Presence Of Vasculitis Research Articles

Idiopathic Large-Vessel Vasculitis Presenting as Acute Abdomen and Mesenteric Ischemia

Objective: The term “large-vessel vasculitis” encompasses the spectrum of primary vasculitis that causes chronic granulomatous inflammation predominantly of the aorta and its major branches. The two major categories of large-vessel (LV) vasculitis are giant cell arteritis and Takayasu arteritis. LV vasculitis of unknown etiology presenting as mesenteric ischemia is presented here. Materials and Methods: This is a single-center retrospective analysis of 17 patients who presented with mesenteric ischemia over a period of 7 years. Their age ranged from 18 to 69 years. There were five females and the rest were male. The presenting symptoms were abdominal pain, nausea, malaise, and low-grade fever. Peripheral pulses were normal. Computed tomography (CT) aortogram showed typical wall edema and inflammatory tissue encasing the superior mesenteric artery, celiac artery, and the adjacent aorta in all the patients. Thrombosis and localized dissection in the superior mesenteric and celiac arteries was seen in some. Erythrocyte sedimentation rate and C-reactive protein were elevated, but other vasculitis markers were normal. Results: All of them were treated with glucocorticoids with immediate relief from the symptoms. Anticoagulation was given for those with thrombus, stenosis, and dissection. None had any intervention. At 1-year follow-up, there was no recurrence, and all were asymptomatic. Conclusion: Idiopathic LV vasculitis can present as mesenteric ischemia and acute abdomen. CT aortogram typically shows inflammatory tissue around the visceral artery and the aorta. The etiology is still uncertain. However, this condition has many similarities with LV giant cell arteritis (GCA-LV). Hence, all investigations should be done to rule out GCA. They respond very well to glucocorticoids. Correct diagnosis can avoid an unnecessary surgery. Long-term follow-up is required in order not to miss any late complications This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

A Late Presentation of Unilateral Idiopathic Retinal Vasculitis, Aneurysms, and Neuroretinitis (IRVAN) Syndrome

Although uncommon, idiopathic retinal vasculitis, aneurysms, and neuroretinitis (IRVAN) syndrome can lead to vision threatening complications if not diagnosed and treated appropriately. This case report describes an atypical presentation of unilateral IRVAN syndrome, which resulted in significant visual loss in an elderly male. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/a-late-presentation-of-unilateral-idiopathic-retinal-vasculitis-aneurysms-and-neuroretinitis-irvan-syndrome)

Anti-glomerular basement membrane disease with intense nephrotic syndrome: a new case report.

Anti-glomerular basement membrane (anti-GBM) disease was usually described as a small vessel vasculitis presenting with acute kidney injury, haematuria and non-nephrotic proteinuria. We report a case of anti-GBM disease revealed by an intense nephrotic syndrome. The urinary protein level was 12g/day. Renal biopsy only showed crescent glomerulonephritis with linear staining of IgG in direct immunofluorescence without other glomerulonephritis. Immunoglobulin G (IgG) anti-GBM antibody titer was elevated.

69-Year-Old Man With Nasal Congestion and Pre-Syncope

69-Year-Old Man With Nasal Congestion and Pre-Syncope

P208 A case of double positivity

Abstract Background/Aims In the absence of renal or lung involvement, the investigation of small vessel vasculitis does not routinely include checking of anti-glomerular basement membrane (GBM) antibodies. We present a patient with an MPO-ANCA positive vasculitic neuropathy who later developed anti-GBM positive glomerulonephritis. We review the literature on patients who are “double-positive” for ANCA and GBM antibodies and speculate on the utility of routinely screening all vasculitis patients for GBM antibodies in the absence of manifestations of Goodpasture’s syndrome. Methods We describe a 70 year old female who presented feeling generally unwell with reduced mobility due to discomfort in her feet. Examination was unremarkable, except for reduced temperature and joint position sense in the feet. CRP was elevated and she was found to have a weakly positive MPO titre of 32. She was treated as an MPO-ANCA positive vasculitis and neuropathy with steroids and Mycophenolate Mofetil, with a good clinical and biochemical response. Fifteen months into her treatment, routine monitoring revealed an acute kidney injury with a creatinine of 459 umol/l from a baseline of 88. Anti GBM titres were requested for the first time as part of the renal screen. MPO antibody titres were only 9, but GBM antibody titres were markedly elevated (>200). Renal biopsy showed focal necrotising glomerulonephritis, in keeping with anti-GBM disease. She was treated with steroids, cyclophosphamide and plasma exchange by the renal physicians. She remains dialysis dependant. Results In our patient, GBM antibodies were only requested once she developed relevant clinical manifestations of anti-GBM disease. This in contrast to nephrology practise, where ANCA and GBM antibodies are routinely requested simultaneously in the context of nephritis. It could be speculated that if the GBM antibody status had been detected sooner, we may have altered her management and possibly improved her disease course. Anti-GBM disease and ANCA-associated vasculitis are both rare conditions. However, co-existence of the two uncommon diseases in individual patients is well-described and more than chance occurrence. A study showed that 5% of ANCA-positive cases were also positive for anti-GBM antibodies, while one-third of anti-GBM positive samples had detectable ANCA. Multiple studies have shown that low level ANCA antibodies develop months to years before onset of anti-GBM disease, as seen in our patient as well.The dominant disease phenotype in double positive patients is anti-GBM disease rather than vasculitis. It is very rare for isolated anti-GBM disease to relapse. In contrast, half of double positive patients experience disease relapse and therefore, require long-term follow-up and maintenance immunosuppression. Conclusion Awareness of this double positive phenomenon is important. Rheumatologists do not routinely check anti-GBM in all vasculitis. This case highlights the importance of doing so, especially in atypical presentation of vasculitis or in relapse and refractory disease. Disclosure S. Khalid: None. J. Li: None. S. Young Min: None.

Cerebral Lymphocytic Vasculitis presenting with behavioral changes and mimicking hemorrhage vs. neoplasm on neuroimaging (2739)

Cerebral Lymphocytic Vasculitis presenting with behavioral changes and mimicking hemorrhage vs. neoplasm on neuroimaging (2739)

“Triple-positive” renal limited vasculitis presenting with rapidly progressive glomerulonephritis

Rationale: Coexistence of anti-glomerular basement membrane (anti-GBM) disease with anti-neutrophil cytoplasmic antibody (ANCA) in a case of glomerulonephritis is often identified as a “double-positive” disease. Interestingly, the majority of “double positive” ANCA is myeloperoxidase (MPO)-ANCA and some of the MPO-ANCA positive cases showed intrarenal arteritis, suggesting an ANCA-associated kidney lesion. Proteinase 3-ANCA positive diseases are also rarely reported. Patients positive for all three antibodies, i.e., triple-positive patients, are extremely rare. Patient's Concern: A 53 year-old female presented with anasarca and oliguria of 2 months’ duration. Diagnosis: Pauci-immune type renal limited crescentic glomerulonephritis positive for MPO-ANCA, proteinase 3-ANCA, and anti-GBM antibody (triple-positive). Interventions: Intravenous high dose cyclophosphamide, oral azathioprine, intravenous methylprednisolone, and plasma exchange as per British Health Professionals in Rheumatology Guidelines. Outcomes: After one-month follow-up, anasarca and proteinuria were lessened, serum creatinine was normalized, titers of MPO-ANCA levels were decreased, and anti-GBM antibody levels were normalized. Lessons: Triple-positive renal limited vasculitis is rare and response to combined immunosuppressive therapy and plasma exchange can contribute to successful remission.

Patient perceptions of health-related quality of life in giant cell arteritis: international development of a disease-specific patient-reported outcome measure.

ObjectivesGCA is a large vessel vasculitis (LVV) presenting with headache, jaw claudication, musculoskeletal and visual involvement. Current treatment is glucocorticoids and anti-IL-6 tocilizumab in refractory disease. The objective of this study was to explore the impact of GCA and its treatment on people’s health-related quality of life (HRQoL), to inform the development of a disease-specific patient-reported outcome measure (PROM) for use in clinical trials and practice.MethodsParticipants from the UK and Australia, with biopsy- or imaging-confirmed GCA, were interviewed to identify salient aspects of HRQoL in relation to GCA and its treatment. Purposive sampling included a range of demographic and disease features (cranial, LVV-GCA and visual involvement). Inductive analysis identified individual themes of importance, then domains. Candidate questionnaire items were developed from the individual themes, refined by piloting, cognitive interviews and a linguistic translatability assessment.ResultsThirty-six interviews were conducted to saturation with participants with GCA from the UK (25) and Australia (11). Mean age was 74 years, 23 (63.9%) were female, 13 (36.1%) had visual loss and 5 (13.9%) had LVV-GCA. Thirty-nine individual themes within five domains were identified: physical symptoms; activity of daily living and function; participation; psychological impact; and impact on sense of self and perception of health. Sixty-nine candidate items were developed from individual themes; piloting and refinement resulted in a 40-item draft questionnaire.ConclusionThis international qualitative study underpins the development of candidate items for a disease-specific PROM for GCA. The draft questionnaire is now ready for psychometric testing.

Medium- and Large-Vessel Vasculitis.

Systemic vasculitides are multisystem blood vessel disorders, which are defined by the size of the vessel predominantly affected, namely small, medium, or large vessels. The term "large vessel" relates to the aorta and its major branches; "medium vessel" refers to the main visceral arteries and veins and their initial branches. The most common causes of large-vessel vasculitis are giant cell arteritis and Takayasu arteritis, and those of medium-vessel arteritis are polyarteritis nodosa and Kawasaki disease. However, there is some overlap, and arteries of any size can potentially be involved in any of the 3 main categories of dominant vessel involvement. In addition to multisystem vasculitides, other forms of vasculitis have been defined, including single-organ vasculitis (eg, isolated aortitis). Prompt identification of vasculitides is important because they are associated with an increased risk of mortality. Left undiagnosed or mismanaged, these conditions may result in serious adverse outcomes that might otherwise have been avoided or minimized. The ethnic and regional differences in the incidence, prevalence, and clinical characteristics of patients with vasculitis should be recognized. Because the clinical presentation of vasculitis is highly variable, the cardiovascular clinician must have a high index of suspicion to establish a reliable and prompt diagnosis. This article reviews the pathophysiology, epidemiology, diagnostic strategies, and management of vasculitis.

Pediatric ANCA- Associated Vasculitis Presenting with Acute Postoperative Subglottic Stenosis: A Case Report

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often present with nonspecific constitutional symptoms. However, there has been increasing recognition of a subgroup of young, female AAV patients who are at higher risk for isolated subglottic airway disease. Case Presentation: We report a rare case of a 17-year-old female with undiagnosed ANCA-associated vasculitis who presents with acute, life threatening subglottic stenosis requiring emergent intubation after routine surgery. Conclusion: Much of the research on ANCA-associated vasculitis have been based on adult cases, however many pediatric patients may not follow the same diagnostic and clinical presentations. Clinicians should be vigilant in recognizing a subgroup of vasculitis patients who are often young, female, and have a higher prevalence of subglottic stenosis. Symptoms should be promptly worked up and initiation of immunosuppressive therapy is necessary to prevent life threatening complications and end-organ damage. Keywords: ANCA-associated vasculitis, Subglottic stenosis, Granulomatosis with polyangiitis.

An extraordinary case of oral leukocytoclastic vasculitis demonstrating igd deposition around dermal vessels and within mucosal keratinocytes

Cutaneous vasculitides include a widespread and heterogeneous cluster of diseases affecting the blood vessels that are clinically characterized by polymorphic skin lesions, including palpable purpura, urticarial and/or necrotic-ulcerative lesions. Often, they can be manifestations of a systemic disease. Selected cases occur in the mouth. A 75-year-old female presented to her physician for the sudden appearance of blisters in her mouth, with severe orodynia and no history of other diseases or medication intake. A skin biopsy of the oral mucosa yielded a diagnosis of leukocytoclastic vasculitis. The direct immunofluorescence and immunohistochemistry stains demonstrated deposits of IgD, IgG, IgA, IgM, kappa, lambda, C1q, C3c, albumin and fibrinogen at the upper dermal neurovascular plexus. IgD also demonstrated positive nucleolar staining of the keratinocytes. Our case involves a rare presentation of oral cutaneous vasculitis with immune deposits of several immunoglobulins, complement, albumin and fibrinogen. Our case adds importance to studies of the IgD role in antigenic complex immune responses, especially in the mouth.

Pulmonary Embolism and Global T-Wave Inversion as the Concomitant Presentation of IgA Vasculitis

Pulmonary Embolism and Global T-Wave Inversion as the Concomitant Presentation of IgA Vasculitis

Acute Hemorrhagic Infancy of Edema: A Purpuric Rash in 6-Month-Old Infant

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis presenting with purpura, ecchymosis, fever, and edema. Pediatricians must effectively differentiate AHEI from other diseases that have similar presentations but are more severe in order to treat appropriately without overutilizing expensive, unnecessary diagnostic tests. In this article, we describe a case of a 6-month-old previously well male who presented to our institution with a worsening rash and fever. In this case, the patient’s age and benign clinical manifestations without systemic involvement favored the diagnosis of AHEI over more serious conditions. This case is a valuable example of the clinical findings of AHEI and the effectiveness of conservative therapy once a diagnosis is made for pediatricians, especially emergency and urgent care physicians.

Sarcoid vasculitis presenting with erythema nodosum-like lesions.

Sarcoid vasculitis presenting with erythema nodosum-like lesions.

Assessment of T Regulatory cells in Egyptian patients with Behcet’s disease as a prognostic marker of Uveitis

ABSTRACT Behçet’s disease is an autoimmune type of vasculitis presenting by attacks of oral, skin and genital ulcers, as well as ophthalmic disorders that cause blindness in 25% of affected patients. Circulating T regulatory cells suppress atypical autoimmune responses and help developing immune self-tolerance. An X chromosome encoded forkhead transcription factor (Foxp3) is essential for proper T reg cell development and function. Aim of the work; The aim of the study was to determine the correlation between T reg cell percentage of expression, Behçet’s uveitis pathogenesis, and disease manifestations. Materials and Methods; Forty-four subjects were involved in this study: 15 subjects diagnosed with Behcet’s uveitis (including a female) in addition to 29 healthy age and sex-matched controls. The percentage of CD25 and Foxp3 expression in peripheral blood CD4+ T-lymphocytes was determined by flowcytometry. X-rays and magnetic resonance imaging (MRI) were used to assess patients with suspected Behcet’s arthritis. Percentages of CD4+CD25−Foxp3+and CD4+CD25+Foxp3+ T reg cell expression were higher in Behcet’s uveitis patients compared to the controls without a significant difference. Conversely, CD4+CD25+Foxp3 − T reg cell percentage was found to be higher in the controls than in Bechet’s uveitis cases without a significant difference. However, the mean fluorescence intensity of Foxp3 factor necessary for Treg cell function was significantly higher in the control group compared to Behcet’s uveitis cases (p = 0.02). Results of the current work indicate the protective role played by T reg cells (especially the Foxp3+ group) against Behcet’s uveitis that could be used as a valuable prognostic marker.

An Isolated Presentation of Ischemic Retinal Vasculitis by the Great Masquerader

Abstract Ischemic retinal vasculitis is a sight-threatening inflammatory ocular condition that involves the retinal blood vessels. It is often associated with vascular occlusion and retinal hypoperfusion that may lead to neovascularization and subsequently preretinal and vitreous hemorrhages. It can be idiopathic or secondary to infection, neoplasia, or any other systemic diseases. Although it is rare, tuberculosis, which is an airborne disease caused by Mycobacterium tuberculosis, can manifest as retinal vasculitis without any evidence of pulmonary or other ocular structures involvement. We present a case of a 16-year-old girl with sudden blurred vision in the right eye. Based on fundoscopic examination and fluorescein fundus angiography, the right eye had severe bilateral ischemic vasculitis and neovascularization. Both Mantoux and tuberculosis spot test (T-Spot) further supported the presumptive clinical diagnosis of ocular tuberculous ischemic retinal vasculitis. Subsequently, pan-retinal photocoagulation sessions were commenced together with antituberculosis therapy and resulted in improved vision. We also include a literature review of similar reported cases where occlusive retinal vasculitis is the only presentation of ocular tuberculosis. Although this case highlights the possibility of ischemic retinal vasculitis as the only feature of ocular tuberculosis, a high degree of suspicion is necessary when dealing with this great masquerader.

Microscopic Polyangiitis (MPO-ANCA) and Henoch-Schoenlein purpura overlap in a Child

MPO-ANCA and Henoch-Schoenlein purpura overlap are extremely rare in the pediatric population. We present a case with biopsy-proven IgA renal depositions and positive MPO in a 6-year-old child with the clinical presentation of vasculitis with cutaneous and renal involvement. The child was successfully treated with a combination of steroids and cyclophosphamide.

A P-Anca Positive Patient With Recently Treated Vasculitis Presenting with Shortness of Breath and Fever: A Diagnostic Dilemma

A P-Anca Positive Patient With Recently Treated Vasculitis Presenting with Shortness of Breath and Fever: A Diagnostic Dilemma

An unusual precipitant of acute heart failure-ANCA-associated vasculitis in a patient with ischaemic cardiomyopathy: a case report.

BackgroundAntineutrophil cytoplasmic antibody (ANCA)-associated pulmonary renal vasculitis is an uncommon disease entity. Its presentation as acute heart failure for the first time in a patient with established coronary artery disease (CAD) is even rarer. We present here a case of such an association and an approach to managing this clinical situation.Case summaryA 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV. He was having hypertension, uncontrolled diabetes mellitus, chronic kidney disease (CKD), and CAD. He also underwent a percutaneous coronary intervention to left anterior descending in the past for acute coronary syndrome and had moderate left ventricular dysfunction. He was being managed as a case of acute decompensated heart failure (ADHF) and was mechanically ventilated. Suddenly his ventilator requirement increased and endotracheal aspirate contained blood. The chest radiograph showed bilateral hilar infiltrates. Simultaneously he also had recurrent episodes of ventricular tachycardia (VT) requiring direct current (DC) cardioversion. Blood investigations showed deranged renal function and severe hyperkalaemia, but no evidence of coagulopathy. High-resolution computed tomography chest showed features of diffuse alveolar haemorrhage. Further investigations revealed high titres of c-ANCA and raised inflammatory biomarkers. A diagnosis of ANCA-associated vasculitis presenting as acute on CKD with dyselectrolytaemia (hyperkalaemia) leading to VT was made. Apart from standard management for associated illness, he was treated with plasma exchange, steroids, and cyclophosphamide to which he responded and was later on discharged.DiscussionAntineutrophil cytoplasmic antibody-related pulmonary renal vasculitis can lead to rapidly progressing renal failure and may present as ADHF in a patient with existent CAD. The associated VT storm in our patient can be attributed to hyperkalaemia secondary to acute renal failure. A multidisciplinary approach is required for the successful management of such a complex clinical scenario.

Antineutrophil cytoplasmic antibody-associated vasculitis in a patient with diffuse scleroderma

Scleroderma is a multiorgan connective tissue disease characterized by autoantibody production and fibroproliferative stenosis of the microvasculature. The development of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in patients with scleroderma is a rare association. Renal and pulmonary complications occur more frequently than expected in scleroderma/ANCA-associated vasculitis overlap. The definitive diagnosis usually made based on autoantibody titers and biopsy of involved tissue. We present a case of diffuse scleroderma with ANCA-associated vasculitis presenting with neurological symptoms.