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A granulomatose com poliangeíte (GPA), previamente denominada granulomatose de Wegener, é uma doença sistémica caracterizada por inflamação granulomatosa necrotizante e vasculite que envolve Download full- size image. A granulomatose de Wegener é uma vasculite sistémica rara e idiopática caracterizada pelo atingimento dos pequenos vasos. A doença atinge. Pregnancy and Wegener granulomatosis: a case report. Visits. Download PDF A granulomatose de Wegener (GW) é uma vasculite com manifestações.


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INtrODuCtION. Wegener's Granulomatosis (WG) is a necrotizing vasculitis that 1. rheumatologist of the rheumatology Department of Hospital Geral de Fortaleza (HGF). 2. .. com necrose papilar renal em granulomatose de Wegener. J Bras. J Bras Pneumol. ;31(Supl 1):SS6. Granulomatose de Wegener. S A granulomatose de Wegener é uma doença sistêmica caracterizada por vasculite . Dermatol. vol no.3 supl.1 Rio de Janeiro /June . Macieira J. Granulomatose de Wegener localizada com manifestação ocular: relato de caso.

These experiments were mainly conducted at the concentration camp in Dachau in Southern Germany and many victims were members of Polish clergy.

Granulomatose de Wegener : aspect en IRM cérébrale

We found no evidence to prove the active participation of Dr Wegener in such experiments. A ghetto for Jews, many of whom were brought there from Germany proper, was established. In , some people lived there under appalling conditions [ 14 ]. Ghetto inmates were selected for deportation to the nearby concentration camp in Chelmno and died there, mainly by carbon monoxide poisoning in converted buses. The local health authorities were deeply involved in the process of ghettoization and selection [ 16 ] although few perpetrators faced trial [ 15 ].

There is evidence that, at least occasionally, victims of these transports were autopsied by Wegener [ 9 ]. The loss of many documents around the end of World War II may offer some explanation for this lack of prosecution.

Neurological Complications of Wegener's Granulomatosis

Later, Staemmler was appointed head of the Department of Pathology at the University of Breslau and took Wegener with him. An ardent supporter of the Nazi regime [ 17 ], Staemmler was appointed there with political purpose.

Professor Staemmler has also served the national socialism by giving talks […]. Martin Staemmler, Wegener's head of the department, was a prolific author on racial hygiene [ 4 , 17 ]. These vasculitis are late and don't occur in the limited form of the disease.

Vasculitis, in its granulomatous form in the renal tissue, is very rare. Originally these antibodies are associated with necrotizing vasculitis and they can be subdivided according to its stimulating antigen. Financial Support: None.

Granulomatose de Wegener : aspect en IRM cérébrale

Granulomatosis with polyangiitis, a new nomenclature for Wegener's Granulomatosis - case report.. An Bras Dermatol. Vasculitis, neutrophilic dermatoses and related disorders.

Rook's textbook of dermatology.

London: Wiley Blackwell; Cutaneous vasculitis. New York: Elsevier, Rheumatoid factor was positive in 5 cases. Cyclophosphamide and corticosteroids alleviated the symptoms in 6 of 7 patients. The ocular and systemic condition remained stable in seven patients. One patient expired due to the severity of the disease and another was lost to follow-up.

The ocular manifestations, systemic extent of disease, management and outcome in the nine patients of WG are listed in [Table - 1]. The results of the laboratory investigations are listed in [Table - 2].

One case Case 1 is presented for illustration. Case report A year-old male presented in December with a history of pain, redness and photophobia in the left eye of 4 months' duration. He gave a history of taking oral corticosteroids following which he developed severe cough and fever.

He was on antituberculosis drugs for a diagnosis of an abscess in the left lung. Systemic history was remarkable, including a productive cough with one episode of haemoptysis and early morning knee and the ankle joint stiffness. Slitlamp examination of the right eye revealed sectoral superior scleral congestion with stromal infiltrates at the limbus. The left eye examination revealed necrotising scleritis with areas of uveal prolapse superiorly between Fundus examination of the right eye was normal and the left eye showed an area of retinitis corresponding to the area of necrotizing scleritis along with haemorrhages and retinal oedema.

Intraocular pressures were normal. On repeat investigation the ESR was raised to mm 1st hour, and the serial chest X-rays showed progression of the left lung cavity and appearance of another cavity in the right lung [Figure - 2]. Bronchoalveolar lavage revealed Pseudomonas fluorescens. Direct smear and culture for Mycobacterium tuberculosis were negative.

The patient was started on systemic antibiotics oral norfloxacin mg twice daily, oral metronidazole mg twice daily, intravenous gentamicin 80 mg twice daily and hourly instillation of prednisolone acetate eye drops.

Four days later, his systemic condition worsened with development of palpable purpuras and ecchymosis on the arms, forearms, legs [Figure - 3] and back and swelling of the ankle and the knee joints. Ocular examination now showed progression of the retinitis towards the posterior pole [Figure - 4].

Renal biopsy showed crescentric glomerulonephritis [Figure - 5]. CT scan of the thorax showed minimal pleural and pericardial effusion with cavities in the right and left lungs [Figure - 6]. A diagnosis of WG was made. Ultrasound-guided aspiration of the fluid from the cavity in left lung showed a haemorrhagic, sterile fluid.

In view of his elevated blood urea and creatinine levels, he underwent dialysis.

Granulomatose de Wegener

Oral cyclophosphamide was continued for 6 months. His haematological status was closely monitored.Less toxic immunosuppressing medications such as rituximab , methotrexate , azathioprine , leflunomide , or mycophenolate mofetil are used. This inflammation leads to narrowing of the vessels and results in reduced blood flow to, and loss of tissue in, various organ systems of the body.

The patient was started on systemic antibiotics oral norfloxacin mg twice daily, oral metronidazole mg twice daily, intravenous gentamicin 80 mg twice daily and hourly instillation of prednisolone acetate eye drops.

Renal biopsy in these patients showed a crescentric glomerulonephritis.

The most common symptoms are kidney inflammation, unintended weight loss, skin lesions especially on the feet and lower legs, nerve damage, and fevers. Professor Staemmler has also served the national socialism by giving talks […]. Generally, methotrexate, mycophenolate mofetil and azathioprine are less toxic than cyclophosphamide.

There may be muscle wasting and tissue death gangrene of the tips of the fingers and toes.