Type Of Vasculitis Research Articles

Clinical and Demographic Features of 195 Pediatric Patients with Henoch-Schönlein Purpura, 12-Year Single Center Experience in Shiraz

Background: Henoch-Schönlein purpura (HSP) is one of the most common systemic types of vasculitis in children. Although it is a self-limited disease, life-threatening complications such as nephritis may occur. Early diagnosis and follow up might improve the long term outcome in renal involvement. There are few studies that have evaluated HSP in Iran. Objectives: The purpose of this study was to investigate demographic, laboratory data and clinical presentations of admitted HSP patients in a tertiary referral center, over a twelve-year period. Methods: This retrospective descriptive study evaluated 195 patients, diagnosed with HSP, who were admitted to Namazi Hospital in southwest of Iran (2006 - 2018). Demographic, clinical and laboratory findings, as well as treatment outcome of HSP patients were collected. Results: There were 118 males and 77 females with the mean age of 6.7 ± 3.21 years. About 70 (36%) patients showed common cold symptoms two weeks before HSP presentations. Admission course was 1 - 17 days (mean 4.55 ± 2.83) and autumn was recorded with the highest number of admitted patients (44.1%). In the course of hospitalization, 100% of the patients presented with palpable purpura, 61.02% with joint pain and 19.49% with abdominal pain. Moreover, 17.95% of the patients were noted with renal involvement. Laboratory data shows that more than half of patients (54%) had leukocytosis, only 9% of patients had positive CRP but all the patients had high erythrocyte sedimentation rate (ESR). Total of 43.1% of the patients received corticosteroids. Conclusions: The observed number of male patients with HSP was higher than females and the highest frequency of the HSP cases was observed in autumn. Joint pain and abdominal pain were the predominant clinical presentations, following skin purpura. The presented data can help with further HSP diagnosis and treatment plan.

The prevalence and impact of depression in primary systemic vasculitis: a systematic review and meta-analysis

ObjectiveTo describe the prevalence of depression among patients with primary systemic vasculitides (PSV); compare prevalence according to vasculitis type and against controls; and examine the impact of depression on PSV outcomes.MethodsWe searched Medline, PubMed, Scopus and Web of Science using a predefined protocol in accordance with PRISMA guidelines. We included all studies that reported the prevalence or impact of depression in PSV. We also included polymyalgia rheumatica (PMR) given its association with giant cell arteritis (GCA). Meta-analyses of prevalence estimates were performed using random-effects models and reported as percentages (95% confidence interval).ResultsWe reviewed a total of 15 studies that described the prevalence of depression, categorised into small (n = 10) and large vessel vasculitis (n = 7). Pooled prevalence estimate for depression in a small vessel (predominantly ANCA-associated) vasculitis was 28% (95% CI 20–38%) with significant heterogeneity (I2 = 93%). Depression prevalence in large-vessel vasculitis (Takayasu and GCA/PMR) was 24% (95% CI 17–34%), again with significant heterogeneity (I2 = 96%). One study reported 56% prevalence of depression in medium vessel disease. The prevalence of depression in small vessel vasculitis was higher than healthy controls. In these patients, depression and depressive symptoms were associated with poorer quality of life, adherence, and work disability, but not disease activity or damage.ConclusionDepression is highly prevalent among patients with primary systemic vasculitis and associated with poorer outcomes across a range of measures in studies of small vessel disease. Further studies are needed for depression in medium and large vessel vasculitides.

A rare case of acute onset vasculitis in pregnancy

Vasculitis in pregnancy is a rare condition. When a patient presents, it is usually a pregnant woman with a previous diagnosis of vasculitis. We present a case of vasculitis presenting for the first time during pregnancy with a remarkable recovery following the delivery. A 32 year old, previously healthy primi gravida presented at a gestation of 28 weeks and four days with a history of fever for three days. She developed a vasculitic type rash over both lower limbs. Investigations revealed urinary sediments, proteinuria, haematuria, and pulmonary consolidation, suggesting skin, renal and pulmonary involvement. Patient did not improve despite adequate treatment with antibiotics and antiviral treatment. Not only the investigations done to identify a possible aetiology leading to a secondary vasculitis but also the tests done to classify a primary vasculitis, became negative. Skin biopsy suggested leucocytoclastic small vessel vasculitis. Patient symptomatically improved with methylprednisolone pulse therapy and plasmapheresis. Termination of pregnancy was considered with a view of commencing cytotoxic therapy to prevent permanent pulmonary and renal injury. On day 18th of the illness, live baby was delivered by Caesarean section at a gestation of 31 weeks. Patient improved dramatically following the delivery. Patient became completely asymptomatic over few days, proteinuria and pulmonary fibrosis reversed in about one months’ time with steroids.

P0444SEASONALITY HAS NO ASSOCIATION WITH TIMING OF DIAGNOSIS OF RENAL ANCA ASSOCIATED VASCULITIS

Abstract Background and Aims Seasonal variation of ANCA associated vasculitis (AAV) and a possible link to extrinsic infective triggers is predominantly based on single centre epidemiological data. Despite frequent confounding factors including difficulty in identifying the precise time of disease onset, seasonality may be associated with the type of vasculitis and may impact the incidence of renal involvement. Therefore, the aim of this study was to explore if there is an association between seasonality, severity and incidence of biopsy-proven renal vasculitis in the Scottish population. Method Using the Scottish renal biopsy registry, we identified all adult native renal biopsies performed across Scotland between 2014 and 2018 with a diagnosis of AAV, including microscopic polyangiitis (MPA) and granulomatosis with polyangitis (GPA). Demographic data including ANCA antibody status, histological diagnosis, estimated glomerular filtration rate (eGFR) and proteinuria at presentation were recorded. Seasons were defined as autumn (September – November), winter (December-February), spring (March – May) and summer (June - August). Statistical analysis was performed using multivariate ANOVA analysis and Student’s t-test in parametric data. Results 339 cases of biopsy proven AAV were identified and included in the analysis. In this cohort, 53% were female with mean patient age of 65.6 years (± 13). Mean estimated glomerular filtration rate (eGFR) at the time of diagnosis of AAV was 32 (± 27.2) mL/min/1.73m2 and median urinary protein creatinine ratio (uPCR) was 146mg/mmol (IQR 79.8 – 271.3). Diagnosis of MPA n=209(62%) was more common than GPA n=130(38%) and patients with MPA were significantly younger at presentation (63.5 ± 13.6 ‘vs’ 67 ± 12.7 years, p = 0.017). Otherwise, these groups did not differ in mean eGFR (MPA 29.6 ± 25.7 ‘vs’ GPA 34.8 ± 27.6 mL/min/1.73m2) or median uPCR (MPA 147, IQR 78.6 – 286.5 ‘vs’ GPA 139, IQR 80.5 – 261 mg/mmol) at onset. We observed a mean of 3.5 (± 1) new cases of MPA and 2.1 (± 0.7) new cases of GPA per month, with no significant difference observed in month-to-month comparison. Seasonal analysis showed mean occurrence of 11.4 (± 4.5) cases of MPA in autumn, 11.2 (± 4.9) in winter, 10.6 (± 1.5) in spring and 8.6 (± 1.9) in summer months. In GPA, mean 6.6 (±2.7) cases occurred each autumn, 5.4 (± 3) in winter, 7.2 (± 2.9) in spring and 6.4 (± 0.9) in summer months. Overall, no significant differences in monthly or seasonal incidence across 5 years of monitoring were detected. Similarly, we observed no difference in renal function at presentation during different seasons for MPA (mean eGFR range 21.8 – 37.6 mL/min/1.73m2, uPCR median range 112 – 167.5 mg/mmol) or GPA (mean eGFR range 32-37 mL/min/1.73m2; uPCR median range 110 – 244 mg/mmol). Conclusion Our data suggest that there is no seasonal variation in the incidence of AAV diagnosed on kidney biopsy in patients living in Scotland. Additionally, patients present with similar levels of kidney function regardless of season. Thus traditional holiday periods i.e. Easter/Christmas do not seem to lead to a delay in diagnosis. This is the first study to consider seasonality in a complete national cohort and suggests that seasonal extrinsic factors do not play a major role in the pathogenesis leading to AAV onset.

AB0488 CAN A GCA RISK STRATIFICATION SCORE BE HELPFUL IN CLINICAL PRACTICE?

Background:Giant cell arteritis (GCA) is the most common type of large vessel vasculitis. Typically it presents in patients over the age of 50 with a combination of temporal headaches, scalp tenderness, jaw claudication, raised inflammatory markers and visual disturbance. The diagnosis of GCA is often challenging and there is a difficult balance of over and under investigation. There have been several proposed scoring systems to help clinicians risk stratify patients who may present with suspected GCA. One such scoring system, published in 2017, showed clinical utility in a large international multi-centre study. Following analysis by logistic regression on data from 530 biopsies, Ing et al. developed a parsimonious prediction model comprising 5 candidate criteria: age, jaw claudication, ischemia-related loss of visual acuity, platelet count and logCRP (Figure 1).[1]Objectives:Increasingly, ultrasound doppler imaging is recognised and accepted as satisfactory means of confirming the diagnosis of GCA, with the presence of the halo sign characteristic for GCA. The aim of our study was to determine whether this GCA prediction model accurately predicts positive temporal artery biopsies in a large, real world UK cohort. In addition, we assessed whether this model accurately predicts positive temporal artery ultrasounds.Methods:A retrospective cohort study was performed using electronic medical records of patients referred for temporal artery biopsy (TAB) and temporal artery ultrasound (USTA) for suspected GCA. All TAB performed at the Royal Wolverhampton NHS Trust between June 2014 - June 2018 and all USTA performed between January 2015 - January 2019 were analysed. Patients who undergo USTA for suspected GCA at our centre routinely have bilateral temporal and axillary arteries scanned. Patients were excluded if they already had a previous diagnosis of GCA (and the clinical question was suspected flare), or if there was insufficient information available.Results:The total number of patients who underwent a confirmatory diagnostic test (either TAB or USTA) for suspected GCA was 187. Thirteen of these patients met the exclusion criteria, the remaining 174 patients were included for analysis. 126/174 patients underwent a TAB, 63/174 had an USTA. 15/174 had both these were included in the USS cohort because for all these patients the ultrasound was the first diagnostic test performed (Table 1). Our results appear to closely mirror the original multi-centre results with regards to prediction of biopsy positive GCA, with the centiles closely following those in the inception cohort. 0% of the ‘low’ risk probability biopsy cohort were misclassified - none had a positive biopsy. However, 8% of the ‘low’ risk probability ultrasound cohort were misclassified - 2 had a positive ultrasound.Table 1.Investigation outcome summaryTotal number of patients who underwent TAB +/or USS TA for?GCA187 - 13 patients rejectedN = 174TAB = 111USS = 63Of these 15 patients hadbothUSS & TABPositive TAB =31 (28%)Negative TAB =80 (72%)Positive USS =24 (38%)Negative USS =39 (62%)Conclusion:Our study, highlights that a probability score for GCA derived from a large multi-centre cohort of patients who were biopsy positive, predicts ultrasound positivity with similar accuracy. Our work reveals that scoring systems are not infallible but can be helpful in guiding clinical decision making

P0823CHARACTERISATION OF HISTOLOGY PROVEN GLOMERULAR DISEASES IN THE MALTESE POPULATION: A NATIONAL CROSS-SECTIONAL SURVEY

Abstract Background and Aims The incidence and characterisation of glomerular diseases in Malta has not been investigated. This survey aims at determining the nationwide patterns, incidence and spectrum of biopsy-proven glomerular diseases. Method This is a retrospective analysis of all native kidney biopsy specimens performed in the adult population in Malta between January 2014 and December 2018. Demographic data and clinical variables were extracted from histopathology reports. Pathological diagnosis was based on findings obtained through light microscopy, immunofluorescence and electron microscopy. Results A total of 281 sufficiently sampled and conclusive native kidney biopsies were performed in Malta. The median age at biopsy was 52.9 years (interquartile range: 25), 58.7% were males and 93.1% were Maltese patients. The most common finding on biopsy was diabetic nephropathy (13.5%, n=38). Chronic kidney disease which was progressing faster than expected was the most common reason for performing a biopsy in these cases (65.8%, n=25/38). Ischaemic glomerulopathy was seen in 13.2% of cases (n=37). Podocytopathies accounted for 19.2% (n=54) of biopsies. Focal segmental glomerulosclerosis (FSGS) was seen in 12.8% (n=36); 58.3% (n=21/36) of which considered secondary to other conditions. Minimal change disease (MCD) accounted for 5.7% (n=16), nephrotic syndrome being the most common indication for biopsy (87.5%, n=14/16). Membranous nephropathy (MN) was identified in 11.0% of biopsies (n=31). Anti-Phospholipase A2 antibody status was available for 17 of these patients, 58.8% being positive (n=10/17). Immunoglobulin A nephropathy accounted for 11.7% (n=33). An active urinary sediment was the main indication for biopsy in 78.8% of these cases (n=26/33). Thin basement membrane was present in 2.1% (n=6). Lupus nephritis (LN) was present in 5.7% (n=16) of biopsies; class III LN in 50% (n=8/16), class IV LN in 37.5% (n=6/16); features of class V lupus were present in 31.3% (n=5/16). Only one biopsy showed isolated class V LN. Systemic involvement was present in the vast majority of patients with LN (81.3%, n=13/16). Pauci-immune glomerulonephritis was identified in 5.7% (n=16) of biopsies. Microscopic polyangiitis was the commonest type of pauci-immune ANCA-positive vasculitis (75.0%, n=12/16). Anti-glomerular basement membrane (GBM) disease accounted for only 1.1% (n=3). All patients with anti-GBM disease and 37.5% (n=6/16) of ANCA-positive vasculitis developed a rapidly progressive course requiring dialysis. Interstitial nephritis accounted for 5.7% (n=16) of biopsies; 31.2% (n=5/16) attributed to drugs. Haematological disease related glomerulonephritis accounted for 2.1% (n=6), infection related glomerulonephritis 1.1% (n=3), fibrillary glomerulonephritis 1.4% (n=4), membranoproliferative glomerulonephritis 0.7% (n=2) and other pathologies 6.4% (n=18). Conclusion This is the first nationwide study investigating the histopathological patterns and incidence of biopsy-proven glomerular diseases in Malta. The most common biopsy-proven glomerular disease is diabetic nephropathy, followed by focal segmental glomerulosclerosis. and ischemic glomerulopathy.

Ischemic Colitis in Buerger’s Disease: Case Presentation and Review

Buerger’s disease is a type of vasculitis that predominantly affects small to medium arteries and the veins of the upper and lower extremities. Intestinal vessels are rarely involved. This is a case report of a 38-year-old male, smoker, with known Buerger’s disease who was found to have ischemic colitis of the sigmoid colon on biopsy and inferior mesenteric artery occlusion on computed tomography (CT) angiography. Intestinal ischemia is a rare complication in Buerger’s disease. Patients may present with vague abdominal symptoms. Given the very low incidence of intestinal involvement, social history and clinical correlation are of chief importance for early detection. Smoking cessation is paramount, as it is the mainstay treatment of the underlying disease.

Checkpoint inhibitors related renal vasculitis and use of rituximab.

e15145 Background: As the percentage of cancer patients eligible for checkpoint inhibitor therapy (CPI) is increasing rapidly over the past couple years and approaching 45%, more cases of CPI related nephrotoxicity, including a rare subset with vasculitis, are reported. To learn more about the clinical presentation of CPI associated renal vasculitis, its mechanism, treatment options, and prognosis, we describe cases from our institution and reviewed the literature for related cases. Methods: We retrospectively reviewed the charts of all cancer patients from 2014 to 2020 who were diagnosed with CPI related nephrotoxicity and underwent a kidney biopsy. Results: We identified 6 cases of renal vasculitis reach with different malignancy, 4 had pauci-immune GN and one of which had IgA vasculitis. 4 of the patients were on nivolumab and 4 of the patients had negative ANCA serology. All patients had microscopic hematuria and severe acute kidney injury (AKI) with Creatinine > 4.5 mg/dl upon diagnosis and 2 patients requiring renal replacement therapy (RRT). One patient had concurrent lung involvement. All patients were treated by discontinuing CPI and initiating corticosteroids and rituximab. 3 patients received plasmapheresis who required RRT or had lung involvement. 2 patients died within 8 months of diagnosis due to malignancy progression and none of the cases had a relapse of vasculitis. Conclusions: We demonstrated that CPI can be associated with different types of renal vasculitis that is predominantly ANCA negative and manifest as severe kidney. Despite the lack of strong evidence, treatment as primary ANCA vasculitis with corticosteroids and rituximab is well tolerated with favorable renal outcome.

Unilateral cyanosis on tongue as an unusual appearance in emergency department: a case report

Introduction: Unilateral and bilateral tongue cyanosis usually occurs due to the Raynaud syndrome and in the underlying severe types of vasculitis and rheumatology.Case Presentation: The present study was conducted on a 54-year-old woman who referred to the emergency department with complaints of sudden and painless discoloration of the left half of the tongue. The patient had no history of disease other than diabetes controlled with glibenclamide. Clinical examination of the head and neck revealed evidence of unilateral cyanosis in the left half of the tongue without pain, whose discoloration did not improve with warming of the tongue.Conclusion: Cyanosis in the emergency department can be managed appropriately by considering some parameters including history taking, history of cyanosis occurrence, history of cardiopulmonary disease, cold sensitivity and history of rheumatologic diseases, presence or absence of nail clubbing, arterial blood oxygen saturation and arterial blood gas test results. These parameters can be effective in designing a treatment regimen, while differentiating the causes of central from peripheral cyanosis.

Case presentation of Behcet’s syndrome with neurological involvement

Vasculitis is a heterogeneous group of conditions with mostly non-specific etiology and diverse clinical presentation, defined by inflammation of the blood vessels, leading to ischemic damage to certain organs, including the nervous system. The etiology is multifactorial, with a genetic predetermination (the HLA-system is involved), and environmental factors. In almost all cases of vasculitis, there is a triggering factor, which is not always diagnosed and determined. There is no common diagnostic standard for each type of vasculitis. Important hallmarks for the diagnosis are age, sex, and ethnicity of the patient. The clinical presentation is diverse and in correlation to the extent, localization, and degree of the vascular involvement. Laboratory and roentgenology tests are done, aimed at finding the potential type of vasculitis and the expected specific involvement of the damaged organs, including the central nervous system. To quantify the extent of the progression of the condition, the Birmingham Vasculitis Activity scoring system is used. It is an aggregation of symptoms, which when used can help determine the changes and make a prognosis for the presentation of the patient. We present a case with an established and confirmed diagnosis of Behcet’s disease and the dynamics presented on the magnetic resonance imaging during several hospitalizations over a two-year period.

Utility of the Brief Illness Perception Questionnaire to Monitor Patient Beliefs in Systemic Vasculitis.

To assess the validity and clinical utility of the Brief Illness Perception Questionnaire (BIPQ) to measure illness perceptions in multiple forms of vasculitis. Patients with giant cell arteritis (GCA), Takayasu arteritis (TA), antineutrophil cytoplasmic antibody-associated vasculitis (AAV), and relapsing polychondritis (RP) were recruited into a prospective, observational cohort. Patients independently completed the BIPQ, Multidimensional Fatigue Inventory (MFI), Medical Outcomes Study 36-item Short Form survey (SF-36), and a patient global assessment (PtGA) at successive study visits. Physicians concurrently completed a physician global assessment (PGA) form. Illness perceptions, as assessed by the BIPQ, were compared to responses from the full-length Revised Illness Perception Questionnaire (IPQ-R) and to other clinical outcome measures. There were 196 patients (GCA = 47, TA = 47, RP = 56, AAV = 46) evaluated over 454 visits. Illness perception scores in each domain were comparable between the BIPQ and IPQ-R (3.28 vs 3.47, P = 0.22). Illness perceptions differed by type of vasculitis, with the highest perceived psychological burden of disease in RP. The BIPQ was significantly associated with all other patient-reported outcome measures (rho = |0.50-0.70|, P < 0.0001), but did not correlate with PGA (rho = 0.13, P = 0.13). A change in the BIPQ composite score of ≥ 7 over successive visits was associated with concomitant change in the PtGA. Change in the MFI and BIPQ scores significantly correlated over time (rho = 0.38, P = 0.0008). The BIPQ is an accurate and valid assessment tool to measure and monitor illness perceptions in patients with vasculitis. Use of the BIPQ as an outcome measure in clinical trials may provide complementary information to physician-based assessments.

P182 Prevalence and mortality of ANCA-associated vasculitis in England

Abstract Background The contemporary prevalence of ANCA-associated vasculitis (AAV) in England is unknown. Hospital Episode Statistics (HES) contain data on every hospital and day case NHS admission in England since 1997. In collaboration with the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS) we validated the diagnosis of AAV using ICD codes in HES. The positive predictive value of these codes was 86%, which compares favourably to a median coding accuracy of 80% in a recent systematic review of NHS coding studies. This justifies using this novel dataset for population-based epidemiology with coverage of the whole population of England. Methods We worked within NCARDRS enabled by their Section 251 legal permissions (CAG 10-02(d)/2015). We extracted all cases of AAV from HES 2011/12 to 2016/17 using ICD-10 codes M313 Granulomatosis with polyangiitis (GPA), M317 Microscopic polyangiitis (MPA), and M301 Eosinophilic granulomatosis with polyangiitis (EGPA). We used the Summary Care Record to check vital status and record date of death where appropriate. We estimated point prevalence on 1 July 2016 using ONS mid-year population estimates for England in 2016 as the denominator. Standardised mortality ratio (SMR) was calculated using the Office for National Statistics death summary tables 2016 to provide expected number of deaths for each 5-year age-band and sex. Results We identified 9,890 patients who were coded as having AAV during a hospital admission 2011-2017. This included 6,856 (69.3%) with GPA, 964 (9.8%) with MPA and 2,070 (20.9%) with EGPA. On 1 July 2016, our dataset found 8,040 people in England were living with ANCA associated vasculitis. We estimate the prevalence was 14.55 (95% CI: 14.23-14.87)/100,000 adult population. The median age of these patients was 65.3 years (interquartile range 52.3-74.2). 47% were female. The prevalence of GPA was 9.97/100.000 (95% CI: 9.71-10.24), MPA was 1.40/100,000 (95% CI: 1.30-1.50), and EGPA was 3.18/100,000 (95% CI: 3.03-3.33). People with AAV were 4.6 times more likely to die than the background population of the same age and sex (Standardised Mortality Ratio = 4.58). Conclusion There are no recent UK prevalence estimates for all types of ANCA-associated vasculitis. Studies in Australia, Germany, Southern Sweden and the USA have found estimated prevalence to be between 4.6-18.4 cases per 100,000 individuals. Our estimate of 14.6/100,000 in England is consistent with this, and towards the higher end of the range. However, our estimates underestimate the prevalence of MPA compared to other studies, and further work is needed to increase the routine identification of cases of MPA. Further work within NCARDRS using their unique data linkages will enable more specific AAV case ascertainment as well as nationwide population-based studies on cause of death and studies using the database of English prescriptions dispensed in the community. Disclosures F.A. Pearce None. B. Griffiths None. C. Mukhtyar None. R. Al-Jayoussi None. R.A. Watts None. J. Aston None. M. Bythell None. S. Stevens None. P.C. Lanyon None.

Do cytokines correlate with refractory Kawasaki disease in children?

BackgroundKawasaki disease (KD) is a type of pediatric vasculitis. Ten to twenty percent of children with KD do not respond to initial intravenous immunoglobulin (IVIG) treatment which called refractory Kawasaki disease. If untreated, approximately 15–25% of KD patients have complications. Therefore, it is important to predict whether KD is resistant to IVIG at an early stage. The aim of this study was to determine whether cytokines are predictors of refractory KD in children. MethodsWe retrospectively reviewed the medical records of 265 children diagnosed with KD who received IVIG within 10 days of fever onset at Beijing Children’s Hospital. Refractory Kawasaki disease was defined as persistent or recrudescent fever beyond 36 h after IVIG. Before IVIG and 3 days after temperature normalization following IVIG treatment, the concentrations of cytokines in the serum including interferon gamma (IFN-γ), tumor necrosis factor-alpha (TNF-α), interleukin-10 (IL-10), interleukin-6 (IL-6), interleukin-4 (IL-4), interleukin-2 (IL-2) and other conventional inflammatory mediators including white blood cells counts (WBC), C-reactive protein (CRP), erythrocyte sedimentation rate (ESR) and albumin were detected. The patients were divided into 2 groups: IVIG-sensitive group and refractory group. ResultsOf the 265 patients, 47 (17.7%) were refractory. After treatment with IVIG, the serum levels of IFN-γ, TNF-α, IL-10 and IL-6 in both groups were significantly lower than those before treatment (P < 0.05). Before treatment, the serum levels of IFN-γ, TNF-α, IL-10 and IL-6 in refractory group were significantly higher than those in IVIG-sensitive group (P < 0.05). There were no significant differences in IL-4 or IL-2 levels between the 2 groups. The results of logistic regression analysis showed that IFN-γ, IL-6, fever duration and serum albumin were independent risk factors for refractory KD. The area under the receiver operating characteristic curve (ROC curve) for IFN-γ was 0.781, and the cut-off value for refractory was 7.37 pg/ml, while the area under the ROC curve for IL-6 was 0.837, and the cut-off value for refractory was 70.13 pg/ml. ConclusionsIFN-γ and IL-6 were independent risk factors for refractory Kawasaki disease in children. KD patients with serum levels of IFN-γ above 7.37 pg/ml and IL-6 above 70.13 pg/ml before treatment were prone to refractory.

Measuring Vasculitis with Numbers: Outcome Scores.

Primary Systemic Vasculitides (PSV) are a heterogeneous group of diseases. Outcome scores are important to evaluate vasculitis patients in a more structured and standard way and these help physicians to predict patients with poor prognosis or high risk of relapse. Furthermore, we need reliable outcome measures for clinical trials. There are a number of vasculitis outcome scores available in the clinical practice with different strengths and limitations. These are mainly measures of disease activity, disease damage, response to treatment and quality of life. Birmingham Vasculitis Activity Score (BVAS) and its pediatric version aim to evaluate a wide scope of PSV. On the other hand, some outcome studies have focused on a single vasculitis type since the whole group includes different diseases with heterogeneous clinical features. The aim of this review is to provide an overview on outcome measures currently being used in the evaluation of patients with PSV. We mainly focus on immunoglobulin A vasculitis/Henochschönlein purpura, Kawasaki disease, anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, polyarteritis nodosa, Takayasu arteritis and Behçet's disease.

Management of urticarial vasculitis: A worldwide physician perspective

BackgroundUrticarial vasculitis (UV) is a rare type of leukocytoclastic vasculitis characterized by long lasting urticarial skin lesions and poor response to treatment. As of yet, no clinical guidelines, diagnostic criteria, or treatment algorithms exist, and the approaches to the diagnostic workup and treatment of UV patients may differ globally. We conducted an online survey to examine how UV patients are diagnosed and treated by international specialists and to reveal the greatest challenges in managing UV patients worldwide. MethodsDistribution of the questionnaire included an email to individuals in the World Allergy Organization (WAO) database, with no restrictions applied to the specialty, affiliation, or nationality of the participants (November 2018). The email contained a link (Internet address) to the online questionnaire. Responses were anonymous. The link to the questionnaire was further sent to the network of Urticaria Centers of Reference and Excellence (UCARE) in the Global Allergy and Asthma European Network (GA2LEN) as well as to the Turkish Dermatology Society and the Japanese Society of Allergology, who distributed the link to their members. In addition, the survey link was posted online in the group of the Russian Society of Allergologists and Immunologists. ResultsWe received 883 completed surveys from physicians in 92 countries. UV was reported to be rare in clinical practice, with an average of 5 patients per physician per year. More than two-thirds of physicians reported wheals, burning of the skin, and residual hyperpigmentation in 60–100% of UV patients. The most frequently reported reason for receiving referrals of patients with UV was to establish the diagnosis. The most important features for establishing the diagnosis of UV were wheals of longer than 24 hours duration (72%), the results of skin biopsy (63%), and post-inflammatory hyperpigmentation (46%). The most common tests ordered in UV patients were complete blood count, erythrocyte sedimentation rate, C-reactive protein, complement components, antinuclear antibodies, and skin biopsy. Physicians considered UV to be of unknown cause in most patients, and drugs and systemic lupus erythematosus to be the most common identifiable causes. Two of 3 physicians reported that they use second-generation antihistamines in standard dose as the first-line therapy in patients with UV. The greatest perceived challenges in the management of UV were the limited efficacy of drugs and the absence of clinical guidelines and treatment algorithms. ConclusionsUV is a challenging disease. Skin biopsy, a gold standard for UV diagnosis, is not performed by many physicians. This may lead to misdiagnosis of UV, for example, as chronic spontaneous urticaria, and to inadequate treatment. International consensus-based recommendations for the classification of UV and the diagnostic workup and treatment, as well as prospective studies evaluating potentially safe and effective drugs for the treatment of UV, are necessary.

Investigation of the frequency and clinical manifestations of Wegener vasculitis among other vasculitis patients in rheumatology ward of Vali-asr hospital during the past 10 years

Background and objective: Vasculitis is a rare disease and its prevalence varies according to age, race, time and geographical location. Due to the fact that no study has been conducted in Iran to investigate the frequency, clinical and laboratory symptoms of vasculitis in a considerable population of vasculitis patients, so the need for this study is felt. The aim of this study was to evaluate the frequency and clinical signs of granulomatosis with polyangitis (GP) patients who admited in rheumatology ward of Valiasr hospital in the last 10 years.Methods and material: In this study, all patients with a possible diagnosis of vasculitis in the rheumatology ward of Valiasr Hospital during the past 10 years were evaluated. Then the GP patients were evaluated for clinical, laboratory, radiological, pathological findins, outcome, mortality, and major complications. Results: During the last 10 years, 223 vasculitis patients have been admitted to Valiasr hospital. Of these, 87 % were primary vasculitis and 13% were secondary vasculitis. The highest frequncy was related to GP (21.52 %) followed by Behcet's disease. Women and men were included 52.08 % and 47.91 % of GP patients, respectively. Upper and lower airway involvement and totally ear, nose and throat (ENT) involvement were the most common clinical symptoms in GP patients.Conclusion: According to the finding of this study, GP vasculitis was the most common type of vasculitis that ENT involvements were the most common clinical symptoms in this patients. However, more studies are required to confirm these data in Iran.

Childhood-onset Takayasu Arteritis.

Childhood-onset Takayasu Arteritis (cTAK) is a rare, large-vessel type of vasculitis seen in children, mainly affecting the aorta and its major branches. Clinical manifestations are often severe and arise as a result of systemic and local inflammation, along with end-organ ischemia. Disease flares are common and the disease burden is high, with a significant rate of morbidity and mortality. Recent advances in understanding the underlying disease pathobiology resulted in the use of pathway-targeting agents, such as TNF- or IL-6 inhibitors with improved disease control. Nonetheless, the prognosis often remains guarded and the accrued damage is significant. This review aims at summarizing the recent evidence and observations regarding this condition, with a focus on pediatric publications.

Handgrip strength is a comorbidity marker in systemic necrotizing vasculitides and predicts the risk of fracture and serious adverse events.

Sarcopenia has been associated with poor outcomes in various medical and surgical conditions. However, its impact in systemic necrotizing vasculitides (SNV) had never been characterized. We aimed to assess the prevalence, associated factors and prognostic impact of sarcopenia in SNV. Patients with SNV were successively included in a prospective longitudinal study assessing comorbidities. At inclusion, we evaluated sarcopenia by assessing skeletal muscle mass index using DXA and muscle strength using handgrip strength. Vasculitis and treatments-related events were recorded and analysed using Cox models. One hundred and twenty patients were included. At inclusion, low handgrip strength (<30 kg for men and 20 kg for women) was identified in 28 (23%) patients, while no patient exhibited low skeletal muscle mass index (<7.23 kg/m2 for men and 5.67 kg/m2 for women). Low handgrip strength was associated with age (P<0.0001), type of vasculitis (P=0.01), vasculitis damage index (P=0.01), history of falls (P=0.0002), osteoporosis (P=0.04), low serum albumin (P=0.003) and prealbumin (P=0.0007), high CRP (P=0.001), high FRAX® tool (P=0.002) and low bone mineral density at femoral neck (P=0.0002). After median follow-up of 42 months, low handgrip strength was associated with higher risk of bone fracture [HR 4.25 (1.37-13.2), P=0.01] and serious adverse events [HR 2.80 (1.35-5.81), P=0.006]. Handgrip strength is associated in SNV with nutritional status and comorbidities such as bone disease, and seems to predict, as in other medical conditions, the risk of fracture and serious adverse events during follow-up. In contrast, assessment of skeletal muscle mass index in this population remains uncertain.

Mesenchymal stem cell and hematopoietic stem cell transplantation for vasculitis

Vasculitis poses a great therapeutic challenge to the treating physician. Steroids remain the mainstay of the treatment and biologics are emerging as popular agents. Similar to other immune-mediated diseases, refractory vasculitis seems to be a suitable candidate for bone marrow and mesenchymal stem cell transplantation. Preliminary data show stem cell-based therapies are feasible and effective in severe, treatment-resistant forms of several types of vasculitis. However, due to the fact that patients are rare, clinical data are sparse. Further prospective studies are needed to confirm these preliminary results. In addition, mostly partial remissions are achieved after stem cell transplantation; thus optimization of the therapeutical regimens is necessary through international cooperation.

Púrpura Henoch-Schönlein bullosa en pediatría. Reporte de caso

Introducción. La púrpura Henoch-Schönlein (PHS) es la forma más común de vasculitis en la infancia; esta se da en pequeños vasos sanguíneos y no es frecuente que genere complicaciones graves como la vasculitis bullosa, tal como sucedió en el caso que se presenta a continuación.Presentación del caso. Paciente masculino de cinco años con un cuadro clínico consistente en artralgias y aparición de lesiones purpúricas en miembros inferiores. Luego de ser diagnosticado con PHS, presentó lesiones bullosas, por lo que fue hospitalizado y se inició manejo analgésico y tópico; durante su estadía en el hospital se vigiló su función renal y, ya que no presentó otras complicaciones, se dio de alta.Conclusión. Las publicaciones disponibles sobre PHS sugieren que su presentación cutánea bullosa en pediatría no es frecuente y que no siempre se relaciona con un compromiso renal y/o gastrointestinal como la variante clásica; sin embargo, siempre debe vigilarse la función de estos sistemas sin importar la gravedad dérmica de esta vasculitis.