lesions but also hydrocephalus, meningitis and vasculitis. Positive results were commonly found in CSF antigen and serology. Mortality was high, and treatment with liposomal amphotericin B followed by itraconazole may decrease mortality.

Endothelial Dysfunction, Inflammation and Coronary Artery Disease: Potential Biomarkers and Promising Therapeutical Approaches.

Coronary artery disease (CAD) and its complications are the leading cause of death worldwide. Inflammatory activation and dysfunction of the endothelium are key events in the development and pathophysiology of atherosclerosis and are associated with an elevated risk of cardiovascular events. There is great interest to further understand the pathophysiologic mechanisms underlying endothelial dysfunction and atherosclerosis progression, and to identify novel biomarkers and therapeutic strategies to prevent endothelial dysfunction, atherosclerosis and to reduce the risk of developing CAD and its complications. The use of liquid biopsies and new molecular biology techniques have allowed the identification of a growing list of molecular and cellular markers of endothelial dysfunction, which have provided insight on the molecular basis of atherosclerosis and are potential biomarkers and therapeutic targets for the prevention and or treatment of atherosclerosis and CAD. This review describes recent information on normal vascular endothelium function, as well as traditional and novel potential biomarkers of endothelial dysfunction and inflammation, and pharmacological and non-pharmacological therapeutic strategies aimed to protect the endothelium or reverse endothelial damage, as a preventive treatment for CAD and related complications.

Understanding the Cryoglobulinemias.

PURPOSE OF THE REVIEW: Cryoglobulins are immunoglobulins with the ability to precipitate at temperatures <37 °C. They are related to hematological disorders, infections [especially hepatitis C virus (HCV)], and autoimmune diseases. In this article, the state of the art on Cryoglobulinemic Vasculitis (CV), in a helpful and schematic way, with a special focus on HCV related Mixed Cryoglobulinemia treatment are reviewed. RECENT FINDINGS: Direct - acting antivirals (DAA) against HCV have emerged as an important key in HCV treatment to related Cryoglobulinemic Vasculitis, and should be kept in mind as the initial treatment in non-severe manifestations. On the other hand, a recent consensus panel has published their recommendations for treatment in severe and life threatening manifestations of Mixed Cryoglobulinemias. HCV-Cryoglobulinemic vasculitis is the most frequent form of CV. There are new treatment options in HCV-CV with DAA, with an important number of patients achieving complete response and sustained virologic response (SVR). In cases of severe forms of CV, treatment with Rituximab and PLEX are options. The lack of data on maintenance therapy could impulse future studies in this setting.

Successful multiple-exchange peritoneal dialysis in a patient with severe hematological toxicity by methotrexate: case report and literature review / Diálise peritoneal com múltiplas trocas bem-sucedida em paciente com grave toxicidade hematológica por metotrexato: relato de caso e revisão de literatura

Abstract Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.

Resumo Apesar de sua toxicidade, o metotrexato é um medicamento eficaz no controle de várias doenças. A mielossupressão, um de seus principais efeitos adversos, aumenta em gravidade e frequência nos pacientes com insuficiência renal. Apresentamos o caso de um homem de 68 anos de idade com doença renal terminal relacionada à vasculite associada ao ANCA em diálise peritoneal, que recebeu a medicação em dose baixa em função da atividade da doença e que teve como complicação pancitopenia grave com mucosite, tratada com medidas de suporte e diálise peritoneal com múltiplas trocas. Revisamos 20 casos publicados até o presente momento sobre pancitopenia associada a metotrexato em pacientes em diálise. Foi identificada alta morbidade e mortalidade, razão pela qual seu uso nesse tipo de paciente não é recomendado. No entanto, quando esta complicação ocorre, uma opção terapêutica pode ser o uso de diálise peritoneal com múltiplas trocas, além da terapia de suporte para toxicidade medicamentosa. Maiores estudos são necessários para demonstrar o papel da diálise peritoneal com múltiplas trocas na remoção desse medicamento.

Successful multiple-exchange peritoneal dialysis in a patient with severe hematological toxicity by methotrexate: case report and literature review.

Methotrexate is an effective medication to control several diseases; however, it can be very toxic, being myelosuppression one of its main adverse effects, which increases in severity and frequency in patients with renal failure. We present the case of a 68-year-old man with chronic, end-stage renal disease associated with ANCA vasculitis, under treatment with peritoneal dialysis, who received the medication at a low dose, indicated by disease activity, which presented as a complication with severe pancytopenia with mucositis that improved with support measures and multiple-exchange peritoneal dialysis. We reviewed 20 cases published to date of pancytopenia associated with methotrexate in patients on dialysis and found high morbidity and mortality, which is why its use in this type of patient is not recommended. However, when this complication occurs, a therapeutic option could be the use of multiple-exchange peritoneal dialysis in addition to supportive therapy for drug-related toxicity, although it is recognized that studies are required to show the role of multiple-exchange peritoneal dialysis in the removal of this medication.

Vascular sclerosing effects of bleomycin on cutaneous veins: a pharmacopathologic study on experimental animals.

BACKGROUND:: Varicose veins and the complications of venous disease are common disorders in humans. OBJECTIVE:: To study the effects of bleomycin as a potential new sclerosing agent and its adverse events in treating varicose veins. METHODS:: Bleomycin-loaded liposomes 0.1ml was injected in the dorsal ear veins of white New Zealand rabbits. Sodium tetradecyl sulfate was used as a positive control. Normal saline was used as negative control. The blood vessels of the treated ears were photographed before and at one hour and two, eight and 45 days after treatment. Biopsies from the treated areas were obtained for histological examination. Blood samples were collected to determine any possible toxicity. RESULTS:: Bleomycin by itself was ineffective; therefore, liposomes were used as a vector to deliver bleomycin to the vein lumen. Subsequently, bleomycin started showing its sclerosing effects. Toxicity monitoring showed no apparent hematologic, pulmonary, hepatic or renal toxicities. This study revealed that bleomycin induced vasculitis, which led to vascular occlusion, which was observed on day 1 and day 8. No bleomycin-related injury was noted by histopathological examination of lung sections. The calculation of the lung/body weight coefficient indicated that edema was present in the experimental groups compared with the negative and positive controls. STUDY LIMITATIONS:: Relatively small number of experimental animals used. CONCLUSIONS:: This study showed that bleomycin-loaded liposomes were able to induce vasculitis and vascular occlusion without any toxicity or complications. It might be useful, hence, to treat patients suffering from Varicose veins and other ectatic vascular diseases with this agent.

Vascular sclerosing effects of bleomycin on cutaneous veins: a pharmacopathologic study on experimental animals

Abstract: Background: Varicose veins and the complications of venous disease are common disorders in humans. Objective: To study the effects of bleomycin as a potential new sclerosing agent and its adverse events in treating varicose veins. Methods: Bleomycin-loaded liposomes 0.1ml was injected in the dorsal ear veins of white New Zealand rabbits. Sodium tetradecyl sulfate was used as a positive control. Normal saline was used as negative control. The blood vessels of the treated ears were photographed before and at one hour and two, eight and 45 days after treatment. Biopsies from the treated areas were obtained for histological examination. Blood samples were collected to determine any possible toxicity. Results: Bleomycin by itself was ineffective; therefore, liposomes were used as a vector to deliver bleomycin to the vein lumen. Subsequently, bleomycin started showing its sclerosing effects. Toxicity monitoring showed no apparent hematologic, pulmonary, hepatic or renal toxicities. This study revealed that bleomycin induced vasculitis, which led to vascular occlusion, which was observed on day 1 and day 8. No bleomycin-related injury was noted by histopathological examination of lung sections. The calculation of the lung/body weight coefficient indicated that edema was present in the experimental groups compared with the negative and positive controls. Study limitations: Relatively small number of experimental animals used. Conclusions: This study showed that bleomycin-loaded liposomes were able to induce vasculitis and vascular occlusion without any toxicity or complications. It might be useful, hence, to treat patients suffering from Varicose veins and other ectatic vascular diseases with this agent.

Informe de evaluación científica basada en la evidencia disponible: vasculitis / Scientific evaluation report based on available evidence: vasculitis

INTRODUCCIÓN: Las vasculitis son un conjunto heterogéneo de enfermedades sistémicas caracterizadas por la inflamación y en cierta medida destrucción de los vasos sanguíneos, por ende, afectan la perfusión o permeabilidad de dichos vasos (1,2). La vasculitis asociada a ANCA se caracteriza por ser una vasculitis necrotizante de pequeños vasos (arteriola, capilar y vénula) con afectación ocasional de arterias y venas viscerales, sin depósitos inmunes en las lesiones y asociadas a la presencia de autoanticuerpos contra el citoplasma de los neutrófilos, cuyos antígenos son la mieloperoxidasa (MPO) y la proteinasa 3 (PR3). TECNOLOGÍA SANITARIA ANALIZADA: Rituximab. EFICACIA DE LOS TRATAMENTOS: Entre las conclusiones que se pueden extraer acerca de la efectividad del Rituximab para el tratamiento de las Vasculitis asociadas a ANCA se encuentran: - Rituximab podría aumentar levemente la tasa de inducción de remisión. La certeza de la evidencia es baja. - Existe incertidumbre sobre si Rituximab podría aumentar o disminuir la mortalidad general. La certeza de la evidencia es muy baja. - Rituximab podría aumentar la incidencia de infecciones. La certeza de la evidencia es baja. - Existe incertidumbre sobre si Rituximab podría favorecer o no el desarrollo de neoplasias. La certeza de la evidencia es muy baja. EVALUACIÓN ECONÓMICA: No se evaluó esta dimensión, en conformidad con el Título III De las Evaluaciones Favorables de la Norma Técnica N° 0192 del Ministerio de Salud, sobre el proceso de evaluación científica de la Evidencia establecido en el artículo 7° de la ley N°20.850. CONCLUSIÓN: Para dar cumplimiento al artículo 28° del Reglamento que establece el proceso destinado a determinar los diagnósticos y tratamientos de alto costo con Sistema de Protección Financiera, según lo establecido en los artículos 7°y 8° de la ley N°20.850, aprobado por el decreto N°13 del Ministerio de Salud, se concluye que el presente informe de evaluación se considera no favorable, dado que la evidencia presentada es de certeza muy baja, de acuerdo a lo establecido en el Título III. de las Evaluaciones Favorables de la Norma Técnica N° 0192 de este mismo Ministerio.

Informe de evaluación científica en la evidencia disponible: vasculitis / Scientific evaluation report on the available evidence: vasculitis

INTRODUCCIÓN: Las vasculitis son un conjunto heterogéneo de enfermedades sistémicas caracterizadas por la inflamación y en cierta medida destrucción de los vasos sanguíneos, por ende, afectan la perfusión o permeabilidad de dichos vasos. La vasculitis asociada a ANCA se caracteriza por ser una vasculitis necrotizante de pequeños vasos (arteriola, capilar y vénula) con afectación ocasional de arterias y venas viscerales, sin depósitos inmunes en las lesiones y asociadas a la presencia de autoanticuerpos contra el citoplasma de los neutrófilos, cuyos antígenos son la mieloperoxidasa (MPO) y la proteinasa. Dentro de la clasificación de las vasculitis, el consenso internacional de Chapel Hill del 2012 establece una agrupación de dichas enfermedades de la siguiente manera: Vasculitis de grandes vasos, vasculitis de medianos vasos, vasculitis de pequeños vasos (asociadas a anticuerpos ante citoplasma de neutrófilos (ANCA), asociadas a complejos inmunes), vasculitis de vaso variable, vasculitis de un solo órgano, vasculitis asociadas a enfermedades sistémicas y vasculitis asociadas con una probable etiología. La vasculitis asociada a ANCA se caracteriza por ser una vasculitis necrotizante de pequeños vasos (arteriola, capilar y vénula) con afectación ocasional de arterias y venas viscerales, sin depósitos inmunes en las lesiones y asociadas a la presencia de autoanticuerpos contra el citoplasma de los neutrófilos, cuyos antígenos son la mieloperoxidasa (MPO) y la proteinasa 3 (PR3). Su nomenclatura fue actualizada en 2012 e incluye a la "granulomatosis con poliangiitis" (GPA, previamente granulomatosis de Wegener), la poliangiitis microscópica (MPA), la "eosinofilia y granulomatosis con poliangiitis" (EGPA, previamente enfermedad de Churg-Strauss) y la vasculitis limitada a riñón. Las incidencias anuales de GPA y MPA son relativamente similares y varían entre 2 y 12 casos por millón de habitantes al año, con una prevalencia que se encuentra entre 23 a 160 casos por millón de personas. Por su parte, EPA resulta más rara que las condiciones recién nombradas, con una incidencia de 1-4 casos por millón de habitantes y una prevalencia de aproximadamente 10-20 casos por millón de personas. TECNOLOGÍAS SANITARIA DE INTERÉS: Rituximab es un anticuerpo monoclonal quimérico murino/humano, obtenido por ingeniería genética, que representa una inmunoglobulina glucosilada con las regiones constantes de la IgG1 humana y las secuencias de la región variable de las cadenas ligeras y cadenas pesadas murinas. Este anticuerpo se produce a partir de un cultivo en suspensión de células de mamífero (células de ovario de hámster chino) y se purifica mediante cromatografía de afinidad y de intercambio iónico, incluyendo procedimientos específicos de inactivación y de eliminación viral. EFICACIA DE LOS TRATAMIENTOS: Entre las conclusiones que se pueden extraer acerca de la efectividad del Rituximab para el tratamiento de las Vasculitis asociadas a ANCA se encuentran: - Rituximab podría aumentar levemente la tasa de inducción de remisión. La certeza de la evidencia es baja. Existe incertidumbre sobre si Rituximab podría aumentar o disminuir la mortalidad general. La certeza de la evidencia es muy baja. Rituximab podría aumentar la incidencia de infecciones. La certeza de la evidencia es baja. Existe incertidumbre sobre si Rituximab podría favorecer o no el desarrollo de neoplasias. La certeza de la evidencia es muy baja. ALTERNATIVAS DISPONIBLES: Enfermedad localizada: La terapia de inducción contempla metotrexato y corticosteroides. Por otro lado, la terapia de mantención considera corticosteroides más azatioprina o leflunomida. Enfermedad sistémica precoz: En la inducción se recomienda metotrexato o ciclofosfamida y corticosteroides. Por su parte, en la terapia de mantención comprende el tratamiento con corticosteroides más azatioprina. Enfermedad generalizada: La recomendación para la inducción es ciclofosfamida y corticosteroides, mientras que para la terapia de mantenimiento considera corticosteroides más azatioprina o micofenolato mofetilo. Enfermedad severa: Se recomienda en la inducción utilizar ciclofosfamida y corticosteroides más plasmaféresis y en la terapia de mantenimiento corticosteroides más azatioprina o micofenolato mofetilo. Enfermedad refractaria. En la inducción se recomiendan las siguientes alternativas de tratamiento inmunoglobulinas, micofenolato, 15-deoxyspergualina, globulina antitimocítica, infliximab, rituximab. Mientras que en la terapia de mantención no existe consenso. RESULTADOS DE LA BÚSQUEDA DE EVIDENCIA: Se encontraron dos revisiones sistemáticas relevantes para la pregunta que se está intentando responder, estas fueron publicadas en los años 2014 y 2015. Además, se encontró un Resumen de Epistemonikos (11) sobre la efectividad del Rituximab para inducir la remisión en las vasculitis asociadas a ANCA, que sigue métodos similares a los que se utilizan en los informes de evidencia para Ley Ricarte Soto. Dicho Resumen de Epistemonikos considera a la revisión sistemática de Silva-Fernández et al 2014 como eje de su análisis. Luego de la realización de este Resumen, se publicó una Revisión Cochrane que se focalizó en intervenciones para adultos con vasculitis renal. CONCLUSIÓN: Para dar cumplimiento al artículo 28° del Reglamento que establece el proceso destinado a determinar los diagnósticos y tratamientos de alto costo con Sistema de Protección Financiera, según lo establecido en los artículos 7°y 8° de la ley N°20.850, aprobado por el decreto N°13 del Ministerio de Salud, se concluye que el presente informe de evaluación se considera no favorable, dado que la evidencia presentada es de certeza muy baja, de acuerdo a lo establecido en el Título III. de las Evaluaciones Favorables de la Norma Técnica N° 0192 de este mismo Ministerio.

O rituximabe como uma opção para pacientes com vasculite sistêmica grave refratária à terapia convencional: relato de sete casos e revisão de literatura / Rituximab as an alternative for patients with severe systemic vasculitis refractory to conventional therapy: report of seven cases and literature review

Resumo O maior entendimento das bases fisiopatológicas e do comportamento das vasculites sistêmicas, aliado ao desenvolvimento de regimes terapêuticos com perfil de segurança e eficácia cada vezes melhores, modificou drasticamente o prognóstico dos pacientes diagnosticados com essas entidades clínicas. Recentemente, o emprego do rituximabe no tratamento de pacientes com vasculites ANCA associadas em ensaios clínicos randomizados se mostrou uma opção importante em casos selecionados, especialmente pacientes refratários ou intolerantes à terapia-padrão com ciclofosfamida e corticosteroides. O presente artigo traz o relato de sete casos de vasculites sistêmicas com tratamento bem-sucedido com rituximabe.

Abstract The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids. This article presents the report of seven cases of systemic vasculitis successfully treated with rituximab.

Rituximab as an alternative for patients with severe systemic vasculitis refractory to conventional therapy: report of seven cases and literature review.

The greater understanding of pathophysiology and behavior of systemic vasculitis, together with the development of therapeutic regimens with increasingly better safety and efficacy profiles, dramatically changed the prognosis of patients diagnosed with these clinical entities. Recently, the use of rituximab in the treatment of patients with ANCA-associated vasculitis in randomized clinical trials showed an important alternative in selected cases, especially patients refractory or intolerant to standard therapy with cyclophosphamide and corticosteroids. This article presents the report of seven cases of systemic vasculitis successfully treated with rituximab.

Inflammatory myopathy as the initial presentation of cryoglobulinaemic vasculitis.

Cryoglobulinaemic vasculitis is characterised by immunoglobulin deposition at low temperatures. The most common manifestations are cutaneous involvement, arthralgias, Raynaud's phenomenon, peripheral neuropathy and renal disease. Myopathy is unusual and only a few cases have been reported. Here, we present a 31-year-old woman who developed progressive muscle weakness involving upper and lower extremities, dysphagia, paraesthesias and palpable purpura. Diagnostic studies revealed elevated creatine kinase, diffuse myopathic and sensorimotor axonal neuropathy on electromyography and nerve conduction studies, and inflammatory myopathy on muscle biospsy. Cryoglobulin levels were elevated on two occasions. She responded favourably to cyclophosphamide and high-dose corticosteroids. Cyclophosphamide was continued for 1 year followed by methotrexate. Prednisone was gradually tapered and discontinued 1 year later. She remained in clinical remission after 4 years of follow-up. This case suggests that cryoglobulinaemia should be considered in the differential diagnosis of a patient presenting with inflammatory myopathy.

[Meningovascular neurosyphilis with basilar artery thrombosis, a case report and literature review]. / Neurosífilis meningovascular con trombosis de la arteria basilar.

Herein a case is described of a 54-years old patient, HIV negative, with cerebro-vascular disease by basilar artery thrombosis secondary to meningovascular neurosyphilis. Neurosyphilis is the impairment at any stage of the central nervous system by Treponema pallidum subspecies pallidum and includes asymptomatic and symptomatic forms of infection. The presentation can take many forms, depending on the location and extent of tissue damage. The currently recommended treatment is crystalline penicillin, 4 million units every 4 hours for 14 days.

Neurosífilis meningovascular con trombosis de la arteria basilar / Meningovascular neurosyphilis with basilar artery thrombosis, a case report and literature review

Se presenta el caso clínico de un paciente de 54 años, negativo para VIH, con enfermedad cerebrovascular por trombosis de la arteria basilar, secundaria a neurosífilis meningovascular. La neurosífilis es el compromiso del sistema nervioso central por Treponema pallidum subespecie pallidum en cualquier estadio de la entidad e incluye las formas asintomáticas y sintomáticas de la infección; sus formas de presentación son diversas y dependen de la localización y la extensión de las lesiones. La recomendación actual es el tratamiento con 4 millones de unidades de penicilina cristalina cada 4 horas por 14 días.

Herein a case is described of a 54-years old patient, HIV negative, with cerebro-vascular disease by basilar artery thrombosis secondary to meningovascular neurosyphilis. Neurosyphilis is the impairment at any stage of the central nervous system by Treponema pallidum subspecies pallidum and includes asymptomatic and symptomatic forms of infection. The presentation can take many forms, depending on the location and extent of tissue damage. The currently recommended treatment is crystalline penicillin, 4 million units every 4 hours for 14 days.

Vasculitides throughout history and their clinical treatment today.

Therapeutic management of the vasculitides is closely linked to modern rheumatologic advances, particularly as it relates to the discovery and first clinical use of glucocorticoids. These compounds were introduced in the late-1940s for the treatment of rheumatoid arthritis, but soon after, clinicians in Europe and the United States realized that they could have a significant positive impact in systemic vasculitides. However, once it was realized that glucocorticoid use was associated with a high degree of morbidity, the search for better immunosuppressive agents with similar efficacy but improved safety profiles was on. During the past several years, several agents have been utilized for the therapeutic management of systemic vasculitides, and the list keeps growing with the development of newer compounds that have retained efficacy but with a better safety profile.

Fatal rhabdomyolysis in systemic lupus erythematosus.

The authors describe herein the sixth lupus case that evolved with rhabdomyolysis. A 36-year-old woman with systemic lupus erythematosus was admitted to our hospital with malaise, myalgia, dysphagia, fever, preserved muscle strength, leukocytosis (15,600 cells), and increased creatine kinase of 1,358 IU/L that reached 75,000 IU/L in few days. She denied the use of myotoxic drugs and alcohol. Urine 1 showed false positive for hemoglobinuria (myoglobin) without erythrocytes in the sediment, confirming the diagnosis of rhabdomyolysis. Secondary causes were excluded. She was treated with hyperhydration and alkalinization of urine. Despite treatment, the patient developed pulmonary congestion and she died. The authors also review in this article rhabdomyolysis in patients with systemic lupus erythematosus.