Epidemiologic Features of Systemic Vasculitides in the Southeast Region of Brazil: Hospital-Based Survey.

BACKGROUND/OBJECTIVE: The epidemiology of vasculitis is variable in different geographic areas, and this issue has not been approached in Brazil yet. The objective of this study was to assess the frequency of vasculitis in specialized centers in Brazil. METHODS: This cross-sectional study was performed in 9 vasculitis outpatient clinics from 6 different states mainly from the Southeast and the Northeast regions of Brazil between 2015 and 2017. Diagnosis and/or classification criteria for Behçet disease (BD), Takayasu arteritis (TA), giant cell arteritis (GCA), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and cryoglobulinemic vasculitis (CryoVas) were used to include patients with at least 6 months of follow-up in this hospital-based survey. RESULTS: A total of 1233 patients with systemic vasculitis were included from the Southeast region. Behçet disease was the most frequent vasculitis (35.0%) followed by TA (26.4%), GPA (16.2%), PAN (5.8%), GCA (5.8%), EGPA (4.3%), MPA (3.4%), and CryoVas (3.0%). Up to 7.8% of vasculitis patients had a juvenile onset, and the frequency of vasculitides found in children and adolescents was as follows: TA (52.6%), BD (24.7%), GPA (12.4%), and PAN (10.3%). No cases of EGPA, MPA, and CryoVas were diagnosed before the age of 18 years. As a comparator, 103 vasculitis patients were included in the Northeast of Brazil where TA was found in 36.9% and BD in 31.1% of vasculitis cases. No GCA cases were found in the Northeast part of Brazil. CONCLUSIONS: Similar to the epidemiology of vasculitis in Asia, BD and TA are the most frequent vasculitis in Southeastern Brazilian referral centers.

Use of (99m)Tc-anti-TNF-α scintigraphy in a patient with non-radiographic axial spondyloarthritis.

The aim of this study was to describe the use of (99m)Tc-anti-TNF-α scintigraphy for detecting inflammation of the sacroiliac joints in a patient with non-radiographic axial spondyloarthritis. A 47-year-old female patient, non-smoker and non-drinker, complained of a low back pain inflammation, which began 4 years before her condition have exacerbated to morning stiffness and anterior uveitis in the last 6 months. Initially diagnosed as mechanical low back pain, she irregularly took non-steroidal anti-inflammatory drugs and corticosteroids, without significant long-lasting results. Radiographic findings were negative. There was increased uptake of (99m)Tc-anti-TNF-α in an area corresponding to the topography of ileum and sacroiliac right joint upon (99m)Tc-anti-TNF-α scintigraphy. Magnetic resonance imaging (MRI), the most used image diagnosis tool, showed minimum impregnation of gadolinium in the right sacroiliac joint and at the iliac face of the inferior third of the right sacroiliac joint. We suggest that (99m)Tc-anti-TNF-α can facilitate early diagnosis of patients with non-radiographic axial spondyloarthritis. More studies are now ongoing.

A rabdomiólise está associada à febre dengue em um paciente lúpico / Rhabdomyolysis associated with dengue fever in a lupic patient

Esse relato descreve o caso de uma mulher com lúpus eritematoso sistêmico (LES) que sofreu rabdomiólise em seguida à sua infecção pelo vírus da dengue. Foram relatados apenas alguns casos de LES com manifestação de rabdomiólise, nenhum deles associados à febre dengue. A princípio, a paciente apresentava-se com febre alta, mialgia, astenia muscular, leve cefaleia, poliartralgia e trombocitopenia, lembrando uma exacerbação lúpica, mas considerando que o número de pessoas infectadas pela dengue na época era alto e tendo em vista que os sintomas das duas condições são parecidos, foi solicitada sorologia para dengue. Transcorridos alguns dias, a paciente apresentou rabdomiólise, tendo então sido tratada com medicamentos imunossupressivos, alcalinização urinária e hidratação vigorosa, medidas que melhoraram seus danos musculares e a condição inflamatória. A sorologia positiva para dengue nos foi disponibilizada apenas depois da instauração do tratamento descrito acima. A paciente recebeu alta em estado assintomático. Esse caso demonstra a grande semelhança entre a febre dengue e uma exacerbação lúpica; isso deve alertar o clínico para que, especialmente durante uma epidemia, faça uma cuidadosa diferenciação entre essas doenças, de forma a estabelecer uma terapia correta e eficiente.

This report describes the case of a woman with systemic lupus erythematous (SLE) that developed rhabdomyolysis after being infected by dengue virus. There are only a few cases of SLE accompanied by rhabdomyolysis, none of them associated with dengue fever. Initially, the woman presented high fever, myalgia, muscular weakness, mild headache, polyarthralgia and thrombocytopenia reminding a lupus flare, but since the number of people infected by dengue at that time was high and the symptoms from both conditions are similar, a dengue serology was requested. After a few days, the patient developed rhabdomyolysis. She was then submitted to immunosuppressive drugs, urinary alkalization and vigorous hydration, which improved her muscle damage and inflammatory condition. The positive dengue serology was only available after the therapy above had been established. She was discharged in an asymptomatic state. This case demonstrates how alike dengue fever and a lupus flare are, warning clinicians that, especially during an epidemic, both diseases should be carefully differentiated in order to establish a correct and efficient therapy.

[Rhabdomyolysis associated with dengue fever in a lupic patient]. / A rabdomiólise está associada à febre dengue em um paciente lúpico.

This report describes the case of a woman with systemic lupus erythematous (SLE) that developed rhabdomyolysis after being infected by dengue virus. There are only a few cases of SLE accompanied by rhabdomyolysis, none of them associated with dengue fever. Initially, the woman presented high fever, myalgia, muscular weakness, mild headache, polyarthralgia and thrombocytopenia reminding a lupus flare, but since the number of people infected by dengue at that time was high and the symptoms from both conditions are similar, a dengue serology was requested. After a few days, the patient developed rhabdomyolysis. She was then submitted to immunosuppressive drugs, urinary alkalization and vigorous hydration, which improved her muscle damage and inflammatory condition. The positive dengue serology was only available after the therapy above had been established. She was discharged in an asymptomatic state. This case demonstrates how alike dengue fever and a lupus flare are, warning clinicians that, especially during an epidemic, both diseases should be carefully differentiated in order to establish a correct and efficient therapy.

Giant cell arteritis: a multicenter observational study in Brazil.

OBJECTIVE: To describe demographic features, disease manifestations and therapy in patients with giant cell arteritis from referral centers in Brazil. METHODS: A retrospective cohort study was performed on 45 giant cell arteritis patients from three university hospitals in Brazil. Diagnoses were based on the American College of Rheumatology classification criteria for giant cell arteritis or temporal artery biopsy findings. RESULTS: Most patients were Caucasian, and females were slightly more predominant. The frequencies of disease manifestations were as follows: temporal headache in 82.2%, neuro-ophthalmologic manifestations in 68.9%, jaw claudication in 48.9%, systemic symptoms in 44.4%, polymyalgia rheumatica in 35.6% and extra-cranial vessel involvement in 17.8% of cases. Aortic aneurysms were observed in 6.6% of patients. A comparison between patients with biopsy-proven giant cell arteritis and those without temporal artery biopsies did not yield significant differences in disease manifestations. All patients were treated with oral prednisone, and intravenous methylprednisolone was administered to nearly half of the patients. Methotrexate was the most commonly used immunosuppressive agent, and low-dose aspirin was prescribed to the majority of patients. Relapses occurred in 28.9% of patients, and aspirin had a protective effect against relapses. Females had higher prevalences of polymyalgia rheumatica, systemic manifestations and jaw claudication, while permanent visual loss was more prevalent in men. CONCLUSIONS: Most of the clinical features of Brazilian giant cell arteritis patients were similar to those found in other studies, except for the high prevalence of neuro-ophthalmic manifestations and permanent blindness in the Brazilian patients. Aspirin had a protective effect on relapses.

MRI in seronegative spondyloarthritis: imaging features and differential diagnosis in the spine and sacroiliac joints.

OBJECTIVE: Radiologists should be familiar with MRI findings suggestive of spondyloarthritis and its differential diagnosis. Because most publications describing these features are found in the rheumatologic literature, the purpose of this review is to present these imaging findings of axial spondyloarthritis to radiologists. CONCLUSION: New imaging outcomes have improved the diagnosis and follow-up of spondyloarthritis and the assessment of therapeutic modalities. Diagnostic criteria include MRI of the sacroiliac joint, which facilitates earlier diagnosis.

Giant cell arteritis: a multicenter observational study in Brazil

OBJECTIVE: To describe demographic features, disease manifestations and therapy in patients with giant cell arteritis from referral centers in Brazil. METHODS: A retrospective cohort study was performed on 45 giant cell arteritis patients from three university hospitals in Brazil. Diagnoses were based on the American College of Rheumatology classification criteria for giant cell arteritis or temporal artery biopsy findings. RESULTS: Most patients were Caucasian, and females were slightly more predominant. The frequencies of disease manifestations were as follows: temporal headache in 82.2%, neuro-ophthalmologic manifestations in 68.9%, jaw claudication in 48.9%, systemic symptoms in 44.4%, polymyalgia rheumatica in 35.6% and extra-cranial vessel involvement in 17.8% of cases. Aortic aneurysms were observed in 6.6% of patients. A comparison between patients with biopsy-proven giant cell arteritis and those without temporal artery biopsies did not yield significant differences in disease manifestations. All patients were treated with oral prednisone, and intravenous methylprednisolone was administered to nearly half of the patients. Methotrexate was the most commonly used immunosuppressive agent, and low-dose aspirin was prescribed to the majority of patients. Relapses occurred in 28.9% of patients, and aspirin had a protective effect against relapses. Females had higher prevalences of polymyalgia rheumatica, systemic manifestations and jaw claudication, while permanent visual loss was more prevalent in men. CONCLUSIONS: Most of the clinical features of Brazilian giant cell arteritis patients were similar to those found in other studies, except for the high prevalence of neuro-ophthalmic manifestations and permanent blindness in the Brazilian patients. Aspirin had a protective effect on relapses.

Dermatomiosite vesiculobolhosa como manifestação paraneoplásica de tumores sincrônicos de próstata e língua: relato de caso / Paraneoplastic vesiculobullous dermatomyositis with synchronic prostate and tongue tumors: case report

A dermatomiosite é uma miopatia inflamatória que cursa com manifestações cutâneas. No adulto acima de 50 anos, pode estar associada a neoplasias malignas, comportando-se como um grande sinalizador de malignidade. OBJETIVO: Demonstrar essa associação com aspectos peculiares: dermatomiosite de apresentação atípica paralela a dois tumores sincrônicos, em geral, não relacionados àquela. RELATO DO CASO: Descrevemos o caso de um paciente do gênero masculino de 72 anos, que desenvolveu dermatomiosite, inicialmente apenas com quadro cutâneo clássico, evoluindo com lesões vesiculobolhosas, e, meses após, com miopatia. Após extensa investigação, foi localizado um adenocarcinoma de próstata. Houve remissão da doença após tratamento do câncer e administração de glicocorticoide. Durante a retirada gradual do glicocorticoide, ocorreu reativação da dermatomiosite, sendo reiniciadas investigações, que revelaram a presença de carcinoma escamoso de língua. Tratada esta neoplasia, houve remissão completa, mesmo após a retirada total do corticoide. CONCLUSÃO: Trata-se de um caso raro por envolver as formas menos usuais de apresentação da dermatomiosite, tanto com relação ao quadro cutaneomuscular, quanto à sua associação a tumores de próstata e língua (tumores nunca antes relatados juntos). Este caso demonstra a importância da investigação minuciosa em busca de neoplasias na abordagem desses pacientes.

Dermatomyositis is an inflammatory myopathy with skin manifestations. In the adult over the age of 50 years, it can be associated with malignant neoplasias, being, thus, a signal of malignancy. OBJECTIVE: To show the association of dermatomyositis of atypical presentation with two synchronous tumors, usually not related to that. CASE REPORT: We report the case of a 72-year-old male, who developed dermatomyositis, initially with only classic skin findings, which progressed to vesiculobullous lesions, and, months later, to myopathy. After extensive investigation, prostate adenocarcinoma was diagnosed. After treatment of the cancer and administration of glucocorticoid, the disease went into remission. During gradual glucocorticoid withdrawal, dermatomyositis recurred, and the new investigation revealed the presence of squamous cell carcinoma of the tongue. After treating this neoplasia, complete remission occurred, even after total corticoid withdrawal. CONCLUSION: This is a rare case involving less usual dermatomyositis presentation forms, relating to the cutaneous-muscle findings and the association with prostate and tongue tumors (tumors never reported together). This case demonstrates the importance of a careful investigation, searching for neoplasias, when approaching such patients.

Paraneoplastic vesiculobullous dermatomyositis with synchronic prostate and tongue tumors: case report.

UNLABELLED: Dermatomyositis is an inflammatory myopathy with skin manifestations. In the adult over the age of 50 years, it can be associated with malignant neoplasias, being, thus, a signal of malignancy. OBJECTIVE: To show the association of dermatomyositis of atypical presentation with two synchronous tumors, usually not related to that. CASE REPORT: We report the case of a 72-year-old male, who developed dermatomyositis, initially with only classic skin findings, which progressed to vesiculobullous lesions, and, months later, to myopathy. After extensive investigation, prostate adenocarcinoma was diagnosed. After treatment of the cancer and administration of glucocorticoid, the disease went into remission. During gradual glucocorticoid withdrawal, dermatomyositis recurred, and the new investigation revealed the presence of squamous cell carcinoma of the tongue. After treating this neoplasia, complete remission occurred, even after total corticoid withdrawal. CONCLUSION: This is a rare case involving less usual dermatomyositis presentation forms, relating to the cutaneous-muscle findings and the association with prostate and tongue tumors (tumors never reported together). This case demonstrates the importance of a careful investigation, searching for neoplasias, when approaching such patients.

Endotracheal inflammatory pseudotumor: the role of interventional bronchoscopy.

We present the case of a 31-year-old woman with recurrent pneumonia. A chest roentgenogram, a chest computed tomographic scan, and a rigid bronchoscopy revealed a mass in the left mainstem bronchus, which was protruding into the trachea and right mainstem bronchus, with no extraluminal component. Endoscopic biopsies confirmed an inflammatory myofibroblastic tumor. The patient was treated with two sessions of bronchoscopic resection, followed by medical therapy with corticosteroids and azathioprine. She has been followed-up for 31 months without complications or recurrence. Although the usual treatment for this type of entity is surgical excision, endoscopic resection may be an adequate oncologic procedure when the tumor is completely endoluminal, as in this case.