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)

This study assayed serum levels of FVIII Rag as a marker of endothelial injury in patients not only with frank connective tissue disease but also in those presenting with Raynaud's phenomenon and in families of those with systemic sclerosis. Elevated levels of FVIII Rag were found in 62% of patients with systemic sclerosis (SS), 38% with systemic lupus erythematosus (SLE), 67% with mixed connective tissue disease (MCTD) and in 17% with primary Raynaud's phenomenon. Twenty per cent of first degree relatives of patients with SS also demonstrated high levels of FVIII Rag and certain antibodies, namely those reacting with U1RNP and the centromere. The association between elevated FVIII Rag and antibodies linked to Raynaud's and vasculitis lends support to antibody involvement in pathogenesis. High levels of FVIII Rag in family members may reflect an increased susceptibility of endothelium to injury particularly since relatives also have a higher frequency of clinical features such as Raynaud's phenomenon.
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PMID:Factor VIII related antigen in connective tissue disease patients and relatives. 228 86

The data are available on concentrations of Willebrand factor antigen (FVIII Ag) in 43 patients with rheumatoid arthritis (RA), 19 patients with livedo vasculitis (LV) and 56 donors. The measurements were made with solid-phase enzyme immunoassay. RA patients were found to display significantly higher concentrations of FVIII Ag (1.88 +/- 0.17 IU/ml) versus donors (1.06 +/- 0.05 IU/ml, p < 0.001) and LV patients (1.08 +/- 0.09 IU/ml, p < 0.001). No significant differences existed between FVIII Ag concentrations in LV patients and donors (p > 0.05). In 12 (28%) out of 43 RA patients FVIII Ag levels rose to 3 standard deviations from the mean in donors. Hyperproduction of FVIII Ag was associated with skin vasculitis symptoms in RA patients (p = 0.0004), more frequent occurrence of antinuclear factor (p = 0.02). Elevated concentrations of FVIII Ag did not relate to other extraarticular RA symptoms and general rheumatic inflammation, X-ray stage, RF titer, cryoglobulins, enhanced ESR, C-reactive protein.
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PMID:[The von Willebrand factor antigen in patients with rheumatoid arthritis: a method for its determination and the clinical significance]. 805 12

We describe a 51-year old-woman with haemorrhagic syndrome manifested as spontaneous bruising, and prolonged and abundant menstrual bleeding. She had hed these symptomes for six months prior to admission, but denied lifelong bleeding tendency. Gynaecological examination revealed a myomatouse uterus. Occasionally, the patient had crops of erythemo-exudative skin lesion which were shown on biopsy to represent urticarial (leukocytoclastic) vasculitis. No evidence of systemic connective tissue disease was found. Haemostasis testing revealed: prolonged bleeding time, decreased platelet adhesiveness to glass beads, lowered FVIII : C, absence of ristocetin-induced platelet aggregation and unmeasurable FVIII : Af and vWf. There was no evidence for an inhibitor to FVIII : C. However, patient's plasma, when mixed ana partes equales with normal plasma, reduced the FVIII : Ag and vWf level to 48% and 46% respectively, as compared to the mixture of normal plasma and buffer. When incubated at room temperature, patient's washed platelets spontaneously released vWf and aggregated in the presence ristocetin. However, when patient's plasma was added aggregation was inhibited. On the basis of these findings and the lack of anamnestic data suggestive of congenital haemorrhagic disorder, we concluded that the patient had acquired von Willebrand disease due to an inhibitor to vWf and FVIII : Ag, associated with urticarial vasculitis and myomatouse uterus. To our knowledge, this association has not yet been reported.
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PMID:[Acquired von Willebrand disease in a patient with urticarial vasculitis and myomatouse uterus]. 2121 30

Patients with hemophilia suffer from repeated episodes of hemarthrosis leading to chronic inflammation and synovitis. Radiosynovectomy is an effective nonsurgical modality that can reduce inflammation, pain, and hemarthrosis in such cases. We describe an adolescent male with severe Hemophilia A, who developed arterial vasculitis and perivasculitis targeting the brachiocephalic, right common carotid, and right subclabvian arteries occurring within few days after difficult Phosphorus-32 radiosynovectomy, possibly as a complication of the procedure. Despite prophylaxis with recombinant FVIII therapy, he developed chronic synovitis and underwent radionuclide synovectomy with P-32 injection to the left ankle and right knee. Five days later, he developed pain in the lower right neck and right upper chest. Computed tomography and magnetic resonance imaging and angiography demonstrated inflammation involving the arteries of the right thoracic inlet. Geiger-Mueller meter indicated increased radioactivity not only in the left ankle and right knee but also in the right upper chest. Detection of radioisotope at the right thoracic inlet corresponding to the area of vasculitis was indicative of likely deposition of the P-32 isotope in an area exposed to maximum cardiac output and increased blood flow, leading to subclavian, carotid, and innominate arteritis with surrounding edema.
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PMID:Remote arterial vasculitis as a possible complication of Phosphorus-32 Radiosynovectomy. 3040 63