Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Several previous reports suggest an association between treatment of patients with interferon-alpha (IFN-alpha) and development of autoantibodies and autoimmune disease. We here summarize the experience from a group of 135 patients with midgut carcinoid tumors treated with natural leukocyte IFN-alpha or recombinant IFN-alpha (rIFN-alpha). An unusual high incidence of antimicrosomal antibodies (MsAb) or anti-thyroglobulin antibodies (TgAb) and thyroid disease manifested as hyperthyroidism, hypothyroidism or a biphasic Hashimoto-like disease was seen, with female predominance. The incidence of antinuclear antibodies (ANA) was also increased, but equally in both sexes. Antibodies to parietal cells were found in 5 cases and 4 patients with pernicious anemia were detected. Two patients developed vasculitis of leukocytoclastic type and one a syndrome resembling systemic lupus erythematosus. Some patients treated with rIFN-alpha develop anti-IFN antibodies. Such antibodies may also be autoantibodies reacting with autologous IFN-alpha. They can neutralize the biologic activity of administrated IFN preparation and cause therapeutic failure. The implications of the various autoimmune manifestations during IFN-alpha treatment are discussed.
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PMID:Autoimmune phenomena in patients with malignant carcinoid tumors during interferon-alpha treatment. 185 11

Vascular diseases are not encountered very often in gastroenterology, though in cases of ischemic colitis a coronary heart disease is often present. In addition, heart diseases such as coronary heart disease, atrial fibrillation, and congestive heart failure are important risk factors for ischemic colitis and should be treated to avoid further ischemic episodes of the gut. The most common extraintestinal manifestations of Crohn's disease and ulcerative colitis are observed in the eye, on the skin, and in the liver region. Involvement of the cardiovascular system does not seem to be very common, though systematic epidemiological data on the significance of these extraintestinal complications--which certainly influence the prognosis--are lacking. Other patients with Crohn's disease or ulcerative colitis suffer from vasculitis, which reflects a further manifestation of inflammatory diseases affecting the cardiovascular system. Another important complication is activation of coagulationfactors, especially during active flare-up of intestinal disease; this can result in thromboembolic events. Systematic studies or investigations on the epidemiology of cardiovascular complications are still lacking, so that an overview of the published data is given. Metastatic tumors of the heart are rare, but there are case reports of cardiac metastases in patients with carcinomas of the colon. Carcinoid heart syndrome, another cardial complication of malignant disease, can prejudice the prognosis of patients with neuroendocrine tumors of the gastrointestinal tract.
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PMID:[Concomitant cardiovascular conditions in intestinal illness]. 1718 44

Retroperitoneal fibrosis is a syndrome characterised by the presence of fibrosclerotic tissue in the retroperitoneum, often encasing the ureters. In most cases, retroperitoneal fibrosis is idiopathic, but may also be associated with large vessel vasculitis at distant sites, with the so-called IgG4-related sclerosing disease, as well as with exposure to some medications, infections, malignancies, surgery, or radiation. 18-Fluorodeoxyglucose (FDG) positron emission tomography (PET) is a nuclear medicine technique which is able to accurately identify in vivo areas characterised by elevated glucose metabolism, such as inflammatory, infective, and neoplastic lesions. There is mounting evidence suggesting that FDG-PET may have a role in assessing disease activity in idiopathic retroperitoneal fibrosis, but the role of FDG-PET in secondary retroperitoneal fibrosis is less established. Herein, we present four patients with retroperitoneal fibrosis of different etiology (isolated idiopathic, associated with large-vessel involvement, associated with carcinoid tumour, and secondary to pergolide) who underwent FDG-PET as part of their workup. The implications of FGD-PET results in the diagnosis and treatment of retroperitoneal fibrosis of different etiology are discussed.
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PMID:Role of 18F-fluorodeoxyglucose positron emission tomography in the workup of retroperitoneal fibrosis. 2141 82

A 66-year-old woman presented with dry cough and subsequent intermittent breathlessness and wheeze. During this time she was treated by her general practitioner for chronic obstructive pulmonary disease. When her symptoms worsened and her lung function had deteriorated, a high resolution CT scan was undertaken. This showed bilateral fine nodules. Investigations did not support a diagnosis of vasculitis and sarcoidosis was suspected. A lung biopsy was performed that showed diffuse idiopathic neuroendocrine cell hyperplasia. At follow-up, to date, the patient remains symptomatic but has not progressed or developed a carcinoid tumour.
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PMID:Diffuse idiopathic neuroendocrine cell hyperplasia: an unusual cause of breathlessness and pulmonary nodules. 2279 6

Granulomatosis with polyangiitis (GPA), previously termed Wegener's Granulomatosis, is an autoimmune small vessel vasculitis which is highly associated with antineutrophil cytoplasmic antibodies (ANCA) and has varied clinical manifestations. Diagnosis hinges on identifying a combination of clinical features of systemic vasculitis, positive ANCA serology, and histological evidence of necrotizing vasculitis, necrotizing glomerulonephritis, or granulomatous inflammation from a relevant organ biopsy. The American College of Rheumatology has also developed a classification criteria focusing specifically on nasal or oral inflammation, abnormal chest radiograph, and abnormal urinary sediment, along with granulomatous inflammation, which helps to distinguish GPA from other forms of systemic vasculitis. In the case presented below, the diagnosis of GPA was delayed as the patient had a concomitant atypical endobronchial carcinoid which predisposed to postobstructive pneumonia. Fortunately, the papular lesions that developed across her lower limbs prompted further investigations. The return of appropriate serology coincided with progression to alveolar hemorrhage, offering a more complete clinical picture, and when she responded to the combination of steroid, cyclophosphamide, and plasma exchange, the diagnosis of GPA was cinched.
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PMID:Atypical Endobronchial Carcinoid with Postobstructive Pneumonia Obscuring the Diagnosis of Granulomatosis with Polyangiitis. 2634 44

Giant cell arteritis (GCA), a systemic vasculitis of unknown origin, may appear rarely as a paraneoplastic syndrome. Cases secondary to pulmonary neuroendocrine tumors have not been reported. A 75-year-old female developed prednisone-responsive GCA/polymyalgia rheumatica (PMR) shortly followed by syndrome of inappropriate antidiuretic hormone secretion. An 8 mm carcinoid lung tumor with positron emission tomography normal uptake was found. After a thoracoscopic tumor resection the patient experienced complete clinical and laboratory remission. This is the first report of GCA with PMR in the context of carcinoid lung tumor. It emphasizes the role of paraneoplastic vasculitis as a possible cause of GCA.
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PMID:Giant cell arteritis and polymyalgia rheumatica as first manifestation of typical pulmonary carcinoid tumor. 2721 83