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PubMedDate not supplied

[Systemic vasculitis].

This paper is dedicated to a general and synthetic review of a large and heterogeneous group of diseases, entitled "Systemic Vasculites". Despite its rarity in clinical practice, they can be severe and even fatal, if a proper diagnosis and adequate treatment is not made. Among the several classifications, we have adopted the Chapel Hill classification based on its pathogenesis. As they are multiple entities, we will describe only the essential clinical aspects of the diverse vasculitis, including the diagnostic criteria of the American College of Rheumatology, which are very sensitive and specific. We will conclude with a note on the global strategy of treatment of each entity.

Humans↗
PubMed2005

Primary systemic vasculitis: clinical features and mortality.

BACKGROUND: Wegener's granulomatosis (WG), Churg Strauss syndrome (CSS) and microscopic polyangiitis (MPA) are primary systemic vasculitides (PSV), the clinical features of which have been described from tertiary centres. AIM: To provide the first clinical description of MPA from a general hospital and compare clinical features with WG and CSS. DESIGN: Retrospective analysis of patient records. METHODS: Records of 99 PSV patients attending a single hospital, from 1988 to 2000, were reviewed for: clinical features, date/age at diagnosis, sex, duration of illness, anti-neutrophil cytoplasmic antibodies (ANCA), treatment, comorbidity and deaths. Cases were classified using ACR, CHCC and Lanham criteria/definitions. Birmingham vasculitis activity scores (BVAS) and damage index (VDI) were calculated. Survival was assessed using Cox proportional hazards model and standardized mortality ratios (SMRs). RESULTS: Compared to previous reports there was more ENT (29%) and respiratory (29%) but less renal (92%) involvement in MPA, and less ENT involvement in WG (81%). CSS showed high neurological (72%), cardiovascular (28%) and gastrointestinal (17%) involvement and the highest median (range) VDI (p = 0.01 vs. WG; p = 0.001 vs. MPA). BVAS1 was significantly lower in MPA than in WG [median (range) 15 (4-29) vs. 21 (6-39), (p = 0.001)] but not in CSS [20 (7-28), p = 0.08]. SMR (95%CI) for PSV was 4.8 (3.0-6.6); 5-year survival was 45.1% for MPA, 75.9% for WG and 68.1% for CSS. Age was a significant risk, but only to the same extent as in the reference population. When age was adjusted for, no other significant factor was found. DISCUSSION: The clinical characteristics seen here are similar to those in previous series. There are difficulties in using the MPA CHCC definitions in classification. There is a high proportion of neurological involvement in CSS, causing permanent damage. MPA may have a poorer prognosis than WG or CSS.

Aged↗
PubMedDate not supplied

[Alterations of hemostasis in systemic vasculitis].

The authors revised the published data on coagulation abnormalities observed in patients suffering from primary vascular diseases or vasculitides associated with systemic diseases of the connective tissue. These patients present a tendency toward thrombosis as a result from platelet activation and endothelial cell injury, together with altered fibrinolytic activity. The proliferative events secondary to platelet activation and endothelial injury remain present after cessation of the inflammatory process and may play a role at the end stage obstruction of the vascular lumen always seen in these diseases.

Animals↗
PubMedDate not supplied

Recent data on the pathology of systemic vasculitis .

In connection with two case reports of connectivities associated with marked vascular lesions, difficult to classify, the authors are reviewing the available data on the role played by vasculitis in the pathogeny of such pathologic states. The chronologic relationship between the occurrence of the systemic vascular involvement and the sensitization to certain drugs, as well as the exposure to various antigens and infections, have suggested the concept of hypersensitization or development of immune disorders with important pathogenic role in these connectivites. The immunodepressive genetic factors, either inborn or acquired, might influence the release and activation of certain slow viruses. These recent discoveries are opening new prospects for an effective etiopathogenic treatment in such diseases.

Adult↗
PubMed2000

[Long-term prognosis and outcome of patients with primary systemic vasculitis: initial results in Germany].

Some outcome parameters have been shown to represent valuable measures for the long-term assessment of primary systemic vasculitides (PSV). These parameters include disease extent, disease activity, damage, and the health-related quality of life. We describe a concept for the establishment of a detailed documentation system for PSV with a duration of at least 5 years to obtain a reliable characterization of these patients. This database will provide the basis for a PSV cohort and the development of an interdisciplinary patient education concept followed by an evaluation with a multicenter, trial.

Germany↗
PubMed2005

Classification and diagnostic criteria in systemic vasculitis.

Approximately 20 different primary forms of vasculitis are recognized, not all of which have been included in classification schemes or consensus statements regarding nomenclature. A variety of classification schemes have been proposed over the past 50 years, many predicated upon the size of the primary type of vessel involved in a given disease, as well as other considerations that include demographic features, organ tropism, the presence or absence of granulomatous inflammation, the role of immune complexes in pathophysiology and the association of autoantibodies with some forms of vasculitis. All classification schemes to date have had shortcomings owing to the substantial gaps in knowledge about vasculitis, but the American College of Rheumatology criteria for the classification of some forms of vasculitis are useful for the purpose of including patients in research studies. The Chapel Hill Consensus Conference has clarified some existing controversies in nomenclature of the systemic vasculitides. Robust diagnostic criteria for the various forms of vasculitis have, however, remained elusive.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2004

[Systemic vasculitis during parasitosis].

A RARE EVENTUALITY: Although parasite infections are frequent, observations of vasculitis related to parasitosis are, however, very rare. REGARDING THE MECHANISM: The simultaneous occurrence of a parasitosis and vasculitis may be the consequence of either the direct implication of a parasite observed in the histological lesions in the onset of alteration in the vascular wall, or of immunopathological phenomena occuring during the anti-parasite immune response, or a fortuitous association. THE HUMAN PARASITOSIS IMPLIED: In most cases, vasculitis associated with parasitosis is an isolated event with varied clinical aspects. Such cases have been reported in toxoplasmosis, trichinosis, strongyliasis, ascaridiosis, sarcocystosis, amibiasis, leishmaniosis and toxocarosis.

Humans↗
PubMed1999

Circulating antineutrophil autoantibodies in a child with isolated central nervous system vasculitis.

An 8-year old girl with history of twisted neck and painful swelling on the left side of the neck was found to have malfunction of glossopharyngeal and hypoglossal nerves on the left side. Magnetic resonance angiography revealed a giant aneurysm of the internal carotid artery surrounded by a widespread inflammatory tumor. Cerebral angiography disclosed a large, false aneurysm with almost complete compression of the internal carotid artery. Circulating antineutrophil cytoplasmic autoantibodies (titer 1:2560) and high levels of antibodies against antiproteinase 3 were detectable. This observation indicates that these autoantibodies may be a diagnostic tool in children in whom an undiagnosed central nervous system inflammatory disease is present.

Angiography, Digital Subtraction↗
PubMed1999

Serologic and clinical response to treatment of systemic vasculitis and associated autoimmune disease with intravenous immunoglobulin.

BACKGROUND: Autoimmune vasculitides cannot always be controlled by steroids and immunosuppressive drugs. Intravenous immunoglobulin (IVIg) treatment was found beneficial in several vasculitides including systemic and organ-specific diseases. In this article we tested whether the beneficial clinical response of IVIg treatment in vasculitides was accompanied by a decrease in vasculitis-associated autoantibody levels. METHODS: Ten patients diagnosed as having vasculitis were treated with high-dose (2 g/kg) IVIg monthly, in a 5-day schedule. In all the patients, other therapeutic measures failed to control disease progression prior to IVIg treatment. Each patient received between 1 and 6 treatment courses. All patients were evaluated for the levels of 5 autoantibodies (Abs) related to vasculitis before and after each treatment course. RESULTS: In 6 out of the 10 patients, a beneficial clinical response followed IVIg treatment. Moreover, no treatment-related adverse effects were observed in any of the patients. Anti-myeloperoxidase antibodies and cytoplasmic-antineutrophil cytoplasmic antibodies levels decreased concomitantly with the clinical improvement observed in the patients with Churg-Strauss vasculitis and Wegener's granulomatosis, respectively. Levels of cytoplasmic-antineutrophil cytoplasmic antibodies (ANCA) with specificity for bacteridial/permeability-increasing protein and human lysosomal-associated membrane protein increased after each treatment course, but returned to normal values before the following one. CONCLUSIONS: When other therapeutic measures, such as immunosuppressive therapy, fails to control disease manifestations in patients with vasculitides, IVIg is a possible effective intervention method with a high response rate. IVIg probably exerted its effects on disease progression via different mechanisms. Among these mechanisms, a decrease in relevant Ab levels is often found (probably by anti-idiotypes in IVIg), and thus ANCA levels are expected to associate with disease activity.

Adult↗
PubMed1997

[Systemic vasculitis as the initial presentation of a left atrial myxoma].

Left atrial myxoma remains a diagnostic challenge. The authors briefly review previously reported cases and their individualistic clinical and laboratory features. This report documents an unusual clinical presentation, initially directing attention to the central nervous system as well as bilateral tenderness and weakness of the extremities. The illness was considered to be a vasculitis until thirty-one months later when the diagnosis of left atrial myxoma was made. The tumor was successfully removed.

Diagnosis, Differential↗
PubMed1985

Polyarteritis group of systemic vasculitis--new diagnostic criteria.

We have used new diagnostic criteria to define patients with the polyarteritis nodosa (PAN) group of vasculitis. These were the combination of a necrotising glomerulitis without diffuse deposits of immunoglobulins or complement components in a patient presenting with systemic disease and multi-organ involvement. Twenty-two patients who fulfilled these criteria presented to our unit between 1975 and 1982. The diagnosis of PAN was confirmed by traditional histological criteria in eight. We anticipate that the use of these criteria will lead to earlier diagnosis and thereby improve the management of this potentially lethal disorder.

Adult↗