Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “Systemic Vasculitis”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 289 records · Page 16Linked to original sources
PubMed1982

Systemic vasculitis in a district general hospital 1972-1980: clinical and laboratory features, classification and prognosis of 80 cases.

Between 1972 and 1980 a histological diagnosis of vasculitis was made on 80 patients from a district general hospital. These were divided into a polyarteritis nodosa (PAN) group, a rheumatoid vasculitis (RV) group and a heterogeneous group of other vasculitides. There was considerable overlap between the clinical and laboratory features in the three groups. Non-specific symptoms (fever and myalgia), leucocytosis and eosinophilia were the most useful features for distinguishing PAN from the other two groups. Hepatitis B infection was rare (two patients) and hypocomplementaemia was a feature of RV but not of PAN. The overall mortality was similar in each group. However, in PAN deaths due to vasculitis were more common within six months of diagnosis. Features associated with a poor prognosis were renal impairment, cutaneous and intestinal vasculitis in PAN; and neuropathy, weight loss and histological evidence of vasculitis at rectal biopsy in RV. Cytoxic drugs combined with corticosteroids were associated with an improved prognosis compared with corticosteroids alone in the PAN group. Pulmonary involvement was associated with less severe renal disease but with a six month mortality similar to that in the whole PAN group. Systemic vasculitis is not uncommon in a district general hospital population. The overlapping clinical and laboratory features in different vasculitic diseases stress the problems in classification between PAN and other groups. Patients with systemic disease complicated by necrotizing arteritis have a severe, life threatening disease which may respond to aggressive cytotoxic therapy.

Adolescent↗
PubMed1993

The role of the endothelium in systemic vasculitis.

The critical role played by the vascular endothelium in the development of vasculitic disease will be discussed within this article, attention being focused on the pathogenesis of the primary vasculitides, particularly Wegener's granulomatosis, microscopic polyarteritis and Kawasaki disease. Under normal circumstances the endothelium constitutes a barrier to the efflux of plasma proteins into the extracellular tissues, serves to maintain an anti-coagulant environment and forms a platform upon which many important biological processes take place. Alteration of the balance of these functions by inflammatory mediators and cytokines leads to the formation of a pro-coagulant environment, fluid leakage and increased adhesivity of the endothelium for neutrophils and lymphocytes. Both neutrophils and lymphocytes have the capacity to damage the endothelium directly, by the secretion of oxygen radicals, enzymes and other cytotoxic molecules, or indirectly by the secretion of cytokines which may alter the biological properties of endothelial cells, a process called endothelial activation. The role played by antibodies to the endothelium and to neutrophil cytoplasm components in mediating vascular injury in systemic vasculitis is also discussed and, finally, injury precipitated by components of the coagulation/thrombotic system.

Arteritis↗
PubMed2002

Systemic vasculitis complicating hairy cell leukaemia treatment with cladribine.

A single course of cladribine has been used commonly over the past decade to treat hairy cell leukaemia, with an impressive rate of complete remission and few serious adverse effects. Although vasculitis has been reported in the course of hairy cell leukaemia, it has only rarely been reported as the consequence of cladribine treatment. We describe a 73-year-old woman who developed serious systemic vasculitis with associated thrombocytopenia in the course of treatment with cladribine.

Aged↗
PubMed1993

Anti-idiotype antibodies to anti-myeloperoxidase autoantibodies in patients with systemic vasculitis.

The presence of anti-idiotype antibody (anti-id) activity to anti-myeloperoxidase autoantibodies (anti-MPO) was measured by assessing binding of IgG in patients' sera to F(ab')2 fragments of polyclonal heterologous anti-MPO antiserum. Thirteen paired acute and remission sera and three groups of sequential sera from patients with systemic vasculitis were studied. The levels of anti-MPO fell as disease activity subsided with treatment. However, levels of anti-id activity against these anti-MPO rose as patients entered remission. This effect was still seen after controlling for binding to F(ab')2 constant regions and after polyethylene glycol precipitation of immune complexes. In the sequential studies, levels of anti-MPO and anti-MPO anti-id tended towards a reciprocal relationship. These observations point to dynamic interactions between autoantibody and complementary anti-idiotype antibodies in this system and a role for idiotypic networks in the regulation of anti-neutrophil cytoplasm antibodies.

Acute Disease↗
PubMed1994

Endothelial cell activation in patients with systemic vasculitis.

Vascular endothelial cells respond in vitro to a number of stimuli, and in particular to cytokines, by undergoing functional and morphological alterations which endow them with the capacity to promote inflammatory reactions. We studied this process of endothelial cell activation in 20 skin biopsies from 18 patients with systemic vasculitis. At sites of cutaneous inflammation, blood vessels were lined with swollen endothelial cells which expressed increased levels of intercellular adhesion molecule-1 (ICAM-1) and vascular cell adhesion molecule-1 (VCAM-1), and were associated with a mononuclear cell inflammatory infiltrate. Neutrophil infiltration was only found in the presence of endothelial leucocyte adhesion molecule-1 (ELAM-1), which was expressed in 15/20 biopsies. ELAM-1 and VCAM-1 were associated with the presence of inflammatory cytokines which induce expression of these molecules in cultured endothelial cells. Endothelial activation in vivo appears to parallel that observed in vitro, and is likely to be important in determining the nature of an inflammatory response.

Adolescent↗
PubMed1998

[Anti-neutrophil cytoplasmic antibodies ANCA in glomerulonephritis and systemic vasculitis with renal involvement--the frequency of occurrence and ability for granulocyte activation in vitro].

We analysed the occurrence of anti-neutrophil cytoplasmic antibodies (ANCA) in sera of 191 patients with glomerulonephritis (76 females and 115 males) by the standard indirect immunofluorescence method (IIF). The presence of ANCA was demonstrated in sera of 4.4% (8/181) patients with idiopathic glomerulonephritis (GN) and in 30% (3/10) of patients with rapidly progressive glomerulonephritis (RPGN), as a form of renal limited vasculitis. In the experimental part of our study we analysed the influence of GN ANCA-negative sera on the neutrophil function in vitro and compared with the effect of ANCA-positive sera (titre > or = 4:40) from systemic vasculitis (SV) patients with renal involvement. The activation of neutrophils was established by reactive oxygen species (ROS) production and the ability of superoxide anion to reduce ferrocytochrome c. Among 30 ANCA-negative GN sera 20% (6/30) revealed the ability to activate neutrophils isolated from healthy donor. Remaining ANCA-negative GN sera and all sera from normal healthy individuals (negative control group) did not affect the neutrophil function and did not induce the superoxide anion production. Their effect was similar to the second negative reference system without serum. Only 33% (3/9) of high titre ANCA-positive sera (> or = 1:40) from SV patients were able to activate neutrophils and to produce the superoxide anion with following ferrocytochrome c reduction, but the effect of activation was most powerfully expressed (three times greater than by GN ANCA-negative sera). The remaining ANCA-positive sera and all SV ANCA-negative sera did not affect the neutrophil function in vitro. These experimental data indicate that the presence of ANCA in GN sera is not necessary to induce neutrophil activation in vitro. On the other hand the influence of the SV ANCA-positive sera was most powerful expressed, although only 33% of sera were able to activate neutrophils in vitro. Our results indicate that not always ANCA presence in serum was connected with the ability to neutrophil activation in vitro. It is possible that in ANCA-negative sera other factors were able to activate neutrophils in vitro, but the effect of activation was markedly lower.

Adult↗
PubMed1995

Antigen specificity in hydralazine associated ANCA positive systemic vasculitis.

The anti-hypertensive agent hydralazine can cause a lupus-like syndrome characterized by serosal inflammation, arthralgias and rashes. The kidneys however are usually spared. The condition is characterized by circulating immune complexes and antinuclear antibodies, whilst antibodies against double-stranded DNA are rare. Hydralazine can also cause a systemic vasculitis with a pauci-immune rapidly progressive glomerulonephritis, which is associated with autoantibodies directed against components of the neutrophil cytoplasm. In this study, ten patients with hydralazine-induced vasculitis had antibodies with specificities for both myeloperoxidase and lactoferrin. We suggest that this particular pattern of autoantibodies, together with antibodies with reactivity against nuclear components including double-stranded DNA, are characteristic findings in hydralazine-induced vasculitis. In addition, renal involvement appears to be more common in this group of patients with vasculitis than in those with the lupus-like syndrome.

Adult↗
PubMed2003

Recombinant protein to analyze autoantibodies to proteinase 3 in systemic vasculitis.

The presence of antineutrophil cytoplasmic autoantibodies with specificity for proteinase 3 (PR3-ANCA) usually is detected by enzyme-linked immunosorbent assay (ELISA) with purified PR3 as a substrate. We studied the technical performance of direct and capture ELISA using a recombinant proteolytically inactive form of PR3 produced in the baculovirus expression system for the detection of PR3-ANCA in 114 patients with systemic vasculitis at diagnosis. We found that ELISA using recombinant PR3 produced in insect cells is a promising alternative for ELISA with native PR3. We found a correlation between tests using recombinant or native PR3, as well as correlation of the ELISA results with ANCA titers measured by the indirect immunofluorescence technique. However, the specificity for ANCA-associated vasculitis of ELISA with recombinant PR3 was lower than ELISA using native PR3. Compared with the direct assay, capture ELISA is a more sensitive method for PR3-ANCA detection, with both native and recombinant PR3, and its results depend on the monoclonal antibody used to capture the antigen.

Animals↗
PubMed2003

[Stem cell transplantation in primary systemic vasculitis].

BACKGROUND: Many patients with various autoimmune diseases fail to respond to conventional immunosuppressive therapy and develop irreversible organ damage. In some cases the complications might be fatal. Furthermore, prolonged immunosuppression with cyclophosphamide or corticosteroids often leads to long-term side effects, cumulative organ damage, and development of secondary malignancies. THERAPY: Thus, short-term, high-dose immunosuppressive therapy with autologous stem cell transplantation might be an alternative for otherwise refractory patients. This concept has been used mainly in patients with scleroderma, systemic lupus erythematosus, rheumatoid arthritis, and multiple sclerosis. PATIENTS: Patients with primary systemic vasculitis (PSV) seem to be suitable candidates for high-dose chemotherapy (HDCT). Although complete, long-term remissions are possible with standard immunosuppressive therapy, there is a subgroup of patients who cannot be cured by standard therapy. They need long-term immunosuppression and eventually die due to progression of the disease or cumulative therapy-related toxicity. In large series, the 5-year mortality rate was > 20%. The severity of vasculitis rather than the distinction between subgroups should determine whether HDCT might be a life-saving treatment. RESULTS AND CONCLUSION: Careful patient selection with the help of scoring systems and determination of the optimal time in the course of disease are now the major goals in the approach to HDCT. First reports as well as our own single-center experience in HDCT with a limited number of patients with severe PSV have shown that long-term remissions are possible even in patients refractory to conventional immunosuppression.

Adrenal Cortex Hormones↗
PubMed1997

Alpha-1-antitrypsin phenotyping in ANCA-associated diseases: one of several arguments for protease/antiprotease imbalance in systemic vasculitis.

The vasculitic lesions observed in Wegener's granulomatosis may be partly the consequence of proteases released following activation of neutrophils by ANCA. The activity of these proteases, including proteinase 3 (PR3) and elastase, is normally closely restricted to the inflammation site by a large excess of circulating alpha-1-antitrypsin (alpha1AT). Patients with ANCA-positive systemic vasculitis may exhibit a protease/antiprotease imbalance either genetically determined in the rare patients with deficient alpha1AT phenotypes, or more often acquired through both alpha1AT inactivation in various pathological conditions and possible inhibition of PR3/alpha1AT complexation by anti-PR3 ANCA. This imbalance may at least contribute to disease spreading or aggravation.

Animals↗
PubMed1988

The lung in systemic vasculitis.

Pulmonary vasculitis is usually caused by one of three disorders: (1) Wegener's granulomatosis (WG); (2) Churg-Strauss syndrome (CSS), or allergic angiitis and granulomatosis; or (3) a nonspecific small vessel systemic necrotizing vasculitis (SNV), or microscopic polyarteritis. WG, the most common cause of lung vasculitis, has features of a granulomatous vasculitis of the upper airway and lung and widespread small vessel vasculitis involving the kidneys and other organs. The features of pulmonary WG overlap with those of malignancy and infectious granulomatous lung disease; accurate diagnosis generally requires open lung biopsy. CSS is defined by the triad of asthma, eosinophilia, and systemic vasculitis. Easily accessible tissues should be biopsied, but the clinical features are so distinctive that tissue biopsy is not invariably required for diagnosis. CSS must be differentiated from other diseases that cause pulmonary infiltrates with eosinophilia, including infections. Nonspecific SNV causes diffuse alveolar hemorrhage due to pulmonary capillaritis. Concomitant segmental necrotizing glomerulonephritis is almost always present. Diagnosis is made by renal biopsy, compatible extrarenal features, exclusion of nonimmune causes of lung hemorrhage, and exclusion of WG to the extent possible.

Churg-Strauss Syndrome↗
PubMedDate not supplied

Antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis after immunisation with bacterial proteins.

OBJECTIVE: There is circumstantial evidence for a role for infections in the development of the small vessel vasculitides associated with antineutrophil cytoplasmic antibodies (ANCA). The aim of this study was to determine whether the immunisation of rats with bacterial proteins could result in circulating ANCA, T cells with specificity for ANCA antigens, and a systemic vasculitis. METHODS: Adult male Wistar rats were immunised with pasteurised sonicated S. aureus (n = 7), E. coli (n = 8), purified protein derivative (PPD, n = 5), myeloperoxidase (MPO, n = 5) or phosphate-buffered saline (PBS, n = 5), in complete and in incomplete Freund's adjuvant. ANCA were assayed by indirect immunofluorescent (IIF) examination of normal rat neutrophils, and in ELISAs using human proteinase 3 (PR3), MPO and bactericidal/permeability-inreasing protein (BPI). The T cell response to PR3, MPO and BPI was assessed by a whole blood T cell proliferative assay in vitro, and by a delayed type hypersensitivity (DTH) response in vivo. Kidney and bowel were examined histologically for evidence of vasculitis and colitis. RESULTS: One rat from each group immunised with S. aureus or E. coli developed pauciimmune segmental glomerular sclerosis. The rat immunised with E. coli had additionally an arteritis affecting renal interlobular and gut vessels. This rat had circulating C-ANCA, that produced granular cytoplasmic neutrophil fluorescence with central accentuation, but the target antigen could not be determined in ELISAs using human PR3, MPO or BPI. In animals immunised with S. aureus or E. coli, there was no significant T cell proliferative or DTH response specific for human PR3, MPO or BPI. CONCLUSION: The development of ANCA and vasculitis in a rat immunised with bacterial proteins indicates that the relationship between infections and ANCA should be investigated further.

Animals↗
PubMed1993

Long-term remission of intractable systemic vasculitis with monoclonal antibody therapy.

Monoclonal antibodies that target T cells offer an alternative to conventional immunosuppressive drugs in the management of autoimmune disease. "Humanisation" of such monoclonal antibodies makes their clinical use less likely to be prone to the risk of cross-species sensitisation than treatment with rodent antibodies. We describe humanised monoclonal antibody therapy in four patients with severe systemic vasculitis unresponsive to immunosuppressive drugs. Substantial and sustained benefit was seen in three of the four patients, although one of these three patients developed anti-idiotypic antibodies that had to be removed by plasma exchange.

Antibodies, Anti-Idiotypic↗
PubMed1993

Alpha 1-antitrypsin genetic polymorphism in ANCA-positive systemic vasculitis.

Alpha 1-antitrypsin (alpha 1-AT) is the major inhibitor of proteinase 3 (PR3), the main target antigen of antineutrophil cytoplasm antibodies (ANCA) in Wegener's granulomatosis. alpha 1-AT is encoded by a polymorphic gene, with over 75 alleles, defining severely, medium and non-deficient protease inhibitor (PI) phenotypes. We describe the association of severely and medium deficient PI phenotypes with anti-PR3 positive systemic vasculitis, and postulate a pathogenetic role for alpha 1-AT deficiency and the occurrence of ANCA, with specificity for PR3 in a subgroup of patients with Wegener's granulomatosis.

Adult↗
PubMed1991

[Necrotizing extracapillary glomerulonephritis without immunoglobulin deposits. Renal localization of systemic vasculitis with anti-cytoplasm polynuclear antibodies].

Necrotizing and crescentic glomerulonephritis without immunoglobulin deposits (also called pauci-immune glomerulonephritis) is the cause of approximately 50 percent of acute renal failures of glomerular origin. Our study, based on 40 case-records selected on histological criteria, and on data from the literature, shows that in most cases, if not all, this type of glomerulonephritis is part of a wider systemic vasculitis which predominantly affects the glomerular capillaries. Anti-neutrophil cytoplasm antibodies are detected in three-quarters of the cases, with a specific distribution that varies according to the clinical features. In spite of the patients' age (mean: 62 years), the severity of glomerular lesions and the extra-renal diffusion of the disease, 60 percent of these patients can be cured and recover a normal, or at least acceptable renal function, provided the corticosteroid and sometimes immunosuppressive therapy is initiated at an early stage.

Adolescent↗
PubMed2003

Central nervous system vasculitis.

Vasculitis of the central nervous system (CNS) is classified as primary angiitis or as vasculitis secondary to a variety of diseases. A wide spectrum of clinical features may occur. A definite diagnosis is hampered by the difficulty in obtaining tissue for histology. Consequently, a diagnosis is frequently made on the basis of clinical presentation, brain magnetic resonance imaging, and cerebral angiography without pathologic confirmation. Recent experience shows that there are multiple other conditions that can mimic CNS vasculitis, many of which have different therapies. Most patients with CNS vasculitis should be treated aggressively with a combination of immunosuppressive medications. The prognosis is greatly improved with early recognition and therapy.

Autoantibodies↗
PubMed2005

Central nervous system vasculitis caused by propylthiouracil therapy: a case report and literature review.

Antineutrophil cytoplasmic antibodies (ANCA) are associated with vasculitis, including vasculitis induced by drugs such as the thionamides. The affected organ systems in thionamide-induced vasculitis have been primarily renal, musculoskeletal, and dermatologic. We describe the first case of thionamide-induced central nervous system vasculitis presenting as confusion, with complete resolution after discontinuation of propylthiouracil. We review the literature and summarize 42 additional cases of thionamide-induced ANCA-positive vasculitis since 1992. Propylthiouracil was responsible in 93% of cases and the predominant ANCA pattern on immunofluorescent staining was perinuclear (p-ANCA). Clinical improvement occurred after drug discontinuation in 93%, steroid therapy was used in some cases. The mean duration of treatment with thionamides was 35 months prior to presentation. Long-term medical treatment with thionamides for hyperthyroidism may increase the risk of this severe side effect.

Central Nervous System Diseases↗
PubMed2005

Central nervous system vasculitis secondary to parvovirus B19 infection in a pediatric renal transplant patient.

Central nervous system (CNS) vasculitis secondary to chronic parvovirus B19 (B19) infection presenting with recurrent neurological findings is a very rare disorder during childhood. Here we report a 12-year-old boy with a renal transplant who had chronic B19 infection with skin eruptions and recurrent episodes of encephalopathy with focal neurological deficits. B19 DNA was detected in blood, bone marrow, and skin biopsy specimens. Repeat cranial magnetic resonance (MR) imaging during each episode of encephalopathy showed variable focal findings, and MR angiography revealed vasculitic changes with narrowing of the cerebral arteries. We hypothesized that the CNS vasculitis might be associated with the chronic B19 infection. At the time of his fourth presentation with the same clinical findings, we administered intravenous immunoglobulin (IVIG) (1 g/kg per day, 2 consecutive days), which we continued for 6 months, at monthly intervals. IVIG therapy resulted in remission and has been effective not only for the clearance of B19, but also for the improvement of clinical and radiological findings of CNS vasculitis. We suggest that chronic B19 infection should be considered in immunocompromised patients with suspected CNS vasculitis. IVIG should be considered as a part of the treatment.

Brain Diseases↗