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PubMed1996

Clinical disease activity in systemic vasculitis.

Long-term follow-up data indicate that the systemic vasculitides are chronic relapsing diseases with high morbidity. It is therefore of paramount importance to distinguish activity, which requires cytotoxic or immunosuppressive therapy, from damage requiring rehabilitation. Damage due to underlying disease or treatment highlights the need for more effective, less toxic treatment regimens. An integrated package for clinically based assessment of disease activity in systemic vasculitis, the Vasculitis Integrated Assessment Log, has been accepted for further evaluation and implementation by the European Community Study Group for use in therapeutic trials. The added value of serologic tests and inflammatory markers in clinical assessment is becoming more clear, and unsuspected disease activity has been revealed by biopsy and imaging of standard sites or affected areas. Integration of these data should enable characterization of high- and low-risk patient subgroups to determine specific therapies.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1999

Systemic vasculitis and atypical infections: report of two cases.

Two cases of systemic vasculitis are described; one presenting with adult Henoch-Schonlein purpura secondary to a concomitant Chlamydia infection and the other with leucocytoclastic vasculitis and mesangioproliferative glomerulonephritis secondary to a recent parvovirus B19 infection. Association of chlamydial infection has not previously been described with Henoch-Schonlein purpura and this infection should, perhaps, be added to the list of aetiologies of this disease. Parvovirus B19 causing significant urinary sediment abnormalities associated with mesangioproliferative glomerulonephritis and leucocytoclastic vasculitis has also not been described previously.

Adult↗
PubMed2005

[Structural and metabolic characteristics of erythrocytes in patients with systemic vasculitis].

We studied the functional and metabolic changes of erythrocytes in systemic vasculitis (SV). The below was detected: an increasing intensity of blood chemiluminescence, a higher concentration of TBA-positive substances in erythrocyte membranes, inhibited Na+, K+ - and Ca2+ -activated and Mg2+ -related ATPases in erythrocytes and a reduced osmotic and acidic resistance of erythrocytes. An enhanced aggregation ability of erythrocytes and their inhibited desagregation were observed in hemorrhagic vasculitis an especially in microscopic polyangiitis. The revealed changes characterize the significance of the erythrocyte pathology in thrombosis and development of hemorrhagic disorders in SV patients.

Adolescent↗
PubMed1988

[Vesical neuropathy in systemic vasculitis: 3 cases].

Bladder neuropathy was diagnosed in 3 patients with systemic vasculitis (temporal arteritis: 1 case; periarteritis nodosa: 2 cases). Clinical characteristics were: dysuria, diminished or abolished bladder sensation leading to indolent bladder retention. Urodynamic investigation showed hypotonic and underactive detrusor, increased detrusor compliance, hyposensitive bladder, and/or overactive urethral closure. Needle electrode examination showed signs of denervation of periurethral muscles; sacral evoked latencies were increased, favouring pudenal nerve alterations. Symptoms and urodynamic abnormalities resolved following corticosteroid therapy. Clinical and therapeutic implications of bladder neuropathy in necrotizing vasculitis are emphasized.

Adult↗
PubMed1996

New insights into the epidemiology of systemic vasculitis.

Because of the relative rarity of the conditions, epidemiologic investigation of systemic vasculitis is a challenging prospect. Previous work in this area has been primarily in the form of descriptive studies and therapeutic trials, which have increased awareness of these syndromes among clinicians. However, compared with other diseases, little is known about the epidemiology of the systemic vasculitides. Although significant progress has been made during the past year to estimate incidence and prevalence rates of disease, particularly for giant cell arteritis and Kawasaki disease, further studies are needed to determine risk factors for initial expression and relapses of vasculitis, biologic markers of disease progression, comorbidities, long-term outcomes, and cost of care and disability.

Humans↗
PubMed2005

Standardised work-up programme for fever of unknown origin and contribution of magnetic resonance imaging for the diagnosis of hidden systemic vasculitis.

BACKGROUND: Fever of unknown origin (FUO) is a diagnostic challenge. Rheumatologists are often in charge of patients with FUO because the vasculitides, especially, are potential and common causes of FUO. OBJECTIVE: To evaluate the value of a standardised investigation to identify the cause of FUO. METHODS: A standardised work-up programme for patients with FUO was started at the beginning of September 1999. The rate of identified causes of FUO was compared between all patients with FUO admitted to a tertiary care centre of rheumatology between January 1996 and August 1999 (control group) and September 1999 and January 2003 (work-up group). In January 2002 magnetic resonance imaging (MRI) was added to the investigation. RESULTS: 67 patients with FUO were identified--32 before and 35 after institution of the work-up programme. Before implementation 25% of all patients with FUO remained undiagnosed, after implementation 37%. After institution of the investigation the percentage of patients with vasculitides increased significantly from 6% (n = 2) to 26% (n = 9, p = 0.047, Fisher's exact test). This increase could be attributed to the addition of MRI in 2002. When all patients with FUO before 2002 (n = 55) and thereafter (n = 12) were compared the prevalence of systemic vasculitis increased from 11% (n = 6) to 42% (n = 5, p = 0.021). CONCLUSION: Implementation of a standardised work-up programme for FUO did not improve the overall rate of diagnosis. Addition of MRI significantly increased the diagnosis of systemic vasculitis as the underlying cause of FUO. MRI should be included in the investigation of patients with FUO when vasculitis is suspected.

Adult↗
PubMed1997

Bilateral adrenal enlargement as a first sign of systemic vasculitis.

In this case-report we describe the fatal outcome of systemic vasculitis. A 51-year-old man was hospitalised with constant abdominal pain, chest pain, anorexia, fatigue, weight loss, dyspeptic complaints, and a period of high fever at home. Bilateral adrenal enlargement was found without a plausible cause. Endoscopy revealed a reflux oesophagitis grade I, which was treated with famotidine. His complaints disappeared without further treatment. Five days after release from hospital the patient was re-admitted with subfebrile temperature followed by an Addison's crisis due to primary adrenal failure. Laboratory tests for systemic illness were all negative. He was treated with high-dose corticosteroids. Right adrenal biopsy revealed haemorrhage, possibly of older age. After 10 days he returned with severe kidney and heart failure. He was transported to another hospital for haemodialysis. Unfortunately the patient passed away because of cardiac arrhythmias. Postmortem investigation revealed inflammation of middle-sized and small arteries in the adrenal glands, heart, lung and thyroid. In the kidneys, mesangio-proliferative glomerulonephritis was found. A definite classification of the vasculitis could not be made because of the high-dose corticosteroids therapy. Possibly, the haemorrhage of both adrenal glands was caused by venous thrombosis due to the hypercoagulable state, which is often observed in vasculitis.

Adrenal Gland Diseases↗
PubMed2005

[Prevalence of antiepithelial cell antibody in systemic vasculitis and identification of the target antigen thereof].

OBJECTIVE: To investigate the prevalence of antiepithelial cell antibody (AECA) in systemic vasculitis (SV) and the target antigen thereof. METHOD: Sera of 113 patients with SV of different kinds, 46 patients with Behcet's disease, 23 patients with Takayasu arteritis, 19 patients with Wegener's granulomatosis, 8 patients with polyarteritis nodosa, 7 patients with microscopic polyangiitis, and 10 patients with Churg-Strauss syndrome were collected to detect the protein expression of AECA by Western blotting, with the protein of the endothelial cells of the line EA. hy926 line as substrate. Two-dimensional electrophoresis combined with immunoblotting, liquid chromatography-electrospray ionization mass spectrography was used to detect the target antigens related to vasculitis. Sera of 57 patients with systemic lupus erythematosus (SLE), 25 patients with rheumatoid arthritis (RA), and 20 healthy persons were collected as controls. RESULTS: The AECA-positive rate of the SV patients was 69.0%, not significantly different from that of the SLE patients (66.7%), but significantly higher than those of the RA patients (6.7%, P < 0.01) and healthy persons (0, P < 0.01). The AECA from the SV patients reacted with the endothelial cell (EC) antigens with the molecular size of 26 to 125 kDa, and the AECA from the SLE patients reacted with the EC antigens with the molecular size of 15 to 97 kDa. The EC antigens with the molecular size of 47 kDa was commonly found in the sera of the AECA-positive SV patients and SLE patients, however, was not found in the RA, and polymyositis-dermatomyositis patients. The EC protein reacted by 47 kDa protein was identified by proteomic techniques as alpha-enolase. CONCLUSION: A group of heterogeneous antibodies, AECA can be found frequently in patients with SV and SLA. AECA reacts against a common EC antigen, alpha-enolase.

Adolescent↗
PubMed2003

Increased circulating levels of proteinase 3 in patients with anti-neutrophilic cytoplasmic autoantibodies-associated systemic vasculitis in remission.

In systemic small vessel vasculitides, patients form autoantibodies against neutrophil granular proteins, anti-neutrophilic cytoplasmic autoantibodies (ANCA). Some correlation is seen between ANCA titre and disease activity, but whether this is cause or effect is still unknown. It has been reported that levels of proteinase 3 (PR3), one of the main ANCA antigens, are increased in patients with active disease. An increased level of circulating antigen could mean a predisposition to autoimmunity. In order to explore this we measured PR3 levels in patients with stable disease. In addition we measured neutrophil gelatinase-associated lipocalin (NGAL) as a specific marker of neutrophil degranulation, cystatin C as a marker of renal function as well as C-reactive protein (CRP), IL-6 and sTNFr1 as markers of inflammation. PR3, NGAL, IL-6 and sTNFr1 were measured in plasma by the ELISA technique. In the PR3 ELISA, we used anti-PR3 monoclonal antibodies as capture-antibodies and affinity-purified rabbit-anti-PR3 antibodies for detection. PR3-ANCA, myeloperoxidase (MPO)-ANCA, CRP and cystatin C were measured by routine methods. PR3 was significantly raised (P < 0.0001) in vasculitis patients (median 560 micro g/l, range 110-3,940, n = 59) compared with healthy blood donors (350 micro g/l, 110-580, n = 30) as well as disease controls (360, 110-580, n = 46). No correlation was seen with disease activity, inflammation or renal function. The raised NGAL levels correlated strongly with decreased renal function (r = 0.8, P < 0.001). After correcting for this, slightly increased levels (110, 42-340, n = 59) were observed compared with healthy blood donors (81, 38-130, n = 25), but not compared with the disease controls (120, 57-260, n = 48). In the disease controls, there was a significant correlation between NGAL and proteinase 3 (r = 0.3, p < 0.05), but this was not the case in the vasculitis patients. Whether patients had PR3-ANCA or MPO-ANCA was of no significance. In our measurements, we found significantly raised levels of PR3 in plasma from patients with small vessel vasculitis, regardless of ANCA specificity. This was not due to decreased renal function, ongoing inflammation or neutrophil activation. Plausible mechanisms for this include defects in the reticuloendothelial system, genetic factors and selective neutrophil degranulation or leakage.

Adult↗
PubMed2005

Therapeutic effect of anti-TNF-alpha antibodies in an experimental model of anti-neutrophil cytoplasm antibody-associated systemic vasculitis.

The therapeutic options for anti-neutrophil cytoplasm antibody (ANCA)-associated systemic vasculitis (AASV) remain limited and hampered by adverse effects. One potential novel therapeutic avenue involves inhibition of TNF-alpha, with encouraging uncontrolled data in humans with one agent (infliximab) but disappointing controlled data from another (etanercept). For investigating the potential role of TNF-alpha as a therapeutic target in AASV, the effect of an anti-rat TNF-alpha mAb (CNTO 1081) in a rat model of AASV was investigated. For testing the effect of TNF-alpha blockade in this model, starting on day 28 after immunization (a point when glomerulonephritis is established), animals were randomized to treatment with CNTO 1081 or control mouse IgG. Treatment with CNTO 1081 significantly reduced albuminuria (mean 1.1 +/- 0.3 mg/24 h CNTO 1081 versus 8.0 +/- 1.9 controls; P < 0.05) and crescent formation (0% CNTO 1081 versus 60% controls; P < 0.05). Lung hemorrhage was also reduced (CNTO 1081: median score 0, range 0 to 2; controls: 2, range 1 to 3; P < 0.05). When analyzed by intravital microscopy, there was a 43% inhibition of leukocyte transmigration in mesenteric venules in response to topical CXCL1 (a neutrophil chemoattractant) in the CNTO 1081 group compared with controls (P < 0.001). Anti-myeloperoxidase antibody titers were similar in both groups throughout the study. In conclusion, these findings indicate that TNF-alpha plays an important role in the pathogenesis of experimental autoimmune vasculitis and suggest that blockade of this cytokine with an mAb is effective in treating established vasculitis. The therapeutic action of anti-TNF-alpha reagents may be mediated, in part, by suppression of the enhanced leukocyte-endothelial interactions in this disorder.

Animals↗
PubMed1997

Systemic vasculitis and epithelioma. A report of three cases with a literature review.

Coexistence of a systemic vasculitis and an epithelioma is rare. The prevalence of malignancies in patients with vasculitis has been estimated at about 5%, with about two thirds hematological malignancies and one third solid tumors, although the actual figure may be higher. We report three cases of vasculitis in patients with solid tumors. A review of the literature yielded 77 additional cases. A total of 56 cases were fully documented. The sex ratio was 1.26, and mean age was 62.5 years. The most common vasculitides were leukocytoclastic vasculitis (33%), polyarteritis nodosa (16.5%) and Henoch-Schönlein purpura. The most common sites of cancer formation were the lung (23%), the digestive system (17.5%) and the kidney (14%). The diagnosis of vasculitis antedated that of cancer in most patients. Radical cancer treatment was often followed by resolution of the vasculitis and vasculitis recurrences occurred concomitantly with tumor recurrences in some patients, suggesting that the vasculitis was a paraneoplastic syndrome. Patients with unexplained chronic vasculitis with or without a history of cancer should undergo extensive investigations for an occult neoplasm.

Aged↗
PubMed2004

[Differential diagnosis of multiple sclerosis and systemic vasculitis].

Two groups, each included 12 patients, with confirmed diagnosis of multiple sclerosis (age 19-35 years) and with systemic vasculitis (age 21-47 years) were compared in the study. Differential clinical immunological and tomographical features of these two states are described.

Biomarkers↗
PubMed1994

Lymphocytic enteritis and systemic vasculitis in sheep.

Lymphocytic enteritis and systemic lymphocytic vasculitis may be a new or previously unrecognized syndrome in sheep suffering from chronic wasting. Seven cases in three separate flocks were seen over an eight-year period at Veterinary Laboratory Services in Brighton, Ontario. Most of the animals were between five and twelve months of age and exhibited weight loss and inappetance, with or without diarrhea. All were Suffolks or crossbred Suffolks. In one of the flocks, there were also several sheep with lymphoma. Postmortem examination of carcasses and ancillary testing did not reveal an etiology; however, the intestinal and vascular lesions resembled those described in certain viral diseases, such as malignant catarrhal fever and Border disease, and immune-mediated diseases, such as polyarteritis nodosa. Submission for necropsy of sheep that exhibit signs of chronic wasting is encouraged, to help establish the etiology of the disease and its significance to the industry.

Animals↗
PubMed2003

Are environmental factors important in primary systemic vasculitis? A case-control study.

OBJECTIVE: To investigate the association between primary systemic vasculitis (PSV) and environmental risk factors. METHODS: Seventy-five PSV cases and 273 controls (220 nonvasculitis, 19 secondary vasculitis, and 34 asthma controls) were interviewed using a structured questionnaire. Factors investigated were social class, occupational and residential history, smoking, pets, allergies, vaccinations, medications, hepatitis, tuberculosis, and farm exposure in the year before symptom onset (index year). The Standard Occupational Classification 2000 and job-exposure matrices were used to assess occupational silica, solvent, and metal exposure. Stepwise multiple logistic regression was used to calculate the odds ratio (OR) and 95% confidence interval (95% CI) adjusted for potential confounders. Total PSV, subgroups (47 Wegener's granulomatosis [WG], 12 microscopic polyangiitis, 16 Churg-Strauss syndrome [CSS]), and antineutrophil cytoplasmic antibody (ANCA)-positive cases were compared with control groups. RESULTS: Farming in the index year was significantly associated with PSV (OR 2.3 [95% CI 1.2-4.6]), with WG (2.7 [1.2-5.8]), with MPA (6.3 [1.9-21.6]), and with perinuclear ANCA (pANCA) (4.3 [1.5-12.7]). Farming during working lifetime was associated with PSV (2.2 [1.2-3.8]) and with WG (2.7 [1.3-5.7]). Significant associations were found for high occupational silica exposure in the index year (with PSV 3.0 [1.0-8.4], with CSS 5.6 [1.3-23.5], and with ANCA 4.9 [1.3-18.6]), high occupational solvent exposure in the index year (with PSV 3.4 [0.9-12.5], with WG 4.8 [1.2-19.8], and with classic ANCA [cANCA] 3.9 [1.6-9.5]), high occupational solvent exposure during working lifetime (with PSV 2.7 [1.1-6.6], with WG 3.4 [1.3-8.9], and with cANCA 3.3 [1.0-10.8]), drug allergy (with PSV 3.6 [1.8-7.0], with WG 4.0 [1.8-8.7], and with cANCA 4.7 [1.9-11.7]), and allergy overall (with PSV 2.2 [1.2-3.9], with WG 2.7 [1.4-5.7]). No other significant associations were found. CONCLUSION: A significant association between farming and PSV has been identified for the first time. Results also support previously reported associations with silica, solvents, and allergy.

Adolescent↗
PubMed2003

Investigation of quality of life, mood, pain, disability, and disease status in primary systemic vasculitis.

OBJECTIVE: To assess quality of life (QOL) and psychological adjustment in primary systemic vasculitis (PSV), and to assess their relationship to disease-related measures. METHODS: Fifty-one PSV patients completed questionnaires assessing QOL (Short Form 36 [SF-36]), disability (Health Assessment Questionnaire [HAQ]), and mood (Hospital Anxiety and Depression Scale [HADS]). Illness-related measures, disease activity, and permanent damage scores (the Birmingham Vasculitis Damage Index [BVDI] modified, and the Birmingham Vasculitis Activity Scale modified) were collected. Aspects of the HAQ's psychometric properties were evaluated. RESULTS: PSV patients' SF-36 scores, except for mental health, were significantly lower than the norms, indicating poorer QOL. Using the HADS, 43.2% of patients reported increased anxiety symptoms and 25.5% increased depressive symptoms. Patients with increased pain when compared with those with little or no pain had significantly impaired scores in all SF-36 subscales, except for mental health; and they scored significantly worse on depression, fatigue, problems with sleep, and symptom severity. Patients with neuropathic symptoms and those taking high levels of steroids had significantly impaired scores on some of the relevant measures. There were no significant correlations between the modified BVDI scores with the SF-36 subscales or with the other self-report disease-related measures. The HAQ showed high internal consistency and high concurrent and discriminant validity. CONCLUSION: Many aspects of QOL are significantly impaired in PSV. Self-reported pain and disease symptoms, SF-36 scores, and depression and anxiety levels are significant indicators of the adverse impact of vasculitis on patients' lives that need to be assessed and managed.

Adaptation, Psychological↗
PubMedDate not supplied

Pharmacokinetics of cyclophosphamide (CP) and 4-OH-CP/aldophosphamide in systemic vasculitis.

Cyclophosphamide given in association with corticosteroids has markedly improved the prognosis of systemic vasculitis. Little information has been reported on cyclophosphamide pharmacokinetics in these diseases and data evaluating its metabolite, 4-hydroxycyclophosphamide/aldophosphamide, pharmacokinetics and concentrations are lacking. Cyclophosphamide was administered as a 1-h intravenous infusion every 3 weeks for six cycles to ten vasculitis patients. Serum cyclophosphamide and 4-hydroxycyclophosphamide/aldophosphamide concentrations were assayed on the first cycle of the treatment by reversed-phase high-pressure liquid chromatography with ultraviolet detection. The mean (+/- SD) 4-hydroxycyclophosphamide/aldophosphamide and cyclophosphamide areas under the serum concentration-time curves were, respectively, 1.86 +/- 1.12 and 154.1 +/- 62.7 mg/L x h with a ratio of 1.30 +/- 0.76%. The mean maximum serum 4-hydroxycyclophosphamide/aldophosphamide was reached 2.3 h after cyclophosphamide administration. The mean (+/- SD) cyclophosphamide and 4-hydroxycyclophosphamide/aldophosphamide half-lives were, respectively, 5.5 +/- 3.1 and 7.6 +/- 2.3 h. The results are consistent with those obtained for cancer patients, in spite of a wide interpatient variability of concentrations and pharmacokinetic parameters.

Adult↗
PubMed2003

Systemic vasculitis associated with alphal-antitrypsin deficiency.

We describe a rare case of systemic vasculitis associated with alpha1-antitrypsin (alpha1-AT) deficiency. Mutational analysis of the alpha1-AT gene in this patient revealed a homozygous alpha1-AT Mnichinan variant. Alpha1-AT possesses broad-spectrum inhibitory activity against many serine proteases, including human neutrophil elastase, to help maintaining the crucial balance between proteases and protease inhibitors. The increase in free protease activity in the context of alpha1-AT deficiency may induce exacerbation of the vasculitis. This serious genetic defect severely affects the balance between a protease and a protease inhibitor at the pathological site.

Female↗