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PubMed2001

[Vasculitis].

Systemic vasculitis is a rare condition that usually takes a chronic and often unpredictable course. Long-term outcome is crucially dependent on a close cooperation between the primary caregiver and the specialist. The tentative diagnosis is based on the medical history and clinical examination and verified by laboratory diagnostics, high-resolution imaging and histological assessment. The primary aim of therapy is to induce remission and provide emergency treatment of any vital organs that may be in jeopardy. Later on, remission maintenance and glucocorticoid sparing are important targets. During this phase, it is important to differentiate between disease activity and disease damage when optimizing the drug regimen. In addition to the basic treatment, arterial blood pressure should be controlled, screening for cardiovascular risk factors conducted and infections identified early. Nowadays, good outcomes can be achieved thanks to improved diagnostic tests, a broader therapeutic spectrum and more effective monitoring.

Diagnosis, Differential↗
PubMed2006

Pantoprazole and perinuclear antineutrophil cytoplasmic antibody-associated vasculitis.

Systemic vasculitis may, at times, be drug-induced and associated with antineutrophil cytoplasmic antibodies (ANCA). However, pantoprazole, a commonly used and well-tolerated proton pump inhibitor, has not previously been reported to cause ANCA-associated syndromes. We describe a patient who developed interstitial nephritis, cutaneous vasculitis, a perinuclear ANCA staining pattern (pANCA) on immunofluorescence, and anti-myeloperoxidase antibodies (MPO-ANCA) in association with pantoprazole. We review various immune-mediated syndromes reported in association with proton pump inhibitors, including one report of omeprazole associated with interstitial nephritis and the development of ANCA.

2-Pyridinylmethylsulfinylbenzimidazoles↗
PubMed1997

Retroviral-associated vasculitis of the nervous system.

Vasculitis may involve the central and peripheral nervous system in HIV-infected patients. Central nervous system vasculitis is rare with HIV infection and most are owing to opportunistic infections including varicella, CMV, fungal, tuberculosis, and syphilis. Vasculitis of the peripheral nerve may cause mononeuritis multiplex or polyneuropathy, sometimes as the first symptom of HIV or after AIDS has developed. Symptoms may be limited to the peripheral nerve. The etiology may be infection of endothelial cells, hepatitis B or HIV-induced immune complexes, or dysregulation of cytokines and adhesion molecules. Treatment with steroids alone is often effective; IVIg and cytotoxic agents have also been used. It is uncertain whether vasculitis of the nervous system is ever due to other retroviruses (HIV-2, HTLV-1, and HTLV-2).

Acquired Immunodeficiency Syndrome↗
PubMed1984

Clinical and prognostic significance of vasculitis as an early manifestation of connective tissue disease syndromes.

The courses of 18 patients with arthritis and vasculitis in the first 2 years after onset of disease (mean follow-up, 54 months) were studied. The patients were categorized as having rheumatoid vasculitis, systemic vasculitis, and undifferentiated connective tissue syndrome. These patients cannot be distinguished on basis of organ involvement by vasculitis or histopathologic findings on biopsy, but can be separated clinically by the extent of joint involvement and the presence or absence of rheumatoid factor and antinuclear antibody. Early onset of vasculitis is associated with a poor outcome, especially in patients with rheumatoid arthritis, with rapid progression to vasculitic involvement of the viscera, resulting in death. On the basis of the 54-month follow-up period involving this selected series, the prognosis of patients with systemic vasculitis and undifferentiated connective tissue syndrome appears more favorable than that of patients with rheumatoid vasculitis.

Adult↗
PubMed1997

Rapid screening assay for anti-GBM antibody and ANCAs; an important tool for the differential diagnosis of pulmonary renal syndromes.

BACKGROUND: Pulmonary renal syndrome is encountered in several diseases such as Goodpasture's syndrome, antineutrophil cytoplasmic antibody (ANCA) associated systemic vasculitis, systemic lupus erythematosus (SLE) and infection-associated or drug-induced glomerulonephritis. To preserve organ function it is of vital importance to make the correct diagnosis and institute adequate therapy early, in the acute phase. METHOD: An enzyme-linked immunosorbent assay (ELISA), specially designed as a rapid screening assay for antiglomerular basement membrane (anti-GBM) antibody, proteinase-3 (PR3-) ANCA and myeloperoxidase-(MPO-) ANCA were evaluated from 1060 serum samples drawn from patients with clinically suspected pulmonary renal syndrome or rapidly progressive glomerulonephritis (RPGN). RESULTS: Of the 1060 serum samples, 142 were positive for anti-GBM antibody (n = 19), PR3-ANCA (n = 60), or MPO-ANCA (n = 73). Of the 142 samples, 10 were double positive. Reanalysis of positive sera with a quantitative ELISA yielded results manifesting good correlation with those of the the rapid screening assay. Of 918 sera found to be negative in the rapid screening assay, 105 were also tested with IIF, 11 being found to be positive. However, these 11 sera manifested no specificity for PR3 or MPO, but some were specific for bactericidal/permeability-increasing proteins, lactoferrin or elastase ANCAs. Two of the patients whose sera yielded negative results in the rapid assay had systemic vasculitis. CONCLUSION: The ELISA thus detects the true antibodies to PR3, MPO, and GBM, whereas IIF detects additional specificities. The findings suggest the rapid assay results to be of high positive predictive value, and the assay to be of high diagnostic specificity and sensitivity and thus useful in the diagnostic workup in suspected cases of RPGN or pulmonary renal syndrome.

Anti-Glomerular Basement Membrane Disease↗
PubMedDate not supplied

[Clinical chorioretinal aspects in systemic immune vasculitis. II].

Systemic immune vascular pathology raises very often diagnosis and differential diagnosis difficulties generated by the multitude and polymorphism of its clinical symptomatology. Chorioretinal modifications, sometimes particular, may complete the general clinical picture of the systemic immune vasculitis, favouring a correct clinical diagnosis. At the same time, a better knowledge of the clinical picture of some general systemic affections may lead to a correct diagnostic interpretation of these chorioretinal modifications. The paper presents the author's own classification of the main systemic immune vasculitis and also several rare affections (with immunologic implications) with emphasis on the vascular chorioretinal syndrome and on the clinical general one.

Chorioretinitis↗
PubMed2001

Vasculitis of the nervous system.

Vasculitis is inflammation of the blood vessels, which may involve either the central nervous system (CNS), or the peripheral nervous system (PNS), or both. This involvement may be primary and restricted to the CNS, and rarely to the PNS."Primary angiitis of the CNS" is the term used to describe isolated CNS involvement by vasculitis, in which neither the clinical presentation and behaviour of the disease, nor the histopathology is uniform. This heterogeneity indicates a spectrum, depending on the type and extent of the vascular involvement seen within the CNS, covering a group of disorders, rather than a single disease. This may explain the different prognosis and response to treatments. In clinical practice vasculitis of the nervous system, secondary to a known cause or underlying disease is more commonly seen than as a primary disorder. Primary systemic vasculitides and connective-tissue disorders, Behçet's Disease, lymphoproliferative diseases and other malignancies, some infections and related conditions, drugs and substance abuse are some of the conditions known to cause vasculitis in the nervous system. There is a broad variety of pathogenetic mechanisms. Both the CNS and the PNS may be involved, either separately or together.

Connective Tissue Diseases↗
PubMed1989

Update on systemic necrotizing vasculitis.

The systemic necrotizing vasculitides are classified into vasculitic syndromes on the basis of the pattern of clinical and pathologic involvement. The vasculitides have certain common clinical and laboratory abnormalities. Systemic necrotizing vasculitis is diagnosed on the basis of clinical features, and the vascular nature of the disease is determined by biopsy of involved tissue or angiography. The outcome is dependent on the extent of visceral involvement. Vascular inflammation influences the physiologic features of the vessel and may trigger vasoconstriction. Although glucocorticoids combat the inflammation, they may augment vasoconstriction and platelet aggregation. These effects must be considered in designing a management approach and in evaluating the cause and management of ischemic complications.

Arachidonic Acids↗
PubMed1999

Validity of a vasculitis activity index for systemic necrotizing vasculitis.

OBJECTIVE: To establish the validity of an index designed to measure activity in systemic necrotizing vasculitis (SNV). METHODS: The Vasculitis Activity Index (VAI) was designed to incorporate appropriately weighted clinical measurements that reflect disease activity in SNV. We performed a pilot study to guide the modification and subsequent testing of the initial design. The data necessary to calculate the VAI are direct ratings by a clinical observer of the degree of activity in 9 organ systems and 3 indirect measures of vasculitis activity. These data are recorded on 0-4 visual analog scales. Physician's global assessment (PGA) is used as the "gold standard" measurement of disease activity. The VAI was validated using 2 independent data sets: the questionnaire data set, derived from test case histories ("paper cases") sent to 100 practicing rheumatologists, and the clinic data set, obtained from use of the VAI in 204 regular care visits of 74 patients with SNV. RESULTS: The VAI correlated highly with the PGA: Pearson's correlation coefficient R = 0.84 (95% confidence interval [95% CI] 0.80-0.88) for the questionnaire data set, and R = 0.92 (95% CI 0.90-0.94) for the clinic data set. The mean of the interobserver coefficients of variation for the test case histories was lower for the VAI than for the PGA (mean difference 0.45; P = 0.002), indicating that the VAI has less interobserver variation than does the PGA. The change in VAI between clinic visits for individual patients correlated highly with the change in PGA (R = 0.88, 95% CI = 0.83-0.91). The VAI data collection form requires about 1 minute to complete, including computation of the score. CONCLUSION: The VAI is a valid measure of vasculitis activity that correlates highly with the PGA. In addition, the VAI has less interobserver variation than the PGA and has a high level of sensitivity to change over time. Additional testing of the VAI appears warranted.

Health Surveys↗
PubMed1996

[Systemic necrotizing vasculitis: case report].

Systemic vasculitis presents a group of clinically pathologic syndromes with acute inflammatory process in its basis which commonly occurs on small blood vessels destroying their walls with fibrinoid necrosis. It often occurs on several organs and systems depending on the form, size and number of the diseased blood vessels. This paper is a case report on a female patient suffering from a serious systemic necrotizing vasculitis with clinically demonstrated ulceronecrotizing skin changes and purpura together with appearance of coronary ischemia; rhythmic disorders of heart rate and gastrointestinal bleeding and cardiac arrest in one moment. It contains a description of the course of the disease and a good result after application of immunosuppressive therapy with high doses of corticosteroids, cyclosporin A and plasmaphereses.

Blood Vessels↗