Vasculitis

Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a group of rare systemic autoimmune diseases characterised by necrotising inflammation of small blood vessels and usually associated with circulating ANCA. The pathophysiology of AAV is complex, involving many aspects of the innate and adaptive immune system. Neutrophils are central to the pathogenesis of AAV as they are both the target of the autoantibody and effector cells mediating vascular injury. We describe mechanisms for ANCA induced activation of neutrophils, the pathogenic mechanisms by which this leads to endothelial cell injury, and how neutrophil crosstalk modulates other aspects of the immune system in AAV...

Intravenous immunoglobulin (IVIG) therapy has emerged as a promising treatment option for various dermatological autoimmune diseases due to its immunomodulatory potential and low incidence of severe side effects. Despite its widespread use, the mechanism of action of IVIG in treating autoimmune diseases remains a topic of debate. IVIG is derived from the plasma fractionation of a large pool of donors, primarily consisting of the IgG isotype. Its main mechanisms of action involve neutralizing circulating autoantibodies via the F(ab')2 portion, inhibiting complement-mediated tissue destruction, and reducing the half-life of circulating autoantibodies through the Fc portion...

INTRODUCTION AND IMPORTANCE: Although pulmonary artery involvement is well recognized, the incidence of interstitial lung disease (ILD) with Takayasu arteritis is very rare. The pathophysiology of ILD in Takayasu is still incompletely understood, in contrast to several studies establishing the relationship between ANCA-associated vasculitis and ILD. The management of this patient involved a multidisciplinary approach with long-term follow-up. CASE PRESENTATION: The authors present a case of HRCT-proven interstitial lung disease in a patient with Takayasu arteritis and heart failure...

INTRODUCTION: Kawasaki disease (KD) is a pediatric vasculitis that can result in coronary artery aneurysm (CAA) formation, which is a dangerous complication. Treatment with intravenous immunoglobulin (IVIg) significantly decreases the risk of CAA, possibly through competitive binding to Fc-gamma receptors (Fc γ Rs), which reduces the binding of pathological immune complexes. However, ~20% of children have recrudescence of fever and have an increased risk of CAA. Therefore, we aimed to identify genetic markers at the FCGR2/3 locus associated with susceptibility to KD, IVIg resistance, or CAA...

Urticarial vasculitis is characterized by persistent urticarial lesions lasting over 24 h. Urticarial vasculitis is often triggered by medications, infections, and autoimmune disorders. However, vaccinations against viral and bacterial pathogens have recently been documented to induce urticarial vasculitis. We describe the case of a 67-year-old woman who was presented with an extensive erythematous and purpuric rash without systemic symptoms 3 days after an influenza vaccination. She was diagnosed with normocomplementemic urticarial vasculitis based on clinical findings, normal complement levels, and histopathological findings of leukocytoclastic vasculitis...

Granulomatosis with polyangiitis (GPA) is a rare type of small to medium vessel necrotizing vasculitis that usually affects vessels of the upper or lower airways and kidneys. Cardiac involvement in GPA is often subclinical and if clinically significant has been rarely reported, even less so as an initial presentation. We describe the case of a 44-year-old man who presented with what appeared to be inferior ST-segment elevation myocardial infarction and was found to have small vessel vasculitis of the coronary arteries with associated myocarditis as a presenting manifestation of GPA, which was ultimately treated with steroids, rituximab, and avacopan...

Takayasu arteritis is a rare vasculitis of the aorta and its branches. Here, we present a teenaged athlete with coronary arteritis and pyoderma gangrenosum, the youngest person whose case is described in the literature. She received immune modulation and percutaneous coronary intervention, with subsequent in-stent restenosis and cardiac arrest requiring coronary artery bypass grafting.

BACKGROUND: Multiple sclerosis (MS) and Takayasu's arteritis (TAK) are two autoimmune diseases that affect the Central nervous system (CNS), but the relationship between them has not been established. CASE PRESENTATION: Here we report the emergence of MS during treatment. Takayasu's arteritis in a 24-year-old Iranian woman with a severe presentation. She was treated aggressively with IV methylprednisolone 1 g/day for 3 days and continued with oral prednisolone, also IV cyclophosphamide monthly...

OBJECTIVES: We aimed to compare inflammatory markers and determine their potential role in distinguishing secondary leukocytoclastic vasculitis (SLV) from idiopathic leukocytoclastic vasculitis (ILV). MATERIALS AND METHODS: We included in this cross-sectional study patients with cutaneous leukocytoclastic vasculitis (CLV) diagnosed on cutaneous biopsy. We assessed clinical and laboratory data and then calculated platelet-to-lymphocyte ratio (PLR), neutrophil-to-lymphocyte ratio (NLR), C-reactive protein (CRP)-to-albumin ratio (CAR), and fibrinogen-to-albumin ratio (FAR)...

No abstract text is available yet for this article.

BACKGROUND: There are no recent studies with a focus on the histopathology of erythema nodosum leprosum (ENL). OBJECTIVES: To describe the histopathological spectrum of ENL. MATERIALS AND METHODS: Digital records from the pathology department were searched, and 125 slides were included. The histopathologic findings were recorded using a pre-designed proforma. RESULTS: Several patterns were noted with the most common being a superficial and deep, perivascular and peri-appendageal, well-circumscribed dermal infiltrate that was seen in 70 (56...

BACKGROUND: Pyoderma gangrenosum (PG) is a reactive neutrophilic inflammatory dermatosis with a varied clinicopathologic presentation. It commonly manifests as rapidly progressive painful ulcers, mimicking varied conditions including infections, vasculitis, and malignancies, and is a diagnosis of exclusion. There are scarce data on PG from the Indian subcontinent. AIM AND OBJECTIVES: The aim of the study was to study the clinicopathologic profile of patients with PG and their underlying systemic associations...

BACKGROUND: Clinical course prediction of patients with interstitial lung disease (ILD) admitted to the intensive care unit (ICU) for acute respiratory failure (ARF) can be challenging. This study aimed to characterize the prognostic value of admission chest CT-scan in this situation. METHODS: We retrospectively included ILD patients admitted to a French ICU for acute respiratory failure requiring oxygen. Patients with lymphangitis carcinomatosis and ANCA vasculitis were excluded...

Kawasaki Disease is multisystem vasculitis affecting young children and infants. While the diagnosis of a typical form of Kawasaki Disease is obvious, there are some patients who do not fulfill the classic diagnostic criteria for the disease which is termed as 'incomplete Kawasaki Disease' or 'Atypical Kawasaki Disease'. We present a case of a 6 months old child with fever who after failing to respond to IV antibiotics showed considerable improvement after administering aspirin and Intravenous Immunoglobulin thus diagnosed as Atypical Kawasaki Disease...

BACKGROUND: Behcet's disease (BD) is a rare and recurrent autoinflammatory disorder characterized by systemic vasculitis, frequently manifested as recurrent aphthous stomatitis (RAS). We aim to identify specific serum proteins to discriminate between BD and idiopathicRAS. METHOD: Peripheral blood was collected from 12 BD patients, 12 idiopathic RAS patients, and 21 healthy volunteers. The serum samples underwent Tandem Mass Tag-based mass spectrometry analysis...

We describe a 75-year-old male who presented to the emergency department with generalized weakness and was ultimately diagnosed with acute renal failure secondary to pauci-immune necrotizing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. The patient's clinical course was complicated by a perforated gastric ulcer and severe malnutrition, necessitating involvement from multiple specialists. The case highlights the challenges of this rare vasculitis and the complications that can arise from the disease and its treatment...

BACKGROUND: Behçet syndrome (BS) is a vasculitis of variable vessels with multiple organ manifestations. OBJECTIVE: This article gives an overview of innovations in the last 2 years. MATERIAL AND METHODS: A literature search was carried out using the keyword "Behcet" in 2022-2024 in PubMed. The selection of suitable articles was based on the relevance. RESULTS AND CONCLUSION: With respect to the pathophysiology it is now clear that BS occupies an intermediate position between autoinflammatory and autoimmune clinical pictures...

Noninfectious aortitis is occasionally detected incidentally, either on imaging or on histopathologic review after open thoracic aortic surgery. It can present as a clinically asymptomatic, seemingly focal lesion, as diffuse inflammation throughout several aortic segments but sparing the branch vessels, or as a manifestation of a widespread systemic condition. Treatment differs based on etiology, so once identified, all patients with aortitis need a thorough evaluation, laboratory tests, complete large-vessel imaging, and a referral to a vasculitis expert...

OBJECTIVES: ANCA-associated vasculitis (AAV) are chronic diseases with relapses that associate organic damage because of the disease and its treatment. Avacopan is a new treatment indicated for AAV. We present the first experiences with avacopan in Spain as part of an Early Access program. METHODS: Patients with AAV who started avacopan between June 2022-September 2023 were included. For comparison, a historical cohort of patients diagnosed with AAV around the same time and treated without avacopan was also included...

No abstract text is available yet for this article.