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PubMedDate not supplied

[Pulmonary silicosis complicated by systemic vasculitis].

A patient is described who had been--as a cutter of crystal--for 40 yrs exposed to the silica dust. At regular medical examinations at out-patients' department for pulmonary diseases first stage of pulmonary silicosis had been found and confirmed at the Institute for Occupational Health. Nine years after the retirement the clinical symptoms of febrility, cough, joint and muscular pains appeared together with the difficulties in swallowing and dyspnea even at minor physical activities. During the treatment the systemic vasculitis, probably visceral erythematodes, was established with satisfactory response to the corticosteroid therapy.

Humans↗
PubMed1984

Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg-Strauss syndrome.

Drawing on our experience of 16 cases and a review of the English literature, we propose that CSS is under-diagnosed because of exclusive emphasis upon pathologic recognition of the disorder. The classical histological picture comprises a necrotizing vasculitis, eosinophilic tissue infiltration and extravascular granulomas, but it is only found in a minority of cases, and is not pathognomonic of the condition (69, 108). On the other hand, the clinical pattern of the disorder is most distinctive, and CSS can be readily identified on clinical grounds. Typically, it begins with allergic rhinitis, which is often complicated by nasal polyposis and sinusitis. Asthma and peripheral blood eosinophilia are essential features, often accompanied by pulmonary infiltrates. The systemic vasculitis of CSS resembles that of PAN, but severe renal disease is uncommon (the typical renal lesion is a focal segmental glomerulonephritis), and cardiac involvement accounts for 50% of deaths. Diagnostic difficulties arise from the close relationship of CSS to other granulomatous, vasculitic and eosinophilic disorders. CSS is usefully regarded as a point of overlap between these three disease spectrums (Fig. 5). Individual components of each spectrum can occur in the course of CSS; hence cases may be reported as PAN developing as a complication of Löffler syndrome or eosinophilic gastroenteritis (37, 57, 66). The hypereosinophilia of CSS tends to be less severe and more steroid-responsive than in HES, and evidence of eosinophil degranulation was not found in the patients we studied. Complement abnormalities are not a prominent feature of the disorder, and circulating immune complexes were detected in only two cases; both contained IgM. This may be of pathogenetic significance as IgM deposition was a dominant feature in four of the five cases with positive renal immunofluorescence. IgE levels were elevated in all patients studied during the vasculitic phase, and skin-prick tests were positive in 8 of 10 patients tested. CSS responds well to treatment with steroids, although some patients benefit from the addition of immunosuppressive agents. The vasculitic illness is usually of limited duration, but relapses can occur, and should be detected and treated early. Major problems in the post-vasculitic phase stem from hypertension and persisting peripheral nerve damage. Allergic upper and lower respiratory tract disease is an important cause of morbidity in the pre- and post-vasculitic periods.

Adult↗
PubMed2000

Pathogenic role of anti-endothelial cell antibodies in systemic vasculitis.

Antiendothelial cell antibodies (AECA), a heterogeneous group of antibodies quite distinct from the ANCA family, have been detected in variety of diseases which share a varying degree of vessel wall damage. This review is mainly focused on Wegener's granulomatosis, Takayasu's arteritis and Kawasaki syndrome, which provide the best examples to evaluate the pathogenic and prognostic value of AECA. There is increasing evidence to show that AECA might be pathogenic in inducing autoimmune vascular disease. It is relevant to note that the presence and titre of AECA has been correlated with disease activity in systemic vasculitis. Experimental in vitro and in vivo models support a potential pathogenic role for AECA in sustaining immune-mediated vessel inflammation. Rather than being cytotoxic to endothelial cells, AECA are able to up-regulate the expression of adhesion molecules (E-selectin, intercellular adhesion molecule-1 and vascular cell adhesion molecule-1) and to induce the secretion of cytokine and chemokine which, in turn, cause leukocyte recruitment and adhesion. A recent idiotypic animal model has provided further evidence that AECA can be pathogenic.

Autoantibodies↗
PubMed1995

Neutrophil and recombinant myeloperoxidase as antigens in ANCA positive systemic vasculitis.

Myeloperoxidase (MPO) is one of the major autoantigens recognized by anti-neutrophil cytoplasm antibodies. The association of this antigen with specific disease entities requires that there is a source of pure antigen present in large quantities. Further delineation of the molecular mechanisms involved in the antigen-antibody interaction requires the ability to manipulate the molecule. The expression of recombinant MPO in Chinese hamster ovary cells has produced a source of pure protein, suitable for molecular studies. We have shown that this protein is an antigen recognized by 95% of anti-MPO antibodies from patients with systemic vasculitis. This recombinant molecule will be of use in providing an additional specific solid-phase assay for these antibodies and further forms of this protein which mirror the antigenicity of native MPO more exactly may replace chemically purified antigen. It will also be of great value in studies examining the epitopes recognized by anti-MPO antibodies and in studies of immunoregulation and T cell activation.

Animals↗
PubMed1997

Renal granulomas in systemic vasculitis. EC/BCR Project for ANCA-Assay Standardization.

Renal granulomas are a relatively infrequent finding in the kidney biopsy. They have been described in a number of syndromes such as Wegener's granulomatosis, anti-GBM glomerulonephritis, and sarcoidosis, and are commonly believed to be indicative of a fulminant clinical course. In leading textbooks, diverse definitions of renal granulomas are presented, which has led to controversies in identifying them. This, in combination with their rare occurrence, makes it difficult for the general pathologist to identify them. We present the clinical data of 16 patients with renal granulomas, from a total group of 157 patients with systemic vasculitis. Their renal functioning was not significantly different from the other 141 patients in whose renal biopsies renal granulomas were present. Furthermore, we present two practical definitions for the recognition of renal granulomas in the kidney biopsy, and we show a number of examples of their various histopathological shapes.

Adult↗
PubMed1995

Antineutrophil cytoplasmic autoantibodies, autoantigens, and systemic vasculitis.

Antineutrophil cytoplasmic antibodies (ANCA) encompass a heterogeneous group of autoantibodies targeting antigens in neutrophils (PMN), monocytes, and endothelial cells. ANCA are routinely detected by the indirect immunofluorescence technique (IFT) and at least three different patterns of fluorescence can be distinguished which have been assigned the acronyms cANCA, pANCA and aANCA. cANCA is mostly induced by proteinase 3 (PR3) antibodies (PR3-ANCA), and pANCA by myeloperoxidase (MPO) antibodies (MPO-ANCA), while aANCA has unidentified subspecificity. Over the past decade, ANCA have been the subject of extensive investigation. They have proved to be of significant value both as diagnostic tools and for follow-up in several forms of systemic vasculitis (e.g. Wegener's granulomatosis, WG; microscopic polyarteritis, MPA; Churg-Strauss syndrome, CSS) which are now termed 'ANCA-associated vasculitides'. Furthermore, it is suspected that the presence of ANCA is an important factor in the pathogenesis of these disease groups. Data regarding the detection of ANCA and their diagnostic value and role in the pathogenesis of vasculitic disorders will be discussed in this review. Growing evidence points to a pathophysiological and diagnostic relevance of the distribution of the ANCA target antigens PR3 and MPO (presence in the circulation, on cell membranes, and in tissue extracellularly). An autoimmune process has been implicated in the pathogenesis of ANCA-associated vasculitis, but it is uncertain which mechanism underlies the induction of the ANCA-related immunoresponse. In this paper mechanisms such as antigenic cross-reactivity between human PMN proteins and extrinsic antigens by molecular mimicry, idiotypic immunoglobulin regulation, and T-cell reactivity to PR3 and MPO will be discussed.

Antibodies↗
PubMed2006

Increased neutrophil membrane expression and plasma level of proteinase 3 in systemic vasculitis are not a consequence of the - 564 A/G promotor polymorphism.

Several findings link proteinase 3 (PR3) to small vessel vasculitis. Besides being a major target of anti-neutrophil cytoplasm antibodies (ANCA), previous findings have shown increased circulating levels of PR3 in vasculitis patients, increased levels of neutrophil membrane-PR3 (mPR3) expression and a skewed distribution of the - 564 A/G polymorphism in the promotor region of the PR3 gene. In this study we elucidate how these three findings relate to each other. The plasma concentration of PR3 was measured by enzyme-linked immunosorbent assay (ELISA), mPR3 expression by fluorescence activated cell sorter (FACS) and the gene polymorphism by real-time polymerase chain reaction (PCR). We compared results from 63 patients with ANCA-associated systemic vasculitis (AASV) with 107 healthy blood donors. In accordance with previous reports, AASV patients had increased plasma concentrations of PR3 compared to healthy controls (mean 224 microg/l versus 155 microg/l, P < 0.0001). They also showed an increased number of mPR3-positive neutrophils (60%versus 42%, P < 0.001). However, contrary to a previous report, we found no skewed distribution of the polymorphism in PR3 gene. There was a weak correlation between mPR3 mean fluorescence intensity (MFI) and plasma PR3 among healthy controls and myeloperoxidase-ANCA (MPO-ANCA)-positive patients (r = 0.24, P = 0.015 and r = 0.52, P = 0.011, respectively). In conclusion, increased plasma PR3 and high expression of mPR3 are associated with small vessel vasculitis, but neither of them is a consequence of the - 564 A/G polymorphism of the PR3 gene promotor.

Case-Control Studies↗
PubMed1995

Use of proteinase 3 purified by reverse phase HPLC to detect autoantibodies in systemic vasculitis.

The vasculitis autoantigen proteinase 3 was purified from neutrophil primary granules using reverse phase high performance liquid chromatography. It was shown to be free of important contaminants, was enzymatically active and was antigenic to sera containing antineutrophil cytoplasmic antibodies with a cytoplasmic pattern (C-ANCA) by indirect immunofluorescence. Development of an enzyme-linked immunosorbent assay showed that this preparation could be used to detect autoantibodies in Wegener's granulomatosis and microscopic polyangiitis. Assays based on reverse phase HPLC-purified proteinase 3 will be valuable in diagnosis and monitoring of treatment, and should increase our understanding of the autoimmune response in primary systemic vasculitis.

Amino Acid Sequence↗
PubMed1996

Azurocidin is a novel antigen for anti-neutrophil cytoplasmic autoantibodies (ANCA) in systemic vasculitis.

We isolated a 27-kD protein using cation exchange chromatography from an acid extract of neutrophil granules. N-terminal amino acid sequence analysis of the first 10 residues showed that this protein is azurocidin, a member of the family of neutral serine proteinase found in the neutrophil, which shares amino acid sequence homology with the three other neutral serine proteinases, elastase, proteinase 3 (PR3) and cathepsin G, but unlike them is without proteolytic activity. To test whether, in addition to these proteases, azurocidin might be a target for the humoral autoimmune responses associated with human vasculitis, 185 indirect immunofluorescence (IIF)-positive ANCA sera, made up of four groups of sera with specificities for PR3 (n=37), myeloperoxidase (MPO; n=50), bactericidal/permeability-increasing protein (BPI; n=41) and sera that recognized none of them (triple negative, n=57), and 46 normal sera were screened for IgG anti-azurocidin antibodies using an ELISA incorporating purified azurocidin. Twenty of the 185 IIF-positive sera and 2/46 normal sera displayed reactivity with azurocidin. Positive sera could blot the 27-kD band by Western blot analysis. Further study of the 20 positive sera revealed that: (i) 10 also had autoreactivity for MPO, of which six additionally recognized lactoferrin; (ii) two had reactivity with BPI; (iii) the remaining eight sera were positive only for azurocidin. All 20 sera were from patients with systemic vasculitis, and four of the six sera with triple reactivity (for azurocidin, MPO and lactoferrin) were from patients with hydralazine-induced vasculitis. We concluded that: (i) azurocidin is a novel ANCA antigen; (ii) anti-azurocidin antibodies from a subgroup of patients might represent the consequence of a drug-induced multi-clone activation.

Adult↗
PubMed1990

Clinical course of anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and systemic vasculitis. The Glomerular Disease Collaborative Network.

OBJECTIVES: To determine the spectrum of clinical manifestations in patients with anti-neutrophil cytoplasmic autoantibody (ANCA)-associated glomerulonephritis; to determine renal and patient survival in these patients; to compare survival among patients treated with corticosteroids alone, corticosteroids plus intravenous cyclophosphamide or corticosteroids plus oral cyclophosphamide; and to assess the correlation of disease manifestations and treatment response with ANCA subtypes and serial autoantibody titers. DESIGN: Inception cohort study; mean follow-up of 24 months. SETTING: Collaborative network of 120 university and private practice nephrologists (The Glomerular Disease Collaborative Network). PARTICIPANTS: Seventy patients with ANCA and pauci-immune necrotizing and crescentic glomerulonephritis, of whom 59 were treated with either corticosteroids alone (14 patients), corticosteroids plus oral cyclophosphamide (30 patients), or corticosteroids plus intravenous cyclophosphamide (15 patients). MAIN RESULTS: Of the 70 patients, 18 had renal-limited disease (idiopathic crescentic glomerulonephritis); 15, nonpulmonary extrarenal disease consistent with polyarteritis nodosa; and 37, pulmonary disease consistent with Wegener granulomatosis or alveolar capillaritis. There were overlapping manifestations of disease between patients with autoantibodies producing a cytoplasmic pattern and patients with autoantibodies producing a perinuclear pattern; however, the perinuclear pattern occurred more frequently in patients with renal-limited disease. Renal and patient survival was 75% at 24 months, and no difference in survival was seen between patients with renal-limited disease and those with systemic disease. No differences in survival were seen between patients treated with oral cyclophosphamide and those treated with intravenous cyclophosphamide; however, the comparative data from patients treated with corticosteroids alone were inconclusive. In general, autoantibody titers correlated with response to treatment and disease activity, but there were exceptions. CONCLUSIONS: Patients with ANCA have various forms of necrotizing vascular inflammation, ranging from renal-limited disease to widespread systemic vasculitis, including polyarteritis nodosa and Wegener granulomatosis. Oral corticosteroids with either oral or intravenous cyclophosphamide appear to be equally effective therapy for ANCA-associated glomerulonephritis.

Adolescent↗
PubMed1991

Canine leishmaniasis associated with systemic vasculitis in two dogs.

Two cases of canine leishmaniasis associated with systemic necrotizing vasculitis are described. The main macroscopic lesions were haemorrhagic in type; histopathological changes confirmed a vascular lesion affecting small arteries of several organs (skin, intestinal tract, kidney, urinary bladder, mesenteric lymph nodes, adrenal gland, myocardium, lung, eye and choroid plexus). The presence of the parasite was confirmed with a specific immunocytochemical stain. The possibility of an immunological aetiology of the vasculitis and its classification is discussed.

Animals↗
PubMed1997

Reinfection with Chlamydia pneumoniae may induce isolated and systemic vasculitis in small and large vessels.

It is a common clinical experience that the onset of the so called non infectious vasculitides is often preceded by upper respiratory tract symptoms. A specific agent is only occasionally recovered. We report five cases in Sweden with manifestations of vasculitis from different organs. In three of the five patients the onset was preceded by upper respiratory tract symptoms. All patients had serologic findings indicating Chlamydia pneumoniae infection and all required corticosteroid treatment for symptomatic recovery. One was diagnosed as an aseptic meningitis. Another was diagnosed as a cerebral arteritis, probably a variant of a giant cell arteritis. A third patient had symptoms similar to a polymyalgia rheumatica engaging the thighs. Two patients had an acute myocardial infarction. One of them had Cogan's syndrome. The other also had pulmonary and hepatic engagement and an elevated level of anti basement membrane IgM antibodies, though not to the Goodpasture antigen. He had no renal involvement. The diagnosis of Chlamydia pneumoniae infection was based on the detection of species-specific IgA, IgG and IgM antibodies to Chlamydia pneumoniae using microimmunofluorescence technique, MIF. Four of the 5 cases exhibited a fourfold increase in antibody titers, and the fifth case was found to have high levels of IgG and IgA antibodies, suggesting recent infection. Investigations for other infectious agents were negative in all patients. The serologic findings in those patients are consistent with a pattern of reinfection with Chlamydia pneumoniae. We therefore suggest that reinfection with Chlamydia pneumoniae may induce isolated and systemic vasculitis in virtually any organ of the body.

Adult↗
PubMed2000

No strong association between alleles of tumour necrosis factor alpha and interleukin-1 receptor antagonist and corneal melting associated with systemic vasculitis.

AIMS: To investigate polymorphism within the tumour necrosis factor alpha (TNF-alpha) promoter region and within the interleukin-1 receptor antagonist (IL-1Ra) gene in a group of patients with vasculitis associated corneal melting. METHODS: The polymorphic regions at position -308 on the TNF-alpha promoter region and in intron 2 of the IL-1Ra gene were amplified by the polymerase chain reaction (PCR). The resultant products were separated by electrophoresis on agarose gels and visualised by ethidium bromide staining. Genotype and allele frequencies for the 20 patients were compared with healthy controls from the same geographical area. RESULTS: The allele frequencies in the patient and control groups respectively for the TNF-alpha and IL-1Ra sites studied were as follows: TNF1, 82.5% and 80.2%; TNF2, 17.5% and 19.8%; IL-1Ra*1, 82. 5% and 78.3%; IL-1Ra*2, 15% and 20%; IL-1Ra*3 2.5% and 1.5%. Although there was a trend for the IL1Ra*2 allele to be more common in the control group, no allele was found to have a statistically significantly association with the patient group: TNF1 p = 0.89; TNF2 p = 0.89; IL-1Ra*1 p = 0.65; IL-1Ra*2 p = 0.68; IL-1Ra*3 p= 0. 50. CONCLUSIONS: The results suggest that the polymorphic alleles of TNF-alpha and IL-1Ra studied play little or no part in the susceptibility to corneal melting among these patients with systemic vasculitis.

Alleles↗
PubMed2002

Systemic vasculitis with lymphocytic temporal arteritis and Toxocara canis infection.

Vasculitis associated with helminthic infection is rare. We report a case of systemic negative antineutrophil cytoplasmic antibodies vasculitis associated with Toxocara canis infection documented by enzyme-linked immunosorbent assay and Western blot analysis. This case report is unusual because of lymphocytic temporal arteritis and renal involvement. The spontaneous remission of systemic vasculitis without use of steroids or immunosuppressive agents favors a close relationship between vasculitis and the parasite that is more than a coincidental association. To our knowledge, this is the first observation of temporal vasculitis associated with helminthic infection, extending the clinical spectrum of visceral larva migrans.

Aged↗
PubMed1994

Neuroradiology of central nervous system vasculitis.

Evaluation of the patient with suspected vasculitis affecting the nervous system requires consideration of a broad range of differential diagnostic possibilities. Appreciating pertinent neuroradiologic findings as well as understanding the capabilities and limitations of the imaging methods employed is a prerequisite for proper evaluation. Most importantly, integration of the imaging features with clinical and laboratory findings is necessary to maximize the chance of a correct diagnosis.

Aortic Dissection↗
PubMed1995

Evaluation and treatment of central nervous system vasculitis.

Angiitis of the central nervous system (CNS) remains a poorly understood and clinically challenging form of vascular inflammatory disease. Primary angiitis of the CNS (PACNS) has been viewed as a relentless and uniformly fatal disorder if untreated. In addition, recent trends have demonstrated an increasing reliance on angiographic diagnosis without tissue confirmation. It has been suggested that PACNS is clinically more heterogeneous than previously appreciated and may include relatively benign subsets. A reappraisal of diagnostic approaches has suggested caution in the diagnosis of CNS angiitis on purely angiographic grounds. Secondary vasculitis of the CNS is even more heterogeneous. Clinicians involved in the evaluation of patients with presumed CNS vasculitis need to be aware of the clinical spectrum of vascular inflammatory disease within the CNS as well as the strengths and limitations of currently available diagnostic modalities.

Central Nervous System↗
PubMed2003

Localized versus systemic vasculitis: diagnosis and management.

Localized vasculitis restricted to a specific anatomic site or organ is often histologically indistinguishable from more severe, systemic forms of vasculitis. By definition, localized vasculitis involves blood vessels within a confined vascular distribution or single organ without clinical evidence of generalized inflammation. Important factors that determine treatment and prognosis, in what appears initially to be a localized process, include histopathologic type, organ site, and the presence of systemic inflammatory markers and symptoms. The major issue is whether single organ vasculitis is actually an isolated form of the disease in which case surgical excision is curative, or whether the single organ involvement is simply a precursor of more threatening systemic vasculitis. The Birmingham Vasculitis Activity Score is a valuable tool to identify those patients with concurrent systemic involvement. The physician's recognition of a localized versus systemic vasculitic process is important in terms of making the correct diagnosis, prescribing treatment, and arranging appropriate clinical follow-up.

Arteritis↗