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Obstruction of the pulmonary artery by granulomatous vasculitis: a clinical, morphological, and immunological analysis

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The case of a 45 year old woman presenting with fever of unknown origin, headache, polyarthralgia, and chest pain is described. Computed tomography scans and angiography disclosed a mediastinal tumour with a complete obstruction of the right pulmonary artery, positron emission tomography scans showed a tracer accumulation in the walls of the pulmonary artery and the aorta. Thoracotomy was performed and subsequent histological analysis of multiple biopsy specimens led to the diagnosis of a form of giant cell arteritis of the pulmonary artery. Immunological analysis of the cytokine profile of peripheral blood mononuclear cells (PBMC) showed a markedly increased production of tumour necrosis factor α (TNFα) and interleukin 2 (IL2) and a normalisation of TNFα production after successful treatment. In contrast, the production of interferon γ, IL4, IL6, IL10, and IL13 was normal. Furthermore, an expanded subset of double positive CD4+/CD8+ T cells was remarkable, as well as an increased expression of the IL2 receptor on PBMC. Immunomodulatory treatment with steroids and methotrexate was started and led to a rapid resolution of symptoms and morphological changes. The right pulmonary artery, however, remained occluded. The particular features of this case are reviewed with consideration of its clinical, diagnostic, histopathological, and immunological characteristics and are compared with other reported findings.

CASE REPORT

We here report on a 45 year old white woman who was admitted to our department of infectious diseases in January 2001. The clinical symptoms described by the patient consisted of intermittent episodes of fever up to 39°C, polyarthralgia, headache, and chest pain, which predominated during inspiration. Several flare ups of symptoms had been reported during the past 12 months, the last one beginning three weeks before admission.

The patient's medical history was fairly uneventful, showing only one episode of hepatitis A infection as well as an appendectomy several years ago. The …

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    Figure W1 Positron emission tomography (fluorodeoxyglucose-PET). PET scans were performed before the start of immunosuppressive treatment. Both axial (a) and coronal sections (b) show an accumulation of tracer in the aortic wall as well as in the pulmonary artery.
     
     
     
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    Figure W2 Production of tumour necrosis factor alpha (TNF-alpha). FACS analysis of permeabilised peripheral blood mononuclear cells shows the intracellular TNF-alpha production. In normal subjects the number of TNF-alpha producing T cells is low, both in the CD4+ and in the CD8+ T cell subsets, whereas it returns to normal after immunosuppressive treatment.

     

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