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At least 325 records · Page 18Linked to original sources
PubMed2000

Antineutrophil cytoplasmic antibodies (ANCA) and systemic vasculitis: update of assays, immunopathogenesis, controversies, and report of a novel de novo ANCA-associated vasculitis after kidney transplantation.

OBJECTIVES: To characterize antineutrophil cytoplasmic antibodies (ANCA), their major autoantigens, disease associations, and pathophysiology in systemic vasculitides. To describe a patient with a novel de novo ANCA-associated vasculitis after kidney transplantation. METHODS: We reviewed and compiled the literature on ANCA-related topics and systemic vasculitis. Laboratory and clinical data from a cadaveric kidney transplant patient who developed necrotizing vasculitis involving glomerular capillaries, with crescent formation associated with P-ANCA and myeloperoxidase, were analyzed. RESULTS: Large-scale multi-center testing of patient and normal sera by the European ANCA Assay Standardization Project using immunofluorescence assays and enzyme immunoassays indicate the assays have good sensitivity and specificity, and diagnostic utility for ANCA-associated vasculitis. A few investigations covering basic and clinical research with ANCA remain controversial: whether endothelial cells do or do not express a 29-kd neutral serine protease termed proteinase-3 (PR-3), the target of ANCA in most individuals with Wegener's granulomatosis, and whether anti-myeloperoxidase (MPO) ANCAs recognize a restricted number of epitopes on MPO. This issue has relevance for using monoclonal antibodies to treat patients with vasculitis who have adverse effects from immunosuppressive drugs. The two allelic forms of FcgammaRIIa (H131/R131) and the two of FcgammaRIIlb (NA1/NA2) are discussed as possible inheritable genetic elements for vasculitic disorders and for signaling responses. Stimulatory and costimulatory molecules, and cytokine profiles of T lymphocytes are characterized to show that these cells are actively involved in the ANCA-associated vasculitides. The patient described had a de novo ANCA associated small vessel vasculitis which developed after renal transplantation. CONCLUSIONS: There have been significant advances in the development of sensitive and specific ANCA assays. The immunopathogenetic mechanism of ANCA involves the constitutive FcgammaRs, ligands, and signaling responses to activate cytokine-primed neutrophils. This may lead to the generation of reactive oxygen intermediates, degranulation, and secretion of intracellular granule contents, and ultimately inflammation and vasculitis.

Adult↗
PubMed1987

Phenylpropanolamine: an over-the-counter drug causing central nervous system vasculitis and intracerebral hemorrhage. Case report and review.

Phenylpropanolamine (PPA) is the major ingredient in more than 70 over-the-counter preparations including diet pills, nasal decongestants, and the legal "look-alike" stimulants. Structurally and functionally similar to amphetamine and ephedrine, PPA has recently been associated with several neurological manifestations including psychosis, stroke, severe headache, seizures, and intracerebral hematoma. We report a case of intracerebral hematoma and subarachnoid hemorrhage in a young woman with angiographic and biopsy-proven vasculitis of the central nervous system (CNS) induced by PPA in her diet pills. From review of the literature, we distinguish drug-induced vasculitis as a separate entity from primary CNS vasculitis, both clinically and pathologically. This report should alert physicians, in general, to this potentially fatal side effect of PPA, a commonly used over-the-counter drug. Also, neurosurgeons in particular should consider the possibility of drug-induced vasculitis when faced with cases of intracerebral or subarachnoid hemorrhage without apparent cause.

Adult↗
PubMed1998

Uveitis and central nervous system vasculitis.

Vasculitis confined to the central nervous system (CNS) is a rare disease usually characterized by headache and focal neurologic symptoms. Patients with primary vasculitis of the CNS may have symptoms and laboratory findings of systemic disease such as fatigue and elevated erythrocyte sedimentation rate, but by definition, focal inflammation should not be present outside the CNS. We describe 3 patients with uveitis in association with this diagnosis. The recognition of this association adds to the complex differential diagnosis of uveitis in association with CNS disease, and indicates that "isolated" angiitis of the CNS may display clinical features outside the brain and spinal cord.

Adult↗
PubMed2002

[Neurological manifestations of systemic necrotizing vasculitis].

Systemic vasculitides commonly induces peripheral neuropathies. In most cases signs and symptoms of multisystem involvement, including polyarthritis, asthma, renal and skin involvement, accompany peripheral neuropathy. In approximately one third of the patients however peripheral neuropathy is the first and only manifestation of necrotizing vasculitis. Nerve and muscle biopsy is often necessary to confirm vasculitis, which requires histological evidence for diagnosis. A focal or multifocal, axonal, ischemic neuropathy often results from involvement of nerve trunks vasa nervorum. Outcome of the neuropathy is often favorable with corticosteroids but the improvement is very slow and sometimes only partial.

Adrenal Cortex Hormones↗
PubMed2001

Central nervous system vasculitis as a complication of refractory dermatomyositis.

We describe a 47-year-old woman with refractory dermatomyositis (DM) who developed progressive cognitive dysfunction. Magnetic resonance imaging showed a large cerebral infarction, and the diagnosis of central nervous system (CNS) vasculitis was confirmed by both angiogram and brain biopsy. Her DM and CNS vasculitis responded promptly to the institution of daily cyclophosphamide, and her previously refractory disease entered remission.

Brain↗
PubMed1999

Isolated central nervous system vasculitis associated with hepatitis C infection.

Since its identification in 1989, hepatitis C has been implicated in the pathogenesis of an increasing number of diseases previously believed to be primary or idiopathic. We report 2 rarely seen cases of isolated central nervous system (CNS) vasculitis in patients with hepatitis C infection. Patient 1. A 43-year-old man with 4 day right temporal headache developed a left hemiparesis. Weakness was his only physical finding. Computed tomography (CT) scan demonstrated a large right frontotemporal hemorrhage, and angiography revealed focal dilatations and irregularities of multiple branches of the right middle and anterior cerebral arteries. Cerebral decompression was performed and leptomeningeal biopsies showed granulomatous angiitis. Laboratory results were normal except for elevated liver biochemical tests. Later testing for hepatitis C was positive. His neurological symptoms improved with corticosteroids and cyclophosphamide. Patient 2. A 39 yr old male developed 3 days of left sided weakness, slurred speech and difficulty swallowing fluids. Physical findings were limited to his weakness. Magnetic resonance imaging demonstrated a right superior pontine subacute infarct with a small left internal capsule lacunar infarct. Angiography revealed multiple areas of focal narrowing with no areas of abrupt vessel cut off. Cerebral spinal fluid showed 71 PMN, 29 RBC, normal glucose, elevated protein (64 mg/dl), no oligoclonal bands, and low myelin basic protein. Other laboratory analyses were normal including liver biochemical tests. However, hepatitis C serology was positive and mixed cryoglobulins were detected. CNS vasculitis was diagnosed and nearly full recovery was achieved with corticosteroids, cyclophosphamide and warfarin.

Adult↗
PubMed1994

Sensitivities of noninvasive tests for central nervous system vasculitis: a comparison of lumbar puncture, computed tomography, and magnetic resonance imaging.

OBJECTIVE: To determine the sensitivities of lumbar puncture (LP), computed tomography (CT), and magnetic resonance imaging (MRI) for central nervous system (CNS) vasculitis. METHODS: We reviewed the charts and radiologic studies of 20 patients with angiogram positive CNS vasculitis. RESULTS: The patients ranged in age from 7-72 years, with a mean of 43.7 +/- 15.5 years. Twelve patients (60%) were women; 8 (40%) were men. Seven had primary angiitis of the CNS (PACNS), 8 had rheumatological illnesses (4 had systemic lupus erythematosus), and 5 had vasculitis due to other diverse etiologies, including 2 who had CNS infections. LP was positive in 8/15 angiographically proven cases, for a sensitivity of 53% [95% confidence interval (CI): 27-79]. The sensitivity of CT was 65% (11/17) (95% CI: 38-86), and that of MRI 75% (12/16) (CI: 48-93). The use of LP plus either CT or MRI was more sensitive than LP alone: for LP and CT, the sensitivity was 92% (11/12) (CI: 62-100), and for LP and MRI, 100% (12/12) (CI: 74-100). The combination of CT and MRI was not more sensitive than either test alone. CONCLUSION: The sensitivities of LP, CT, and MRI for angiogram positive vasculitis are only modest. CT and MRI may be completely normal in cases of CNS vasculitis when the angiogram is positive. LP and either CT or MRI are of additive benefit in the diagnosis of CNS vasculitis, and should be done before angiography; and, in a patient with both a normal LP and a normal CT or MRI, a CNS angiogram is unlikely to be positive for vasculitis.

Adolescent↗
PubMed1977

Central nervous system vasculitis in rheumatoid arthritis.

The history of a patient with rheumatoid arthritis and necrotizing vasculitis affecting only the central nervous system is reported. Clinical and pathological involvement by this process was present in both cerebral hemispheres, the pons and spinal cord. Review of the literature revealed that cerebral vasculitis in rheumatoid arthritis has been reported rarely and spinal cord vasculitis not at all.

Arthritis, Rheumatoid↗
PubMed2003

Ocular vasculitis.

Systemic vasculitis can affect practically any structure in or around the eye. Although the ocular presentations of vasculitic disease are highly variable and may be quite subtle, it is important for physicians to understand some of the basic concepts, critical findings, and potential significance of ocular signs and symptoms. In addition to preserving sight, awareness of specific ocular syndromes may afford a critical clue to undiagnosed or newly active systemic vasculitis. A brief discussion of the most common ocular syndromes seen in systemic vascular disease is presented, with special attention given to the signs that may be appreciated by non-ophthalmologists. Recent concepts of ocular immunologic therapy are also reviewed, especially as they relate to the use of systemic immunomodulating drugs, because care of these patients often requires close cooperation between ophthalmologists, primary care physicians, and rheumatologists.

Clinical Trials as Topic↗
PubMed1996

Rheumatoid arthritis complicated by pachy- and leptomeningeal rheumatoid nodule-like granulomas and systemic vasculitis.

Rheumatoid nodule is a frequent and characteristic extra-articular manifestation of rheumatoid arthritis (RA). Its involvement of central nervous system is a rare occurrence with only a few reported cases. A 78-year-old man with severe arthritis showing the formation of rheumatoid nodule-like granulomas in the dura and subarachnoid space along with the spleen is presented. The characteristic morphological finding of the granulomas was the presence of neutrophils and the absence of definite fibrinoid necrosis, which differed from the typical features of rheumatoid nodules previously described. The diagnosis should be based on the exclusion of diseases that may cause similar granulomatous reactions including infectious diseases. Additionally, there was systemic necrotizing vasculitis in the dura and multiple cerebral infarcts, although the association between vasculitis and cerebral infarcts was not clear.

Aged↗
PubMed1991

[Systemic vasculitis combined with connective tissue involvement in a patient with yersiniosis].

A clinico-morphological description is presented of a severe systemic disease in 36-year-old male, the trigger factor of which was Yersinia enterocolitica. The patient developed a peculiar nosological variant of systemic necrotic vasculitis of infectious-allergic genesis with signs of nodular periarteritis in the intestine and kidneys in association with diffuse involvement of the connective tissue which resulted 6 months later in a dominating abdominal syndrome with profuse diarrhea, progressive cachexia with a fatal outcome. The diagnosis was established after death.

Acute Disease↗
PubMed2000

Suppression of inflammation in primary systemic vasculitis restores vascular endothelial function: lessons for atherosclerotic disease?

BACKGROUND: Chronic inflammatory rheumatic disorders are associated with excess cardiovascular mortality. This may result from arteriosclerosis following inflammatory damage to the vessel wall by vasculitis. Our hypothesis that vasculitis results in arteriosclerosis by causing vascular endothelial dysfunction was tested in patients with primary systemic necrotizing vasculitis (SNV). METHODS AND RESULTS: Endothelial function was assessed in cross-sectional and longitudinal studies of patients with primary SNV by measuring flow-mediated, endothelium-dependent brachial artery vasodilatation. These patients exhibited marked endothelial dysfunction compared with controls. Remission induction in patients with active primary SNV restored endothelial function. CONCLUSIONS: Endothelial function is significantly impaired in adults with primary SNV, supporting the hypothesis that premature arteriosclerosis in chronic inflammatory rheumatic disorders results from endothelial dysfunction secondary to vasculitis. Normalization of endothelial function after the treatment of primary SNV suggests that early suppression of disease activity in chronic inflammatory rheumatic disorders may reduce long-term vascular damage. The role of inflammation in atheroma formation is increasingly appreciated; this work raises questions regarding the potential for anti-inflammatory therapy in atherosclerosis itself.

Brachial Artery↗
PubMed1991

Antineutrophil cytoplasmic antibody--a useful serological marker for vasculitis.

Systemic necrotizing vasculitides, including polyarteritis nodosa, Churg-Strauss syndrome, "overlap" systemic vasculitis, Wegener's granulomatosis, and idiopathic crescentic glomerulonephritis, are frequent clinical diagnostic problems. These diseases have diverse presentations and are often rapidly progressive, causing irreversible injury to the vessels of the kidneys and lungs before effective immunosuppressive therapy is instituted. Even in their less fulminant forms, they are a cause of significant morbidity and mortality. Antineutrophil cytoplasmic antibody, a recently identified autoantibody, has a high sensitivity and specificity for this spectrum of diseases. The clinical and pathological similarities, the high frequency of antineutrophil cytoplasmic antibody expression, and the similar good response to immunosuppressive therapy suggest that these diseases may be linked by a common pathophysiological mechanisms. Evidence is growing that antineutrophil cytoplasmic antibody plays a central role in this mechanisms. A revision in the classification scheme of vasculitides to recognize that the polyarteritis group (polyarteritis nodosa, Churg-Strauss syndrome, and "overlap" systemic vasculitis), Wegener's granulomatosis, and idiopathic crescentic glomerulonephritis are closely related diseases may be warranted. The clinical and pathological features of systemic necrotizing vasculitides and the current knowledge concerning antineutrophil cytoplasmic antibodies are reviewed.

Autoantibodies↗
PubMed2006

Primary central nervous system vasculitis in children.

OBJECTIVE: Primary angiitis of the central nervous system (PACNS) is a severe and ill-defined neurologic disease. The goal of this study was to characterize the presenting features, treatment, and neurologic outcome of PACNS in children (cPACNS) and to define the predictors of disease progression in order to identify high-risk patients with cPACNS. METHODS: The cohort comprised consecutive patients diagnosed as having cPACNS based on clinical and vascular imaging findings, including identification of arterial stenosis on conventional angiography or magnetic resonance (MR) angiography. Disease progression was defined angiographically at >3 months after initial angiography. Clinical data obtained in prospectively collected standardized assessments and results of laboratory tests, including detection of cerebrospinal fluid abnormalities, were noted, and neuroimaging studies were reanalyzed. Predictors of progression were identified and tested in multivariate regression models. RESULTS: Sixty-two consecutive patients with cPACNS (38 male, 24 female, median age 7.2 years) were included. Two distinct subgroups were identified, those with progressive disease and those with nonprogressive disease. Progressive cPACNS was found in 20 of 62 children and was predicted by a clinical presentation of neurocognitive dysfunction, multifocal parenchymal lesions on MR imaging, and evidence of distal stenoses on angiography. CONCLUSION: The spectrum of PACNS in children includes both progressive and nonprogressive forms. Characteristic features at diagnosis can be used to predict later progression, to identify a distinct high-risk cPACNS cohort, and to help guide selection of patients for immunosuppressive therapy.

Adolescent↗
PubMed1981

Neurologic manifestations of systemic vasculitis. A retrospective and prospective study of the clinicopathologic features and responses to therapy in 25 patients.

A combined retrospective and prospective study of a patient population with either or both biopsy and angiographic evidence of systemic necrotizing vasculitis was undertaken in order to delineate the neurologic manifestations of this syndrome. The type, extent and natural history of the lesions in the peripheral and central nervous system were evaluated, together with the response fo the nervous system disease to corticosteroids or cytotoxic agents or both. In 60 percent of the patients, there was involvement of the peripheral nervous system. Four patterns of neuropathy were seen: mononeuritis multiplex, extensive mononeuritis, cutaneous neuropathy and polyneuropathy. The central nervous system was involved in 40 percent of the patients, manifested predominantly by diffuse and focal disturbances of cerebral, cerebellar and brain stem function. This study demonstrates the complexity and heterogeneity of the neurologic complications of the systemic necrotizing vasculitides. With treatment of the vasculitic process and prevention of further insult, both the peripheral and central nervous system make significant, and at times, dramatic, recovery.

Adolescent↗