Localized Vasculitis Research Articles

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.

Immunohistochemical study of a case ofuterine leiomyoma showing massive lymphoid infiltration and localized vasculitis after LH‐RH derivant treatment

Immunohistochemical study of a case ofuterine leiomyoma showing massive lymphoid infiltration and localized vasculitis after LH‐RH derivant treatment

Acute vasculitis after endovascular brachytherapy

Purpose: Angioplasty effectively relieves coronary artery stenosis but is often followed by restenosis. Endovascular radiation (β or γ) at the time of angioplasty prevents restenosis in a large proportion of vessels in swine (short term) and humans (short and long term). Little information is available about the effects of this radiation exposure beyond the wall of the coronary arteries. Methods and Materials: Samples were obtained from 76 minipigs in the course of several experiments designed to evaluate endovascular brachytherapy: 76 of 114 coronary arteries and 6 of 12 iliac arteries were exposed to endovascular radiation from 32P sources (35 Gy at 0.5 mm from the intima). Two-thirds of the vessels had angioplasty or stenting. The vessels were systematically examined either at 28 days or at 6 months after radiation. Results: We found an unexpected lesion: acute necrotizing vasculitis in arterioles located ≤2.05 mm from the target artery. It was characterized by fibrinoid necrosis of the wall, often associated with lymphocytic exudates or thrombosis. Based on the review of perpendicular sections of tissue samples, the arterioles had received between 6 and 40 Gy. This arteriolar vasculitis occurred at 28 days in samples from 51% of irradiated coronary arteries and 100% of irradiated iliac arteries. By 6 months, the incidence of acute vasculitis decreased to 24% around the coronary arteries. However, at that time, healing vasculitis was evident, often with luminal narrowing, in 46% of samples. Vasculitis was not seen in any of 44 samples from unirradiated vessels (0%) and had no relation to angioplasty, stenting, or their sequelae. This radiation-associated vasculitis in the swine resembles the localized lymphocytic vasculitis that we have reported in tissues of humans exposed to external radiation. On the other hand, it is quite different from the various types of systemic vasculitis that occur in nonirradiated humans. Conclusion: Endoarterial brachytherapy using 32P results in vascular effects beyond the adventitia of the target vessel. This necrotizing vasculitis is causally related to radiation, but its mechanism is unclear and a dose effect is not evident. Quite possibly, local upregulation of inflammatory cytokines contributes to this radiation-associated vasculitis, which only involved some of the arterioles in each sample. It is likely that radiation-associated vasculitis also occurs around human coronary arteries and may result in foci of ischemia. To our knowledge, this lesion has not been previously recognized, either in experimental models or in human specimens examined after angioplasty/brachytherapy.

Odocoileus hemionus deer adenovirus is related to the members of Atadenovirus genus

The Odocoileus hemionus deer adenovirus (OdAdV-1) causes systemic and local vasculitis and proves extremely lethal for mule deer. To characterize the virus, part of the genome flanking the fiber gene was cloned and sequenced. The sequence revealed two open-reading frames that mapped to pVIII hexon-associated protein precursor and fiber protein of several other adenoviruses. The highest amino acid homology for pVIII and fiber was found with the members of the proposed Atadenovirus genus: ovine adenovirus isolate 287 (OAdV-287), bovine adenovirus 4 (BAdV-4) and duck adenovirus 1 (DAdV-1). The homology with bovine adenovirus type 3 (BAdV-3) proved low. The E3 region was not found between the gene for pVIII and fiber. These data suggest that OdAdV-1 is a member of the Atadenovirus genus.

Interferon-associated ischemic optic neuropathy

Ischemic optic neuropathy developed 3 to 6 months after the start of conventional-dose interferon therapy for malignancies in a 53 year-old woman with adenocarcinoma and a 67-year-old man with penile cancer. One case concurrently manifested peripheral neuropathy with histologic evidence of local vasculitis. After follow-up (5 years and 16 months), visual acuity, visual fields, and visual-evoked potentials had improved considerably but were still abnormal.

Experimental adenovirus hemorrhagic disease in white-tailed deer fawns.

Infection with a newly described endotheliotropic adenovirus was the cause of a 1993 epizootic reminiscent of hemorrhagic disease in California mule deer (Odocoileus hemionus columbianus and O. hemionus hemionus). Pulmonary edema and intestinal luminal hemorrhage, or necrotizing stomatitis associated with systemic or localized vasculitis, respectively, were common lesions seen in animals that died during the epizootic. In order to determine if white-tailed deer (Odocoileus virginianus) also are susceptible to infection and fatal disease with the deer adenovirus, eight white-tailed deer fawns (4- to 6-mo-old) were inoculated with purified deer adenovirus. Four were inoculated intravenously and four were inoculated through the mucous membranes. Seven days post-inoculation, one of the fawns inoculated intravenously died. Pulmonary edema and hemorrhagic enteropathy were associated with pulmonary and intestinal vasculitis with systemic multiorgan distribution of endotheliotropic adenovirus as demonstrated by transmission electron microscopy and immunohistochemistry. Adenovirus was reisolated from lung homogenates of the fawn that died of adenovirus hemorrhagic disease.

SPECT in focal enterovirus encephalitis: evidence for local cerebral vasculitis

We report a 4-year-old, left-handed male with focal coxsackievirus A3 encephalitis who presented with seizures and acquired aphasia. Electroencephalography exhibited focal spike discharges over the right frontal regions, but cranial magnetic resonance imaging did not reveal any structural abnormalities. However, brain single-photon emission computed tomography performed during the acute phase disclosed focal hypoperfusion in the right frontal lobe, consistent with decreased regional cerebral blood flow in the territory of some branches of the right cerebral anterior artery. Without specific treatment, the patient recovered completely within 1 month, when brain single-photon emission computed tomography images returned to normal and cranial magnetic resonance imaging still demonstrated no abnormalities. The present case suggests the possible role of transient local cerebral vasculitis in the pathogenesis of focal enterovirus encephalitis.

99Tcm-MAA lung scintigraphy in patients with Behçet's disease: its value and correlation with clinical course and other diagnostic modalities.

Behçet's disease, initially described as local vasculitis involving the oral, genital mucosa and eyes, can affect many organ systems with a varying clinical course. Pulmonary lesions associated with Behçet's disease are rare and reported to have a prevalence of 1.0-7.7%. In this prospective study, 29 patients aged 16-60 years (mean +/- s: 34 +/- 10.5) were investigated for pulmonary involvement with direct radiography, computerized tomography (CT) and intravenous digital subtraction angiography. Lung scintigraphy was also performed to determine its diagnostic value. In addition, patients had a fibreoptic bronchoscopic examination, spirometric test, carbon monoxide diffusion test and methacholine test. Of the 29 patients with Behçet's disease, 17 had pulmonary involvement. The diagnostic criteria for pulmonary vasculitis and tracheobronchial tree involvement were: (1) hilar vascular prominence or mass, new alveolar and reticulonodular infiltrates on chest X-ray; (2) aphthous lesions, bleeding ulcerations, inflamed mucosal appearance on bronchoscopy; (3) vascular thrombosis or aneurysms on CT; (4) occlusion or aneurysms of the main pulmonary arteries or its branches. The value of lung scintigraphy and its correlation with other imaging modalities were investigated to determine if a lung perfusion scan alone can be used to detect pulmonary involvement in Behçet's disease. Twenty-six patients were investigated with lung scintigraphy. Thirteen of 14 patients with lung involvement were identified. Perfusion lung scintigraphy provided true diagnostic information in 96% of our patients. In eight patients without pulmonary involvement, the lung scans were normal. Although DSA specifically demonstrates pulmonary aneurysms, it involves administration of contrast media and cannot show abnormalities distal to the third pulmonary arterial branches. Although vasculitis caused by collagen disorders is physiologically indistinguishable from pulmonary emboli, most perfusion defects resolve within 3 months, which can be used as a differential diagnostic criterion in the appropriate clinical setting. Therefore, pulmonary perfusion imaging can be used to screen patients suspected of having lung involvement, especially in the absence of thrombophlebitis. In patients with thrombophlebitis, a repeat examination at 3 months is recommended to differentiate pulmonary involvement from pulmonary embolism.

URETHRAL DIVERTICULUM: AN UNUSUAL PRESENTATION OF WEGENER'S GRANULOMATOSIS

No AccessJournal of UrologyClinical Urology: Case Report1 Mar 1999URETHRAL DIVERTICULUM: AN UNUSUAL PRESENTATION OF WEGENER'S GRANULOMATOSIS HOWARD B. GOLDMAN, BRIAN F. MANDELL, EMILY E. VOLK, RAYMOND R. RACKLEY, and RODNEY A. APPELL HOWARD B. GOLDMANHOWARD B. GOLDMAN More articles by this author , BRIAN F. MANDELLBRIAN F. MANDELL More articles by this author , EMILY E. VOLKEMILY E. VOLK More articles by this author , RAYMOND R. RACKLEYRAYMOND R. RACKLEY More articles by this author , and RODNEY A. APPELLRODNEY A. APPELL More articles by this author View All Author Informationhttps://doi.org/10.1016/S0022-5347(01)61807-XAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail "URETHRAL DIVERTICULUM: AN UNUSUAL PRESENTATION OF WEGENER'S GRANULOMATOSIS." The Journal of Urology, 161(3), pp. 917–918 References 1 : Urogenital manifestations of Wegener granulomatosis. Medicine1995; 74: 152. Google Scholar 2 : Necrotising urethritis in Wegener's granulomatosis. Brit. J. Urol.1982; 54: 434. Google Scholar 3 : Wegener granulomatosis. Unusual cause of necrotizing urethritis. Urology1979; 14: 66. Google Scholar From the Departments of Urology, Rheumatology and Anatomic Pathology, Cleveland Clinic Foundation, Cleveland, Ohio© 1999 by American Urological Association, Inc.FiguresReferencesRelatedDetails Volume 161Issue 3March 1999Page: 917-918 Advertisement Copyright & Permissions© 1999 by American Urological Association, Inc.MetricsAuthor Information HOWARD B. GOLDMAN More articles by this author BRIAN F. MANDELL More articles by this author EMILY E. VOLK More articles by this author RAYMOND R. RACKLEY More articles by this author RODNEY A. APPELL More articles by this author Expand All Advertisement PDF downloadLoading ...

Lesions and transmission of experimental adenovirus hemorrhagic disease in black-tailed deer fawns.

Adenovirus infection was the cause of an epizootic of hemorrhagic disease that is believed to have killed thousands of mule deer (Odocoileus hemionus) in California during the latter half of 1993. A systemic vasculitis with pulmonary edema and hemorrhagic enteropathy or a localized vasculitis associated with necrotizing stomatitis/pharyngitis/glossitis or osteomyelitis of the jaw were common necropsy findings in animals that died during this epizootic. To study transmission of adenovirus infection in deer and susceptibility of black-tailed deer (Odocoileus hemionus columbianus) fawns to adenovirus infection, six 3-6-month-old black-tailed fawns were divided into two treatment groups. One group was inoculated intravenously and the other group was inoculated through the mucous membranes of the eyes, nose and mouth with purified adenovirus. Each treatment group also included two additional fawns (four total) that were not inoculated but were exposed to inoculated animals (contact animals). One fawn served as a negative control. Between 4 and 16 days postinoculation, 8/10 fawns developed systemic or localized infection with lesions identical to lesions seen in animals with natural disease that died during the epizootic. Transmission was by direct contact, and the route of inoculation did not affect the incubation period or the distribution of the virus (systemic or the localized infection). Immunohistochemical analysis using polyclonal antiserum against bovine adenovirus type 5 demonstrated staining in endothelial cells of vessels in numerous tissues in animals with systemic infection and endothelial staining only in vessels subtending necrotic foci in the upper alimentary tract in animals with the localized form of the disease. All inoculated or exposed animals had staining in the tonsillar epithelium. Transmission electron microscopic examination of lung and ileum from two fawns with pulmonary edema and hemorrhagic enteropathy demonstrated endothelial necrosis and adenovirus virions in endothelial cell nuclei. Adenovirus was reisolated in black-tailed deer pulmonary artery endothelial cells using lung homogenate of the first fawn that developed systemic adenovirus infection. Serum virus neutralization test results suggest that this deer adenovirus is a new serotype.

Isolated Idiopathic Granulomatous (Giant Cell) Vasculitis of the Prostate

Vasculitis involving the prostate is uncommon and usually reflects systemic disease. Isolated prostatic vasculitis is very rare. Most of these cases are morphologically categorized as polyarteritis nodosa. We describe a 72-year-old man with idiopathic granulomatous vasculitis limited to the prostate. The vasculitis was identified incidentally upon microscopic examination of the suprapubic prostatectomy specimen removed for nodular hyperplasia. The transmural inflammation was centered on muscular arteries and veins and it was accompanied by narrowing of the lumen. Vasculitis was not associated with any secondary changes such as infarct or postinfarct fibrosis. Immunophenotyping of the vascular inflammatory infiltrate disclosed abundant macrophages and T-lymphocytes and virtual absence of B-lymphocytes. The patient is alive and well on no treatment for a follow-up period of 14 months. To our knowledge this is the second case report of prostatic involvement in localized idiopathic granulomatous vasculitis. It is important to distinguish cases of isolated granulomatous vasculitis in the prostate from systemic disease, because the latter implies a poor prognosis and requires an aggressive treatment.

Instillation of allogeneic lung macrophages and dendritic cells cause differential effects on local IFN-gamma production, lymphocytic bronchitis, and vasculitis in recipient murine lungs.

Lung allograft rejection is believed to be initiated by donor lung accessory cells, namely macrophages and dendritic cells, interacting with recipient lymphocytes leading to up-regulated Th1 type (IFN-gamma) cellular immunity culminating in graft destruction. The purpose of this study was to determine the individual role of donor lung macrophages and dendritic cells in the rejection response. Utilizing a murine model that reproduces the immunology and histology of acute rejection, C57BL/6 mouse (I-a(b), H-2(b)) lung dendritic cells (DC-enriched lung cells), purified alveolar macrophages (I-a-negative macrophages), or various ratios of I-a-negative macrophages/DC were instilled into BALB/c mouse (I-a(d), H-2(d)) lungs followed by an assessment of local IFN-gamma production and grading of rejection pathology. The data show that DC, and not I-a-negative macrophages, induced IFN-gamma production in recipient lungs. However, the local production of IFN-gamma was not always associated with histological changes characteristic of rejection pathology. In contrast to either cell type alone, instillation of C57BL/6 I-a-negative macrophages and DC, together, were required to induce rejection pathology in BALB/c lungs. In addition, the rejection response was dependent on interactions between donor I-a-negative macrophages and DC.

Experimental adenovirus hemorrhagic disease in yearling black-tailed deer.

An apparently novel adenovirus was associated with an epizootic of hemorrhagic disease that is believed to have killed thousands of mule deer (Odocoileus hemionus) in California (USA) during 1993-1994. A systemic vasculitis with pulmonary edema and hemorrhagic enteropathy or a localized vasculitis associated with necrotizing stomatitis/pharyngitis/glossitis or osteomyelitis of the jaw were common necropsy findings in animals that died during this epizootic. Six black-tailed yearling deer (O. hemionus columbianus) were inoculated with purified adenovirus isolated from a black-tailed fawn that died of acute adenovirus hemorrhagic disease during the epizootic. Three of six inoculated deer also received intramuscular injections of dexamethasone sodium phosphate every 3 days during the study. Eight days post-inoculation, one deer (without dexamethasone) developed bloody diarrhea and died. Necropsy and histopathologic findings were identical to lesions in free-ranging animals that died of the natural disease. Hemorrhagic enteropathy and pulmonary edema were the significant necropsy findings and there was microscopic vascular damage and endothelial intranuclear inclusion bodies in the vessels of the intestines and lungs. Adenovirus was identified in necrotic endothelial cells in the lungs by fluorescent antibody staining, immunohistochemistry and by transmission electron microscopy. Adenovirus was reisolated from tissues of the animal that died of experimental adenovirus hemorrhagic disease. Similar gross and microscopic lesions were absent in four of six adenovirus-inoculated deer and in the negative control animal which were necropsied at variable intervals during the 14 wk study. One deer was inoculated with purified adenovirus a second time, 12 wk after the first inoculation. Fifteen days after the second inoculation, this deer developed severe ulceration of the tongue, pharynx and rumen and necrotizing osteomyelitis of the mandible which was associated with vasculitis and thrombosis of adjacent large vessels and endothelial intranuclear inclusions. Transmission electron microscopy demonstrated adenovirus within the nuclei of vascular cells and immunohistochemistry demonstrated adenovirus antigen within tonsilar epithelium and in rare vessels.

Microvascular abnormalities in patients with vibration white finger.

In vivo nailfold capillary microscopy was performed on 10 men with vibration white finger (VWF) and 10 age-matched male controls. The observed nailfold capillaries required adaptation of Maricq's classifications and addition of new morphological scoring systems. These new classifications produced numerical scores for assessing capillary: dropout, tortuosity, elongation, visualization of subpapillary venular plexus, and the degree of disarrangement of nailfold capillary polarity. Application of these new scores showed, for the first time, a complex pattern of abnormal-nailfold capillaries in patients with VWF. Capillary dropout was evident in 7/10 patients, with an associated disarrangement in nailfold capillary polarity in five. All 10 controls had normal capillary morphology. Tortuosity of the capillary loops and elongation of their length was observed in 30% of patients. These significant morphological alterations seen in VWF suggest a local small-vessel vasculitis.

Localized chronic fibrosing vasculitis of the skin: an inflammatory reaction that occurs in settings other than erythema elevatum diutinum and granuloma faciale.

Erythema elevatum diutinum (EED) and granuloma faciale (GF) are chronic, localized forms of cutaneous leukocytoclastic vasculitis that result in patterned (storiform or concentric) fibrosis. EED often occurs in systemically ill patients as bilaterally symmetrical plaques, papules, or nodules, often over the dorsa of joints. GF occurs as one or a few plaques on the face. Eosinophils and plasma cells are prominent in GF, whereas neutrophils are plentiful in EED. Rarely, extrafacial lesions accompany facial ones in GF, and there are a few reports of upper respiratory tract masses with GF-like histologic features. We report on eight patients with solitary cutaneous lesions with histologic features similar to those of EED or GF, but whose clinical picture was not that of either disease. One, whose histology resembled GF, had a large multinodular dermal and subcutaneous mass that persisted despite attempted resection. Unusual histologic findings in other cases included storiform fibrosis with dense infiltrates of plasma cells, branching nerve fascicles admixed with EED-like changes, and EED-like areas adjacent to zones mimicking a sclerotic fibroma. Chronic fibrosing venulitis can be seen outside the stereotypic settings of GF and EED and is an inflammatory reaction pattern that does not signify a specific diagnosis. Because of transitions between EED or GF-like areas and those of patterned sclerosis with plasma cell-rich infiltrates, we believe that some "inflammatory pseudotumors" of the skin, and perhaps of other sites could be the result of localized vasculitis.

Cutaneous vasculitis

Cutaneous vasculitis shows considerable variation in its clinical and pathological features, according to the size of the involved vessel, the characteristics of the inflammatory infiltrate, and the point in time at which the biopsy is taken. This review approaches vasculitis of the skin from the perspective of the surgical pathologist. Vasculitis is classified by a combination of vessel size and type of inflammation present. Examples of both localized and systemic vasculitis are presented in conjunction with diagnostic algorithms. Clinicopathological scenarios where further prognostic information can be obtained are emphasized. In addition, diagnostic difficulties and the limitations of histopathology in providing a definitive diagnosis are discussed, with reference to other investigations as appropriate.

AIDS-associated intraocular lymphoma causing primary retinal vasculitis

Background: There is an increasing association between ocular-central nervous system (CNS) lymphoma and the acquired immunodeficiency syndrome (AIDS). In this population, the disease generally occurs in a younger age group. The origin of these B-cell neoplasms remains unknown. Method: Case study of a 26-year-old AIDS patient with an incidental finding of localized retinal vasculitis and local vitritis. Disease progression and the failure of antiviral therapy led to early diagnostic vitrectomy with vitreal and retinal biopsy. Cerebrospinal fluid (CSF) evaluation, head magnetic resonance imaging (MRI), and brain biopsy were performed. Results: The diagnosis of ocular non-Hodgkin lymphoma was made by vitreous cytology. Serial sectioning of a retinal biopsy showed no retinal neoplastic infiltration, only perivascular inflammatory cells, CSF was normal. MRI showed multicentric brain mass lesions that enhanced with intravenous contrast. Brain biopsy revealed an immunoblastic, angiocentric, B-cell non-Hodgkin lymphoma. The patient died within two months, despite cerebral and ocular irradiation. Conclusions: The segmentally localized retinal vasculitis-vitritis and absence of retinal infiltration suggested early primary involvement of the vitreous. Coarse perivascular sheathing is characteristic of the mixed retinal vasculitis in this disease. The diagnosis of ocular-CNS lymphoma must be considered in patients with AIDS, however young, with unexplained posterior segment inflammatory disease or subretinal masses.

Pathologic Alterations in the Diabetic Neuropathies of Humans

Pathologic study of nerve tissue is especially useful for the recognition of interstitial events such as inflammation or vascular alterations that cannot be inferred from clinical or electrophysiologic findings and may provide insight into an underlying mechanism or cause. Considerable variation in the natural history and pathologic alterations among diabetic neuropathies suggests that they are heterogeneous. For diabetic polyneuropathy, two mechanisms need to be considered. The first assumes that hyperglycemia induces metabolic derangements that directly affect Schwann cells (or myelin), nodes of Ranvier, or axons. The second assumes that hyperglycemia and metabolic derangement affect the structure and function of endoneurial microvessels, which then induce fiber changes by altering the blood-nerve barrier, inducing hypoxia or ischemia, or by unknown mechanisms. In proximal diabetic neuropathy, there is increasing evidence that the characteristic lesion is an inflammatory (immune) vasculitis that induces ischemic nerve fiber degeneration. Truncal radiculopathy may be due to an inflammatory polyganglionopathy. In cranial nerve III neuropathy, the monophasic course and the pathologic alterations of ischemia suggest that localized vasculitis should be excluded in future cases. Many upper limb mononeuropathies in diabetes mellitus are from carpal tunnel syndrome. Repetitive shear forces, anatomic factors, and excessive stiffness of connective tissues may cause these mononeuropathies.

Serum concentrations of von Willebrand factor and soluble thrombomodulin indicate alteration of endothelial function in inflammatory bowel diseases

Serum concentrations of immunoreactive von Willebrand factor (vWF) and soluble thrombomodulin (TM), and vWF multimer patterns were measured to assess endothelial function in patients with inflammatory intestinal diseases. In Crohn's disease and acute infective diarrhea, vWF concentrations were significantly higher than in normal controls. In all patient groups, multimeric analysis of vWF and the concentration of serum TM were not different from normal controls. The results indicate alteration of endothelial function in inflammatory intestinal disorders. They may be compatible with the presence of localized vasculitis, but indicate that systemic endothelial destruction does not occur in inflammatory bowel disease.

Localized vasculitis of the gastrointestinal tract.

We studied the clinical and pathologic findings of 63 patients with localized vasculitis of the gastrointestinal tract, including 35 partial bowel resections, 14 cholecystectomies, five partial pancreatectomies, six appendectomies, one omentectomy, one gastrectomy, and one esophagectomy. Vasculitis was classified histologically as polyarteritis (n = 33), phlebitis (n = 12), Churg-Strauss angiitis (n = 8), small-vessel vasculitis (n = 6), Buerger's disease (n = 2), and giant-cell arteritis (n = 1). Nineteen of 33 cases of polyarteritis affected the small bowel or gallbladder, and nine patients with polyarteritis had elevated serum antinuclear antibodies or rheumatoid factor. Eight of 12 cases of phlebitis affected the right colon; there were giant cells in four of these 12 cases, a history of medication use in seven of eight cases, and no evidence of serum autoantibodies. Short-term follow-up (mean, 5 years) demonstrated that systemic disease developed in six of 23 patients with polyarteritis (four of whom had elevated serum rheumatoid factor or antinuclear antibodies), the patient with giant-cell arteritis, and one of two patients with Buerger's disease. Systemic vasculitis did not develop in patients with other types of vasculitis. We conclude that patients with gastrointestinal phlebitis, polyarteritis without serum autoantibodies, and small-vessel vasculitis have a low short-term risk for the development of systemic disease.