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)

We report a case of vasculitis with predominant aortic involvement. Vasculitis of large vessels has a limited number of tools for diagnosis and follow-up. A 78-year-old woman was referred to the internal medicine department with a 2-month history of fever of unknown origin (FUO), night sweats, weight loss and markedly elevated ESR and CRP. The results of an extended routine investigation found no infection, malignancy, hypersensitivity or autoimmune disorder. The patient did not suffer from claudication; systolic blood pressure difference between arms was 20 mm Hg. Temporal artery biopsies were negative. 2-18F-Fluorine-2-deoxy-D -glucose positron emission tomography (FDG PET) scan imaging demonstrated intense FDG uptake along the aorta and in the brachio-cephalic and carotid arteries consistent with arteritis. A high dose of corticosteroid therapy (1 mg/kg) was instituted with further tapering. The therapy was followed by complete resolution of the symptoms and pathological FDG uptake on repeated FDG PET. Second-line therapy was not added because of positive conversion of Mantaux test followed by rifampicin prophylaxis. FDG PET should be a part of the work-up of FUO when routine investigation fails to determine its etiology. FDG PET is useful both for diagnosis and assessment of response to therapy for large-vessel vasculitis.
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PMID:Fever due to aortitis. 1636 88

Changes of the affective and cognitive function are usually associated with thyroid gland dysfunction. In autoimmune thyroid disease, these changes can be caused by thyroid dysfunction (hypo- or hyperthyroidism) or associated with the presence of antithyroid antibodies. Even a small change in thyroid hormone concentration is associated with change of cognitive function. In euthyroid older males, variation of total and free thyroxin accounts for about 10% of Wechsler adult intelligence test variance. In euthyroid females, lower cognitive function, measured by Mini Mental test, also correlates with blood thyroxin. Short-term (4 weeks) hypothyroidism induces clinically significant cognitivedysfunction, which is reversible by thyroid hormone substitution. Mild hypothyroidism (TSH less than 10) also induces reversible cognitive dysfunction. In hypothyroidism, PET scanning shows global reduction of brain blood flow and glucose metabolism. Hashimoto's encephalopathy is characterized by corticosteroid reversible encephalopathy associated with the presence of antithyroid antibodies. Encephalopathy can be manifested as multiple stroke-like episodes (vasculitis like), or as diffuse, progressive type characterized by dementia and psychiatric symptoms. In euthyroid patients with Hashimoto's thyroiditis and no evidence of neurological disease, SPECT showed brain perfusion abnormalities. Post mortem and brain biopsy findings can be normal or show perivascular lymphocytic infiltration. Recently, presence of antineuronal antibodies has been found in patients with Hashimoto's thyroiditis. Specific high reactivity against human alpha-enolase was high in patients with Hashimoto's encephalopathy, but absent in patients with other neurological disorders and healthy subjects. Specific antineural antibodies were found in another group of Hashimoto's encephalopathy patients. Furthermore, Ferracci et al, found antithyroid antibodies in the CSF of patients with Hashimoto's encephalopathy.
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PMID:[Autoimmune thyroid disease and brain]. 1640 64

The appearance of vasculitis is often observed following the use of epirubicin hydrochloride in outpatients. As it is important to continue to provide a safe treatment for outpatient with little or no side effects, the aim of this study was to reveal causes underlying the outbreak of vasculitis or the vasculitic state of patients who had undergone instillation of epirubicin hydrochloride as an adjuvant therapy following a breast cancer surgery. The study was conducted based on past investigation records. We extracted relevant factors such as age, previous illness, physical condition and dosage of epirubicin hydrochloride from past records which may all be connected to the outbreak of vasculitis. We administered one dose of epirubicin hydrochloride that was diluted into 50-100 ml of normal saline solution or 5% glucose and instilled over a period of 30 minutes. As a result of this instillation of epirubicin hydrochloride, 35 out of 45 cases displayed vasculitis. We did not observe any relationship among the aged (over 60), previous illness or physical condition (BMI over 25) and the appearance of vasculitis. When a dosage amount of more than 110 mg of epirubicin hydrochloride was administered, 23 out of 29 cases displayed vasculitis. From the past records, it was observed that the cause of vasculitis was dependent on the administrative method itself. As previous studies reported that vasculitis was low when epirubicin hydrochloride was retained in blood vessels for only a short time, our study, however, concentrated on how to administer epirubicin hydrochloride. Our results suggested that an adoption of a single dosage system may suppress the outbreak of vasculitis.
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PMID:[Vasculitic condition following instillation of epirubicin hydrochloride in an outpatient chemotherapy]. 1642 79

Idiopathic hypertrophic cranial pachymeningitis is a rare chronic inflammatory and fibrosing process of unknown etiology. This entity is characterized by fibrosis and thickening of the dura mater and resulting in neurological syndrome. The authors report a 72 year-old woman who presented with progressive bifrontal headache, bilateral visual loss and transient episode of confusion. Neurological examination revealed bilateral optic atrophy, apathy and no focal neurological deficit. Investigations showed anemia of chronic disease, elevated erythrocyte sedimentation rate and polyclonal hypergammaglobulinemia. No specific inflammatory diseases or malignancy such as systemic lupus erythematosus, syphilis, hematologic malignancy were found MRI of the brain revealed thickened and enhanced dura mater and leptomeninges at the inferior aspect of bilateral frontal lobes as well as vasogenic edema of the frontal lobes. Cerebrospinal fluid showed mild pleocytosis, high protein level and normal glucose level. Meningeal biopsy revealed nonspecific inflammatory process of the dura and leptomeninges. There was no granuloma formation or evidence of vasculitis. Special stain for tuberculous bacilli, fungus and malignancy were all negative. The diagnosis of "idiopathic hypertrophic pachymeningitis" was made. The patient was treated with oral prednisolone 45 mg/day. Her headache was improved, but the profound vision loss in both eyes remained unchanged after 2 years of follow-up. Prednisolone was tapered within 18 months. Idiopathic hypertrophic cranial pachymeningitis usually involves dura at tentorium cerebelli, cavernous sinus and base of the skull. The extensive involvement at the anterior cranial fossa is extremely rare.
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PMID:Extensive anterior cranial fossa idiopathic hypertrophic pachymeningitis: a case report and review of the literature. 1651 96

Anti-neutrophil cytoplasmic antibodies (ANCA) not only are triggered by target protein myeloperoxidase (MPO) and proteinase 3 (PR3) of polymorphonuclear neutrophil (PMN) but also react with primed PMN to exert the inflammatory process in vasculitis syndrome. To clarify the crucial role of PMN in ANCA-associated vasculitis and the related mechanism, PMN was cultured with monoclonal antibody MPO-ANCA and PR3-ANCA to determine the function of phagocytosis, Interleukin- 8 (IL-8) production, glucose uptake, and TNF-related apoptosis induced ligand (TRAIL) production. The spontaneous membrane expression of MPO and PR3 on PMN could be significantly increased by lipopolysaccharide (LPS) and TNF-alpha, but not by IL-8 or GRO-alpha. The PMN-stimulating activity of ANCA was demonstrated by enhancing phagocytosis, IL-8 production, and glucose uptake that was more prominent by MPO-ANCA. The PMN stimulation by ANCA was not through protein kinase, H2O2, or superoxide anion radicals as their inhibitors exerted no effect on ANCA-mediated activation. On the other hand, ANCA also accelerated PMN apoptosis and increased TRAIL production. These results demonstrate that activation-induced cell death (AICD) mechanism could be initiated in PMN with existence of ANCA. In conclusion, MPO-ANCA is more potent in stimulating PMN than PR3-ANCA. ANCA-activated PMN is not only responsible for the amplified inflammatory process in blood vessel but also initiates immune circuit via triggered macrophage/monocyte by apoptotic PMN through the mechanism of AICD elicited by ANCA.
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PMID:Anti-myeloperoxidase antibodies enhance phagocytosis, IL-8 production, and glucose uptake of polymorphonuclear neutrophils rather than anti-proteinase 3 antibodies leading to activation-induced cell death of the neutrophils. 1657 89

We report on the case of a 60-year-old woman with complaints of fatigue, coughing, anorexia, atypical chest pain, recurrent fever, and also ear pain and hearing loss. A test for anti-neutrophil cytoplasmic antibody (ANCA) was myeloperoxidase positive with p-ANCA specificity. Laboratory acute phase parameters were increased. A 2-deoxy-2-[(18)F]fluoro-D: -glucose positron emission tomography/computed tomography investigation showed pathological uptake in the aorta ascendens, with no other involvement of the large vessels. After therapy with methylprednisolon intravenously and later prednisolon orally with methothrexate, her general condition and hearing loss improved both subjectively and objectively. "Atypical" Cogan's syndrome was diagnosed on the basis of sensorineural deafness with improvement on steroids and large-vessel vasculitis of the aortic arch.
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PMID:The role of PET/CT in Cogan's syndrome. 1763 64

A 9-week-year-old boy was admitted to the pediatric clinic after 12 hours of fever and diarrhoea. On admission he had a fever of 40 degrees C, rhinitis and moderate diarrhoea. Blood tests were normal, except for elevated CRP (89 mg/L). The tentative diagnosis was viral gastroenteritis, and peroral rehydration with a hypertone glucose-salt-mixture was started. CRP reached a maximum of 199 mg/L, and the boy stayed febrile throughout the next days. Stool examination revealed Enterovirus. After several clinical examinations, findings included a reactive cervical glandular node and mild conjunctivitis. When the child had been febrile for five days, he was admitted to an echocardiography, which showed dilated coronary arteries with abnormal caliber variations and a small amount of pericardial fluid. Since he fulfilled only three of the five criteria of classical Kawasaki disease, he was diagnosed as having neonatal, incomplete Kawasaki disease. Kawasaki disease is an immunologic vasculitis that appears mainly in children between three months and 12 years of age. Only 2% of patients with Kawasaki disease are less than three months old. Neonates with Kawasaki disease often have an atypical presentation of symptoms and have a particular risk of developing coronary artery aneurysms with potentially fatal outcome. With this case-report we will stress the importance of considering Kawasaki disease in neonates with fever of unknown origin.
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PMID:[A 9-week-old boy with fever and diarrhoea]. 1794 Nov 56

We report on a 11-year-old boy who had 2 acute hemiparesis episodes over a period of 1 month. He suffered from headache and fatigue since 1 year. He could not remember neither a tick bite nor a local erythematous skin lesion. The diagnosis of neuroborreliosis was based on intrathecal production of specifics antibodies. Furthermore, the CSF/blood glucose ratio was decreased (0.14), which was rarely described. Cranial MRI showed left capsulothalamic inflammation and a vasculitis. The patient was successfully treated by ceftriaxone. Neuroborreliosis should be considered in all children with stroke-like episode, even in the absence of a history of a tick bite.
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PMID:[Acute hemiparesis revealing a neuroborreliosis in a child]. 1815 90

Positron emission tomography is an imaging technique based on the use of radiopharmaceuticals. The most extended one is the glucose analogue 18F fluorodeoxyglucose (FDG) that is deposited where there is an increase of glycolytic metabolism, whether this is caused by neoplastic, inflammatory or infectious diseases. It is used in oncology for the initial staging, to assess response to treatment, residual disease, recurrent diagnosis and restaging, but specifically among the different types of tumor. It also has a field in the study of large vessel vasculitis, in granulomatous diseases and in dementias.
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PMID:[Diagnostic limitations of positron emission tomography. What are we seeking?]. 1826 95

Hypertrophic cranial pachymeningitis (HCP) is an uncommon disorder with few studies correlating clinical, imaging and histopathological features. The aim of this study was to describe clinical and laboratory observations and therapeutic options of patients with HCP. Eleven patients with HCP (M:F 6:5; age range, 23-52 years) were evaluated over 10 years. Etiology was ascertained by MRI and laboratory tests and confirmed by biopsy of meninges and/or brain (7), nasal mucosa (1), mediastinal lymph node (1), muscle (2) or conjunctiva (2). Salient clinical features were headache (7), multiple cranial neuropathies (8), visual disturbances (6), seizures (2) and hemiparesis (2). Abnormal tests included: rapid erythrocyte sedimentation rate (3), positive serum venereal disease testing (1), chest CT abnormalities (4/6) and positive Mantoux test (2/5). Cerebrospinal fluid changes (10/11) revealed the following: cell count 0-47/mm(3); protein 14-95 mg/mL; and glucose of 44-79 mg/mL. Contrast MRI revealed a variable extent of thickened dura mater in all patients. Histopathology (n=11) confirmed chronic inflammation (100%) and provided specific etiology in six (vasculitis [2], sarcoidosis [2], tuberculosis [1], Wegener's granulomatosis [1]). Treatment included steroids only (4), anti-tubercular therapy with steroids (5), penicillin (1) and cyclophosphamide and plasmapheresis (1). During follow-up (27.0+/-26.3 months) there was significant recovery (9/9). On serial imaging (4), the lesion remained the same in three and resolved partially in one patient. HCP, despite frequently posing diagnostic and therapeutic challenges, has favorable outcome when treated appropriately.
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PMID:Hypertrophic cranial pachymeningitis in countries endemic for tuberculosis: diagnostic and therapeutic dilemmas. 1828 Jan 67


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