Type Of Vasculitis Research Articles

The presence of anti-neutrophil extracellular trap antibody in patients with microscopic polyangiitis.

Although ANCA is the major autoantibody in patients with ANCA-associated vasculitis, previous studies have suggested the presence of anti-neutrophil extracellular trap (NET) antibody in patients with microscopic polyangiitis (MPA), one type of ANCA-associated vasculitis. In this study, we aimed to determine the prevalence and pathogenic role of anti-NET antibody (ANETA) in MPA. We examined the presence or absence of ANETA in sera obtained from 19 MPA patients by indirect immunofluorescence. We compared the clinical parameters, including age, sex, MPO-ANCA, creatinine, CRP, MPO-DNA complexes and vasculitis activity, in ANETA-positive and ANETA-negative MPA patients. We investigated the serum NET induction and degradation abilities of ANETA-positive and ANETA-negative MPA patients with reference to healthy controls (n = 8). Furthermore, we assessed the relationship between ANETA and the effect of IgG depletion on the serum NET degradation ability. ANETA was present in 10 of the 19 MPA patients. There was no significant difference in the clinical parameters in ANCA-positive and ANCA-negative MPA patients. Although the NET induction ability was higher and the NET degradation ability was lower in MPA sera than those in healthy controls, these abilities were not different between ANETA-positive and ANETA-negative MPA sera. Interestingly, the NET degradation ability in some sera with ANETA was markedly increased by IgG depletion. Some MPA patients produce ANETA and some ANETA possess an inhibitory function against the serum NET degradation ability. Although further studies are needed, ANETA is worthy of attention in order to understand the pathophysiology of MPA.

Nomenclature of cutaneous vasculitides - German translation of the dermatologic addendum to the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides.

The nomenclature system agreed upon by the Chapel Hill Consensus Conference (CHCC) with respect to the terminology and precise definition of the various types of vasculitis has gained widespread interdisciplinary and international acceptance. As the revised version from 2012 (CHCC 2012) does not address the special features of vasculitis of the skin, it has recently been supplemented with an addendum containing the nomenclature of cutaneous vasculitides (D-CHCC). The present article provides the German translation of the terms and defintions of the D-CHCC as well as additional explanatory comments. The goal is to enable German-speaking health care providers to more readily apply these defined terms - which are based on interdisciplinary consensus - in everyday clinical practice and to facilitate assessment of their practicability and relevance in patient care. If the majority of cutaneous vasculitides diagnosed can be matched with any of the defined entities presented herein, it might subsequently be possible to develop a classification system and diagnostic algorithms.

Oral manifestations of granulomatosis with polyangiitis - Clinical and radiological assessment

Background/purposeGranulomatosis with polyangiitis (GPA) is a type of primary systemic vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). GPA mainly affects medium and small vessels and may manifest in different organs, most commonly upper respiratory tract. Oral lesions occur in 6–13% of GPA patients and might be the first symptom observed by the patient. This study presents the evaluation of orofacial manifestations of patients with GPA. Materials and methodsProspective study was conducted between June 2014 and November 2017 in Department of Allergy and Immunology at University Hospital in Krakow. Patients diagnosed with GPA, after medical assessment, were examined including precise dental inspection, bacterial and fungal flora of oral cavity evaluation and Cone Beam Computed Tomography (CBCT) imaging. ResultsNine patients were enrolled in the study. Characteristic for GPA strawberry gingivitis was observed in one patient. Bone destruction and inflammatory lesions in paranasal sinuses was confirmed by CBCT in 55.6% of patients. Fungal infection was revealed in 66.7% of patients. ConclusionThese findings oblige dentists to consult patient with laryngologist or internal medicine physician to establish further diagnostic approach, because early diagnosis of GPA is crucial for implementing appropriate treatment and preventing chronic organ damage.

Significance of histological crescent formation in patients with IgA vasculitis (Henoch-Sch\xf6nlein purpura)-related nephritis: a cohort in the adult Chinese population

BackgroundIgA vasculitis (IgAV, formerly Henoch-Schönlein purpura) is a type of systemic vasculitis. This study aimed to explore the clinicopathological features, treatment and renal outcomes of adult IgAV-related nephritis (Henoch-Schönlein purpura nephritis) patients with different degrees of crescent formation.MethodsAdult patients with biopsy-proven IgAV-related nephritis in Nanjing Jinling Hospital were enrolled and divided into three groups as follows: control (no crescents, n = 257), group 1 (crescents < 25%, n = 381), and group 2 (crescents ≥25%, n = 60). The clinicopathological features, treatment and renal outcomes were compared among the three groups.ResultsThere were no significant differences in gender and age at biopsy among the three groups. Groups with more crescents had shorter renal durations and higher prevalence of macroscopic hematuria, proteinuria and nephrotic syndrome than the control group. The presence of renal insufficiency at biopsy was similar, whereas laboratory findings indicated that patients with ≥25% crescents had higher levels of serum creatinine and blood urea nitrogen than the control and group 1. Histologically, the incidence of glomeruli-Bowman’s capsule adhesion and capillary necrosis were proportional to the degree of crescent formation. Patients with more crescents received more positive immunosuppressive therapies. During follow-up, the levels of proteinuria and hematuria were in remission after treatment, and patients without crescents had lower levels of proteinuria. At the last follow-up, the renal function had deteriorated in the control and group 1, whereas the levels of serum creatinine at biopsy and last follow-up were similar in group 2. There was a significant difference in renal survival from end-stage renal disease (ESRD) or 50% decline in renal function among the three groups (log-rank, P = 0.030). However, no association between crescent formation and renal outcomes was found after adjusting potential confounders.ConclusionsAdult IgAV-related nephritis patients with more crescents had more-severe renal manifestations and worse treatment responses, whereas the proportions of crescents were not associated with higher risks for ESRD or 50% decline in renal function. A more suitable pathological classification standard is needed to predict renal prognosis.

An evaluation of depression, anxiety and fatigue in patients with Behçet's disease.

Behçet's disease (BD) is a type of systemic vasculitis and inflammatory disease of unknown etiology, which is associated with fatigue and lower quality of life (QoL). The aim of this study was to assess the relationship between Behçet's Disease Current Activity Form (BDCAF) and Behçet's Disease Quality of Life (BDQoL), depression, anxiety and fatigue in Behçet's disease. We conducted a cross-sectional study of 155 BD patients and 107 healthy subjects in a single center over a 1year period. All participants completed the Multidimensional Assessment of Fatigue (MAF) questionnaire, and the Hospital Anxiety and Depression (HADS) scale. Disease activity in the BD patients was assessed using the BDCAF, and the physician's global assessment (PGA). BD patients also completed the BDQoL questionnaire. There was no significant difference in age and gender between the groups. BD patients had significantly higher HADS-anxiety (HADS-A), HADS-depression (HADS-D) and MAF scores than the healthy control group (P<0.05). BD patients with active disease had significantly higher MAF and HADS-A scores compared to inactive BD patients (P<0.05). MAF scores showed positive correlations with HADS-A, HADS-D, BDCAF and BDQoL. Fatigue and anxiety are common in clinically active BD patients compared with healthy control subjects and inactive BD patients.

Pulmonary Thromboembolism in Behcet’s Disease: A Case Report

Behcet’s disease is a vasculitis which presents as recurrent oral and genital aphthous ulceration, uveitis and skin lesions. Unlike other types of vasculitis, venous system involvement is a common manifestation of Behcet’s disease. Despite the high incidence of deep vein thrombosis in Behcet’s disease, pulmonary artery thromboembolism (PTE) is a rare complication. In this article, we report on a 44-year old man who experienced recurring painful oral ulcers, bilateral panuveitis, superficial phlebitis and positive pathergy which had led to a diagnosis of Behcet’s disease 12 years earlier. He developed sudden onset dyspnea, pleuritic chest pain and hemoptysis two days before admission. The patient had tachypnea and mild respiratory distress at the time of admission. CT angiography showed filling defects in the inter-lobar arteries of both lungs with sub-segmental consolidation. A lung perfusion scan showed multiple segmental and sub-segmental perfusion defects in both lungs which did not match the ventilation scan. A diagnosis of PTE was made and anticoagulation with enoxaparin and warfarin was begun. The tachypnea, chest pain and hemoptysis disappeared after three days and he was discharged after 10 days with warfarin. His disease was in complete remission at eight months after discharge.

A10216 Serum MCP-1 levels are increased in systemic vasculitis of pations with hypertension with renal involvement

Objectives: Monocyte chemotactic protein 1 (MCP-1), which is able to adjust the migration of monocytes/macrophages, osmosis and raise monocytes and T lymphocyte cells to participate in a variety of inflammation, are closely linked to various types of systematic vasculitis. However, serum MCP-1 levels have not been evaluated in patients with small and medium vessel vasculitis (SMVV), especially in those with PAN. The study was performed to investigate the level of serum MCP-1 in determining the SMVV. Methods: We reviewed all patients admitted to our center for the etiology screening of hypertension between January 2013 and December 2016. All vasculitis are diagnosed by a rheumatologist and fulfilled the American College of Rheumatology (ACR) 1990 criteria. Serum samples were collected in 43 patients with systemic vasculitis with hypertension and 43 healthy controls (HC). Serums MCP-1 were measured using commercially available ELISA kits. Results: The serum MCP-1 levels were significantly higher in patients with systemic vasculitis, compared with healthy controls (p < 0.001). Furthermore, it was significantly increasing in patients with renal than non-renal involvement (p = 0.001) and HC (p < 0.001). However, there was no significant statistical differences between active and inactive phase. Serum MCP-1 levels with patients in systemic vasculitis were positive correlation with serum creatinine levels (r = 0.387, p < 0.010). Furthermore, there was a closely correlation between 24-hour proteinuria and MCP-1 levels (r = 0.404, p < 0.014). Conclusion: Serums MCP-1 were significantly higher in patients with systemic vasculitis compared with HC, especially in patients with renal involvement. Serum MCP-1 might be as a potential biomarker tools in the systemic vasculitis with renal involvement.

A case report of granulomatous polyangiitis complicated by tuberculous lymphadenitis

Rational:Granulomatous polyangiitis (GPA) is a type of vasculitis involving medium and small arteries, typically affecting the upper and lower respiratory tract with coexisting glomerulonephritis. GPA is also characterized by necrotizing granulomatous inflammation and the presence of antineutrophil cytoplasm antibodies (ANCA). So far, various infections have lead to elevation of titers of serum ANCA, making it difficult to diagnose.Patient Concerns:We report a 50-year-old woman who was diagnosed as tuberculous lymphadenitis. During the treatment by anti-tuberculosis (TB) drugs, rapidly progressive renal failure and pleurisy had appeared with elevated titer of PR3-ANCA. Renal biopsy revealed crescentic glomerulonephritis.Diagnosis:Renal biopsy revealed crescentic glomerulonephritis and diagnosis of GPA was made.Interventions:Steroid therapy had been started with continuation of anti-TB drugs.Outcomes:Renal dysfunction had gradually recovered and pleurisy had disappeared with decreasing titer of PR3-ANCA.Lessons:This is the first report of GPA complicated by TB infection. When we encounter a case with rapidly progressive renal failure during the TB infection, complication of GPA should be suspected as 1 of the different diagnosis.

Vascular calcification in patients with large-vessel vasculitis compared to patients with hyperlipidemia

ObjectiveCalcification of the coronary arteries, aorta, and branch vessels can occur in both large-vessel vasculitis (LVV) and atherosclerosis. The study objective was to determine the location and amount of vascular calcification in patients with LVV versus hyperlipidemia (HLD) and to identify risk factors associated with vascular calcification in LVV. MethodsPatients with giant cell arteritis (GCA), Takayasu's arteritis (TAK), and HLD underwent non-contrast computed tomography of the aorta and branch vessels. Vascular calcification in 14 specific arterial territories (4 segments of the aorta, 9 branch arteries, and the coronary arteries) was quantified throughout the large arteries by a cumulative Agatston score. Multivariate linear regression analyses were used to identify associations between traditional and disease-specific risk factors and total Agatston score. ResultsA total of 88 subjects, including GCA (n = 29); TAK (n = 22); and HLD (n = 37), participated. Prevalence of vascular calcification in the aorta and branch vessels significantly differed in the coronary arteries (HLD = 67%, GCA = 35%, TAK = 9%, p < 0.01). Total Agatston scores were higher in GCA (median 3260, range 25–18,138) versus HLD (460, 19–17,215) (p < 0.01) but did not significantly differ between GCA and TAK (1944, 52–47,520) (p = 0.53). In multivariable regression analysis, age, type of vasculitis, and prednisone use was associated with vascular calcification in LVV. ConclusionThe prevalence of coronary artery calcification is lower in LVV compared to HLD, but the amount of total vascular calcification throughout the large arteries is greater in LVV. Both traditional and disease-specific risk factors are associated with vascular calcification in LVV.

Increased Serum MCP-1 Levels in Systemic Vasculitis Patients with Renal Involvement.

Monocyte chemotactic protein 1 (MCP-1) plays a significant role in inflammation pathways by affecting monocyte/macrophage migration, the number of monocytes and T lymphocytes, and osmosis. Inflammation is closely linked to various types of systematic vasculitis. Here we characterized serum MCP-1 levels in systemic vasculitis patients. This cross-sectional study included serum samples collected from 43 patients with systemic vasculitis and 43 healthy controls (HCs). Serum MCP-1 levels in the samples were measured using commercially available enzyme-linked immunosorbent assay (ELISA) kits. Serum MCP-1 levels were significantly higher in patients with systemic vasculitis relative to HCs (parentheses indicate quartile values) [134.65 (73.74, 262.75) pg/mL versus 59.1 (37.41, 90.18) pg/mL, P < 0.001]. Furthermore, systemic vasculitis patients having renal involvement had significantly higher MCP-1 levels relative to patients without renal involvement [196.16 (104.41, 310.35) pg/mL versus 73.74 (41.24, 145.95) pg/mL, P = 0.001] and HCs [196.16 (104.41, 310.35) pg/mL versus 59.10 (37.41, 90.18) pg/mL, P < 0.001]. Serum MCP-1 levels in systemic vasculitis patients were positively correlated with serum creatinine levels (r = 0.387, P < 0.010) and with 24-h proteinuria (r = 0.404, P < 0.014). Receiver operating characteristic (ROC) analysis showed that the cutoff value for MCP-1 to distinguish systemic vasculitis from HCs was 72.73 pg/mg, and the area under the ROC curve was 0.772. The sensitivity and specificity were 76.7% and 72.1%, respectively. Serum MCP-1 levels were significantly higher in patients with systemic vasculitis than in HCs, especially in patients with renal involvement. Thus, serum MCP-1 has the potential to be a biomarker for systemic vasculitis with renal involvement.

Endothelial dysfunction in patients with eosinophilic granulomatosis with polyangiitis

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of vasculitis associated with asthma and eosinophilia. Endothelial dysfunction has been well documented in other types of vasculitis but not in EGPA. Thirty patients (10 men and 20 women) diagnosed with EGPA and remaining in a remission, and 58 controls (24 men and 34 women) matched for age, sex, and body mass index, were enrolled in the study. We assessed each participants for typical risk factors of cardiovascular diseases and measured serum levels of vascular cell adhesion molecule-1 (VCAM-1), interleukin 6 (IL-6), and thrombomodulin. We also measured flow-mediated dilatation (FMD) of the brachial artery and intima-media thickness (IMT) of the common carotid artery using ultrasonography. Patients with EGPA had 20% higher serum level of VCAM-1 (p < 0.001) and 41.9% of thrombomodulin (p < 0.001). They also had 38.8% lower relative increase of FMD (FMD%) (p < 0.001), indicating endothelial dysfunction. These differences remained significant also after adjustment for potential confounders. Laboratory and ultrasonographic parameters of endothelial injury were correlated to the markers of inflammation and impaired kidney function. Determinants of lower FMD% in a simple regression model were pack-years of smoking (β = − 0.3 [95% confidence interval (CI) − 0.5 to − 0.1]), serum level of IL-6 (β = − 0.36 [95% CI − 0.62 to − 0.1]), and thrombomodulin (β = − 0.34 [95% CI − 0.6 to − 0.08]). EGPA patients are characterized by inflammatory endothelial injury that is likely related to the pathogenesis of the disease. Proper immunosuppressive treatment is the best method to prevent atherosclerosis and future cardiovascular events, the patients may also benefit from additional preventive interventions.

Vasculitis associated with immune checkpoint inhibitors-a systematic review.

Recent experimental and genetic studies have implicated the role of programmed cell death protein 1 (PD-1), programmed cell death protein-ligand 1 (PDL-1), and cytotoxic T lymphocyte-associated antigen 4 (CTLA-4) in the pathogenesis of medium and large vessel vasculitis. This study sought to evaluate the occurrence and nature of vasculitis associated with cancer treatment using immune checkpoint inhibition (anti-PD-1, anti-PDL-1, and anti-CTLA4). A systematic review of the medical literature was conducted by searching all available clinical data up to February 2018 in several databases and search engines including Cochrane Library, Embase, Google Scholar, Medline, Scopus, Web of Science, and Clinicaltrials.gov . Searches included the following FDA-approved anti-PD1 (nivolumab and pembrolizumab), anti-PDL1 (atezolizumab, avelumab, and durvalumab), and anti-CTLA4 (ipilimumab). The vasculitis cases were compiled and classified based on the 2012 revised Chapel Hill Consensus Conference nomenclature. The clinical feature of the vasculitis cases and their relationship to immune checkpoint inhibition was assessed. There were 53 cases of vasculitis of which 20 were confirmed. The main reported type of vasculitis was large vessel vasculitis and vasculitis of the central and peripheral nervous system. All cases resolved with either holding the immune checkpoint inhibitors and/or administering glucocorticoids. No death related to vasculitis was reported. Vasculitis, namely large vessel and vasculitis of the nervous system, is associated with immune checkpoint inhibition. Results of this study add to the growing evidence regarding the relationship between immune checkpoints and vasculitis and suggest that the pathway may be a therapeutic target.

Churg-Strauss Syndrome in a Seven-year-old Boy: A Case Report

Churg-Strauss syndrome (CSS) is a type of vasculitis of small-to-medium sized vessels. This syndrome is known by a history of bronchial asthma with systemic necrotizing vasculitis and peripheral blood hypereosinophilia. It is currently called eosinophilic granulomatosis with polyangiitis (EGPA). This disease affects both genders and all age groups, but it is very rare among children. CSS diagnosis is based on clinical findings such as asthma, eosinophilia, rhinosinusitis, and signs of vasculitis in major organs. In cases where steroids alone or in combination with other immunosuppressive agents are used as treatment, the outcome and long-term survival are usually satisfying. In comparison with other types of systemic vasculitis, the mortality rate of this syndrome is low. In this study, we present the case of a 7-year-old boy with poorly controlled bronchial asthma since three years of age. This case had developed purpuric skin lesions, sinusitis, arthritis, and weakness of the limbs with symptoms of mononeuritis multiplex at the age of seven. After being admitted to our hospital, a series of studies, including complete blood count-diff, chest X-ray, paranasal sinus radiography, brain magnetic resonance imaging, nerve conduction study, spirometry, and serological tests for autoantibodies, were performed and he was diagnosed with CSS. Thereafter, he received regular corticosteroid therapy in combination with methotrexate, and his symptoms were generally well-controlled with the beginning of the treatment. The clinical characteristics, diagnosis, and management of CSS in children are also reviewed in this study.

Treatment of Eosinophilic Granulomatosis with Polyangiitis: A Review.

Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic granulomatosis with polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic granulomatosis with polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities. Its conventional treatment relies mainly on agents that decrease inflammation: corticosteroids and immunosuppressant adjunction for severe manifestations. New therapeutic approaches are needed for refractory disease, relapses and issues associated with corticosteroid dependence, especially for asthma manifestations. Drugs under evaluation mostly target eosinophils and B cells. Results of low-evidence-based trials suggested possible efficacies of biologicals: B-cell-blocking rituximab and anti-immunoglobulin E omalizumab. Recently, the first large-scale randomised controlled trial on eosinophilic granulomatosis with polyangiitis proved the efficacy of anti-interleukin-5 mepolizumab. That finding opens a new era in eosinophilic granulomatosis with polyangiitis management, with mepolizumab approval but also in future drug evaluations and trial designs for eosinophilic granulomatosis with polyangiitis. Additional studies are needed to determine which patients would benefit most from targeted therapies and achieve personalised treatment for patients with eosinophilic granulomatosis with polyangiitis. Herein, we review eosinophilic granulomatosis with polyangiitis characteristics and provide an overview of established and novel pharmacological agents.

Nocuous Skin Manifestations of Spironolactone:Drug Induced Leukocytoclastic Vasculitis

The author discusses a case of drug induced vasculitis caused by spironolactone which is very rare complication of the drug. The authors give account on the type of vasculitis caused by drug induced allergy and also the differential diagnoses if the presentation is unusual with only hematuria and kidney injury. The benefits of Spironolactone and the side effects are also illustrated.

Nocuous Skin Manifestations of Spironolactone: Drug Induced Leukocytoclastic Vasculitis

The author discusses a case of drug induced vasculitis caused by spironolactone which is very rare complication of the drug. The authors give account on the type of vasculitis caused by drug induced allergy and also the differential diagnoses if the presentation is unusual with only hematuria and kidney injury. The benefits of Spironolactone and the side effects are also illustrated.

Tocilizumab for the treatment of giant cell arteritis

ABSTRACTIntroduction: Giant cell arteritis (GCA) is the most frequent type of vasculitis, occurring in people older than 50 years. So far, treatment has been limited to corticosteroids and methotrexate only.Areas covered: Interleukin-6 (IL-6) plays a role in the pathophysiology of GCA. This review covers recent advances in the treatment of GCA with tocilizumab (TCZ), which specifically binds to both soluble and membrane-bound IL-6R and inhibits IL-6R-mediated signaling.Expert commentary: Two randomized controlled trials recently showed the efficacy of the IL-6 receptors inhibitor monoclonal antibody TCZ for the induction and maintenance of remission in patients with new-onset and relapsing GCA. Furthermore, addition of TCZ to prednisone led to a reduction in the cumulative prednisone doses required to control GCA. The profile of adverse events was balanced across treatment groups and no safety concerns were raised during the trial.

Epigenetic hypomethylation and upregulation of NLRC4 and NLRP12 in Kawasaki disease.

INTRODUCTIONKawasaki disease (KD) is a type of childhood febrile systemic vasculitis. Inflammasomes control inflammatory signaling and are related with the development of KD. In this study, we performed a survey of transcripts and global DNA methylation levels of inflammasome sensors of NOD-like receptors (NLRs) and the downstream interleukin 1β (IL-1β).MATERIALS AND METHODSIn this study, for the chip studies, we recruited a total of 18 KD patients, who we analyzed before receiving intravenous immunoglobulin (IVIG) and at least 3 weeks after IVIG treatment, as well as 36 non-fever controls by Illumina HumanMethylation 450 BeadChip and Affymetrix GeneChip® Human Transcriptome Array 2.0. A separate group of 78 subjects was performed for real-time quantitative PCR validations.RESULTSThe expressions of mRNA levels of NLRC4, NLRP12, and IL-1β were significantly upregulated in KD patients compared to the controls (p<0.05). Once KD patients underwent IVIG treatment, these genes considerably decreased. In particular, the methylation status of the CpG sites of these genes indicated a significant opposite tendency between the KD patients and the controls. Furthermore, mRNA levels of IL-1β represented a positive correlation with NLRC4 (p=0.002). We also observed that the mRNA levels of NLRP12 were lower in KD patients who developed coronary arterial lesions (p<0.005).CONCLUSIONThis study is among the first to report epigenetic hypomethylation, increased transcripts, and the upregulation of NLRC4, NLRP12 and IL-1β in KD patients. Moreover, a decreased upregulation of NLRP12 was related to coronary arterial lesion formation in KD patients.

Infection-associated vasculitides.

Vasculitides are disorders characterized by inflammation of the vessel walls, often caused by autoimmunity, but sometimes as a result of microbial invasion. Almost all types of microbes including bacteria, viruses, protozoa and fungi have been incriminated in the pathogenesis of vasculitis. Accurate etiological diagnosis is important since immunosuppressive treatment may lead to further deterioration if infection is the cause of vasculitis. Clinical features sometimes provide clues to the etiology. Further evaluation requires a focused and cost-effective plan of laboratory investigation. The investigations aim at establishing the diagnosis of vasculitis and identify the causative organism. An accurate diagnosis of vasculitis optimally requires histological examination and imaging. For infection-associated vasculitis, the identification of the organism requires studies of stained specimens, cultures, and/or detection of antigens and antibodies. Ideally, the treatment involves administration of an appropriate antimicrobial. In non-self-limiting types of vasculitides, glucocorticoids are needed when the symptoms are progressive, with vital organs involvement, and sometimes, when there is no antimicrobial agent of proven efficacy against the incriminated agent. Additional immunosuppressive agents or interventions must be considered when the disease is severe and/or post-infective immune mechanisms are involved in the pathogenesis, e.g., severe HBV- or HCV-associated vasculitides. Available preventative vaccinations are also crucial. The incidence of HBV-associated vasculitides dramatically decreased following HBV vaccination campaigns, and other infection-associated vasculitides may as well in the future.

Primary central nervous system vasculitis associated with lymphoma

To record the clinical findings, response to therapy, and course of patients with primary CNS vasculitis (PCNSV) associated with lymphoma. We reviewed the histories of 936 patients with a diagnosis of any type of vasculitis and lymphoma who were seen at the Mayo Clinic over a 32-year period. Ten patients with both PCNSV and lymphoma were identified. We compared the findings in these 10 patients with those from 158 patients with PCNSV without lymphoma seen over 29 years. Ten of a total of 168 (5.9%) patients with PCNSV also had a history of lymphoma: 6 with Hodgkin lymphoma (HL) and 4 with non-HL (NHL). A granulomatous vasculitis was found in all 8 patients with cerebral biopsies, accompanied by vascular deposits of β-amyloid peptide in 2. In 7 patients, medical diagnostic workup for PCNSV revealed the lymphoma. Compared to the 158 patients with PCNSV without lymphoma, patients with lymphoma were more frequently male (p = 0.04), had increased gadolinium leptomeningeal enhancement (p = 0.03) at presentation, and had more neurologic disability at last follow-up (p = 0.01). No significant differences in treatment response were observed in the 2 groups (p = 0.202). Considering all 168 patients, increased disability at last follow-up was associated with increasing age at diagnosis (odds ratio [OR] 1.4), lymphoma (OR 5.9), and cerebral infarction (OR 3.2), while reduced disability was associated with gadolinium-enhanced lesions (OR 0.43) and amyloid angiopathy (OR O.23). Lymphoma may be diagnosed simultaneously with PCNSV, suggesting an immunologic paraneoplastic mechanism.