Search

Background: Ultrasonographic evaluation of fetal thymus size may be used to predict the adverse perinatal outcome in pregnant women with vasculitis., Aim: To compare fetal thymus size in pregnant women with vasculitis and healthy pregnant women and to evaluate whether fetal thymus size predicts the adverse perinatal outcome., Methods: Twenty-two pregnant women with previously diagnosed vasculitis, 18 of them with Behçet's disease, three with Takayasu arteritis, and one with Wegener's granulomatosis, were included in the case group. The control group comprised 66 healthy pregnant women whose gestational ages matched the case group. Thymic thoracic ratio (TTR) was measured to assess fetal thymus size in the view of three vessels and trachea., Results: In the case group, fetal TTR was significantly lower (0.32 ± 0.03 vs. 0.36 ± 0.02, p = < 0.001). Fetal TTR was significantly lower in those using prednisone than those not (p = .001) in the case group. There was no significant difference in fetal TTR between colchicine used and not used (p = .078) in the case group. Also, for the TTR, a sensitivity of 100% and a specificity of 92% were achieved with a cut-off value of 0.33 for predicting adverse perinatal outcomes., Conclusion: The fetuses of pregnant women with maternal vasculitis had a smaller TTR. The small fetal thymus may alert clinicians to possible adverse perinatal outcomes and, with other supporting risk factors, may help predict adverse perinatal outcomes in pregnant women with vasculitis., Competing Interests: Declaration of Competing Interests The authors declare that they have no conflict of interest., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Clinical and autopsy features in three cases of Wegener's granulomatosis are presented. The disease is characterized by the triad of necrotizing granulomatous lesions of the respiratory tract, segmental necrotizing angiitis of arteries and veins, and necrotizing glomerulitis commonly terminating in uremia. Correct antemortem diagnosis was made in two of the three cases-in one by recognition of the characteristic triad and in the other by a biopsy from the antrum. Pathological lesions were chiefly in respiratory tract, spleen, kidneys and blood vessels. Necrotizing granulomatous lesions were present in the lungs. Glomerular lesions were particularly severe and wide-spread in two of the three patients, leading to uremia. Trabeculitis of the spleen, an unusual lesion, was a noticeable feature in all three. Fibrinoid necrosis, with little or no inflammatory infiltration, was the prevailing type of vascular necrosis. The characteristics of the disease process suggest a hypersensitivity mechanism. Similarities to, but significant differences from, polyarteritis nodosa are recognized.

Objective Von Willebrand factor (VWF) antigen plays a role in vascular inflammation and thrombosis, both of which are important in the pathogenesis of ANCA-associated vasculitis (AAV). Previous work found that VWF correlates with disease activity in childhood-onset primary CNS vasculitis. We sought to determine the relationship between VWF and disease activity over time in children with AAV. Methods AAV patients with more than one VWF level measured were included in this retrospective study, and the relationships between active vasculitis, VWF and other disease measures were analysed. Generalized estimating equations analysis was used to account for repeated VWF measurements within a patient. Repeated measures correlation was used to determine associations of paired laboratory observations. Diagnostic performance was evaluated using receiver operating curve analysis. Results A total of 732 total VWF measurements were collected in 33 AAV patients. VWF antigen levels were higher during active disease [median 2.03 IU/ml, interquartile range (IQR) 1.35, 2.55] compared with inactive disease (median 1.18 IU/ml, IQR 0.94, 1.53). VWF antigen was the only variable that was significantly associated with active disease (odds ratio 3.01, P  < 0.001, 95% CI 2.3, 3.93). The effect of VWF did not show a substantial difference between the disease subtypes. There was a moderate positive correlation between VWF antigen and disease activity, with an acceptable sensitivity and specificity rates. Conclusion Increased VWF antigen levels correlate with active vasculitis in this paediatric-onset AAV cohort and may be used as an additional biomarker in childhood AAV. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Here, we report a case of granulomatosis with polyangiitis presenting with unilateral parotid gland enlargement and later developed skin lesions on the lower extremities and abdomen. Although rare, salivary gland enlargement may be the presenting sign of Wegener's granulomatosis or other ANCA associated vasculitides. [ABSTRACT FROM AUTHOR]

Wegener's granulomatosis or Granulomatosis with polyangiitis is a rare multisystem autoimmune vasculitis disease which may become life threatening due to the various system involvement. Granular hyperplastic gingivitis, the so called "strawberry gingivitis" is a rare manifestation of this disease, yet pathognomic when present. Early diagnosis and treatment is very important. [ABSTRACT FROM AUTHOR]

Key Clinical Message Here, we report a case of granulomatosis with polyangiitis presenting with unilateral parotid gland enlargement and later developed skin lesions on the lower extremities and abdomen. Although rare, salivary gland enlargement may be the presenting sign of Wegener's granulomatosis or other ANCA associated vasculitides.

Wegener's granulomatosis is a necrotising vasculitis affecting both arterioles and venules. The classical triad involves acute inflammation of upper airway along with inflammation of lower respiratory tract and renal involvement, however other organ system may also be affected. Our patient presented with severe unilateral earache, ear discharge, hearing loss and ipsilateral facial nerve palsy as the manifestations of the disease, which are rarely reported in medical literature1. [ABSTRACT FROM AUTHOR]

Abstract Background Granulomatosis with polyangiitis (GPA) or Wegener’s granulomatosis is an autoimmune disorder with a wide spectrum of manifestations that mostly primarily presents with respiratory symptoms such as cough, dyspnea, and hemoptysis and leads to a high mortality rate if left untreated. It is a relatively uncommon condition, characterized by necrotizing granulomatous vasculitis of small- and medium-sized vessels. Recent studies have shown that hyperactivation of immune cells in patients with the coronavirus disease 2019 (COVID-19) leads to elevated levels of various autoantibodies and inflammatory cytokines including interferon-gamma (IFN-γ) and tumor necrosis factor-α (TNF-α). There are the same factors that involve in the pathogenesis of autoimmune diseases such as GPA. Case presentation While there have been several reported cases of COVID-19 occurring in patients receiving immunosuppressant treatment for GPA, here we report a case of a 72-year-old woman with a history of coronavirus disease 2019 (COVID-19) who suddenly suffered unilateral vision and hearing loss and peripheral facial palsy on the same side. Chest computed tomography (CT) demonstrated a subpleural consolidation in the inferior lobe of the left lung. Based on the radiology report, chest CT evidence was due to a history of COVID-19 pneumonia. CT scans of the paranasal sinus showed pansinusitis and necrosis of the nasal septum. According to the available evidence, mucormycosis was clinically suspected, and the patient underwent endoscopic sinus surgery. Eventually, the histopathological analysis revealed a diagnosis of Wegener’s granulomatosis. Conclusions Since GPA and its complications can be prevented only through strong clinical suspicion and early diagnosis, our presentation of this case aims to increase awareness of autoimmune diseases in COVID-19 patients even after recovery.

Abstract Background Granulomatosis with polyangiitis (GPA) is a rare systemic vasculitis. Its severity ranges from indolent disease to fulminant that may cause death. With treatment, remission is seen in more than 80% of cases, although relapse is still common. There have been studies showing that there may be factors to predict relapse in GPA. Based on relapses, the decision to start treatment and/or to monitor the patients more closely is made. Therefore, predicting the relapse of GPA can be effective in controlling the disease. Our aim was to investigate possible factors for relapse in GPA. We recruited 254 patients diagnosed with GPA who were under treatment at Alzahra hospital affiliated to Isfahan University of Medical Sciences (from 2013 to 2020) and Amir Alam Hospital affiliated to Tehran University of Medical Sciences (from 2020 to 2022) to plan a retrospective study. Chi-squared or Fisher’s exact tests were performed to compare categorical variables, while the Mann–Whitney U test was used to compare continuous variables. Results Analysis of our patients (aged 20–80,122 females) showed that 147 patients (57.9%) experienced relapse. Mean age in relapse group was 43.8 ± 16.6 and in no-relapse group was 45.6 ± 15.1 (P > 0.05). Among many potential predictors, we observed in multivariate analysis that positive PR3-ANCA (proteinase 3-antineutrophilic cytoplasmic antibodies) (P = 0.007, OR:2.62,CI:1.29–5.31),nose manifestations (P = 0.004, OR 3.00, CI 1.43–6.26), mucosal membranes involvement (P = 0.009, OR 4.21, CI 1.43–12.38), and gastrointestinal tract (GI) complications (P = 0.03, OR 5.64, CI 1.14–27.90) were significant predictors of GPA relapse. Conclusion Among clinical and laboratory features we studied, positive PR3-ANCA, nose manifestations, mucosal membranes involvement, and GI complications were independent predictors of relapse in patients with GPA.

ABSTRACT: Granulomatosis with polyangiitis formerly known as Wegener's granulomatosis was first described by German pathologist Friedrich Wegener in 1936. It is a multi-system necrotizing noncaseating granulomatous vasculitis which affects small to medium-sized vessels. It can involve any organ system, most commonly the lungs and kidneys. American College of Rheumatology requires 2 of 4 criteria for diagnosis: Positive biopsy for granulomatous vasculitis, urinary sediment with red blood cells, abnormal chest radiograph and oral/nasal inflammation. Here we present a case of Granulomatosis with polyangiitis with brief review of literature.

Granulomatosis with Polyangiitis (GPA) is an uncommon condition belongs to the group of ANCA-associated necrotizing vasculitides. It is characterized by necrotizing granulomatosis of upper and lower respiratory system with coexisting glomerulonephritis. Its signs and symptoms are largely varied due to a wide spectrum of involvement sites. In the present case report, we describe a case of 26-year-old female presented to our centre with history of bilateral ear discharge, earache, and dry cough with a deviated mouth angle to left. Diagnostic work up was done and patient was treated with pulse methylprednisolone, plasmapheresis, and rituximab. Timely diagnosis and prompt initiation of the treatment has prevented the GPA progression and helped the patient to improve quickly. Currently, the patient is doing well and is in remission. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis is a rare disease, with small vessel vasculitis leading to progressive damage of vital organs. The mainstay of treatment is immunosupressive therapy. Exacerbations of the disease are prevalent and damages of tissues may result in organs failures. In our paper we present a case of a patient, who was qualified to biologic therapy with rituximab due to ongoing ear-nose-throat (ENT) symptoms worsening. Remission of rhinolaryngologic complaints was not met despite rituximab treatment, however significant improvement in hearing occurred. [ABSTRACT FROM AUTHOR]

Objective: The concept of otitis media with ANCA-associated vasculitis (OMAAV) was recently proposed by the study group of the Japan Otological Society. However, little is known about the effect of ear involvement on the clinical features and prognosis of AAV. We investigate this issue in this study.Methods: We retrospectively examined 36 patients diagnosed with OMAAV and 44 patients diagnosed with AAV without ear involvement (non-OMAAV) at Ehime University Hospital from 2013 to 2018. We collected serological findings including ANCA type and titer, C-reactive protein (CRP), serum creatinine level, organ involved at initial diagnosis, treatment, remission, disease relapse, and mortality from medical records. We investigated whether clinical features and outcomes differed between the OMAAV and non-OMAAV groups.Results: Age, ANCA titer, and CRP at initial diagnosis were not significantly different between the two groups, and the rate of intravenous cyclophosphamide (IVCY) use also did not differ. The proportions of patients with concurrent eye involvement, facial palsy (FP), and hypertrophic pachymeningitis (HCP) were significantly higher in the OMAAV than in the non-OMAAV group (p = 0.005, 0.005 and 0.049, respectively), while both renal and peripheral nerve involvement were significantly less common in OMAAV patients (p = 0.04). Among the 30 patients with renal involvement, serum creatinine level at diagnosis was significantly lower in the OMAAV group (p = 0.04). The mortality rate was 8.3% in OMAAV and 6.8% in non-OMAAV cases, but this difference was not significant. The rate of relapse was 33.3% in OMAAV and 13.6% in non-OMAAV cases; this difference was significant (p = 0.04).Conclusions: Serological measurements of disease activity did not differ between the groups. Eye involvement, FP, and HCP, however, were significantly more common in AAV with ear involvement. In addition, renal involvement was less common and renal impairment was milder in AAV with ear involvement. These findings can be considered clinical features. The relapse rate was significantly higher in AAV with ear involvement. [ABSTRACT FROM AUTHOR]

Wegener's granulomatosis or granulomatosis with polyangiitis (GPA) is multisystemic vasculitis. Kidney involvement in GPA often presents with rapidly progressive renal failure and requires urgent treatment. A 60-year-old female presented with prolonged history of fever, generalized weakness, decreased appetite, and weight loss over 4 months. Her renal function was normal; urine culture was sterile. On further evaluation, she was found to have large, hypodense solid lesion in mid pole of the right kidney on CECT. CT guided renal biopsy was done, which showed granulomatous interstitial nephritis with focal crescents. On further evaluation, she was found to have high titers of anti-MPO antibody. She was started on steroid and methotrexate with subsidence of fever. Follow-up after 12 months showed resolution of the lesion. GPA solely presenting as solid mass like lesion in the kidney is extremely rare presentation. Early diagnosis and prompt initiation of the treatment can prevent the progression of the disease. [ABSTRACT FROM AUTHOR]

Wegener's granulomatosis is a vasculitis that affects small to medium blood vessels, generally occurring at the age of 64-75 years with an incidence of 8-10 / 1,000,000 people. Meningoencephalitis manifestations are rarely found in Wegener’s granulomatosis with the incidence only in the range of 0-7%. Anti-neutrophil cytoplasmic antibody (ANCA) is often used in diagnosing Wegener’s granulomatosis, but negative ANCA can be found in 10-20% of cases. A 30-year-old man came with a decrease in consciousness. His anamnesis and physical examination showed signs that led to Wegener’s granulomatosis, despite negative ANCA examination results. After getting therapy according to the management of the Wegener’s granulomatosis there is a clinical improvement in the patient. This case is raised because it is a rare case and needs sharpness in establishing a diagnosis. Wegener's granulomatosis can cause very bad progress, but if handled properly, complete remission can be achieved.

Abstract Background ANCA-associated vasculitis is a life-threatening, systemic autoimmune disease. There is an increased risk of organ infarction but in many cases this is asymptomatic. We described here the first reported case of PR3 vasculitis presenting with symptomatic bilateral renal wedge infarction. Case presentation A 19-year old Caucasian woman with no past medical history presented on a number of occasions over a number of weeks with progressively more severe back pain, fevers and arthralgia. On the final presentation she was noted to have developed splinter haemorrhages and her blood tests revealed impaired renal function along with elevated inflammatory markers. She was subsequently found to have high titres of serum PR3 antibodies and focal necrotising glomerulonephritis on renal biopsy, consistent with a diagnosis of PR3 ANCA-associated vasculitis. Cross-sectional imaging revealed multiple wedge infarcts of her spleen and both kidneys, confirmed on contrast-enhanced ultrasound. Large vessel, cardiac and thrombophilic causes of thromboembolism were excluded. She was treated with high-dose corticosteroids and CD20 monoclonal antibodies (rituximab) and at time of writing, 4 months after initial presentation, has entered clinical remission. Conclusions Here we describe the first reported case of PR3 vasculitis presenting with symptomatic renal wedge infarction. In patients with vasculitis who present with flank or back pain, infarction of abdominal organs should be considered in the differential. Both splenic and renal infarctions are likely underdiagnosed in the setting of ANCA-associated vasculitis but may have clinical impact in contributing to infection risk and the degree or renal recovery, respectively.

Objectives This study aimed to examine the sensitivity of muscle biopsy (MB) in ANCA-associated vasculitis (AAV), identify factors predicting MB positivity and assess the prognostic value of a positive MB. Methods We conducted a single-centre retrospective study of AAV with an MB performed at diagnosis. AAV classification [granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA)] followed the European Medicines Agency algorithm. A logistic regression model was used to identify the factors associated with MB positivity. Survival curves were generated using the Kaplan–Meier method. Results Among 276 AAV patients (1995–2018), 101 had an MB. Seventy-eight patients were included: 33 with GPA, 25 with MPA and 20 with EGPA. MB samples were positive in 45 cases (58%): 17 GPA, 16 MPA and 12 EGPA. Univariate analysis focussed on GPA and MPA, revealed that the MB yield was higher in females [22/31 (71%) vs 11/27 (41%); P  = 0.02] and in anti-MPO patients [25/37 (68%) vs 6/19 (32%) for anti-PR3; P  = 0.01]. By multivariate analysis, three factors predicted MB positivity: anti-MPO ANCA [odds ratio (OR) 10.67 (CI 2.09, 81.68)], female sex [OR 5.3 (CI 1.16, 32.35)] and neutrophil count [OR 1.33 (CI 1.07, 1.8)]. MB positivity had no impact on relapse, death or end-stage renal disease–free survival. Conclusions MB is a safe and efficient diagnostic tool for AAV. Predictors of MB yield include ANCA type, sex and neutrophil count. MB cannot substitute for kidney biopsy when indicated, but should be considered in other cases. [ABSTRACT FROM AUTHOR]

Because of a similar organ involvement and histopathological features, IgG4-related disease (IgG4-RD) may mimic some forms of granulomatosis with polyangiitis (GPA). However, several cases of clear coexistence or overlap of both diseases have been reported. We describe a case of a 47-year-old man presenting with a renal mass and a nasal crusting showing histopathological features of IgG4-RD in both territories. Cytoplasmic/proteinase 3 (PR3) antineutrophil cytoplasmic antibodies (ANCA) were positive and the patient subsequently developed kidney failure and nephritic syndrome that led to a renal biopsy re-evaluation revealing changes compatible with segmental necrotising glomerulonephritis and GPA. Remission induction therapy with prednisone and rituximab was started and clinical and laboratory parameters returned to normal. After administering a maintenance regimen based in rituximab 500 mg every six month the patient remained asymptomatic during 4 years of follow-up and free of prednisone the last 18 months. Although coexistence or overlap of GPA and IgG4-RD may be established in some clinical scenarios, the possibility of widening the spectrum of a single disease is also postulated. [ABSTRACT FROM AUTHOR]

Abstract Background Granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration. Case presentation We describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Our patient initially presented with localised ENT involvement, but despite treatment with methotrexate, she deteriorated. Granulomatous inflammatory tissue around the skull base resulted in cavernous sinus syndrome, facial nerve palsy, palsies of cranial nerves IX-XII (Collet-Sicard syndrome), and the rare complication of cranial diabetes insipidus due to pituitary infiltration. The glossopharyngeal, vagus and accessory nerve palsies resulted in severe dysphagia and she required nasogastric tube feeding. Her neurological deficits substantially improved with treatment including high dose corticosteroid, cyclophosphamide and rituximab. Conclusions This case emphasises that serious morbidity can arise from localised cranial Wegener’s granulomatosis in the absence of systemic disease. In such cases intensive induction immunosuppression is required. Analysis of previously reported cases of pituitary involvement in GPA reveals that this rare complication predominantly affects female patients.

The association of granulomatosis with polyangiitis and pregnancy is rare and therapeutic options are limited by the risk of teratogenicity and fetotoxicity. There is a paucity of published literature to guide clinical decision-making in these cases. We report the case of a 26-year-old woman with no medical history who presented at 21 weeks of gestation with a bilateral sudden loss of hearing and erosive rhinitis. The diagnosis of granulomatosis with polyangiitis was confirmed radiologically and biologically. Corticosteroids were not enough to stabilize the disease and she received intravenous immunoglobulins with remission. A successful delivery of a healthy male newborn was done at 36 weeks. A review of all published literature on granulomatosis with polyangiitis in pregnancy between 1970 and 2017 is presented. Trial registration : Not applicable. [ABSTRACT FROM AUTHOR]

ENT involvement is common in ANCA‐associated vasculitis (AAV), particularly in GPA and EGPA. Early recognition and treatment is important for good outcomes, yet evidence suggests that UK ENT surgeons may not consistently recognise the early features of AAV, despite a similar incidence to vestibular schwannoma. AAV is a rapidly advancing field, with significant developments in the understanding of its pathogenesis, classification and treatment over the past decade. Relevant vasculitis mimics are also discussed with a particular focus on the increasing prevalence of vasculitis mimics driven by an increase in recreational cocaine use, as well as the emergence and reclassification of several other vasculitis mimics in the head and neck. This article reviews key recent updates in the vasculitis literature, with a particular focus on those relevant to recognition and diagnosis of AAV for the ENT surgeon. Strengths and limitations of relevant diagnostic testing are discussed, and a method of evaluation of patients with features of AAV presenting to ENT services is outlined. [ABSTRACT FROM AUTHOR]

Ischemic stroke is an incredibly rare manifestation of granulomatosis with polyangiitis. It is important for the clinician to be aware of this unusual complication so that efforts can be made to reduce the risk of this event. [ABSTRACT FROM AUTHOR]

Introduction: Granulomatosis with polyangiitis (GPA; also known as Wegener’s granulomatosis) is a primary systemic vasculitis involving the ear, nose and throat system (ENT) and lower respiratory tract. Because of the lack of knowledge regarding the clinical findings of GPA due to the limited number of studies, the current study was designed to investigate the prevalence and nature of the otology manifestations in the disease course. Materials and Methods: In the current prospective study, patients with a diagnosis of GPA from 2012–2016 were included. A definitive diagnosis was made based on the history, physical examination (otomicroscopy, Rinne and Weber test), audiometry, tympanometry, cytoplasmic and perinuclear anti-neutrophil cytoplasmic antibody (C-ANCA and P-ANCA) investigations, and pathologic studies. Results: Twenty-seven male and 28 female patients aged 41.6±15.3 years were enrolled. Ear involvement was found in 20 patients (36.3%), and the most prevalent symptom was loss of hearing followed by otalgia and tinnitus. Tinnitus improved in none of the patients. The most prevalent sign was otitis serous followed by mastoiditis and external otitis. The most important audiometry finding was sensorineural hearing loss. Pathological studies using pulmonary samples were more useful for diagnosis. Conclusions: Precise clinical examination is crucial for the early diagnosis of GPA. Otological manifestations are common, especially loss of hearing and otitis serous, and can be the first sign of this disease. Early diagnosis can lead to better treatment of Wegener’s granulomatosis.

A 13-year-old girl was admitted to our hospital because of fatigue, oliguria, and edema of lower extremities. Her laboratory workup showed an acute renal failure (BUN= 225 mg/dl, Creatinine= 13.25 mg/dl). She also had a massive proteinuria. A renal biopsy showed cellular crescentic glomerulonephritis and positive C-ANCA. The serum Creatinine level decreased after dialysis and treatment with high-dose methylprednisolone, rituximab and plasmapheresis. She did not require dialysis after discharge from the hospital. This is a report of a rare case of GPA presenting with 100% cellular crescentic glomerulonephritis. [ABSTRACT FROM AUTHOR]

Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers—members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods: We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results: Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence interval (CI) = 2.3–12.2), respiratory involvement (OR = 3.2; 95% CI = 1.06–9.7), and, in addition, age > 65 (OR = 2.6; 95% CI = 1.05–6.6) were independently associated with death. In MPA, also three variables were observed to be independent predictors of death: PRRT (OR = 5.7; 95% CI = 1.3–25.5), skin involvement (OR = 4.4; 95% CI = 1.02–19.6), and age > 65 (OR = 6.3; 95% CI = 1.18–33.7). Conclusions: In this first multicenter retrospective study of the Polish AAV patients, we have shown that their demographic characteristics, disease manifestations, and predictors of fatal outcome follow the same pattern as those from other European countries, with men possibly suffering from more severe course of the disease. [ABSTRACT FROM AUTHOR]

Objectives We aimed to investigate whether five potential functional haplotypes of the glucocorticoid receptor (GR) gene and a single-nucleotide polymorphism of 11β-hydroxysteroid dehydrogenase type 1 (HSD11B1) are associated with clinical outcome in ANCA-associated vasculitis. Methods Patients diagnosed with ANCA-associated vasculitis (n = 241) were genotyped for five polymorphisms of the GR gene and one polymorphism of the HSD11B1 gene. GR gene haplotypes were predicted based on genotyping results. Relapse-free survival, mortality, renal survival, metabolic adverse events and infections were compared between carriers and non-carriers of GR haplotypes and the HSD11B1 genotype. Results Carriers of haplotype 4 (ER22/23EK + 9β+TthIII1) of GR had a significantly higher 5-year mortality risk [hazard ratio (HR) 4.5 (95% CI 1.6, 12.8)] and had a higher risk of developing end-stage renal disease [HR 7.4 (95% CI 1.9, 28.7)]. Carriers of a minor variant of HSD11B1 more frequently experienced relapse [HR 2.5 (95% CI 1.5, 4.1)] except if they also carried haplotype 1 (Bcl I) of GR. Homozygous carriers of haplotype 1 had a higher risk of developing dyslipidaemia [HR 4.1 (95% CI 1.8, 9.6)]. The occurrence of infections did not differ between GR haplotypes and HSD11B1 genotypes. Conclusion Haplotypes 1 and 4 of GR and a polymorphism of the HSD11B1 gene were associated with clinically relevant inflammatory and metabolic outcomes in ANCA-associated vasculitis. [ABSTRACT FROM AUTHOR]

Objective: To investigate treatment outcomes, hearing outcomes, and adverse effects of rituximab (RTX) for intractable otitis media with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (OMAAV).Methods: Twenty-three patients who met the criteria proposed by the OMAAV study group were included. RTX was used for patients who had difficulty achieving induction of remission using glucocorticoids (GC) and intravenous cyclophosphamide (IVCY).Results: Six patients were treated with RTX (RTX group), while 17 patients did not require RTX for induction of remission (non-RTX group). All six patients in the RTX group achieved remission. Age, sex, and months from onset to diagnosis did not differ significantly between the groups. The air-conduction hearing thresholds at diagnosis and remission were 71.7±6.3dB and 50.1±5.1dB in the RTX group, and 56.8±4.8dB and 35.8±4.8dB in the non-RTX group, respectively. Hearing level at remission was significantly better in the non-RTX group (p<0.05), while hearing gain did not differ significantly between the groups. Infectious complications were similar between the groups.Conclusions: Our findings suggest that RTX is effective and safe for intractable OMAAV patients who have a poor response to GC and IVCY. [ABSTRACT FROM AUTHOR]

Background: Granulomatosis with polyangiitis (GPA) is a systemic vasculitis of the upper and lower respiratory tract along with glomerulonephritis and is very rare in childhood. Its renal manifestations similarity with IgA vasculitis can be misleading. Case presentation: Herein, we report a 12-years-old girl with the clinical picture of IgA vasculitis and renal involvement at the time of presentation, over time, elevated cytoplasmic Anti-neutrophil Cytoplasmic Antibody (C-ANCA) and tissue biopsy confirmed GPA. Conclusion: In the case of a patient with an unusual presentation of IgA vasculitis, to some degree of suspicion, the GPA should be considered. Also, in approach to non-thrombocytopenic palpable petechia and purpura a wide range of differential diagnosis such as infections, ANCA associated vasculitis, and secondary vasculitis should be considered. Therefore, 2 effective method of GPA diagnosis, the high titer of C-ANCA test and tissue biopsy, should be considered simultaneously. [ABSTRACT FROM AUTHOR]

Aim: Antineutrophil cytoplasmic antibody‐associated vasculitides (AAV) are a group of small vessel vasculitis with systemic presentations and considerable morbidity and mortality. Pregnancy in these patients poses a significant therapeutic challenge. There is limited published literature regarding pregnancy in AAV. Methods: Two cases of successful pregnancy outcomes in patients with active AAV are described. A systematic review was conducted on the lines of the PRISMA statement for conducting systemic reviews: PubMed (inception of PubMed until 30 April 2017, English language only) and EmBase databases were searched using the following terms: 'pregnancy' AND 'ANCA associated vasculitis' OR 'granulomatosis with polyangiitis' OR 'eosinophilic granulomatosis with polyangiitis' OR 'microscopic polyangiitis' OR 'Churg‐Strauss syndrome' OR 'Wegener's granulomatosis'. Results: One hundred and thirty‐seven pregnancies were documented in 110 patients of AAV. Vasculitis diagnosis was made before pregnancy in 69, during pregnancy in 32 and after pregnancy in 9 patients. Mean age at the time of pregnancy was 29.3 ± 5.3 years. There were 91 term pregnancies, 28 were preterm pregnancies, 15 abortions and 3 still births; 78 had normal delivery and 26 had caesarian section. Conclusion: Successful pregnancies have been reported in AAV patients. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA), formerly termed Wegener's granulomatosis, is the most common of the pulmonary vasculitides. GPA typically involves the upper respiratory tract, lower respiratory tract (bronchi and lung), and kidney, with varying degrees of disseminated vasculitis. Cardinal histologic features include a necrotizing vasculitis involving small vessels, extensive "geographic" necrosis, and granulomatous inflammation. The spectrum and severity of the disease is heterogeneous, ranging from indolent disease involving only one site to fulminant, multiorgan vasculitis. Circulating antibodies against cytoplasmic components of neutrophils (ANCAs) play a role in the pathogenesis, and often correlate with activity of the disease. Treatment strategies are evolving. Cyclophosphamide (CYC) plus corticosteroids was the mainstay of therapy for generalized, multisystemic GPA since the 1970s. However, within the past decade, rituximab (RTX), a monoclonal antibody directed against B cells, has been shown to be at least as effective (and possibly more effective) as CYC. Furthermore, the use of RTX may reduce the need for maintenance immunosuppression. Optimal therapy for GPA remains controversial, and additional studies are required to determine the role and duration of maintenance therapy following successful induction therapy. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA) previously known as Wegener’s granulomatosis is one of the forms of idiopathic systemic vasculitis. There is very scanty data available on GPA in Asian and Indian population. We studied data of 60 patients from southern India, diagnosed with GPA to describe the physical characteristics, the treatment, and outcome. Patients who fulfilled any two of the four criteria proposed by the American College of Rheumatology, and those with clinical features of GPA with ANCA positivity and histopathological confirmation, were included in the study. Disease activity and damage were assessed by Birmingham Vasculitis Activity Score v. 3 (BVAS v. 3) and Vasculitis Damage Index (VDI), respectively. Relapses were defined as recurrence of GPA of sufficient severity to require treatment or increase in the dose of treatment on a patient who was previously stable. Out of 60 patients, initial BVAS evaluation showed that 57 (95%) patients had severe disease and 3 (5%) patients had limited disease where median BVAS was 21.5 (range 17-44). Follow-up BVAS evaluation for severe disease showed that 13 (22.8%) patients continued with severe disease of which 9 patients did not survive, 24 (42.3%) had remission, 11 (19.2%) had persistent disease, and 9 (15.7%) were lost to follow-up. The mean VDI score was 2.5 ± 2. Renal involvement was established in 42 (70%) patients. Upper and lower respiratory involvement was seen in 38 (63%) patients. Nervous system involvement was noted in the 15 (25%) patients. Articular manifestations were seen in 16 (27%) patients. Diverse clinical manifestation delay early diagnosis and treatment of this potentially treatable vasculitis. Focused approach could expedite early diagnosis and can reduce the mortality. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA), previously known as Wegener’s granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti‐neutrophil cytoplasmic antibody (ANCA)‐negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50‐year‐old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms. Since multiple neurologic deficits were the first manifestation and there were no pulmonary or renal symptoms, our patient was treated for 1 year for suspected infectious or inflammatory diseases such as non‐tuberculous mycobacterial infection or immunoglobulin G4 (IgG4)‐related disease with brain involvement; however, his symptoms would wax and wane. Reanalysis of the laboratory findings including biopsies, finally revealed this case to be compatible with GPA. Here, we report a case of GPA, focusing on the diagnostic pitfalls. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the risk factors and treatment strategies for pneumothorax secondary to granulomatosis with polyangiitis (GPA).Method: Retrospective analysis of cases with pneumothorax secondary to GPA from our own practice and published on literature.Results: A total of 25 patients, 18 males and 7 females, mean age 44 ± 15.7 years, were analyzed. Diagnosis included pneumothorax (11 cases), hydropneumothorax (n = 5), empyema (n = 8) and hemopneumothorax (n = 1). 88% (22/25) patients showed single/multiple pulmonary/ subpleural nodules with/without cavitation on chest imaging. Erythrocyte sedimentation rate and C-reactive protein were both elevated. Corticosteroids and immunosuppressive agents were used in 16 cases. Five cases received steroid pulse therapy, of which 4 patients survived. Pleural drainage was effective in some patients. Seven patients underwent surgical operations. In the 10 fatal cases, infection and respiratory failure were the most common cause. Lung biopsy/ autopsy showed lung/pleural necrotizing granulomatous vasculitis, breaking into the chest cavity, pleural fibrosis, bronchial pleural fistula, etc. The mean age in the death group was greater than the survival group (53 ± 12.9 years vs 40.1 ± 14.7 years, p = 0.05), the ineffective pleural drainage was also higher in the death group (5/5 vs 0/7, p = 0.01).Conclusions: Pneumothorax was seen in the active GPA, due to a variety of reasons, and gave rise to high fatality rate. Aggressive treatment of GPA can improve the prognosis. Older and lack of response for pleural drainage indicates poor prognosis. [ABSTRACT FROM AUTHOR]

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, is the most common form of life-threatening small-vessel vasculitis. Although its exact pathogenesis remains unclear, GPA is believed to belong to the wide complex of autoimmune diseases due to the presence of anti-neutrophil cytoplasmatic antibodies with cytoplasmic staining pattern (c-ANCA) that is expressed in activated neutrophils. GPA predominantly manifests at the upper and lower respiratory tract and the kidneys, but the impairment of multiple organ systems is possible as well. The so-called classical clinical triad of GPA comprises sinusitis, pneumonia, and glomerulonephritis. Despite the fact that there is an elevated risk of mortality for patients suffering from GPA, sudden death due to GPA is a rare and difficult differential diagnosis of sudden natural death in forensic case work. In the present article, the rare case of a 41-year-old male, who died of a sudden death due to previously undiagnosed GPA, is demonstrated. The final diagnosis was feasible by close interdisciplinary collaboration, considering the entire body of findings obtained during autopsy, histopathological investigation, and analysis of the clinical records. Therefore, it remains necessary to point out that especially for rare causes of death, interdisciplinary collaboration is essential in order to concretize the cause of death and exclude rare differential diagnoses of sudden unexpected death of hospitalized patients in forensic case work. [ABSTRACT FROM AUTHOR]