Central Nervous System Involvement in Systemic Autoimmune Rheumatic Diseases-Diagnosis and Treatment.

Central nervous system (CNS) involvement in autoimmune rheumatic diseases represents a significant challenge for clinicians across all specialties. While most reviews on the subject focus on neurological manifestations within a specific rheumatic disease, few descriptions shift from neurological clinical syndromes to achieve rheumatological diagnoses. This narrative review aims to synthesize current knowledge on the diagnosis and management of CNS manifestations occurring in the most prevalent rheumatic conditions in adults. We searched the MEDLINE database using the terms "central nervous system", "rheumatic diseases", "systemic lupus erythematosus", "rheumatoid arthritis", "Sjögren syndrome", and "vasculitis". The search strategy included review articles from 2019 to 2024, published in English, Spanish, or Portuguese. We explored the pathophysiological mechanisms linking autoimmunity to CNS pathology, emphasizing the role of syndromic reasoning, autoantibody profiles, and imaging modalities as tools for diagnosis and determination of inflammatory activity. The review also discusses differential diagnoses through a stepwise approach to neurological syndromes, summarized in diagnostic flowcharts, and presents updated treatment options. Although our approach is primarily semiology-based, the complexity of the subject invites future endeavors involving new technologies, such as functional MRI, MR spectroscopy, and nuclear medicine.

Position statement of the Brazilian society of Rheumatology on mesna use as a preventive therapy for bladder disease in patients with systemic autoimmune diseases and systemic vasculitis under cyclophosphamide treatment.

OBJECTIVE: To review current literature to support the use of mesna as a preventive therapy for hemorrhagic cystitis and bladder cancer in patients with systemic autoimmune diseases and systemic vasculitis treated with cyclophosphamide. MATERIALS AND METHODS: The search for articles was conducted systematically through MEDLINE, LILACS, Cochrane Library, and Embase databases. Only articles in English were selected. For available records, titles and abstracts were selected independently by two investigators. RESULTS: Eighteen studies were selected for analysis. The known adverse effects of cyclophosphamide were hematological toxicity, infections, gonadal toxicity, teratogenicity, increased risk for malignancy and hemorrhagic cystitis. Long-term toxicity was highly dependent on cyclophosphamide cumulative dose. The risk of bladder cancer is especially higher in long-term exposure and with cumulative doses above 36 g. The risk remains high for years after drug discontinuation. Hemorrhagic cystitis is highly correlated with cumulative dose and its incidence ranges between 12 and 41%, but it seems to be lower with new regimens with reduced cyclophosphamide dose. No randomized controlled trials were found to analyze the use of mesna in systemic autoimmune rheumatic diseases and systemic vasculitis. Retrospective studies yielded conflicting results. Uncontrolled prospective studies with positive results were considered at high risk of bias. No evidence was found to support the use of mesna during the treatment with cyclophosphamide for autoimmune diseases or systemic vasculitis to prevent hemorrhagic cystitis and bladder cancer. In the scenarios of high cumulative cyclophosphamide dose (i.e., > 30 g), patients with restricted fluid intake, neurogenic bladder, therapy with oral anticoagulants, and chronic kidney disease, mesna could be considered. CONCLUSION: The current evidence was found to be insufficient to support the routine use of mesna for the prophylaxis of hemorrhagic cystitis and bladder cancer in patients being treated for systemic autoimmune diseases and systemic vasculitis with cyclophosphamide. The use may be considered for selected cases.

Utility of multimodal molecular imaging in the diagnosis and decision-making in arterial diseases.

Arterial diseases are prevalent in the general population, particularly in the elderly, and they are among the main causes of morbidity and mortality worldwide. Nuclear imaging is a useful tool in diagnosis and follow-up in different areas of medicine, and over the last 2 decades, these study modalities have become more relevant in the field of angiology and vascular surgery due to their potential benefit in the interpretation of pathophysiological mechanisms associated with the natural history and severity of diseases that affect the circulation such as vasculitis, degenerative aortic aneurysms (AA), peripheral arterial disease (PAD), and complications following reconstructive procedures such as graft infections. The literature has shown evidence of an important number of radiotracers for specific molecules involved in the activity of these entities and their utility as predictors during surveillance and possible therapeutic targets. The present narrative review aims to describe the use of nuclear medicine, imaging methods, and radiotracers that have been applied in arterial diseases, as well as the advantages and considerations, their importance in the diagnosis and follow-up of these complex groups of patients, and future perspectives.

Peripheral Neuropathy in Systemic Autoimmune Rheumatic Diseases-Diagnosis and Treatment.

Peripheral neuropathy (PN) is frequently observed in systemic rheumatic diseases and is a challenge in clinical practice. We aimed to review the evidence on the subject and proposed a comprehensive approach to these patients, facilitating diagnosis and management. We searched the MEDLINE database for the terms (and its respective Medical Subject Headings (MeSH) terms): "peripheral neuropathy" AND "rheumatic diseases" OR "systemic lupus erythematosus", "rheumatoid arthritis", "Sjogren syndrome", and "vasculitis" from 2000 to 2023. This literature review focuses on the diagnostic workup of PNs related to systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, and systemic vasculitis. For every type of PN, we provide a pragmatic flowchart for diagnosis and also describe evidence-based strategies of treatment.

Abordaje de la enfermedad de Behçet vulvar: comunicación de caso

La enfermedad de Behçet (EB) es un trastorno inflamatorio, multisistémico, recidivante y remitente de etiología desconocida. Una característica clave de la enfermedad es la ulceración orogenital que provoca una morbilidad considerable con gran impacto en la calidad de vida de los pacientes. Su manejo médico consiste en un esquema con colchicina, esteroides o inmunomoduladores. Comunicamos el caso de una paciente con lesiones genitales quien consultó en múltiples ocasiones, recibiendo manejo antibiótico e incluso quirúrgico, con respuesta refractaria a estas intervenciones. Posteriormente, por las características de las lesiones y el comportamiento clínico se sospechó enfermedad de Behçet y se instauró manejo médico con esteroides orales y curaciones con fitoestimulina. Hubo resolución de las úlceras genitales y posteriormente la paciente fue llevada a cirugía para corrección de las sinequias vulvares.

Behçet's disease (BD) is an inflammatory, multisystemic, relapsing-remitting disorder of unknown etiology. A key feature of the disease is orogenital ulceration that causes considerable morbidity with great impact on patient's quality of life. Its medical management consists of a scheme with colchicine, steroids or immunomodulators. We report the case of a patient with genital lesions who consulted on multiple occasions, receiving antibiotics and even surgical management, with refractory response to these interventions. Subsequently, due to the characteristics of the lesion and clinical behavior, Behçet's disease was suspected and medical management with oral steroids and cures with phytostimulin was initiated. The genital ulcers were resolved, and the patient was subsequently taken to surgery for correction of the vulvar synechiae.

Necrotizing Scleritis as a Manifestation of Eosinophilic Granulomatosis With Polyangiitis: A Case Report.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an ANCA-associated vasculitis characterized by the development of necrotizing granulomas rich in eosinophils and vasculitis of small and medium vessels, with compromise of the respiratory tract, peripheral nervous system, and least frequent ocular involvement. We report the case of a 54-year-old Caucasian man with a history of EGPA who presented ocular pain, red eye, vision loss, and evidence of scleral slimming compatible with necrotizing scleritis. The patient was treated with systemic steroids and cyclophosphamide, which reduced the ocular pain but did not improve visual acuity, needing surgical treatment of the scleral coverage defect. While necrotizing scleritis is an unusual manifestation of EGPA, it should be part of the differential diagnosis in patients with red eye or ocular pain as an accurate diagnosis and prompt treatment could reduce local complications.

Systemic disease in leukocytoclastic vasculitis: a focus on direct immunofluorescence findings

Abstract Background Direct immunofluorescence (DIF) panels are usually ordered for clinically suspected cutaneous vasculitis, but their positivity rate is variable, and their prognostic significance is not clear to date. Objective The study aims to investigate the systemic involvement rate in leukocytoclastic vasculitis (LCV) patients and the potential clinical and laboratory associations with systemic involvement, including DIF findings. Methods A retrospective study of patients with histopathologically proven cutaneous LCV examined in the dermatology department between 2013 and 2017 was performed. Results Of the 81 patients (mean age, 50.6 years), 42 (52%) were male. The mean time between the appearance of skin lesions and biopsy was 23.1 days, ranging from 2 to 180 days. DIF showed overall positivity of 90.1%, and C3 was the most frequent immunoreactant (82.7%). Any kind of extracutaneous involvement was present in 47 (58%) of patients, with renal involvement being the most frequent (53.1%), followed by articular (18.5%) and gastrointestinal (11.1%) involvement. The presence of renal disease was associated with the detection of IgG in the lesional skin (p = 0.017), and with the absence of IgM in the lesional skin (p = 0.032). There was a significant association between C3 deposition and joint involvement (p = 0.05). Study limitations This is a single-center study with a retrospective design. Conclusion DIF seems to be a useful ancillary diagnostic tool in the evaluation of cutaneous vasculitis, but the relationship between DIF findings and systemic involvement needs to be further elucidated due to contradictory data in the current literature.

Systemic disease in leukocytoclastic vasculitis: a focus on direct immunofluorescence findings.

BACKGROUND: Direct immunofluorescence (DIF) panels are usually ordered for clinically suspected cutaneous vasculitis, but their positivity rate is variable, and their prognostic significance is not clear to date. OBJECTIVE: The study aims to investigate the systemic involvement rate in leukocytoclastic vasculitis (LCV) patients and the potential clinical and laboratory associations with systemic involvement, including DIF findings. METHODS: A retrospective study of patients with histopathologically proven cutaneous LCV examined in the dermatology department between 2013 and 2017 was performed. RESULTS: Of the 81 patients (mean age, 50.6 years), 42 (52%) were male. The mean time between the appearance of skin lesions and biopsy was 23.1 days, ranging from 2 to 180 days. DIF showed overall positivity of 90.1%, and C3 was the most frequent immunoreactant (82.7%). Any kind of extracutaneous involvement was present in 47 (58%) of patients, with renal involvement being the most frequent (53.1%), followed by articular (18.5%) and gastrointestinal (11.1%) involvement. The presence of renal disease was associated with the detection of IgG in the lesional skin (p = 0.017), and with the absence of IgM in the lesional skin (p = 0.032). There was a significant association between C3 deposition and joint involvement (p = 0.05). STUDY LIMITATIONS: This is a single-center study with a retrospective design. CONCLUSION: DIF seems to be a useful ancillary diagnostic tool in the evaluation of cutaneous vasculitis, but the relationship between DIF findings and systemic involvement needs to be further elucidated due to contradictory data in the current literature.

Incidence and Prevalence of Polymyalgia Rheumatica and Giant Cell Arteritis in a Healthcare Management Organization in Buenos Aires, Argentina.

OBJECTIVE: To estimate incidence and prevalence of polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) in a university hospital-based health management organization (Hospital Italiano Medical Care Program) in Argentina. METHODS: Overall and sex-specific incidence rates (IRs) and prevalence were calculated (age ≥ 50 yrs). Incidence study followed members with continuous affiliation ≥ 1 year from January 2000 to December 2015. Diagnosis as per the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology (ACR) criteria for PMR or the ACR 1990 criteria for GCA. Prevalence was calculated on January 1, 2015. RESULTS: There were 176,558 persons who contributed a total of 1,046,620 person-years (PY). Of these, 825 developed PMR, with an IR (per 100,000 PY) of 78.8 (95% CI 73.4-84.2) overall, 90.1 (95% CI 82.9-97.2) for women, and 58.9 (95% CI 51.1-66.6) for men. Ninety persons developed GCA; the IR was 8.6 (95% CI 6.8-10.4) overall, 11.1 (95% CI 8.5-10.6) for women, and 4.2 (2.2-6.3) for men. There were 205 prevalent PMR cases and 23 prevalent GCA cases identified from a population of 80,335. Prevalence of PMR was 255 per 100,000 (95% CI 220-290) overall, 280 (95% CI 234-325) for women, and 209 (95% CI 150-262) for men; and the prevalence of GCA was 28.6 per 100,000 (95% CI 16.9-40.3) overall, 36.4 (95% CI 20.1-52.8) for women, and 14.2 (95% CI 0.3-28.1) for men. CONCLUSION: This is the first study of incidence and prevalence of PMR and GCA in Argentina. There were similarities and differences with cohorts from other parts of the world, but population-based epidemiologic studies in Latin America are needed.

Particularidades dos Pacientes com Arterite de Takayasu em Idade Mais Avançada: Estudo Coorte, Retrospectivo e Transversal / Characteristics of Older Patients with Takayasu's Arteritis: A Two-Center, Cross-Sectional, Retrospective Cohort Study

Resumo Fundamentos Poucos estudos avaliaram pacientes idosos com Arterite de Takayasu (AT). Objetivo Avaliar o progresso de AT em diferentes grupos etários em seus possíveis efeitos sobre o tratamento medicamentoso e atividade da doença. Métodos este estudo transversal, retrospectivo, do tipo coorte incluiu 66 pacientes com AT. Os pacientes foram entrevistados, e dados dos 12 meses anteriores foram coletados dos prontuários médicos eletrônicos. Os pacientes foram divididos em quatro quartis de acordo com idade atual, e comparados quanto aos dados clínicos e laboratoriais, tratamento, comorbidades, status da doença, e status funcional. Um p<0,05 foi estabelecido como estatisticamente significativo. Resultados Os grupos foram definidos como Q1(22-36 anos, n=16), Q2(37-42 anos, n=18), Q3(43-49 anos, n=17), e Q4(51-66 anos, n=15). A frequência de pacientes com atividade da doença, fadiga, comorbidades e comprometimentos vasculares, e o índice de extensão da doença (DEI. Tak) foram comparáveis entre os grupos. Pacientes com idade mais avançada apresentaram maior duração da doença (p=0,001) e maior comprometimento do status funcional (Q2 versus Q3, p=0,003); menos pacientes usaram prednisona (Q1:43,8%; Q2:33,3%; Q3:11,8%; e Q4:6,7%; p=0,049) e agentes imunossupressores [Q1:100,0%; Q2:66,7%; Q3:58,8% e Q4:46,7%; Q1 versus Q3 (p=0,043) e Q1 versus Q4 (p=0,005) nas análises post-hoc]. Além disso, os níveis de danos da doença, sintomas de uma nova ocorrência de AT, e complicações nos 12 meses precedentes não foram diferentes entre os grupos. Conclusão Pacientes com AT e idade mais avançada requerem mínima intervenção medicamentosa e apresentam maior comprometimento no status funcional, o que pode ser atribuído a fatores relacionados ao envelhecimento.

Abstract Background Few studies have assessed elderly patients with Takayasu's arteritis (TAK). Objectives To evaluate the progression of TAK in different age groups and its possible effects on drug treatment and disease activity. Methods This cross-sectional and retrospective cohort study included 66 TAK patients. Patients were interviewed and data of the 12 preceding months were collected from electronic medical records. The patients were divided into four quartiles according to current age and compared for clinical and laboratory data, treatment, comorbidities, disease status, and functional status. Statistical significance was set at p<0.05. Results The groups were Q1(22-36 years, n=16), Q2(37-42 years, n=18), Q3(43-49 years, n=17), and Q4(51-66 years, n=15). The frequency of patients with disease activity, fatigue, comorbidities and vascular impairments, and the TAK disease extent index were also comparable between the groups. With age, disease duration was longer (p=0.001), fewer patients used prednisone (Q1:43.8%, Q2:33.3%, Q3:11.8%, and Q4:6.7%; p=0.049) and immunosuppressive drugs [Q1:100.0%, Q2:66.7%, Q3:58.8%, and Q4:46.7%; Q1 versus Q3 (p=0.043), and Q1 versus Q4 (p=0.005) in post-hoc analyses], and patients had greater functional status impairment (Q2 versus Q3, p=0.003). In addition, the levels of disease damage, new TAK symptoms, and complications in the preceding 12 months were not different between the groups. Conclusions Older patients with TAK require minimal drug treatment, and have greater impairment of functional status, which may be attributed to aging-related factors.

Manejo anestésico de un paciente con enfermedad de Behçet / Anesthetic management of a patient with Behçet´s Disease Sobre los autores:

RESUMEN Introducción: La enfermedad de Behçet es una enfermedad inflamatoria crónica, recurrente, multisistémica, de etiología desconocida, caracterizada por úlceras orales y genitales recurrentes, inflamación ocular, lesiones cutáneas, artritis, afecciones neurológicas, pulmonares, gastrointestinales y vasculitis sistémica. Objetivo: Describir el manejo anestésico en un paciente portador de enfermedad de Behçet. Presentación del caso: Se reporta el caso de un paciente de 52 años de edad con antecedentes patológicos personales de enfermedad de Behçet que recibe anestesia general para exéresis de adenopatía cervical izquierda metastásica de un carcinoma primario oculto. Conclusiones: El mantenimiento del tratamiento con esteroides, el uso de nadroparina cálcica junto a otras medidas preventivas de la trombosis venosa profunda, el manejo cuidadoso de la vía aérea, la protección ocular y la articular, así como de los puntos de presión y la prevención de la patergia son elementos fundamentales en el manejo de estos pacientes.

ABSTRACT Introduction: Behçet's disease is a chronic, recurrent, multisystemic inflammatory disease of unknown etiology characterized by recurrent oral and genital ulcers, ocular inflammation, skin lesions, arthritis, neurological, pulmonary and gastrointestinal conditions, as well as systemic vasculitis. Objective: To describe the anesthetic management of a patient with Behçet's disease. Case presentation: The case is reported of a 52-year-old male patient with an individual history of Behçet's disease, who receives general anesthesia for removal of left cervical metastatic adenopathy from a hidden primary carcinoma. Conclusions: Keeping the steroid therapy, using calcium nadroparin, together with other measures for preventing deep vein thrombosis; careful airway management, eye and joint protection, as well as attention to pressure points and pathergy prevention are fundamental elements for the management of these patients.

High prevalence of fatigue in patients with Takayasu arteritis: a case-control study in a Brazilian centre.

Objectives: Several studies have shown not only a high prevalence of fatigue but also a reduction in health-related quality of life (HRQoL) in patients with rheumatic diseases. Owing to insufficient research in this area, we aimed to assess the prevalence of fatigue and its contribution to impairment of HRQoL in patients with Takayasu arteritis (TAK). Methods: This single-centre case-control study included 53 TAK patients who were matched by age, BMI and sex with 100 healthy individuals. Aside from the patients' general data, the following information was collected: disease activity, level of activities of daily living (HAQ), physical activity levels and chronic fatigue. Results: The TAK patients and healthy individuals were comparable in terms of current age, BMI and sex distribution. The median disease duration of TAK was 13.0 (7.0-20.0) years, and 11 (20.8%) patients had active disease. Compared with healthy individuals, patients with TAK had a higher prevalence of fatigue and lower HAQ score, physical activity level and intensity, and physical and psychosocial domains of the modified fatigue impact scale (P < 0.01). Moreover, TAK patients had increased fatigue rates compared with the healthy individuals (fatigue severity scale: odds ratio = 2.6; 95% CI = 1.2, 5.4; modified fatigue impact scale: odds ratio = 2.6; 95% CI = 1.2, 5.5). Fatigue was positively correlated with worsening HAQ, CRP levels, daily prednisone dose and disease activity, and negatively correlated with disease duration. Conclusion: TAK patients have a higher prevalence of fatigue, which affects different aspects of the disease, including physical function. Thus, fatigue-focused treatments should also be considered in clinical practice. Trial registration: The Brazilian Clinical Trials Registry (ReBEC), https://ensaiosclinicos.gov.br/, RBR-9n4z2hh.

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.

Arteritis de células gigantes: ¿Cómo debutan pacientes chilenos? Análisis de casos y revisión de la literatura / Giant cell arteritis. Experience in 32 patients

BACKGROUND: Giant cell Arteritis (GCA) is the most common systemic vasculitis in patients over 50 years. Diagnosis is based on clinical, laboratory, imaging and biopsy. Temporal artery biopsy (TAB) may be inconclusive in up to 40% of patients. AIM: To describe disease features of patients diagnosed with GCA. MATERIAL AND METHODS: Review of pathology reports of giant cell arteritis and clinical records of patients seen with the diagnosis between 2000 and 2019. Demographic, clinical, laboratory, histopathology, imaging, treatment and follow-up variables were analyzed. RESULTS: We fetched 32 patients with a median age at diagnosis of 70.5 years (range 57-90), 81% women. Twenty eight percent had polymyalgia. 72% had only cranial symptoms, 12% had extracranial involvement and 13% exclusive extracranial involvement. The median time from onset of symptoms to diagnosis was two months (range 0.5-8). All had elevated erythrocyte sedimentation rate and c reactive protein. A TAB was performed in 27 patients and in 17 (65.4%) it confirmed the diagnosis. Transmural inflam- mation was the most frequent finding. All patients received steroids. Follow-up information was available from 25 patients and 92% received a steroid-spa- ring agent, usually methotrexate (74%). Ninety two percent achieved clinical remission in the first year and 59% had minor relapses during steroid tapering. CONCLUSIONS: Our patients showed frequent extracranial involvement and TAB was a useful diagnostic tool.

Peripheral ulcerative keratitis as presenting manifestation of systemic microscopic polyangiitis: a case report / Ceratite ulcerativa periférica como manifestação de poliangiíte microscópica sistêmica: relato de caso

ABSTRACT Microscopic polyangiitis is a rare autoimmune disease of unknown etiology, characterized by inflammation and necrosis of blood vessels. It forms a part of the antineutrophil cytoplasmic antibody-associated vasculitides-a heterogeneous group of disorders characterized by vasculitis. It is a systemic disease affecting multiple organs. The patients may present with a wide variety of symptoms. Ocular manifestations may present as its initial clinical symptoms, necessitating a multidisciplinary approach for reducing the morbidity and mortality. Early diagnosis aids in the formulation of appropriate treatment and prevention of further complications. Aggressive treatment, including surgery, is often necessary to limit structural damage and preserve visual function. We present the case of an 82-year-old woman who initially presented with peripheral ulcerative keratitis that led to the diagnosis of microscopic polyangiitis.

RESUMO A poliangeíte microscópica é uma doença autoimune rara de etiologia desconhecida, caracterizada por inflamação e necrose dos vasos sanguíneos. Faz parte das vasculites associadas a anticorpos citoplasmáticos antineutrófilos - um grupo heterogêneo de doenças caracterizadas por vasculite. É uma doença sistêmica que afeta vários órgãos. Os pacientes podem apresentar uma grande variedade de sintomas. As manifestações oculares podem apresentar-se como seus sintomas clínicos iniciais, necessitando de abordagem multidisciplinar para redução da morbimortalidade. O diagnóstico precoce ajuda na formulação do tratamento adequado e na prevenção de complicações futuras. O tratamento agressivo, incluindo cirurgia, muitas vezes é necessário para limitar o dano estrutural e preservar a função visual. Apresentamos o caso de uma mulher de 82 anos que inicialmente apresentou ceratite ulcerativa periférica que levou ao diagnóstico de poliangite microscópica.

Different epidemiologic profiles of systemic vasculitis between Brazil and Peru-preliminary results in two referral centers from both countries.

Little is known about the epidemiology of systemic vasculitis in South American countries. The aim of this study is to compare the prevalence of systemic vasculitides in two vasculitis referral centers from Brazil and Peru. A cross-sectional study was performed and all patients above 18 years of age, with at least 6 months of follow-up and who met classification or diagnosis criteria for the most common forms of vasculitis, were included. A total of 562 patients with systemic vasculitis were analyzed, 345 (61.4%) from Brazil and 217 (38.6%) from Peru. The frequency of Behçet's disease (37.9% vs. 1.8%; p < 0.0001), Takayasu arteritis (TAK) (25.2% vs. 6.9%; p < 0.0001), and giant cell arteritis (9.8% vs. 0.9%; p < 0.0001) was higher in the Brazilian center than the Peruvian one. On the other hand, the frequency of microscopic polyangiitis (MPA) (67.3% vs. 2.8%; p < 0.0001) and renal-limited vasculitis (2.8% vs. 0.0%; p = 0.009) was higher in the Peruvian center. No differences were found concerning other forms of vasculitis. At diagnosis, Brazilian patients with TAK, granulomatosis with polyangiitis, and MPA were younger than Peruvian patients. Epidemiologic differences in the frequency of systemic vasculitis are observed between a vasculitis referral center from Brazil and another from Peru. Key Points • Significant differences are observed regarding the epidemiologic profile of systemic vasculitis between Brazil and Peru. • MPA is the predominant form of vasculitis in Peru while BD and TAK are the most frequent forms of vasculitis in Brazil. • The age at diagnosis of TAK, MPA, and GPA was lower in Brazilian patients than in Peruvian patients.

Constitutional symptoms and anemia as the initial manifestation of vasculitis in an older adult. case report

ABSTRACT Introduction: Vasculitis comprises a group of often serious diseases that have an unspecific onset and a late diagnosis. The following report describes a case of vasculitis that may lead to considering this disorder as a differential diagnosis from the beginning of the care process to ensure a comprehensive approach and early treatment initiation that reduce associated morbidity and mortality and improve the success rate of treatments. Case presentation: A 77-year-old female, with a 2-year history of arterial hypertension was admitted to the emergency department of a secondary care center for having experienced symptoms of asthenia, fever, hyporexia and weight loss for a month. The patient was hospitalized for further testing and, given the findings, a possible bacterial translocation secondary to intestinal neoplasm was suspected. Empirical antibiotic treatment was started, but her condition continued to worsen. Complementary tests were performed, although they were not conclusive. Due to the persistence of fever, kidney failure and anemia, a kidney biopsy was performed, revealing arterial vessel with fibrinoid necrosis and associated polymorphonuclear infiltrates, clear signs of an active vasculitis of the microscopic polyangiitis type. Several lines of treatment were used, but the patient evolved unfavorably and died. Conclusions: The presentation of this unusual case intends to contribute to the early diagnosis of this disorder by making medical staff aware of the possibility of considering it when symptoms suggest other diseases, or even when nonspecific symptoms such as anemia and weight loss occur.

RESUMEN Introducción. Las vasculitis son enfermedades graves que suelen tener un debut muy inespecífico y un diagnóstico tardío, lo que implica una alta tasa de fracaso terapéutico. Se presenta un caso de vasculitis que puede contribuir a considerar esta enfermedad como parte del diagnóstico diferencial desde las primeras etapas del proceso de atención para garantizar un abordaje integral y un inicio temprano del tratamiento, lo que reduciría la morbimortalidad asociada a estas entidades y mejoraría la tasa de éxito de los tratamientos. Presentación del caso. Mujer caucásica de 77 años que como único antecedente presentaba hipertensión arterial de dos años de evolución y quien consultó al servicio de urgencias de una institución de segundo nivel por cuadro clínico de un mes de evolución consistente en astenia, febrícula, hiporexia y pérdida de peso. La paciente fue hospitalizada para completar la historia clínica y dados los hallazgos se sospechó de una posible translocación bacteriana secundaria a una neoplasia intestinal y se inició cobertura antibiótica empírica, pero su condición seguía empeorando. Se realizaron pruebas complementarias que no arrojaron resultados concluyentes; ante la persistencia de la fiebre, la insuficiencia renal y la anemia, se realizó una biopsia de riñón que mostró vaso arterial con necrosis fibrinoide e infiltrados de polimorfonucleares asociados, claros signos de una vasculitis activa del tipo poliangeítis microscópica. Se instauró el tratamiento indicado pero la paciente tuvo una evolución desfavorable y falleció. Conclusiones. La exposición de este caso inusual contribuye a que los profesionales de la salud consideren el diagnóstico de vasculitis desde el inicio de la atención, aun cuando la sintomatologia sugiere otras patologías o incluso cuando se presentan síntomas tan inespecíficos como anemia y pérdida de peso, ya que esto ayudará a establecer un diagnóstico temprano.

Distribution of monocytes subpopulations in the peripheral blood from patients with Behçet's disease - Impact of disease status and colchicine use.

The innate immune response has a predominant role in Behçet's disease (BD) pathogenesis, but few studies have assessed monocytes in BD. This study aims to evaluate the profile of monocytes subsets in the peripheral blood of BD patients and healthy controls (HC). Monocytes subsets were identified as classical (CD14+CD16-), intermediate (CD14+CD16dim), and non-classical (CD14dimCD16high) subsets. Patients with BD presented a lower number of total monocytes (p = 0.020) and a lower number (p < 0.0001) of circulating classical monocytes than HC. In contrast, the number of intermediate monocytes was higher in BD patients than HC (p < 0.0001). In BD patients, no associations were observed with the severity of clinical manifestations or therapy. Colchicine was associated with a higher number of non-classical monocytes (p = 0.035). In conclusion, BD patients present an altered distribution of monocytes subsets with a reduction of classical and an increase of intermediate subsets.