Sudden natural death due to takayasu’s arteritis.

A young male aged 23 yrs with alleged history of chest pain was brought to the emergency medicine department of St John’s Medical college hospital. On arrival was declared brought dead. There was no previous significant medical history. A Medico-legal autopsy was done which revealed left anterior descending coronary artery lumen to be occluded by grey white material. On Histo-pathological examination of the heart, it was diagnosed as Takayasu’s arteritis. Takayasu arteritis, also known as Pulseless disease, occlusive thromboaortopathy, and Martorella syndrome, It is a Granulomatous inflammation of unknown aetiology affecting medium and large arteries leading to vessel wall thickening and occlusion . Females are more likely to be affected than males. Patients often notice the disease symptoms between 15- 30 years of age. Symptoms range from malaise, fever, night sweats, weight loss, arthalagia, fatigue and can present with absent pulses, limb claudication, blood pressure, discrepancies, Hypertension, retinopathy Ischemia, postural dizziness, seizures, hemi paresis and many more. Sudden death due to Takayasu’s arteritis affecting coronary artery is rarely reported during medico-legal autopsy, hence this case is reported.

Granulomatose de Wegener: apresentação clínica e tratamento / Wegener´S Granulomatosis: clinical features and management

Granulomatose de Wegener (GW) é uma vasculite necrotizante granulomatosa sistêmica de pequenos vasos, que acomete principalmente o trato respiratório superior e rins. É enfermidade rara, tem altas taxas de morbidade e mortalidade, e pacientes com comprometimento renal têm pior evolução. Relatamos um caso desta relativamente rara enfermidade com o objetivo de revisar, na literatura, as opções terapêuticas, discutir o tratamento instituídoe suas complicações e enfatizar sua apresentação clínica. Paciente do sexo masculino, 69 anos, com quadro de rinossinusite crônica, epistaxe e ulceração de mucosa nasal acompanhadas de lesões purpúreas, hematúria, e insuficiência renal avançada de um mês de evolução apresentou ANCA-c positivo(até diluição de 1:640) e histologia renal com glomerulonefrite crescêntica esclerosante. Foi instituído tratamento agressivo com pulsoterapia(metilprednisolona), seguida de corticoterapia oral, porém, o paciente evoluiu para óbito com 15 dias do tratamento devido a choque séptico. Aapresentação clínica do paciente está de acordo com os relatos prévios da literatura, entretanto, o tratamento instituído foi diferente do recomendado: pulso de ciclofosfamida (CFA) e corticóide. Entretanto, questiona-se: a imunossupressão com CFA era a melhor opção terapêutica, diante da gravidade do processo infeccioso?

Wegener Granulomatosis (WG) is a rare disorder characterized by vasculitis of small arteries, arterioles and capillaries, necrotizing granulomatous lesionsof both upper and lower respiratory tract and glomerulonephritis. The entity carries high morbidity and mortality rates; renal involvement aggravates itsprognosis. A case of this relatively rare disease is reported aiming to review the literature regarding the therapeutic alternatives and discuss our treatmentchoice and the drug-associated complications emphasizing the clinical picture of the patient. A 69 year old man with chronic sinusitis, epistaxis, nasalulcerations, purpura, hematuria, and advanced renal insufficiency for one month, showed high c-ANCA level (positive until 1/640) and focal segmental glomerulonephritis with crescent formation and necrosis on renal histology. The patient was aggressively treated with methylprednisolone followed by oral corticosteroids. Despite 15 day therapy he died from septic shock. The patient’s clinical presentation agreed with the one found in previous studies, but the treatment chosen was different from the most frequent recommendation: cyclophosphamide and corticosteroids. Based on the course of the patient, a questions remains unanswered: would it be immunosuppressive therapy with cyclophosphamide the best choice in face of a severe infectious disease?