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PubMed2003

Echocardiographic and clinical characteristics of aortic regurgitation because of systemic vasculitis.

BACKGROUND: Although systemic vasculitis has been described as one cause of aortic regurgitation (AR), clinical features of this disease entity have not been fully characterized. METHODS: The clinical data of 153 consecutive patients (103 male), who underwent open-heart operation to correct predominant AR from January 1990 to December 2000 were retrospectively analyzed. RESULTS: A total of 16 patients (10%) were found to have AR associated with systemic vasculitis; there were 9 patients with Takayasu's arteritis (TA) and 7 with Behçet's disease (BD). All patients with TA, but only 3 with BD, were female (P <.05). Echocardiography showed redundant motion of the elongated aortic cusp with the frequent periaortic echo-free space in BD, and characteristic motion limitation of thickened cusp with dilated and thickened aorta in TA. All patients with TA, but only 1 patient with BD, were given a diagnosis before operation (P <.05); the others underwent emergency operation to control acute AR of unknown etiology, and were given a correct diagnosis after detection of paravalvular leakage. In TA, paravalvular leakage developed in 1 patient (11%) and another died suddenly with chest pain. In BD, paravalvular leakage developed in 6 patients (86%) who needed repeated operation, and 2 died even after the third operation. The 2-year survival without clinical events (paravalvular leakage, repeated operation, or death) was 76 +/- 15% in TA and 14 +/- 13% in BD (P <.05). CONCLUSIONS: TA and BD are 2 important causes of AR, and show different clinical features. A high rate of paravalvular leakage, especially in BD, warrants cautious systemic evaluations for the cause of AR, and characteristic echocardiographic findings seem to be helpful in the differential diagnosis.

Adult↗
PubMed2003

Central nervous system vasculitis.

Vasculitis is an inflammation of the vessel wall. It may be either primary or secondary. Primary vasculitis includes systemic vasculitis (large, medium, and small-vessel vasculitis) and localized vasculitis (isolated angiitis of the central nervous system and non-systemic vasculitic neuropathy). Secondary vasculitis may be present in connective tissue disorders or may be caused by infections, neoplasms, and substance abuse. Patients presenting with symptoms suggestive of vasculitis require brain neuroimaging, lumbar puncture, and angiography, but only biopsy allows a definite diagnosis.

Cerebral Angiography↗
PubMed1991

Vasculitic syndromes associated with other rheumatic conditions and unclassified systemic vasculitis.

Markers for systemic vasculitis may be useful in both assessment of disease activity and ascribing pathogenesis. Endothelial cell damage may be assessed by Factor VIII-related antigen and perhaps fibrinolysis (plasmin inhibitor/plasmin complex). Antibodies to endothelial cells potentially combine both assessment and pathogenesis. Complement fixing antibodies to endothelial cells, previously reported in systemic lupus erythematosus and Kawasaki syndrome, are also elevated in rheumatoid vasculitis. Their relationship to vascular endothelial cell antibodies, reported in a wide spectrum of systemic vasculitis, is not clear. A direct role for neutrophils in some forms of vasculitis is supported by the overlap of rheumatoid vasculitis with Sweet's syndrome, previously associated with both Behçet's and Crohn's syndrome. An indirect role of persistent neutrophil inflammation at mucosal surfaces is suggested by the vasculitis with antineutrophil cytoplasmic antibodies seen in cystic fibrosis. In other cases, vasculitis may be triggered by direct viral infection such as cytomegalovirus. Experimental models may clarify the confusion. In the MRL mouse, vasculitis appears independent of the B-cell hyperactivity. Experimental models of uveitis also support a role for T cells in the induction of vasculitis.

Animals↗
PubMed1998

Central nervous system vasculitis.

Central nervous system vasculitis occurs in a variety of clinical settings. Some exhibit a distinct age preference; others a tissue tropism. Most frequently encountered are giant cell arteritis (temporal arteritis) and vasculitis secondary to infections. The central nervous system may be involved in the antineutrophil cytoplasmic antibody-associated systemic vasculitides and occasionally neurologic abnormalities appear as a presenting manifestation of disease. Isolated angiitis of the central nervous system, a rare form of vasculitis that is restricted to the central nervous system, must be distinguished from other causes of central nervous system inflammation and from noninflammatory vascular disease. We are learning a great deal about the cellular mechanisms of vascular inflammation in general. Some manifestations of the clinical disease result from histologic features of the infiltrate and the size of affected vessel. However, the local consequences of inflammation, such as increased coagulation and altered vasomotor tone, as well as the systemic consequences, such as activation of the central noradrenergic systems, trigeminovascular system, and hypothalamic pituitary adrenal axis, contribute both to pathogenesis of disease and to recovery.

Behcet Syndrome↗
PubMed1994

[Treatment of systemic vasculitis].

Progress in the treatment of systemic vasculitis have permitted a decrease of mortality but with an increase in iatrogenic morbidity. Steroids remain the cornerstone of the treatment but precise modalities and other concomitant treatments are depending upon the type of vasculitis. In most cases, systemic vasculitis are primary and the treatment, although important, is symptomatic. However, in some cases such as hepatitis B virus-induced polyarteritis nodosa or hepatitis C virus-induced cryoglobulinemia, the treatment can be etiologic and is directed against the antigen responsible for the systemic vasculitis. In the future, a better understanding of pathological mechanisms, particularly of etiologic factors, and new treatment such as monoclonal antibodies should increase the prognosis of systemic vasculitis.

Adrenal Cortex Hormones↗
PubMed1976

Immunologic mechanisms in systemic vasculitis.

Thirty-four patients with systemic vasculitis were studied to determine the possible type and frequency of associated immunologic abnormalities. The patients were divided into three clinical groups--those with systemic vasculitis without respiratory tract involvement, those with systemic vasculitis with respiratory tract involvement (particularly Churg-Strauss vasculitis and Wegener's granulomatosis), and those with limited vasculitis without visceral involvement. A diminished level of serum complement was found in half the patients with systemic vasculitis without respiratory tract involvement. These patients usually had diffuse skin disease that often was associated with the presence of rheumatoid factor and cryoglobulinemia and most likely represented an immune-complex induced disease. The serum IgE often was elevated in patients who had systemic vasculitis with respiratory tract involvement, particularly those with Churg-Strauss vasculitis and Wegener's granulomatosis, and may be a clue to the pathogenesis in this group of patients.

Adult↗
PubMed2002

Scurvy mimicking systemic vasculitis.

An 91 years old woman was hospitalized because of lethargy, shortness of breath and diffuse subcutaneous hemorrhage of legs. Clinical features were consistent with the diagnosis of vasculitis with systemic involvement. However dermatologic characteristics of the legs, in association with malnutrition, suggested vitamin C deficiency which was confirmed by laboratory test. Ascorbic acid supplement dramatically improved her clinical symptoms. This case remarks how scurvy may mimmick a systemic vasculitis.

Aged↗
PubMed1994

[Ophthalmological aspects of systemic vasculitis].

Ocular findings of systemic vasculitis, such as polyarteritis nodosa, giant cell angitis, and aortitis syndrome were reviewed. Systemic lupus erythematosus is not categorized as systemic vasculitis, however its ocular findings are microangiopathic. Therefore review of its ocular findings was included in this paper. The most common fundus finding in these diseases is ischemic optic neuropathy or retinal vascular occlusions. Therefore several points in diagnosis or pathogenesis of optic neuropathy and retinal and choroidal vaso occlusion were discussed. Choroidal ischemia has come to be able to diagnosed clinically, since fluorescein angiography was applied in these lesions. When choroidal arteries are occluded, overlying retinal pigment epithelium is damaged. This causes disruption of barrier function of the epithelium and allows fluid from choroidal vasculatures to pass into subsensory retinal spaces. This is a pathogenesis of serous detachment of the retina. The retinal arterial occlusion formed non-perfused retina. Such hypoxic retina released angiogenetic factors which stimulate retinal and iris neovascularizations and iris neovascularization may cause neovascular glaucoma.

Blood-Retinal Barrier↗
PubMedDate not supplied

[Antineutrophil cytoplasmic antibodies in systemic vasculitis].

The term systemic vasculitis concerns a group of diseases characterized by inflammation of vessels. The diagnosis and follow-up of these conditions is a serious challenge since their classification is difficult and the therapy is usually empiric. Perhaps the greatest breakthrough in the management of these diseases is the recent discovery of serological markers for some vasculitic syndromes. Wishing to determine how far the tests for detection of these antibodies are specific, sera from 63 patients with systemic vasculitides were tested by immunofluorescence of ethanol fixed neutrophils. Two different staining patterns were observed. The cytoplasmic staining (classic pattern) was very specific for Wegener's granulomatosis. In contrast, the perinuclear staining was also present in many vasculitic syndromes such as microscopic polyarteritis and poliarteritis nodosa. In addition, the sera were tested by ELISA, using whole neutrophil cell extract. Although the assay was less specific than the immunofluorescence, the test was helpful in discriminating between true ANCA and atypical fluorescence patterns. Moreover, high levels of antimieloperoxidase antibodies detected by ELISA in a limited number of patients were exclusively associated with the diagnosis of microscopic polyarteritis or Wegener's granulomatosis with renal involvement. Our data confirm the remarkable association of antineutrophil cytoplasmic antibodies with vasculitic syndromes.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2002

Vitronectin- and fibronectin-containing immune complexes in primary systemic vasculitis.

In primary systemic vasculitis anti endothelial cell autoantibodies (AECA) have been described frequently. They represent a heterogeneous group of autoantibodies whose target antigens are mostly unknown. We tried to find AECA-antigens by a co-operative binding assay with a panel of monoclonal antibodies (mAb) directed to human umbilical vein endothelial cells (HUVEC) and extracellular matrix proteins. The mAb were used to bind antigens from lysate of endothelial cells, and binding of human antibodies to these antigens was measured. mAb directed to Vitronectin (VN) and Fibronectin (FN) resulted in enhanced binding of antibodies in sera from patients with Churg Strauss Syndrome (CSS) and Wegener's Granulomatosis (WG) compared to normal sera. Neither free autoantibodies against VN or FN could be detected nor did the addition of endothelial cell lysate influence the binding activity from the patients' sera. This suggests that preformed VN and FN-containing immune complexes (IC) are present in the patient sera. The amount of IC was decreased by incubation with HUVEC, demonstrating that these IC can bind to endothelial cells. However, their involvement in the pathogenesis of the disease is not clearly defined. Our data suggest that there are preformed IC present in sera of patients with CSS and WG that contain VN and FN and bind to endothelial cells.

Antibodies, Monoclonal↗
PubMed2005

Infliximab in a child with therapy-resistant systemic vasculitis.

Treatment of systemic vasculitides is usually based on the use of corticosteroids and other immunosuppressive drugs. We describe a 10-year-old girl with systemic vasculitis resistant to immunosuppressive treatment who had a rapid and impressive response to treatment with infliximab.

Antibodies, Monoclonal↗
PubMed2005

Mycophenolate mofetil in systemic vasculitis.

The systemic vasculitides are a group of potentially life-threatening multi-system autoimmune connective tissue diseases characterized by vascular inflammation. The gold standard therapies include corticosteroids and cyclophosphamide as induction therapy and other agents such as azathioprine and methotrexate to maintain remission. Mycophenolate mofetil (MMF) is increasingly being used in autoimmune disorders and this article reviews the use of this agent in the systemic vasculitides.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2000

What you should know about PR3-ANCA. Evidence for the role of T cells in the pathogenesis of systemic vasculitis.

The pathogenesis of systemic vasculitis is complex and is likely to involve many mechanisms. There is a growing body of evidence that T cells may contribute to the pathogenesis of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides. Predominantly, T cells and monocytes are found in inflammatory infiltrates in patients with Wegener's granulomatosis (WG). The production of ANCA appears to be T-cell-dependent. T lymphocytes from the peripheral blood of patients with ANCA-associated vasculitis have been shown to proliferate in response to proteinase 3 (PR3). These and other findings outlined in this review indicate T-cell involvement, although further studies are still needed to elucidate the exact contribution of T cells to the pathogenesis of systemic vasculitis.

Animals↗
PubMed1996

[Pleuropulmonary manifestations of systemic vasculitis].

Pleuropulmonary manifestations of systemic vasculitis are common, polymorphic and of ambiguous significance: the same pulmonary lesion may reveal a specific manifestation of vasculitis as well as a therapy-induced complication, especially infection which may favor per se a flare-up. Two questions will be successively studied: what are the pleuropulmonary characteristics of Wegener's granulomatosis, Churg-Strauss syndrome, periarteritis nodosa, Behçet's disease, Takayasu's disease and temporal arteritis? What are the major adverse effects that may occur in the course of a treated systemic vasculitis?

Humans↗
PubMed1995

[Autoantibodies against neutrophil cytoplasm and their significance in the classification of systemic vasculitis].

In this article new informations about systemic vasculitis were reviewed. Evaluation of ANCA and their antigen specificity is of a great help in classification of systemic vasculitis. Among idiopathic systemic vasculitis anti-serine proteinase antibodies are found in Wegener granuloma, anti-MPO antibodies in Churg-Strauss syndrome and in polyarteritis nodosa. Antibodies against other components of PMNL granules still remain unknown. The main purpose of this review was to underline incidence of systemic vasculitis and the meaning of ANCA in the diagnosis and classification of these diseases.

Autoantibodies↗
PubMed2002

Systemic vasculitis.

The systemic vasculitides are a heterogeneous group of disorders characterized by inflammation of blood vessel walls. They can be classified according to the size of vessel affected and also into primary de novo vasculitides and secondary to other disease processes. Treatments differ and overlap depending on the type of vasculitis. It is therefore important to make the correct diagnosis and treat appropriately to achieve remission as there is a substantial mortality implication.

Journal Article↗
PubMedDate not supplied

Systemic vasculitis.

The systemic vasculitides are comparatively rare but important disorders of childhood. Apart from Henoch-Schönlein purpura, which is the commonest example in paediatric practice, Kawasaki disease is most often encountered. Polyarteritis and Wagener's granulomatosis have a lower incidence but are associated with a high morbidity and mortality. Newer investigative procedures and therapeutic approaches have led to more accurate diagnoses and improved outcome but further advances await a better understanding of the vasculitides: polyarteritis; Kawasaki disease; Wagener's granulomatosis; and ANCA in childhood.

Arteritis↗