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)

An 18-year-old woman was admitted to hospital because of subcutaneous hematoma and fever of unknown origin. Acute myeloid leukemia was diagnosed and empirical antimicrobial treatment and induction chemotherapy were started. After initial defervescence, fever relapsed 2 days after the onset of neutropenia. The CT scan of the lung was consistent with an invasive fungal infection. Treatment with amphotericin B was started and antimicrobial treatment was continued with liposomal amphotericin B because of an increase in creatinine later. The fever persisted and the patient suddenly developed progressive neurological symptoms. CT scan of the head suggested cerebral infarction and angiography of the extra- and intracranial arteries showed signs of vasculitis. Six days after the onset of neurological symptoms cerebral death was diagnosed. Autopsy revealed non-septate, irregularly branched hyphae in various histologic sections including brain. Absidia corymbifera could be isolated from lung tissue confirming the diagnosis of disseminated mucormycosis. In this case, angiographic findings suggested severe cerebral vasculitis which was in fact caused by thromboembolic dissemination of fungal hyphae. This case underlines the fact that cerebral symptoms in febrile neutropenic patients are highly indicative for fungal infections of the brain.
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PMID:Disseminated mucormycosis caused by Absidia corymbifera leading to cerebral vasculitis. 1096 35

Two cases of systemic antineutrophil cytoplasmic antibody (ANCA) vasculitis in the setting of chronic lymphocytic leukaemia and angioimmunoblastic lymphadenopathy type T cell lymphoma are reported. The two patients had fever of unknown origin associated with cutaneous vasculitis and "pulmonary-renal syndrome" with alveolar haemorrhage. Despite anti-infectious treatments, steroids, and chemotherapy, the vasculitis had a fatal paraneoplastic course in several weeks. When infection is excluded in patients with malignancy, atypical features should be promptly investigated for systemic vasculitis, and an ANCA test performed.
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PMID:Systemic antineutrophil cytoplasmic antibody vasculitis associated with lymphoid neoplasia. 1117 97

Few case reports have shown the presence of metastatic tumor cells in renal glomeruli. We report one case with intraglomerular metastasis proved at renal biopsy. A 60-year-old man suffered from weight loss and fever of unknown origin. Urinalysis revealed proteinuria with cellular and granular casts. Because vasculitis was suspected, renal biopsy was performed. Presence of tumor cells occupying the glomerular capillary lumina was shown by means of light microscopy and electron microscopy. Laboratory findings revealed elevated leukocyte count (28.9 x 10(3)/mm(3)), serum granulocyte colony-stimulating factor (G-CSF) (77 pg/mL), and serum CA 19-9 (21,885 U/mL). The patient soon developed disseminated intravascular coagulation and died. Autopsy findings revealed pancreatic cancer showing positive staining for G-CSF and CA 19-9. Tumor cells in the glomerular capillary lumina showed positive staining for CA 19-9 and proliferating cell nuclear antigen (PCNA). These results suggest that the pancreatic tumor cells producing G-CSF were entrapped in the glomerular capillary lumina where they proliferated. This may have been the first step in renal metastasis.
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PMID:Intraglomerular metastasis from pancreatic cancer. 1138 3

Temporal arteritis, the most common form of systemic vasculitis in adults, is a panarteritis that chiefly involves the extracranial branches of the carotid artery. The condition is illustrated in this article by the case of a 79-year-old woman with a dry cough, toothache, tongue infarction, and vision loss. The mean age of onset is 72 years and the disease rarely occurs in persons younger than 50 years. The most common presenting manifestations are headache, jaw claudication, polymyalgia rheumatica, and visual symptoms. Eighty-nine percent of patients have an erythrocyte sedimentation rate greater than 50 mm/h. However, about 40% of patients present with atypical manifestations, including fever of unknown origin, respiratory tract symptoms (especially dry cough), and large artery involvement. Familiarity with such unusual manifestations of temporal arteritis facilitates early diagnosis and treatment, thereby reducing the risk of vision loss.
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PMID:Temporal arteritis: a cough, toothache, and tongue infarction. 1223 25

Giant cell arteritis (GCA) is a systemic large vessel vasculitis. Awareness of various manifestations of GCA is essential for early recognition and prompt treatment so as to prevent complications like blindness. GCA is one of the relatively common causes of fever of unknown origin (FUO) in the elderly in USA and Europe. However, no such cases have been reported from India. A case of GCA presenting as FUO is reported and the literature reviewed.
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PMID:Giant cell arteritis--a rare cause of fever of unknown origin in India. 1224 Aug 61

Microscopic polyangiitis (MPA) is an antineutrophil cytoplasmic antibody (ANCA) associated small vessel vasculitis which can present with various clinical manifestations, for which the mainstay of treatment is systemic corticosteroids and immunosuppressants. We report a case of a 54-year-old female admitted to the hospital because of fever during the last month, leukocytosis and elevated erythrocyte sedimentation rate. Persistence of elevated serum creatinine levels and accompanying hematuria led us to perform a renal biopsy, and MPA was diagnosed on the basis of light and immunofluorescence microscopy. Remission was induced with oral corticosteroids and cyclophosphamide therapy in conjunction with plasmapheresis (PF). The objective of this report was to assess the role of PF in the treatment of MPA and report on its utility in patients with MPA who are not responding to standard therapy or who require unacceptably high doses of steroids or immunosuppressants. In a patient presenting with fever of unknown origin, microscopic polyangiitis should also be considered in the differential diagnosis.
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PMID:MPO-ANCA-associated small vessel vasculitis presenting as fever of unknown origin. Report of one case. 1237 53

Alpha-1-antitripsyn neutralizes the tissue damaging effects of proteases. Alpha-1-antitripsyn deficiency manifests with necrotizing vasculitis. Wegener's granulomatosis is a systemic necrotizing vasculitis that uncommonly affects the gut. The molecular genetics of patients with Wegener's granulomatosis of the gastrointestinal tract have never been characterized. A 63-year-old man with emphysema was admitted with a fever of unknown origin. Initially, this fever was linked to ileocolic Crohn's disease and later attributed to antineutrophil cytoplasm antibody-positive systemic vasculitis. Genetic analysis revealed that the alpha-1-antitripsyn deficiency was due to a previously unreported compound heterozygosity for two mutations (PiZ and PiMProcida). Our findings appear to support the concept that severe alpha-1-antitripsyn deficiency is implicated in the pathogenesis of the Crohn's disease-like milder intestinal manifestations belonging to the spectrum of Wegener's granulomatosis.
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PMID:Intestinal Wegener's granulomatosis in a patient with severe alpha-1-antitrypsin deficiency resulting from a unique combination of two deficiency alleles (PiZ and PiMProcida). 1246 63

Takayasu aortitis (TA) is a chronic inflammatory and fibrotic vasculitis of large- and medium-sized arteries. Early stages of the disease show a panarteritis and inflammatory wall thickening of the aorta and its branches, whereas advanced (fibrotic) stages comprise stenosis, aneurismatic transformation and occlusion. Magnetic resonance imaging visualises early-stage disease with high accuracy and is considered to be the method of choice in the diagnosis of TA. The aim of this article is the detailed comparison of FDG-PET performed with a hybrid camera and MR imaging in five patients with early TA. Five patients (median age 60 years) were enrolled during an ongoing prospective study on [18F]2'-deoxy-2-fluoro-D-glucose (FDG) hybrid camera PET in patients with fever of unknown origin (FUO). These patients underwent MR imaging after establishing the diagnosis of TA. Abnormal FDG uptake in the wall of the aorta was noted in all patients. The bracheocephalic artery and the common carotid arteries were visualized in 3 cases. Increased uptake of the subclavian artery was found in 3 patients and in 4 patients pathological uptake was noted in the ilio-femoral vessels. Of 34 vascular regions studied, 26 (76%) showed elevated FDG uptake. On transversal MR images vessel wall thickening and contrast enhancement of the thoracic aorta was found in 4 patients (ascending aorta/aortic arch: n=2; descending aorta: n=3; abdominal aorta: n=1). Additionally, vessel wall pathologies of the subclavian and the common carotid arteries could be shown in 1 patient and in another patient in the ilio-femoral arteries. No abnormalities were found using contrast-enhanced MR angiography. Of 28 vascular regions studied, 9 (32%) showed vasculitis on MRI. The FDG-PET is a suitable whole-body screening method in the primary diagnosis of early TA, especially in those cases with early disease that present with uncharacteristic symptoms such as FUO. Both MRI and MRA remain indispensable in the exact determination of the pathomorphological changes and in the documentation of complications such as stenosis, aneurismatic transformation and occlusion.
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PMID:Value of F-18 FDG hybrid camera PET and MRI in early takayasu aortitis. 1456 11

The aim of this prospective study was to compare fluorine-18 fluorodeoxyglucose ([(18)F]FDG) positron emission tomography (PET) with magnetic resonance imaging (MRI) in patients with early aortitis, at the time of initial diagnosis and during immunosuppressive therapy. The study population consisted of 15 patients (nine females and six males; median age 62 years, range 26-76 years) who presented with fever of unknown origin or an elevated erythrocyte sedimentation rate or elevated C-reactive protein and who showed pathological aortic [(18)F]FDG uptake. Fourteen of these patients had features of early giant cell arteritis (GCA), while one had features of early Takayasu arteritis. During follow-up, seven PET scans were performed in six patients with GCA 4-30 months (median 19 months) after starting immunosuppressive medication. The results of [(18)F]FDG imaging were compared with the results of MRI at initial evaluation and during follow-up and with the clinical findings. At baseline, abnormal [(18)F]FDG uptake was present in 59/104 (56%) of the vascular regions studied in 15 patients. Seven follow-up PET studies were performed in six patients. Of 30 regions with initial pathological uptake in these patients, 24 (80%) showed normalisation of uptake during follow-up. Normalisation of [(18)F]FDG uptake correlated with clinical improvement and with normalisation of the laboratory findings. All except one of the patients with positive aortic [(18)F]FDG uptake were investigated with MRI and MRA. Thirteen of these 14 patients showed inflammation in at least one vascular region. Of 76 vascular regions studied, 41 (53%) showed vasculitis on MRI. Of 76 vascular regions studied with both PET and MRI, 47 were concordantly positive or negative on both modalities, 11 were positive on MRI only and 18 were positive on PET only. MRI was performed during follow-up in six patients: of 17 regions with inflammatory changes, 15 regions remained unchanged and two showed improvement. Whole-body [(18)F]FDG PET is valuable in the primary diagnosis of early aortitis. The results of [(18)F]FDG PET and MRI in the diagnosis of aortitis in this study were comparable, but FDG imaging identified more vascular regions involved in the inflammatory process than did MRI. In a limited number of patients [(18)F]FDG PET was more reliable than MRI in monitoring disease activity during immunosuppressive therapy.
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PMID:Early diagnosis and follow-up of aortitis with [(18)F]FDG PET and MRI. 1267 2

Design: We evalueted incidence, period and etiology of the fever of difficult diagnosis (FDD) among 169 cases of fever of unknown origin (FUO) observed from 1-90 to 8-98. Materials and methods: we used the general and orientative diagnostic criteria useful for identifying some pathologies and also for distinguishing between infectious and non infectious fevers. Only as last attempt to solve the problem did we adopt ex-adiuvantibus treatments. Results: FDD incidence was 29.58%; we identified four main categories after detailed diagnostic work up:1) Fictitious fevers and abitual hyperthermia; 2) Systemic vasculitis with atypical clinical features; 3) Rheumatic polymyalgia; 4) Steroid-responder fevers. Conclusion: FDD is not a rare event, but of complicated resolution, some remaining unsolved. Our experience suggests that we consider only a few elements at first to distinguish organic from sine materia fevers: age, history of recent surgery, general clinical picture and aspecific indexes of inflammation.
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PMID:[Incidence of fever of difficult diagnosis observed from January 1990 to August 1998] 1275 88


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