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At least 55 records · Page 3Linked to original sources

PubMed2004

Serial magnet resonance angiography in patients with vasculitis and vasculitis-like angiopathy of the central nervous system.

The purpose of the study was to examine the value of the non-invasive magnet resonance angiography (MRA) in the follow-up of cerebral vasculitis (CV) and vasculitis-like angiopathy. We performed follow-up MRA (TOF 3D), MRI and transcranial doppler ultrasound (TCD) in the patients with isolated angiitis of the CNS (2/6), Crohn-disease-associated CV (1/6), and reversible arterial vasoconstriction (RAV) of the CNS (1 migraine, 1 eclampsia and 1 toxic encephalopathy) (3/6). In all patients with RAV MRA showed a complete remission of the vascular alterations after treatment. In the patients with isolated angiitis of the CNS and Crohn-disease-associated CV, partly regressive and partly progressive changes were demonstrated. The MR-angiographically detectable vascular alterations corresponded to the clinical course of the disease, as well as to TCD in all our patients. Success of therapeutic procedures, the need and the intensity of further drug administration could be estimated. The MRA appears to be a valuable non-invasive method in the follow-up of patients with CV and RAV.

Adolescent↗

PubMed1993

[Vasculitis-related acute abdomen in systemic lupus erythematosus--ultrasound appearances in lupus patients with intra-abdominal vasculitis].

Although an acute abdomen often occurs in lupus patients, definitive treatment is commonly delayed due to the difficulty in diagnosis. We performed an abdominal ultrasound (US) in two patients with severe abdominal pain and recognized mural thickenings of intestinal walls. In the first case high dose prednisone was given for severe abdominal pain, with subsequent perforation of sigmoid colon. Pathological specimens showed a thrombotic vasculitis in the mesenteric artery and arteriole of intestinal walls. In the second case physical examination elicited a rebound tenderness. An abdominal X-ray demonstrated a gasless ileus and US also showed marked thickening of intestinal walls, 16 mm in thickness. A CT scan revealed the same findings in the entire bowel. After high dose methylprednisolone was administered, her symptoms markedly improved. The mural thickenings disappeared within 10 days as seen by abdominal ultrasound examination. The US is very useful in order to make an early diagnosis of intestinal vasculitis, showing as edema (but non-specific) of intestinal wall and we can use it safely and repetitively without exposure to radiation, compared with other examinations, such as CT scan and gastrointestinal contrast study etc. The US is not only useful in diagnosis, but also helpful for follow up of patients after the initiation of treatment.

Abdomen↗

PubMed1991

Cutaneous vasculitis, hypersensitivity vasculitis, erythema nodosum, and pyoderma gangrenosum.

Cutaneous vasculitis encompasses a highly heterogeneous group of disorders of diverse etiology, pathogenesis, and clinical features. Recent reexamination of clinical pathologic features have yielded no insights assisting our understanding of current clinical classification schemes and strengthening our clinical approach. Erythema nodosum is a common cutaneous disorder for which new etiologies continue to be described. Pyoderma gangrenosum is an uncommon ulcerative cutaneous condition that remains a therapeutic problem.

Erythema Nodosum↗

PubMed1986

Necrotizing systemic vasculitis with features of both Wegener's granulomatosis and Churg-Strauss vasculitis.

We describe a patient who had nasal biopsy-demonstrated eosinophilic vasculitis and renal biopsy-demonstrated necrotizing glomerulonephritis with tissue eosinophilia. Despite corticosteroid therapy, the patient's renal function deteriorated, and nodular pulmonary infiltrates developed. Both conditions responded dramatically when cyclophosphamide was added to the treatment regimen. The renal disease activity was monitored with the aid of cytodiagnostic urinalysis, a technique of limited, albeit well-established, validity in monitoring renal allograft patients for signs of tissue rejection. This technique provided an improved, semi-quantitative method for examining urine sediment and, in this patient, was helpful as a measure of renal disease activity.

Adult↗

PubMed2002

[Scientifically based patient education exemplified by patients with primary systemic vasculitis. The vasculitis patient education program--development, contents and initial results of evaluation].

Referring to the literature, it is known that structured standardized patient education represents an effective additional treatment in patients with chronic diseases. Most programs are based on cognitive behavioral interventions. Within the last few years, an interdisciplinary approach for providing information on disease, therapies, side effects, coping strategies, nutrition and physiotherapy has been developed for patients with primary systemic vasculitides (PSV) in the vasculitis center of the Medical University of Lübeck/Bad Bramstedt. The contents of the seminars were revised and condensed into five modules. New slides and handouts for patients were developed. To evaluate the new form of the program, a documentation system consisting of patient and physician-administered questionnaires assessing socioeconomic, knowledge and disease-related outcome-parameters was designed. Patients are trained in closed groups (n = 10-15) and asked to complete questionnaires at baseline, 4 weeks and 6 months after training. First results show statistically significant improvement of knowledge and health-related quality of life.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed2003

Extensive dynamics in location, shape, and size of aneurysms in a patient with idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome. Idiopathic retinal vasculitis, aneurysms, and neuroretinitis.

PURPOSE: To describe an unusual case of idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome with rapid dynamics in the number and appearance of the aneurysms. DESIGN: Observational case report. METHODS: Clinical and angiographic data of the patient were reviewed. RESULTS: In the course of only 6 months, preexisting retinal aneurysms resolved while new ones appeared. Changes were observed in the shape and size of preexisting lesions. The resolution of lesions in eyes previously untreated by laser is reported for the first time. CONCLUSIONS: Vascular lesions in IRVAN syndrome may show an unusually rapid turnover. The resolution of aneurysms is a part of the natural course of the disease and may occur without previous retinal laser photocoagulation.

Adult↗

PubMed1997

Treatment of antineutrophil cytoplasm autoantibody-associated systemic vasculitis: initiatives of the European Community Systemic Vasculitis Clinical Trials Study Group.

The diagnosis, treatment, and monitoring of the primary systemic vasculitides associated with circulating antineutrophil cytoplasm autoantibodies (ANCA) have formed the focus of a multicenter collaborative study. Consensus has been reached on criteria for classification, clinical subgroupings by extent and severity of disease, and "standard" and "best alternative" therapeutic regimens. Two series of randomized controlled trials have been designed; their aims are (1) to harmonize current approaches to treatment and (2) to test the value of newer therapeutic agents. In support of these trials, semiobjective scoring systems have been created and validated, and previous standardization of ANCA serologic and histologic analysis has been adopted. The systems of classification and clinical management described herein represent the recommendations of a multidisciplinary study group that hopes to improve the outcome of patients with primary systemic vasculitis by wide dissemination of the collective experience from interested centers.

Adrenal Cortex Hormones↗

PubMed1998

Mixed cryoglobulinaemia, glomerulonephritis, and ANCA: essential cryoglobulinaemic vasculitis or ANCA-associated vasculitis?

Antineutrophil cytoplasmic antibodies (ANCA) are generally believed to be strongly associated with some primary systemic vasculitides (PSV), such as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), which have some clinical manifestations in common and are 'pauci-immune' by immunohistology. This group of PSV has thus been termed 'ANCA-associated vasculitis' (AAV). By contrast, essential mixed cryoglobulinaemic vasculitides (EMC) are clinically heterogeneous and characterized immunologically by complement consumption and by immunocomplex depositions; they are also characteristically ANCA-negative. We report here on two SV-patients in whom the detection of cANCA (proteinase 3-ANCA in one case) in conjunction with glomerulonephritis and various extrarenal vasculitic lesions was suggestive of an AAV. However, demonstration of type II cryoglobulinaemia in conjunction with hypocomplementaemia, and histological proof of immunocomplexes in the glomerulus led to the diagnosis of an EMC, which was associated with hepatitis C virus (HCV) infection in one of the cases. Against the setting of 'false positive' cANCA in EMC, we discuss the differential diagnostic steps as well as current differential therapeutic approaches.

Adult↗

PubMed2001

Cryptogenetic multifocal ulcerous stenosing enteritis: an atypical type of vasculitis or a disease mimicking vasculitis.

BACKGROUND/AIMS: Cryptogenetic multifocal ulcerous stenosing enteritis (CMUSE) is a rare disease whose origin is unknown. The aim of this study was to describe the clinical spectrum of CMUSE, to determine the origin and pathophysiology of the disease, and to propose a treatment strategy. METHODS: A total of 220 French gastroenterology departments were contacted to review patients with unexplained small bowel strictures. Of 17 responses, 12 corresponded to a diagnosis of CMUSE. These patients were hospitalised between 1965 and 1993 and their medical records were reviewed. RESULTS: All patients (mean age 42.1 (4.4) years) had intestinal and five had extraintestinal symptoms (peripheral neuropathy, buccal aphthae, sicca syndrome, polyarthralgia, Raynaud's phenomenon, arterial hypertension). One patient had heterozygous type I C2 deficiency (28 base pair gene deletion). Two to 25 (mean 8.3 (1.9)) small intestine strictures were found. Stenoses of the large jejunoileal arteries were observed on two and aneurysms on three of five mesenteric angiograms. Despite surgery, symptoms recurred in seven of 10 patients and strictures in four. Steroid therapy was effective but caused dependence. One untreated patient died. Small bowel pathology showed superficial ulceration of the mucosae and submucosae, and an inflammatory infiltrate made of neutrophils and eosinophils. CONCLUSIONS: CMUSE is an independent entity characterised by steroid sensitive inflammation of the small bowel which often recurs after surgery. CMUSE may be related to a particular form of polyarteritis nodosa with mainly intestinal expression or with an as yet unclassified vasculitis.

Adolescent↗

PubMed1990