Search

Introduction: Drug-induced vasculitis is reported in almost 10-20 % of vasculitis. Several drugs may be incriminated in their occurrence. Our study aimed to study the epidemiological, clinical, histopathological and evolutionary characteristics of drug-indced vasculitis from a series of cases and to specify the different drugs involved., Methods: We conducted a retrospective study during the period from January 2006 to December 2015 from the cases notified to the regional pharmacovigilance center of Sousse, Tunisia. The diagnosis was established according to the criteria proposed by the group of the American college of rheumatology (ACR)., Results: Our study included thirteen cases of drug-induced vasculitis over a ten-year period, with an mean incidence of 1.3 new cases per year. Mean age of patients was 40.84 years. The mean delay from the treatment onset was 14.46 days with extremes ranging from 5 days to six weeks. Most patients had pure skin involvement. Association with other extracutaneous complaints was present in five cases. Cutaneous biopsy was performed in all patients showing a pathological pattern of leukocytoclastic vasculitis, associated with fibrinoid necrosis, extravasation of red blood cells and allergic capillaritis. The outcome was favorable for all patients. The offending drugs in our series were amoxicillin, pristinamycin, rifampicin, fluconazole, metformin, glimepiride, phenobarbital, gabapentin, fenofibrate, ibuprofen, allopurinol, rituximab and tinzaparin., Conclusion: Anamnestic, clinical, biological and histopathological findings allow the early recognition of drug-induced vasculitis. Adequate treatment prevents systemic spreading and a worse prognosis., (Copyright © 2018. Published by Elsevier Masson SAS.)

Erythema elevatum diutinum (EED) is a rare cutaneous leukocytoclastic vasculitis thought to be related to increased levels of circulating antibodies. It has been shown to be associated with HIV infection, tuberculosis, as well as various autoimmune diseases. A retrospective review of all cases of EED indexed in PubMed between 1990 and 2014 was performed. Inclusion criteria for articles was availability of full text in English and a biopsy-confirmed diagnosis of EED. All other articles were excluded. Cases were stratified by age and anatomic location of the lesions. Treatment response was coded as "complete," "partial," and "none." A total of 133 cases of EED with 381 lesions detailed in case reports and case series were included. Twenty-one cases were associated with HIV. Of 47 patients with reported paraproteinemias, IgA paraproteinemia was found in 57.45%, IgG paraproteinemia in 29.8%, IgM paraproteinemia in 10.6%, and IgD paraproteinemia in 2.1% of cases. Of 40 (30.1%) patients with reported comorbid autoimmune disease, rheumatoid arthritis was associated with 10 cases. Cancer was found to be associated with 9.77% of cases. Seventy-five patients were treated with dapsone, with 36 (48%) achieving complete treatment response, 24 (32%) achieving partial response, and seven (9.3%) achieving no response. Keeping the clinical associations of EED in mind, especially malignancy, is critical in management of the disease. More structured studies need to take place in order to fully define the mechanisms and strength of these associations., (© 2018 The International Society of Dermatology.)

Background: Existing studies of children with vasculitis are limited. The aim of this study was to assess the epidemiology, clinical manifestations, laboratory findings, course, and outcome of Greek children presenting with vasculitic rash., Methods: The relevant data included in the study were collected retrospectively using a standardized form from children who were admitted into our department between 2003 and 2013, with the provisional diagnosis of vasculitis of the skin., Results: The study sample consisted of 95 children (58 boys, 37 girls) with a mean age of 5.9 years. In total, 76 out of 95 (80%) of the children were diagnosed with Henoch-Schönlein purpura, 10/95 (10.5%) with hypersensitivity vasculitis, 6/95 (6.3%) with urticarial vasculitis, and 3/95 (3.1%) with acute hemorrhagic edema of infancy. The mean age of the children was 5.7 years for Henoch-Schönlein purpura, 9 years for hypersensitivity vasculitis, 5.1 years for urticarial vasculitis, and 0.5 years for acute hemorrhagic edema of infancy., Conclusions: (i) The most common vasculitis presenting with skin rash in children is Henoch-Schönlein purpura; (ii) hypersensitivity vasculitis occurs in older children more often when compared to other types of vasculitis; (iii) urticarial vasculitis lesions may be a sign of severe underlying disease; therefore a thorough examination of these patients is warranted; (iv) Despite relapses, the overall prognosis of patients with pediatric skin vasculitides is good, with the exception of those with the urticarial vasculitis type., (© 2017 The International Society of Dermatology.)

Background: Acute hemorrhagic edema of infancy (AHEI) is a rare leukocytoclastic vasculitis of the small vessels occurring at a young age and considered as a benign self-limited disease. Due to its low prevalence, there are limited data on the presentation and complications of this disease., Methods: All computerized files of children who were hospitalized at a tertiary pediatric center due to AHEI over a 10 year period were reviewed. Clinical, laboratory and histopathological data were collected., Results: Twenty-six patients were included in our study, accounting for 0.7 cases per 1000 admissions of children aged 2 years or less. Mean age was 12.9 months. More than two thirds of the children had preceding symptoms compatible with a viral infection. Upon admission, all patients presented with typical findings of a rash and edema. Edema was most profound over the lower extremities (73%). Concomitant viral or bacterial infections were found in six children. Skin biopsy was performed in six patients revealing leukocytoclastic vasculitis. Thirteen children (50%) had systemic involvement including joint involvement (n=9), gastrointestinal hemorrhage (n=4), microscopic hematuria (n=1) and compartment syndrome of the limb (n=1). The latter was diagnosed in a patient with familial Mediterranean fever., Conclusions: Our largest data series highlighted what is known regarding clinical and histological findings in children with AHEI. However, contrary to what was previously reported, we found a higher rate of systemic involvement. Although AHEI is a rare entity, pediatricians should be familiar with its presentation, management and our reported complications.

Clinical manifestation, etiology and outcome of leukocytoclastic vasculitis are little studied. The aim of our study was to examine epidemiological, clinical etiological, and evolutionary characteristics of this entity. We conducted a cross-sectional data collection from medical records of 85 patients with leukocytoclastic vasculitis in the Department of Dermatology at the Farhat Hached University Hospital, Sousse between January 2000 and December 2013. Epidemiological, clinical, paraclinical, etiological data sheets had been completed for each patient. The average age of patients was 47.65 years, ranging between 10 and 78 years. Fifty-three women and 32 men were registered (sex ratio = 0.6). Cutaneous manifestations were dominated by vascular purpura (88.2%). The most common causes of leukocytoclastic vasculitis were systemic diseases (51%), infection (20%) and neutrophilic dermatoses (14.5%). Other causes were drugs (9.1%) and hematologic malignancies (5.4%). The cause of leukocytoclastic vasculitis was not detected in 30 patients (35, 3%). Two predictive factors associated with the acute outcome were retained: the presence of a recent infection (p= 0.014) and drug intake before the rash (p= 0.013). Chronic evolution was positively correlated with antinuclear antibodies (p= 0.009) and cryoglobulinemia (p=0.025). Our study highlights the multitude of causes of leukocytoclastic vasculitis. The search for an underlying disease is an imperative in order to ensure better therapeutic management., Competing Interests: Les auteurs ne déclarent aucun conflit d'intérêt.

Leukocytoclastic vasculitis (LCV) is a heterogenous group of disorders that may manifest as a mild disease isolated to the skin or be a part of life-threatening systemic vasculitis. According to the 2012 Chapel Hill Consensus Conference nomenclature, patients presenting symptoms of LCV confined only to the skin should be defined as suffering from a single-organ cutaneous small vessel vasculitis (SoCSVV). SoCSVV is a benign disease with a good clinical outcome but with a significant risk of relapse and skin ulcer formation.The aim of the current study was to characterize SoCSVV and to identify factors that may be associated with the risk of recurrence and skin ulcers.Medical records of patients with LCV hospitalized at the Department of Dermatology at University Hospital in Cracow in the years 2010 to 2015 were analyzed.A total of 24 patients fulfilled criteria of SoCSVV. Drugs and preceding infections were identified as precipitating factors in 40% and 20% of cases, respectively. Skin lesions other than palpable purpura (i.e., macules, urticarial vasculitis, or ulcers) were identified in almost half of the patients. Interestingly, the presence of macules independently increased the risk of skin ulcer formation (odds ratio = 16; 95% confidence interval: 1.5-176.6; P = 0.0075) in the multivariate logistic regression analysis. One-quarter of patients with SoCSVV experienced relapse during the 6-month follow-up. The greater number of affected skin areas was an independent risk factor of recurrence (odds ratio = 5; 95% confidence interval: 2-45; P = 0.02).SoCSVV was usually associated with drugs and preceding infections. The disease relapses in approximately one-quarter of the patients. The more severe the skin involvement in the course of SoCSVV, the higher is the risk of recurrence.

A 41-year-old Hispanic woman with a 20 pack-year smoking history presented with worsening shortness of breath on exertion that gradually started 2 years ago, then significantly deteriorated over the last 4 months. She was diagnosed with COPD 2 months prior to her presentation and started on treatment with fluticasone propionate and albuterol. Her medical history was relevant for undifferentiated connective tissue disorder diagnosed 5 years prior due to a positive antinuclear antibody test, arthralgia, recurrent urticarial skin rash, peripheral neuropathy, abdominal pain, and diffuse body swelling. She was started on treatment with prednisone and azathioprine at the time and had substantial improvement in the occurrence of her urticaria. She also had a history of recurrent laryngeal edema of unclear etiology. She had no history of IV drug abuse, no exposure to animals, was not sexually active, and had no recent travel outside of Florida. There was no significant family history of lung diseases.

Objective: To determine the population-based incidence of leukocytoclastic vasculitis (LCV)., Patients and Methods: This is a retrospective population-based study of all Olmsted County, Minnesota, residents with a skin biopsy-proven diagnosis of LCV from January 1, 1996, through December 31, 2010., Results: A total of 84 patients (mean age at diagnosis, 48.3 years) with newly diagnosed skin biopsy-proven LCV (43 women and 41 men) were identified. The incidence rate (age and sex adjusted to the 2000 US white population) was 4.5 per 100,000 person-years (95% CI, 3.5-5.4). The incidence of LCV increased significantly with age at diagnosis (P<.001) and did not differ between female and male patients. Subtypes of LCV were cutaneous small-vessel vasculitis (CSVV), 38 patients (45%); IgA vasculitis, 25 (30%); urticarial vasculitis, 10 (12%); cryoglobulinemic vasculitis, 3 (4%); and antineutrophil cytoplasmic antibody-associated vasculitis, 8 (10%). LCV was idiopathic in 29 of 38 patients with CSVV (76%) and 24 of 25 patients with IgA vasculitis (96%). Thirty-nine of 84 patients (46%) had systemic involvement, with the renal system most commonly involved (17 of 39 [44%]). Twenty-four of 80 patients (30%) with follow-up data available had recurrent disease. Compared with the Minnesota white population, observed survival in the incident LCV cohort was significantly poorer than expected (P<.001), including the subset of patients with idiopathic CSVV (P=.03)., Conclusion: The incidence of LCV was higher than that reported in previously published studies. Idiopathic LCV was more common in our population-based cohort than that described previously. Overall survival was significantly poorer (P<.001) and should be explored further in future studies., (Copyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.)

Background: Most drug-eluting stents currently in use are coated with a polymer carrying the drug that is released for several weeks. However, a durable polymer may provoke hypersensitive reaction, delayed artery healing, and eventually stent thrombosis. The aim of this study was to investigate the safety and efficacy of a polymer-free paclitaxel-eluting stent (PF-PES) versus a polymer-based PES (PB-PES)., Methods and Results: Eligible patients undergoing percutaneous coronary intervention were randomized 1:1 to receive either PF-PES or PB-PES. The primary end point was late loss at 9 months. Intravascular ultrasound analysis at 9 months and final 2-year clinical follow-up were also performed. From October 2007 to April 2009, 164 patients were enrolled and randomized into 2 groups (PF-PES: n = 84; PB-PES: n = 80). Mean in-stent lumen loss was 0.90 ± 0.59 mm for PF-PES and 0.49 ± 0.52 mm for PB-PES (P < 0.001). Mean neointimal area by intravascular ultrasound was higher in PF-PES than in PB-PES (1.42 ± 1.09 versus 0.51 ± 0.61 mm(2); P < 0.001). At 2 years, a composite end point of all-cause death, any myocardial infarction, and target vessel revascularization occurred in 36.9% for PF-PES and 16.3% for PB-PES (P = 0.004), mainly driven by a higher rate of target vessel revascularization (PF-PES: 35.7%; PB-PES: 13.8%; P = 0.001). One late stent thrombosis was observed in PF-PES., Conclusions: Compared with PB-PES, PF-PES was associated with increased neointimal proliferation and subsequent clinical restenosis. Polymer plays an essential role in the performance of drug-eluting stents., Clinical Trial Registration Url: http://www.clinicaltrials.gov. Unique identifier: NCT01375855., (© 2014 American Heart Association, Inc.)

Objectives: Hypersensitivity vasculitis (HV) and Henoch-Schönlein purpura (HSP) are the most common entities included within the category of cutaneous vasculitis (CV). Palpable purpura and histological changes characterised by the presence of leukocytoclastic vasculitis are common in both conditions. Therefore, considerable overlap between them is often seen. It is especially true when the CV occurs in adults. To further establish clinical differences between these two conditions, in the present study we assessed the main clinical differences between HV and HSP in a wide and unselected series of adults with CV from a defined population., Methods: We reviewed the clinical records of 297 consecutive adults (age >20 years) seen at a single centre between January 1975 and December 2012 that were classified as having HSP or HV according to the criteria proposed by Michel et al. (J Rheumatol 1992; 19: 721-8)., Results: Based on the inclusion criteria, 102 adult patients (71 men/31 women) were classified as HSP and 195 (104 men/91 women) as HV. The mean age was similar in both groups (55.8±16.5 years in HSP and 56.8±18.3 years in HV). Precipitating events, usually an upper respiratory tract infection and/or drug intake, were more frequently observed in HV. Both at the beginning of the disease and when the CV was established clinical manifestations were more frequent in patients with HSP than in those with HV. It was the case for gastrointestinal (57.4% vs. 6.8%; p<0.001), joint (51.5% vs. 36.6%; p=0.01) and renal involvement (86.3% vs. 18.3%; p<0.001). Corticosteroid (56.7% vs. 22%; p<0.001) and cytotoxic drug (19.4% vs. 3.2%; p<0.001) use was also more common in patients with HSP. After a median follow-up of 15.5 (interquartile range- IQR; 3-37) months in HSP and 4 (IQR; 2-12) months in HV, the outcome was better in HV than in HSP. In this regard, complete recovery (72.6% vs. 85.4%; p=0.01) was more commonly observed in HV while residual renal involvement (15.3% vs. 4.2%; p<0.001) was more common in HSP. The disease relapsed in 35.3% of patients with HSP and in 24.4% with HV (p=0.07)., Conclusions: Our results confirm the claim that these two diseases presenting with similar cutaneous involvement are certainly two separate entities with greater systemic involvement and less favourable outcome in HSP.

Urticarial vasculitis (UV) is a subset of cutaneous vasculitis (CV), characterized clinically by urticarial skin lesions of more than 24 hours' duration and histologically by leukocytoclastic vasculitis. We assessed the frequency, clinical features, treatment, and outcome of a series of patients with UV. We conducted a retrospective study of patients with UV included in a large series of unselected patients with CV from a university hospital. Of 766 patients with CV, UV was diagnosed in 21 (2.7%; 9 male and 12 female patients; median age, 35 yr; range, 1-78 yr; interquartile range, 5-54 yr). Eight of the 21 cases were aged younger than 20 years old. Potential precipitating factors were upper respiratory tract infections and drugs (penicillin) (n = 4; in all cases in patients aged <20 yr), human immunodeficiency virus (HIV) infection (n = 1), and malignancy (n = 1). Besides urticarial lesions, other features such as palpable purpura (n = 7), arthralgia and/or arthritis (n = 13), abdominal pain (n = 2), nephropathy (n = 2), and peripheral neuropathy (n = 1) were observed. Hypocomplementemia (low C4) with low C1q was disclosed in 2 patients. Other abnormal laboratory findings were leukocytosis (n = 7), increased erythrocyte sedimentation rate (n = 6), anemia (n = 4), and antinuclear antibody positivity (n = 2). Treatment included corticosteroids (n = 12), antihistaminic drugs (n = 6), chloroquine (n = 4), nonsteroidal antiinflammatory drugs (n = 3), colchicine (n = 2), and azathioprine (n = 1). After a median follow-up of 10 months (interquartile range, 2-38 mo) recurrences were observed in 4 patients. Apart from 1 patient who died because of an underlying malignancy, the outcome was good with full recovery in the remaining patients. In conclusion, our results indicate that UV is rare but not exceptional. In children UV is often preceded by an upper respiratory tract infection. Urticarial lesions and joint manifestations are the most frequent clinical manifestation. Low complement serum levels are observed in a minority of cases. The prognosis is generally good, but depends on the underlying disease.

Cutaneous vasculitis may be associated with malignancies, and may behave as a paraneoplastic syndrome. This association has been reported in a variable proportion of patients depending on population selection. We conducted the current study to assess the frequency, clinical features, treatment, and outcome of paraneoplastic vasculitis in a large unselected series of 766 patients with cutaneous vasculitis diagnosed at a single university hospital. Sixteen patients (10 men and 6 women; mean age ± standard deviation, 67.94 ± 14.20 yr; range, 40-85 yr) presenting with cutaneous vasculitis were ultimately diagnosed as having an underlying malignancy. They constituted 3.80% of the 421 adult patients. There were 9 hematologic and 7 solid underlying malignancies. Skin lesions were the initial clinical presentation in all of them, and the median interval from the onset of cutaneous vasculitis to the diagnosis of the malignancy was 17 days (range, 8-50 d). The most frequent skin lesions were palpable purpura (15 patients). Other clinical manifestations included constitutional syndrome (10 patients) and arthralgia and/or arthritis (4 cases). Hematologic cytopenias (11 cases) as well as immature peripheral blood cells (6 cases) were frequently observed in the full blood cell count, especially in those with vasculitis associated with hematologic malignancies. Specific treatment for vasculitis was prescribed in 10 patients; nonsteroidal antiinflammatory drugs (4 patients), corticosteroids (3 patients), chloroquine (1 patient), antihistamines (1 patient), and cyclophosphamide (1 patient). Ten patients died due to the malignancy and 6 patients recovered following malignancy therapy. Patients with paraneoplastic vasculitis were older, more frequently had constitutional syndrome, and less frequently had organ damage due to the vasculitis than the remaining patients with cutaneous vasculitis. In summary, cutaneous paraneoplastic vasculitis is an entity not uncommonly encountered by clinicians. The most common underlying malignancy is generally hematologic. In these cases the presence of cytopenias and immature cells may be red flags for the diagnosis of cancer. In patients with paraneoplastic cutaneous vasculitis, the prognosis depends on the underlying neoplasia.

Introduction: Urticarial vasculitis is a subtype of vasculitis characterized clinically by urticarial lesions and histologically by necrotizing vasculitis., Objective: To study the clinical and histologic features of urticarial vasculitis in patients seen in the dermatology department of Hospital Universitario Virgen de Rocío in Seville, Spain, and to examine the association between hypocomplementemia and systemic disease., Material and Methods: We performed a chart review of histologically confirmed cases of urticarial vasculitis in the database of our department covering a period of 10 years., Results: Fifteen patients (9 women and 6 men with a median age of 51 years) were included. In 14 patients (93%), the lesions persisted for more than 24hours, and in 9 cases (60%) the lesions resolved leaving residual purpura or hyperpigmentation. Seven patients (47%) had low complement levels in the blood, 12 (80%) had extracutaneous symptoms, and 8 (53%) had associated systemic disease, the most common of which was systemic lupus erythematosus., Conclusions: Urticarial vasculitis may be underdiagnosed. Response to treatment is variable, and hypocomplementemia and extracutaneous symptoms may indicate the presence of associated systemic disease., (Copyright © 2010 Elsevier España, S.L. and AEDV. All rights reserved.)

Aims: To study the clinical and pathological features of cases of apparent solar purpura, with attention to the recently described phenomenon of inflammatory changes within otherwise typical lesions., Methods: We studied 95 cases diagnosed as solar purpura and identified 10 cases (10.5%) in which significant neutrophilic inflammation was present, potentially simulating a leukocytoclastic vasculitis or neutrophilic dermatosis. An additional three cases were identified in subsequent routine practice. The clinical features, including follow-up for subsequent development of vasculitis and histological features were studied., Results: In all cases the histological features were typical of solar purpura, with the exception of inflammatory changes, typically associated with clefting of elastotic stroma. Clinical follow-up information was available for all patients and none developed subsequent evidence of a cutaneous or systemic vasculitis or neutrophilic dermatosis., Conclusions: Inflammatory changes appear to be more frequent in solar purpura than is generally recognised. Awareness of this histological variation and correlation with the clinical findings and evolution is important in avoiding misdiagnosis.

The growth arrest-specific 6 (GAS6) gene product participates in platelet activation and granulocyte interaction with the endothelium. Our case-control study aimed to determine whether polymorphism of GAS6 was associated with predisposing risk or specific clinical manifestations of systemic lupus erythematosus (SLE). Two of the single-nucleotide polymorphisms (SNPs) of the GAS6 gene, GAS6 834 + 7G/A (rs8191974) and GAS6 +1332C/T (rs1803628), were investigated in 83 SLE patients and 89 non-lupus control subjects. We demonstrated that among lupus patients, the GAS6 +1332 T allele was more prevalent in patients with cutaneous vasculitis (allele T 41.7 % in patients with cutaneous vasculitis compared with 18.3 % in patients without vasculitis, odds ratio (OR) 3.2, 95 % confidence interval 1.3-8.0, p = 0.016). In conclusion, GAS6 polymorphism is positively associated with cutaneous vasculitis in SLE patients, which suggests that GAS6 polymorphism could be a genetic marker for SLE with cutaneous vasculitis.

Background: The main morbidity associated with Henoch-Schonlein purpura (HSP) results from glomerulonephritis and associated renal symptoms; thus early and accurate diagnosis would have follow-up implications., Objective: To determine the strength of association of IgA vascular deposition with HSP in a mostly adult and hospital-based population., Methods: We retrospectively analyzed patients with a histologic diagnosis of cutaneous leukocytoclastic vasculitis (LCV) and corresponding direct immunofluorescence (DIF) studies between 2000 and 2010. European League Against Rheumatism criteria were used to diagnose HSP cases clinically. They are purpura or petechiae with lower limb predominance plus at least one of the following: abdominal pain, LCV or proliferative glomerulonephritis with predominant IgA deposit, arthritis/arthralgia, or proteinuria or hematuria., Results: IgA positivity was significantly associated with HSP (P = .0001), and yielded sensitivity of 81% and specificity of 83%. Positive predictive value of IgA staining for HSP was 84%, and negative predictive value was 81%. No other histologic features were significantly associated with HSP., Limitations: Our sample size was relatively small and mostly an inpatient population (79%). We may have missed cases of HSP and a subset of IgA+ LCV patients who did not have clinical findings of HSP by limiting our series to only cases with DIF studies done. Certain clinical data may be incomplete since they were collected retrospectively., Conclusions: Our study confirms a strong association between IgA deposits on DIF and HSP, but also highlights a subset of HSP cases in which vascular IgA deposition in the skin appears to be negative., (Copyright © 2011 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

We present a case of the multicentric plasma cell variant of Castleman's disease (CD) with two rare manifestations. The patient consulted us because of cutaneous vasculitis of the lower limbs, while constitutional symptoms were nearly absent. Imaging studies also revealed pulmonary parenchymal involvement. Furthermore, our patient is the first case in whom association of ankylosing spondylitis with CD is reported. In addition, we present a review of the literature with emphasis on the clinical presentation of CD and its difficult discrimination from autoimmune and infectious disorders. An overview of the therapeutic options is also provided.

Background: Leukocytoclastic vasculitis (LCV) and necrolytic acral erythema (NAE) are skin disorders associated with hepatitis C virus (HCV) infection. However, they have not been found to occur simultaneously in the same patient., Objective: We sought to analyze the role of serum HCV-RNA levels and HCV genotype in the pathogenesis of both LCV and NAE in an attempt to assess whether these two parameters play a role in mutual exclusivity of LCV and NAE in the same patient., Methods: The study included 11 patients with LCV and 13 with NAE, all of whom were infected with HCV. All 24 patients were evaluated for the quantitative levels of HCV-RNA, using real-time polymerase chain reaction. HCV genotyping was performed on 10 patients in each group (N = 20)., Results: Patients with LCV had a higher prevalence of moderate and high levels of HCV-RNA viremia (P = .038) than those with NAE. However, there was no significant difference in HCV genotype between LCV and NAE groups (P = .211)., Limitations: Small number of cases is a limitation., Conclusion: Viral load seems to play a role in determining the response of the skin to HCV infection. High levels of HCV viremia were found to be significantly associated with LCV but not with NAE. HCV viremia may play a role in the development of LCV in HCV-infected patients., (Copyright 2009 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

Unlabelled: BACKGROUNDPapulopustular lesions (PPL) are commonly seen cutaneous lesions in Behçet's disease (BD). Some authorities do not recommend using cutaneous lesions for diagnostic criteria because these lesions are confused with acne vulgaris. Objective To understand if PPL have diagnostic value in BD., Methods: Biopsy was performed on papulopustular lesions of Behçet's patients for histopathological examination to compare with the control group composed of patients with acne vulgaris. Forty two patients with BD and 21 control patients with acne vulgaris were enrolled in the study. Histopathological findings were classified as leukocytoclastic vasculitis, lymphocytic vasculitis, superficial and/or deep perivascular infiltration, and folliculitis and/or perifolliculitis., Results: Of the specimens from 42 Behçet's disease patients, 7 (16.7%) revealed leukocytoclastic vasculitis and 3 (7.1%) had lymphocytic vasculitis, 15 (35.7%) had superficial perivascular and/or interstitial infiltration, 12 (28.6%) had superficial and deep perivascular and/or interstitial infiltration, 5 (11.9%) had folliculitis or perifolliculitis. Of the control group patients, 9 (42.9%) had folliculitis or perifolliculitis, 8 (38.1%) revealed superficial perivascular inflammation and 4 (19.0%) revealed mixed superficial and deep perivascular inflammation. Vasculitis was not encountered in any control group specimens. Vasculitis ratio was statistically higher in Behcet's disease patients (P = 0.016)., Conclusions: We evaluated more patients than previous related studies reported in the literature. Histopathological findings of vasculitis were encountered only in BD group. Non-vasculitis histopathological findings were also encountered in the control group patients. In conclusion, PPL can be used as a diagnostic criterion of BD subsequent to finding vasculitis in histopathological examination of the biopsy specimen of the PPL.

We assessed the dermatological manifestations associated with chronic hepatitis C virus (HCV) infection and their association with liver status. Of 155 patients with chronic HCV infection in Cairo, Egypt, 71 (45.8%) had dermatological manifestations: pruritus without evident skin lesions (21.3%), pigmented purpuric eruption (5.2%), aphthous ulcer and lichen planus (3.9% each), leukocytoclastic vasculitis (2.6%), psoriasis (1.9%), tinea versicolor (1.3%) and other conditions (5.8%). Shrunken liver, splenomegaly and ascites were significantly associated with the presence of skin lesions (relative risk 8.0, 2.7 and 1.8 respectively), and shrunken liver was significantly associated with pruritus (relative risk 2.1). Sex was not associated with any of the skin lesions.

Objective: To determine the clinical profile, etiologic factors, disease associations, and the laboratory profile of adult patients with cutaneous leukocytoclastic vasculitis (LV) presenting to the Dermatology Department at Farwaniya Hospital, Kuwait., Subjects and Methods: The medical records of 57 adult patients (>18 years) with biopsy-proven cutaneous LV from January 2004 to June 2006 at Farwaniya Hospital were reviewed., Results: Of the 57 patients studied, 21 (36.8%) were classified as having primary cutaneous small vessel vasculitis. The most common type of skin lesions found was palpable purpura seen in 29 (50.9%) patients. No possible cause for the cutaneous vasculitis could be identified in 21 patients. Extracutaneous involvement was seen in 32 (56.1%) patients of which 29 had joint involvement. The most frequent laboratory abnormality was an elevated erythrocyte sedimentation rate (ESR). Altered laboratory evaluation in the form of leukocytosis, raised antistreptolysin O titers and urine abnormalities were found in association with systemic vasculitis. IgA deposits were present in 33 patients. Risk factors for chronicity of cutaneous LV were female sex, advanced age, unidentified etiology, absence of fever and presence of joint involvement., Conclusion: The commonest type of LV in Kuwait is primary cutaneous small vessel vasculitis. The most frequent laboratory abnormality associated with LV was increased ESR., ((c) 2007 S. Karger AG, Basel.)

Object: We examined the prognosis of patients with onset of new primary renal vasculitis (PRV) in Miyazaki Prefecture., Patients and Methods: We enrolled and followed-up 56 patients (age, 70.4 +/- 10.9 years, mean +/- SD) with onset of new PRV between January 2000 and December 2004, for a median of 24 months. Patients with PRV were defined according to the EUVAS (European Systemic Vasculitis Study Group) criteria. Outcome and factors predicting unfavorable outcome of death were examined., Results: Among the patients, 25% (n=14) required dialysis therapy immediately at the start of immunosuppressive therapy and of these, renal function recovered in only 3 and 6 died during the first admission. On the other hand, 75% (n=42) did not require immediate dialysis, but 8 patients were introduced to dialysis therapy thereafter. At the end of follow-up, 26 (46%) had survived without dialysis, 10 (18%) were dependent on dialysis and 20 (36%) had died. Infection was the major cause of death (n=11) . The Cox proportional hazards model showed that the presence of lung lesions and immediate dialysis therapy conferred poorer survival rates (HR, 3.32, 95% CI, 1.14 to 9.71; HR 2.73, 95% CI, 1.03 to 7.23, respectively)., Conclusion: A poor survival rate is independently associated with the presence of lung lesions and advanced renal failure at the start of immunosuppressive therapy in patients with PRV. Half of the deaths were due to infection. Thus, PRV should be identified at an early stage and the treatment protocol should prevent infectious complications. These measures should improve the prognosis of patients with PRV.

A retrospective analysis was conducted on 93 adult patients with cutaneous leukocytoclastic vasculitis from St. Vincent's Hospital Melbourne to determine the classification, aetiology, severity and prognosis of this population of patients. We developed a new classification system for the purposes of our study based on modifications to the Chapel Hill Consensus Conference definitions for vasculitic syndromes. The results of our study indicate that an obvious cause was not found in 44.1% of patients. Of the patients with secondary vasculitis, the commonest causes were drugs and infections, accounting for a total of 40.9% of patients. Extracutaneous involvement was found in 39.8% of patients. Patients with symptoms resolving in less than 3 months accounted for 59.1% of the population, whereas 24.8% of patients had either symptoms lasting three or more months or evidence of recurrent symptomatology. There were 6 deaths (6.91%) and the rest were lost to follow up. The majority of patients in this retrospective series were classified as having hypersensitivity vasculitis, which is a relatively benign disorder limited mostly to skin with a low incidence of extracutaneous involvement (15.8%). Nevertheless, evidence of systemic involvement or sepsis need to be excluded as this may have important implications for patient treatment and outcome.

Silica and asbestos exposure are thought to belong to the triggering factors of antineutrophil cytoplasm antibodies (ANCA)-associated vasculitis. We carried out a study to find out whether patients with pulmonary involvement attributable to ANCA-associated vasculitis (AAV) have been exposed to silicon-containing materials. Thirty-one patients (12 women, 19 men, median age 51 years) were interviewed using a structured questionnaire. Occupational exposure to silicon-containing chemicals was reported by 22.6% of the patients (12.9% to SiO2, 9.7% to asbestos), compared with 0% of control subjects (p<0.05). Our findings support the pathophysiologic role of silica in AAV.

The article presents data on involvement of the nervous system of patients with medicamentous allergy characterized by allergic lesions of body vessels. Cerebral allergic vasculitis is often masked by other vascular conditions such as the following: atherosclerosis, high blood pressure, rheumatism and vegetovascular dystonia. The use of the reaction of a specific injury of basophilic leukocytes exposed to penicillin, streptomycin may be a diagnostic test in the determination of the damage of the nervous system in patients with medicamentous allergy.

Vasculitides are clinical syndromes characterized by vessel wall inflammation and resultant organ damage. Pathogenesis involves autoimmune reactions from type I to type IV. Vasculitis may be primary or secondary to infective and connective tissue disorders. The extent of damage may involve multiple organ systems or may remain limited to skin only. As the vasculitic syndromes display a multitude of variable presentations, there can be no uniform laid out guidelines or evaluation scale for the diagnosis of these conditions. High degree of suspicion, a detailed history regarding onset of disease and course of illness, meticulous physical examination, and appropriate laboratory tests to determine organ systems involved and the extent of involvement, lead to the diagnosis of specific syndrome. Anti-neutrophil cytoplasmic antibodies (ANCA), urinalysis, organ-specific biopsy and arteriography are the main diagnostic modalities for the diagnosis of vasculitis.

Unlabelled: Vasculitis can range in severity from a self-limited single-organ disorder to a life-threatening disease with the prospect of multiple-organ failure. This condition presents many challenges to the physician, including classification and diagnosis, appropriate laboratory workup, treatment, and the need for careful follow-up. The physician must not only be able to recognize vasculitis but also be able to provide a specific diagnosis (if possible) as well as recognize and treat any underlying etiologic condition. Most diagnostic criteria are based on the size of vessel involvement, which often correlates with specific dermatologic findings. This may allow the dermatologist to provide an initial diagnosis and direct the medical evaluation. This article reviews the classification and diagnosis of cutaneous vasculitic syndromes and current treatment options; it also presents a comprehensive approach to diagnosing and treating the patient with suspected cutaneous vasculitis. (J Am Acad Dermatol 2003;48:311-40.), Learning Objective: At the completion of this learning activity, participants should be familiar with the classification and clinical features of the various forms of cutaneous vasculitis. They should also have a rational approach to diagnosing and treating a patient with vasculitis.

The vasculitides are a heterogeneous group of diseases that are characterized by blood vessel inflammation and necrosis. They have a wide spectrum of manifestations due to the involvement of arteries and other vessels of various sizes and locations. Classification criteria are useful in improving our understanding of the epidemiology of these conditions but they are not diagnostic criteria. In recent years a progressive increase in incidence has been reported. Both giant cell arteritis and Wegener's granulomatosis are more common in the Northern hemisphere. Environmental factors have been implicated in the pathogenesis of these syndromes. Recent studies in patients with large and small-sized vasculitides support a genetic influence in disease susceptibility. They have confirmed the association of giant cell arteritis and Henoch-Schönlein purpura with HLA-DRB1 alleles. Moreover, the polymorphisms of other genes, such as interleukin-1 receptor antagonist, seem to be implicated in disease severity in patients with cutaneous vasculitis.

Many therapeutic agents, including recently introduced biologic response modifiers, can induce a variety of vasculitic manifestations ranging from small vessel hypersensitivity vasculitis and leukocytoclastic vasculitis to distinct vasculitic syndromes such as Wegener's granulomatosis, polyarteritis nodosa, and Churg Strauss syndrome. The pathogenic mechanisms remain to be defined and appear to be multifactorial, with cell-mediated and humural immune mechanisms playing important roles. Clinical presentation varies in severity from mild to severe and even fatal illness, can be self-limiting, or follows a more chronic protracted course. There are no significant differences in clinical presentation, serologic abnormalities, and pathologic findings with the idiopathic forms of vasculitis. However, it is extremely important to identify the offending drug because the discontinuation of the drug is often followed by a rapid improvement of the underlying vasculitic disorder.

Twenty-one cases of cutaneous vasculitis in small animals (dogs and cats) were reviewed, and cases were divided by clinical signs into five groups. An attempt was made to correlate clinical types of vasculitis with histological inflammatory patterns, response to therapeutic drugs and prognosis. Greater than 50% of the cases were idiopathic, whereas five were induced by rabies vaccine, two were associated with hypersensitivity to beef, one was associated with lymphosarcoma and two were associated with the administration of oral drugs (ivermectin and itraconazole). Only the cases of rabies vaccine-induced vasculitis in dogs had a consistent histological inflammatory pattern (mononuclear/nonleukocytoclastic) and were responsive to combination therapy with prednisone and pentoxifylline, or to prednisone alone. Most cases with neutrophilic or neutrophilic/eosinophilic inflammatory patterns histologically did not respond to pentoxifylline, but responded to sulfone/sulfonamide drugs, prednisone, or a combination of the two.

Objective: To examine the outcome and risk factors for renal sequelae in an unselected population of adults with Henoch-Schonlein Purpura (HSP)., Methods: Retrospective study of adult patients (> 20 years) with biopsy proved cutaneous vasculitis diagnosed as having HSP seen at a single center between 1984 and 1998. Patients were classified as having HSP according to proposed criteria. Only those patients with a followup of at least 1 year were included in this study of renal sequelae., Results: Twenty-eight patients with a mean followup of 5.5 years fulfilled the inclusion criteria. When the study was concluded, 10 patients (36%) had renal sequelae and 2 (7%) had renal insufficiency. Men outnumbered women. However, neither a previous history of drugs, gender, nor age at disease onset was associated with a higher risk of permanent renal involvement. Patients with hematuria at disease onset or renal involvement during the course of the disease more commonly developed renal sequelae (p < 0.001). The presence of anemia (p = 0.05) at the time of diagnosis and the onset in summer (p < 0.05) were also more common in those with permanent renal involvement (renal sequelae). Patients with relapses had also a higher trend to develop renal sequelae (p = 0.07). All patients who fulfilled more than 2 of these 5 risk factors developed permanent renal involvement. With this model we were able to predict renal sequelae in 8 of the 10 patients who had this complication. The Goodman-Kruskal gamma test value was 0.92 (95% CI 0.78-1.00)., Conclusion: In unselected adults with HSP, permanent renal involvement (renal sequelae) is not uncommon. Hematuria at disease onset and persistence of renal manifestations during the course of the disease are significant indicators of possible development of renal sequelae. These manifestations plus other features such as onset in summer, anemia at disease onset, or relapses of the disease may predict the development of renal sequelae in most patients.

Leucocytoclastic vasculitis is a small vessel inflammatory disease mediated mostly by deposition of immune complexes. Infections, medications, chemicals, bacteria, viruses, and diseases associated with immune complexes have been accused in the pathogenesis. Cutaneous leucocytoclastic vasculitis presents as palpable purpura most often localized in the lower extremities, often accompanied by abdominal pain, arthralgia and renal involvement. The clinical diagnosis of leucocytoclastic vasculitis is confirmed histopathologically by skin biopsy. In order to determine the cause of the disease, depending on the patient's history, complete blood cell count, blood cultures, cryoglobulins, serum protein electrophoresis, rheumatoid factor, antinuclear antibody, and autoantibodies to neutrophilic cytoplasmic antigens and complement should be checked. Once the diagnosis of leucocytoclastic vasculitis is made, emphasis should be on the search for an etiological factor and the identification of the involved organs. If possible, the underlying cause should be treated or removed, for example discontinuation of drugs. The prognosis depends on the disease that has the cutaneous leucocytoclastic angiitis as a component, as well as the severity of internal organ involvement. For example, a patient with cutaneous leucocytoclastic angiitis and moderate nephritis as component of Henoch-Schonlein purpura has a much better prognosis than a patient with these same findings as a component of Wegener's granulomatosis. Only if physicians recognize and report severe reactions to regulatory authorities and manufacturers, new drugs associated with a risk of such reactions can be identified.

Objective: To examine the incidence and clinical features of adults with biopsy proven cutaneous vasculitis (CV) associated with drugs., Methods: Retrospective study of an unselected population of adults (age >20 years) with biopsy proven leukocytoclastic CV from 1988 through 1997. Drug associated CV was considered if CV was confirmed by a skin biopsy and there was a history of drug use within one week before the development of CV. Drug associated CV was classified by American College of Rheumatology (ACR) criteria. To differentiate Henoch-Schonlein purpura (HSP) from hypersensitivity vasculitis (HV), the traditional criteria proposed by Michel, et al were used (J. Rheumatol. 1992;19:721-8)., Results: Thirty-three of 138 patients (23.9%) presenting with biopsy proven CV were diagnosed with drug associated CV. The annual incidence rate of biopsy proven drug associated CV in adults was 17.49 cases/million (95% CI 11.53-23.46): men 25.10/million (95% CI 14.85-35.36), women 10.31/million (95% CI 3.92-16.70). Antibiotics (n = 13), especially amoxicillin, and analgesics or nonsteroidal anti-inflammatory drugs (n = 11) were the drugs more commonly associated with CV. All adults with drug associated CV met the ACR classification criteria for HV or HSP. Based on Michel's criteria 26 patients (78.8%) were classified as having HV and 7 HSP. No patient met ACR criteria for other systemic vasculitides. Complete recovery with no sequelae was observed in most cases., Conclusion: Among patients with CV, drug associated disease is common and usually has a good clinical outcome.

The vasculitides constitute a heterogeneous group of diseases characterized by blood vessel inflammation and necrosis with different but frequently overlapping clinical and pathologic manifestations. The incidence of these conditions is frequently controversial. To further investigate the incidence and clinical manifestations of vasculitides, we reviewed the spectrum of these diseases in an unselected population of adults (age > 20 years) from northwestern Spain during a 10-year period. From January 1988 through December 1997, 267 adults were diagnosed as having vasculitis. The overall average annual incidence rate of vasculitis in the region of Lugo, Spain, between 1988 and 1997 for the population older than 20 years was 141.54/million. Primary vasculitis (115.04/million for the population older than 20 years; 81.3%), especially giant cell arteritis (GCA) was the most common group. Small vessel primary vasculitis (hypersensitivity vasculitis and Henoch-Schönlein purpura) was the second most common group. Both GCA and small vessel primary vasculitis had a good outcome. However, although less common, patients with medium and small vessel primary vasculitis, in particular those with polyarteritis nodosa, had a high mortality related to the systemic manifestations of the disease or to the immunosuppressive therapy. Among the group of adults with secondary vasculitis (26.51/million; 18.7%), rheumatic diseases and specifically those occurring in the context of rheumatoid arthritis were the most common group. Patients with secondary vasculitis had clinical or laboratory data that may suggest the presence of an underlying disease. In summary, systemic vasculitides are somewhat more common than previously considered. As in other western countries, GCA constitutes the most common type of vasculitis in northwestern Spain. Better physician awareness may contribute to the progressive increase in the recognition of these conditions.

Objectives: To assess the incidence and clinical features of adults with hypersensitivity vasculitis (HV) and Henoch-SchOnlein purpura (HSP) in a well-defined population., Methods: Retrospective study of an unselected population of adult patients (>20 years) with biopsy-proven cutaneous vasculitis diagnosed as having HV or HSP who presented at a primary hospital between 1988 and 1997. Patients with cutaneous vasculitis secondary to collagen vascular diseases, neoplasia, severe infections, and those with other well-defined clinical entities were excluded. Patients were classified as having either HV or HSP according to the criteria proposed by Michel et al (J Rheumatol 1992;19:721-28)., Results: Fifty-six adults (35 men/21 women), were classified as having HV and 27 adults as having HSP (19 men/8 women). The annual incidence rate for HV was 29.7/million and 14.3/million for HSP. At the onset of the disease, adults with HSP were younger than those with HV (46+/-18 years versus 59+/-18 years in HV; P = .005). Precipitating events were found in 50% of HV and in 30% of HSP patients. A history of drug therapy before the onset of vasculitis was found in 46% of HV and in 26% of HSP (P = .074). At disease onset, skin lesions were the most common manifestation in both groups. During the disease course, adults with HSP had joint manifestations more commonly (59% in HSP v25% in HV; P < .003) and more gastrointestinal (82% v 5% in HV; P < .001) and renal complications (48% v 5% in HV; P < .001). HSP subjects required more aggressive therapy consisting of steroids (P < .001) or cytotoxic agents (P < .001). After 37+/-28 (median, 31) months, complete recovery was observed in 98% of adults with HV. After 40+/-27 (median, 36) months, complete recovery was observed in only 67% of adults with HSP (P < .001). Renal insufficiency was observed in 8% of adults with HSP., Conclusions: In adults, HV and HSP as defined by these criteria, behave as two well-differentiated diseases. HV has a milder course and lack of severe complications, and HSP a higher risk of gastrointestinal and renal complications.

Objective: To study the clinical features and course of patients presenting to a skin referral centre with cutaneous vasculitis., Method: A retrospective review of patients presenting to the National Skin Centre from 1993 to 1995 with cutaneous vasculitis was done. All patients included in the study had histologically proven vasculitis on skin biopsy. The clinical manifestations and laboratory investigations of the patients were recorded. The response to the various drugs given as first line therapy and course of the disease 1 year after initial presentation was reviewed., Results: Forty-seven patients were included in this study. Females outnumbered males in a ratio of approximately 2:1 (32 females versus 15 males). The age of the patients ranged from 14 to 78 years, with a mean of 36 years. The aetiology remained elusive in 70% of cases. Of the known secondary causes, drugs and streptococcal infections were the most frequently implicated. The lower limbs were involved in more than 90% of cases. Cutaneous lesions took the form of palpable purpura, ulcers, nodules and urticaria. Extracutaneous manifestations were present in 47% of patients. The main extracutaneous manifestations were arthralgia/arthritis (21%), microscopic haematuria (16%) and abdominal pain (8%). Direct immunofluorescence on lesional skin was positive in 65% of cases. A raised erythrocyte sedimentation rate was observed in 40% of patients. Positive antinuclear antibodies were detected in 30% of cases. Most patients who were given systemic corticosteroids responded predictably well. The response to other modalities of treatment was more variable. At 1 year follow-up, complete remission was recorded in 47% of the patients, while in 53% of the patients, the disease continued to run a chronic relapsing course.

The study aimed to determine the prevalence of HBs-Ag and anti-HCV antibodies in a group of 43 patients with lichen planus and 19 patients with cutaneous vasculitis versus 30 controls. The results showed that 12 (27.9%) patients with lichen planus were positive for HBs-Ag, 9 (20.9%) were positive for anti-HCV antibodies and 3 (7%) were positive for both. In cutaneous vasculitis patients, 3 (15.8%) were HBs-Ag-positive, 7 (36.8%) were anti-HCV-positive and 3 (15.8%) were positive for both. In the control group, 8 (26.7%) were HBs-Ag positive, 3 (10%) were anti-HCV-positive and 1 (3.3%) was positive for both. These values were not statistically significant.

Objectives: To determine the prevalence and nature of cryoglobulins in a large series of patients with primary Sjögren's syndrome (SS) and identify the clinical and immunologic features related to their presence., Methods: In a cross-sectional study, we investigated 115 consecutive patients (107 women and eight men) with primary SS. All patients fulfilled four or more of the preliminary diagnostic criteria for SS proposed by the European Community Study Group in 1993. Serum cryoglobulinemia was measured in all patients. Serum samples were obtained at 37 degrees C, and cryoglobulinemia was estimated by centrifugation after incubation at 4 degrees C for 7 days. The type of cryoglobulinemia was identified by agarose gel electrophoresis and immunofixation., Results: Cryoglobulins were detected in the sera of 18 (16%) of our patients with primary SS; most were IgMkappa monoclonal/IgG polyclonal. When compared with patients without cryoglobulins, those with cryoglobulins presented a higher prevalence of leukocytoclastic cutaneous vasculitis (56% v8%, P < .001), hypocomplementemia (75% v 2%; P < 0.001) and antibodies to hepatitis C virus (HCV) (47% v8%, P < .001). Liver involvement (clinical signs, biochemical features, or ultrasound/histological data of liver disease) was present in all patients (100%) with cryoglobulins and HCV infection but in only 11% of patients with cryoglobulins without HCV infection (P < .001)., Conclusions: Leukocytoclastic cutaneous vasculitis, hypocomplementemia, and HCV infection are associated with the presence of cryoglobulins in the sera of patients with primary SS. Testing for HCV infection is recommended for patients with SS and cryoglobulinemia because of its high prevalence and its strong association with liver disease.

Objective: To examine the value of the American College of Rheumatology (ACR) 1990 criteria for Henoch-Schönlein purpura (HSP) and hypersensitivity vasculitis (HSV) and the Chapel Hill Consensus Conference (CHCC) definition of cutaneous leukocytoclastic angiitis (CLA) in the classification of cutaneous vasculitis and to estimate the annual incidence of biopsy proven cutaneous vasculitis in a well defined population., Methods: ACR criteria for HSP and HSV and the CHCC definition for CLA and HSP were applied to an unselected cohort of 84 patients with biopsy proven cutaneous vasculitis attending a single district hospital in the United Kingdom between January 1990 and December 1994., Results: Thirty-seven patients fulfilled ACR criteria for HSV, of whom 27 also fulfilled the ACR criteria for HSP. Thirty-two patients met the CHCC definition for CLA and 7 the CHCC definition for HSP. The overall annual incidence of cutaneous vasculitis was 38.6/million (95% CI 30.6-48.1), and for CLA 15.4/million (95% CI 10.6-21.8)., Conclusion: The ACR 1990 criteria for HSP and HSV overlap; the CHCC definitions for CLA and HSP provide clearer distinction. Cutaneous vasculitis is as common as systemic vasculitis.