Abdominal angiostrongyliasis: what does the rheumatologist must know about it?

Abdominal angiostrongyliasis (AA) is a disease caused by Angiostrongylus costaricensis, a nematode that can infect humans accidentally through the ingestion of larvae. Worms live inside intestinal small vessels and can lead to gastrointestinal symptoms and bowel necrosis in otherwise healthy patients. Therefore, abdominal angiostrongyliasis may be important in the differential diagnosis with systemic vasculitides and other rheumatic diseases with vascular involvement. We report a case of abdominal angiostrongyliasis in an 18-year-old woman presenting with necrosis of the terminal ileum.

Acquired factor XI inhibitor in systemic lupus erythematosus--case report and literature review.

OBJECTIVES: Rare patients with systemic lupus erythematosus (SLE) patients exhibit anticoagulants that interfere in the earlier stages of the intrinsic coagulation pathway, such as those involving factor XI (FXI). The objectives of our study were to describe the presence of an acquired inhibitor to FXI causing a life-threatening bleeding disorder in an SLE patient and to review the association of this coagulopathy with SLE. METHODS: We describe the clinical presentation of an SLE patient with an acquired FXI inhibitor. We reviewed the scientific literature using the MEDLINE database searching the following combinations of terms: "SLE and Factor XI," "SLE and Factor XI inhibitor," and "Factor XI inhibitor," from 1964 to 2007. RESULTS: A 20-year-old woman with a 6-year history of SLE was admitted to the hospital because of severe life-threatening abdominal bleeding due to a ruptured ovarian cyst. This hemorrhagic event was related to the presence of an FXI inhibitor. We reviewed another 13 SLE patients with this condition, 8 of whom had bleeding events. Most patients had manifestations of active SLE, and prednisone was used as the primary treatment. CONCLUSIONS: SLE activity seems to be associated with the production of antibodies directed against FXI, which may cause important coagulopathies, especially bleeding events. The inhibitor disappeared after immunosuppressive therapy for SLE in most cases, suggesting that the appearance of this inhibitor is immune mediated. Although the majority of cases with the FXI inhibitor are not fatal, it should be suspected and investigated in SLE patients, especially those with abnormal clotting tests.

Esclerose sistêmica e níveis séricos elevados de organoclorado: uma associação possível? / Systemic sclerosis and elevated organochlorine blood levels: a possible association?

A patogênese da esclerose sistêmica (ES) é complexa e pouco conhecida. Há a participação de ativação imune, dano vascular e excessiva produção de matriz extracelular com deposição de colágeno estruturalmente normal. Fatores genéticos e ambientais contribuem para a expressão da doença nos pacientes. Dentre os fatores ambientais, diversos agentes químicos já foram associados à doença. Descrevemos o caso de uma adolescente de 16 anos, com história prévia de hepatite criptogênica e câncer de lábio, que desenvolveu quadro atípico e rapidamente progressivo de esclerose sistêmica. Inicialmente o quadro era compatível com esclerodermia em placas, porém rapidamente evoluiu para uma forma sistêmica com grave acometimento cardiopulmonar e óbito. Durante a investigação de possíveis fatores etiológicos que pudessem estar relacionados, foram encontrados níveis séricos extremamente elevados de oxiclordano, um agrotóxico organoclorado. Uma possível correlação entre a intoxicação por esse agente químico e o surgimento de ES foi aventada.

The pathogenesis of systemic sclerosis (SS) is complex and not completely understood. Autoimmune mechanisms, vascular damage, and excessive extracelular matrix with collagen deposition play a significant role. Genetic and environmental factors also are decisive to the disease expression. Among environmental factors many chemical agents have been associated with the development of SS. A 16-year-old white woman, with previous history of cryptogenic hepatitis and cancer of the lips, developed an atypical and rapidly progressive form of SS. Initial sclerodermatous plaques progressed to a systemic form with severe cardiopulmonary involvement and death. Diagnostic workup revealed extremely high blood levels of oxychlordane. A possible association of organochlorine intoxication and SS is proposed.

Amiloidose manifestando-se com pseudo-hipertrofia muscular / Amyloidosis presenting as muscle pseudohypertrophy

Amiloidose tipo AL é uma doença rara causada pela deposição extracelular de fragmentos de cadeias leves monoclonais em órgãos e tecidos. Pode apresentar-se com uma ampla variedade de sinais e sintomas, e o acometimento cutâneo-muscular, simulando pseudo-hipertrofia muscular, é um achado muito raro. São descritos dois casos que apresentaram tal manifestação. CASO 1 - Mulher, 61 anos, há quatro meses com história de mialgia e aumento da massa muscular nas cinturas pélvica, escapular e região cervical. Não havia alterações significativas ao exame físico, exceto aparente hipertrofia muscular difusa e discreta macroglossia. CASO 2 - Homem, 51 anos, há dois anos com cansaço e espessamento cutâneo progressivo do dorso, pescoço e braços. Em outros serviços levantou suspeitas diagnósticas de esclerodermia ou de escleredema de Buschke; desde fevereiro de 2007 passou a ser acompanhado nesse serviço e referia, havia cerca de um ano, disfagia para sólidos, disartria e dificuldade para movimentar a língua. Chamava atenção em seu exame o porte físico atlético com musculatura torácica proeminente, porém referia não fazer exercícios físicos. Em ambos os casos, a biópsia cutânea foi realizada com identificação do depósito amilóide por meio da coloração de vermelho congo.

AL amyloidosis is a rare disease secondary to extracellular deposition of light chains fragments in organs and tissues. It can cause a wide variety of signs and symptoms, being the muscular pseudohypertrophy form a very rare finding. CASE 1 - a 61-year-old female had a history of myalgia and increase of muscular mass on pelvic and scapular girdle and cervical region. Besides the generalized muscular hypertrophy and discrete macroglossia, the rest of physical examination was normal. CASE 2 - a 51-year-old male complained of tiredness and progressive cutaneous thickening on his thorax, neck and arms for the last two years. Initially, he was misdiagnosed with either scleredema of Buschke or scleroderma. In February 2007 he was referred to our service, reporting symptoms of dysphagia and difficulty to move his tongue. On physical examination, besides the skin thickness, there was an evident muscle hypertrophy out of proportion the reported exercise practice. In both cases, subcutaneous biopsy was undertaken which revealed amyloid deposit by congo red dye.

Chloroquine ototoxicity.

Chloroquine (CQ), a 4-aminoquinoline drug, has been largely used for the treatment of rheumatoid arthritis and other connective tissue diseases. Besides the well-known retinal toxicity, its use has been suspected of be associated to ototoxicity. Some reports have described mainly sensorineural hearing loss, tinnitus, sense of imbalance, and cochleovestibular manifestations. Differently from what occurs in retinopathy, in which there is a predominance of CQ toxicity, there are reports of alterations in hearing related to either CQ or hydroxychloroquine. Brain-evoked response audiometry seems to be the most sensitive test in detecting early manifestations of cochlear injury caused by CQ when still in a reversible stage. The reversibility of CQ ototoxicity has been debatable, but there is suggestion that such complication can be corrected if the medication is stopped and appropriate therapy, with steroids and plasma expanders, is instituted.

Perfil da automedicaçäo em município do Sul do Brasil / Self-medication profile in a city in South Brazil

Caracteriza o usuário de medicamentos, especialmente aquele que se automedica. Foram entrevistadas 413 pessoas do Município de Santa Maria, Estado do Rio Grande do Sul, Brasil, sobre o consumo de medicamentos no último mês. Dos entrevistados, 69,9 por cento utilizaram medicamentos e destes 76,1 por cento o fizeram através de automedicaçäo. Cefaléia (28,8 por cento) foi a principal queixa motivadora de automedicaçäo. O ácido acetilsalicílico foi a droga mais utilizada (25,4 por cento). Dos fármacos utilizados na automedicaçäo, 51,2 por cento foram indicados por terceiros e 51,7 por cento dessas indicaçöes eram prescriçöes médicas emitidas em consultas anteriores. Idade, grau de escolaridade e acompanhamento médico periódico correlacionaram-se significativamente com automedicaçäo

Lipoma pleural: relato de caso / Pleural lipoma: report of a case

Os autores relataram um caso de lipoma pleural descoberto incidentalmente em radiograma torácico. Os estudos radiográficos näo foram conclusivos quanto à natureza da lesäo. O diagnóstico foi estabelecido por estudo histológico do tumor extirpado por toracotomia, após avaliaçäo citológica inconclusiva, obtida por punçäo aspirativa com agulha fina transtorácica.