Dermatomiositis y su repercusión en la cavidad bucal / Dermatomyositis and its repercussion in the oral cavity

Introducción: La dermatomiositis es una enfermedad multisistémica de probable etiología autoinmune. Esta enfermedad se caracteriza por la aparición de manifestaciones bucomaxilofaciales como afectación de la articulación temporomandibular, secundario con sus respectivas manifestaciones y periodontitis entre otras. Objetivo: Identificar las principales manifestaciones bucomaxilofaciales que se pueden presentar en los pacientes diagnosticados con dermatomiositis. Métodos: Se realizó un estudio observacional, descriptivo, de serie de casos, en 20 pacientes diagnosticados con dermatomiositis y manifestaciones bucomaxilofaciales. Se utilizó la observación dirigida y la revisión documental como técnicas de investigación para identificar la presencia de manifestaciones. Resultados: La mayor cantidad de manifestaciones fueron intrabucales y las más frecuentes afectaron el periodonto de protección (41,8 por ciento), le siguieron las extrabucales en la región labial (28 por ciento). Un total de 16 pacientes tuvieron alteraciones en la articulación temporomandibular, lo que representó un 80 por ciento, y todos los pacientes presentaron periodontopatías (100 por ciento). Conclusiones: En los pacientes con dermatomiositis, predominaron las lesiones en el periodonto de protección, y no hubo gran afectación extrabucal; 16 pacientes presentaron alteración en la articulación temporomandibular, y la lesión intrabucal que más se evidenció fue la periodontopatía(AU)

Introduction: Dermatomyositis is a multisystemic disease of probable autoimmune etiology. This disease is characterized by the appearance of bucomaxillofacial manifestations such as involvement of the temporomandibular joint, secondary with its respective manifestations and periodontitis, among others. Objective: To identify the main oral and maxillofacial manifestations that can occur in patients diagnosed with dermatomyositis. Methods: An observational, descriptive, case series study was carried out in patients diagnosed with dermatomyositis. 20 patients with oral and maxillofacial manifestations, diagnosed with dermatomyositis. Guided observation and documentary review were used as investigative techniques to identify the presence of manifestations. Results: The intraoral location where the most manifestations occurred was in the protective periodontium for 41.8 percent, in the extraoral the labial region, with 28 percent, 16 patients presented alterations in the temporomandibular joint, which represented 80 percent, patients with periodontal disease predominated with 100 percent. Conclusions: Lesions in the protective periodontium predominated, there was no great extraoral involvement, 16 patients presented alteration in the temporomandibular joint, the intra-oral injury that was most evident was periodontopathy(AU)

Radiological findings of breast involvement in benign and malignant systemic diseases / Aspectos radiológicos do envolvimento mamário em doenças sistêmicas benignas e malignas

Abstract Although the primary purpose of periodic mammograms in screening programs is to identify lesions suspected of being carcinomas, the findings are often related to systemic (benign or malignant) diseases, rather than breast cancer. Although the involvement of breast structures in systemic diseases is unusual, it can be included in the differential diagnosis of masses, skin changes, calcifications, asymmetry, and axillary lymphadenopathy. The main diagnostic entities that can be associated with such involvement are diabetes, chronic kidney disease, heart diseases, connective tissue diseases, HIV infection, lymphoma, leukemia, and metastases from primary tumors at other sites. In many cases, information related to knowledge and treatment of chronic diseases is not available to the radiologist at the time of evaluation of the mammography findings. The purpose of this essay is to offer relevant pictorial information to the general radiologist about systemic diseases involving the breast, expanding the range of differential diagnoses in order to avoid unnecessary invasive procedures.

Resumo Embora o objetivo primário da realização periódica da mamografia nos programas de rastreamento seja a identificação de lesões suspeitas para carcinoma mamário, muitas vezes as alterações encontradas não estão relacionadas ao câncer de mama, e sim, a doenças sistêmicas benignas e malignas secundárias de outros sítios. O envolvimento das estruturas mamárias nas doenças sistêmicas é incomum, mas pode ser incluído no diagnóstico diferencial de nódulos, alterações cutâneas, calcificações, assimetrias e linfonodomegalias axilares. As principais entidades diagnósticas que podem estar associadas ao acometimento mamário são o diabetes, a nefropatia crônica, as cardiopatias, as colagenoses, as infecções pelo vírus HIV ou parasitas, o linfoma, a leucemia e as metástases de tumores primários de outros órgãos. Muitas vezes as informações relacionadas ao conhecimento e/ou tratamento de doenças crônicas não estão disponíveis para o radiologista no momento da avaliação da mamografia. O objetivo deste ensaio é oferecer informações iconográficas relevantes a respeito de doenças sistêmicas com envolvimento mamário, permitindo ampliar o leque de diagnósticos diferenciais e evitar eventuais procedimentos invasivos desnecessários.

Manifestação esofágica atípica na esclerose sistêmica difusa: relato de caso / Esophageal atypical manifestation in diffuse systemic sclerosis: case report

A Esclerose Sistêmica (ES) é uma doença difusa do tecido conjuntivo caracterizada pelo envolvimento cutâneo e multissistêmico. O acometimento gastrointestinal, em especial, está presente em 90% de seus portadores e acarreta considerável prejuízo à sua qualidade de vida. A precocidade do diagnóstico do envolvimento gastrointestinal permite o estabelecimento de planos terapêuticos específicos de suas complicações. Relataremos o caso de um paciente diagnosticado com Esclerose Sistêmica do tipo difusa com manifestações pulmonares e esofagogástricas e achado atípico de acometimento esofágico.

Systemic Sclerosis is a diffuse connective tissue disease characterized by cutaneous involvement and multisystemic. The gastrointestinal involvement, in particular, is present in 90% of its carriers, and causes considerable damage to their quality of life. The early diagnosis of gastrointestinal involvement, allows for the establishment of specific treatment plans for its complications. We will report the case of a patient diagnosed with diffuse Systemic Sclerosis, presenting pulmonary and esophagogastric manifestations, as well as an atypical esophageal involvement.

Pseudo-obstrucción lúpica Reto diagnóstico / Lupic pseudo-obstruction A diagnostic challenge

El lupus eritematoso sistémico incluye un gran espectro de manifestaciones clínicas, entre las que se encuentran las gastrointestinales que corresponden a 20% del total y éstas por lo general son consecuencia del tratamiento. Se describe el caso de una mujer joven cuya primera manifestación de una enfermedad del colágeno fue dolor abdominal, y se evidenció un proceso pseudo-obstructivo con diagnóstico definitivo de enfermedad del tejido conectivo. (Acta Med Colomb 2012; 37: 207-210).

Systemic lupus erythematosus includes a broad spectrum of clinical manifestations, including the gastrointestinal manifestations that correspond to 20% of all and which usually result as a consequence of the treatment. We describe the case of a young woman whose first manifestation of collagen disease was abdominal pain, and showed a pseudo-obstructive process with definitive diagnosis of connective tissue disease. (Acta Med Colomb 2012; 37: 207-210).

The Effect of Saffron in Patients with Autoimmune Diseases / 日本東洋医学雑誌

Saffron (<i>Crocus sativus</i> L.) is classified as a beneficial herb in the treatment of “Oketsu,” eliminating blood stagnation. The clinical symptoms of “Oketsu” include feeling cold and arthralgia. The pathological condition of “Oketsu” is considered to be increased blood viscosity and microvascular disorders.However, no useful biomarker has been reported for evaluation of the degree of “Oketsu.” Here, we investigated the clinical effect of saffron on the symptoms of “Oketsu” in patients with autoimmune diseases. At the same time, we measured the plasma levels of platelet factor 4 (PF-4) and beta-thromboglobulin (β-TG) as platelet activation markers. Seventy-one patients (66 women and 5 men, mean age 52.3 ± 16.1) were studied. They were administered saffron (300mg∼900mg) with traditional Kampo medicine. The clinical symptoms of “Oketsu” improved (80.9%, n=38/47) and we measured PF-4 and β-TG in pre-and post-saffron treatment periods. The plasma levels of PF-4 and β-TG significantly decreased after saffron therapy (PF-4 : before 49.6 ± 29.8, after 24.0 ± 19.6ng/ml, β-TG : before 117.5 ± 64.0, after 64.6 ± 47.1ng/ml;paired t-test, p < 0.0001, respectively). These results suggest that saffron is effective in treatment of the symptoms of “Oketsu” in allergy and collagen disease patients. Moreover, PF-4 and β-TG may be useful biomarkers of the degree of “Oketsu.”

Intracranial Fusiform Aneurysms: It's Pathogenesis, Clinical Characteristics and Managements

OBJECTIVE: The objective of this study is to investigate clinical characteristics, management methods and possible causes of intracranial fusiform aneurysm. METHODS: Out of a series of 2,458 intracranial aneurysms treated surgically or endovascularly, 22 patients were identified who had discrete fusiform aneurysms. Clinical presentations, locations, treatment methods and possible causes of these aneurysms were analyzed. RESULTS: Ten patients of fusiform aneurysm were presented with hemorrhage, 5 patients with dizziness with/without headache, 4 with ischemic neurologic deficit, and 1 with 6th nerve palsy from mass effect of aneurysm. Two aneurysms were discovered incidentally. Seventeen aneurysms were located in the anterior circulation, other five in the posterior circulation. The most frequent site of fusiform aneurysm was a middle cerebral artery. The aneurysms were treated with clip, and/or wrapping in 7, resection with/without extracranial-intracranial (EC-IC) bypass in 6, proximal occlusion with coils with/without EC-IC bypass in 5, EC-IC bypass only in 1 and conservative treatment in 3 patient. We obtained good outcome in 20 out of 22 patients. The possible causes of fusiform aneurysms were regard as dissection in 16, atherosclerosis in 4 and collagen disease or uncertain in 2 cases. CONCLUSION: There is a subset of cerebral aneurysms with discrete fusiform morphology. Although the dissection or injury of internal elastic lamina of the cerebral vessel is proposed as the underlying cause for most of fusiform aneurysm, more study about pathogenesis of these lesions is required.

A Case Complicated with Collagen Disease Related with SLE during Hemodialysis / 日本農村医学会雑誌

A 60-year-old man who developed a nephrotic syndrome underwent a renal biopsy, and the case was diagnosed as membranoproliferative glomerulonephritis (MPGN). Despite chemotherapy using steroid, immunosuppresive and anticoagulant drugs, the patient exhibited persistently high urinary protein levels (above 8 g/day), and the renal function deteriorated gradually.<BR>One and half years later, hemodialysis was started, but soon he had a high fever (above 38-40°C). Laboratory data revealed high levels of both antinuclear antibody titer and immune complex (IC) titer, and a low level of CH50.<BR>Considering an active collagen disease like SLE, steroid pulse therapy and plasma exchange were instituted. The therapy was very effective this time.<BR>It is well known that many patients with SLE and a long term history of hemodialysis develop a condition of so called “burn out” in which the activity of SLE declined to allow the withdrawal of steroid therapy. Also well known is the developement of hypocomplementemia in many patients with MPGN.<BR>In our case, it was difficult to clarify what caused the high fever and other clinical symptoms. We must carefully observe the clinical symptoms of SLE.