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...

A young-of-the year, female raccoon was presented with clinical signs of weakness and tremors. The raccoon was euthanized, and autopsy findings included poor body condition, diffuse lymphadenopathy, and pale, firm kidneys with petechial hemorrhages throughout the renal cortex. Histologic lesions included systemic fibrinoid vascular necrosis and severe renal lesions, including lymphoplasmacytic interstitial nephritis and fibrinosuppurative glomerulonephritis. Inflammatory vascular lesions were also present within the uvea, heart, lymph nodes, and the lamina propria of the gastric wall...

BACKGROUND: Exposure to respirable crystalline silica is suggested to increase the risk of autoimmune rheumatic diseases. We examined the association between respirable crystalline silica exposure and systemic sclerosis, rheumatoid arthritis, systemic lupus erythematosus and small vessel vasculitis. METHODS: In a cohort study of the total Danish working population, we included 1 541 505 male and 1 470 769 female workers followed since entering the labour market 1979-2015...

Primary cardiac valve tumours are rare. This is a case report of a 32-year-old non-smoker man with a history of stroke 1 year prior and no other cardiovascular risk factors. The patient was admitted to our acute stroke ward for recurrent left hemiparesis, slurring of speech, facial asymmetry and central retinal artery occlusion. Initial laboratory investigations and ECG were normal. An urgent CT brain showed a large hypodense area at the right frontal, parietal, temporal, occipital region with effaced sulci and right lateral ventricle with midline shift and cerebral oedema in keeping with acute infarction...

A 34-year-old post-partum female having dermatomyositis developed headache and became comatose after a seizure episode. Magnetic resonance imaging of brain showed a massive left ganglio-capsular bleed for which decompressive surgery was done. Computed tomographic angiography showed multiple foci of narrowing and irregularities in distal cerebral vessels. In view of dermatomyositis, the diagnosis of vasculitis was considered and pulse therapy of intravenous methylprednisolone was started. The patient, however, showed no improvement and developed new brain infarcts...

PURPOSE: To describe perfusion patterns of peripheral pulmonary lesions (PPLs) in COVID-19 patients using contrast-enhanced ultrasound (CEUS). PATIENTS AND METHODS: From April 2020 until July 2020, 11 consecutive patients with RT-PCR-confirmed COVID-19 and PPLs sized over 5 mm were investigated by B-mode ultrasound (B-US) and CEUS. The homogeneity of enhancement (homogeneous and inhomogeneous) was examined retrospectively using CEUS. An inhomogeneous enhancement was defined as a perfused lesion with coexisting non-perfused areas (NPA)...

Brain atrophy, typically slowly progressive, is a hallmark of neuropsychiatric (NP) systemic lupus erythematosus (SLE). In this article, we report a case of a young female patient with previously diagnosed SLE, without known NPSLE, with abrupt neurological deterioration and rapidly progressive diffuse atrophy in a few months. A comprehensive diagnostic work-up and follow-up magnetic resonance imaging (MRI), including high-resolution advanced vessel-wall sequences, revealed underlying cerebral vasculitis. The novelty factors that the present report brings are the rapid progressive atrophy demonstrated on follow-up MRI in a patient with SLE, and the depiction of an underlying vasculitis on specific vessel-wall MRI techniques...

We described a case of exuberant cutaneous small-vessel vasculitis in a 27-year-old male with mild CoVID-19 in Brazil. The patient presented painful purpuric papules and vesicobullous lesions with hemorrhagic content located in the larger amount in the lower limbs and, to a lesser extent in the region of the back and upper limbs, saving palms and soles of the feet. Influenza-like syndrome with anosmia and ageusia was reported seven days before the skin lesions. A real-time reverse transcription polymerase chain reaction was positive on a nasopharyngeal swab for SARS-CoV-2...

Giant cell arteritis (GCA) is a systemic vasculitis that classically affects large- and medium-sized vessels in the head and neck but can also manifest extracranially. We report the case of an elderly man who presented with sharp substernal pain, dizziness, and visual changes. He was initially hypotensive and anemic, and imaging showed hemoperitoneum with possible extravasation. Celiac and gastric angiography demonstrated findings consistent with vasculitis and focal extravasation from a left gastric branch...

Due to its rarity and the limited literature, the clinicopathological characteristics of peripheral nerve involvement in immunoglobulin G4 (IgG4)-related disease are unknown. We present two cases of IgG4-related disease, accompanied by peripheral neuropathy, presenting as unilateral ptosis (case 1) and sclerosing cholangitis (case 2), respectively. In both cases, sural nerve biopsy indicated vasculitis as the underlying pathophysiology; the peripheral neuropathy was refractory to corticosteroid therapy. In contrast to the previously proposed pathomechanism of IgG4-related neuropathy (direct lymphoplasmacytic infiltration), the pathological findings in our cases suggest that vasculitis occurs secondary to systemic autoimmune conditions...

OBJECTIVES: To determine the prevalence of vitamin D deficiency in patients with small and medium vessel systemic vasculitis. METHODS: In this cross-sectional study, 25-hydroxy (OH) vitamin D3 levels were measured in adult patients with systemic small and medium vessel vasculitis including antineutrophil cytoplasmic antibody-associated vasculitis (AAV), cryoglobulinaemic vasculitis (CryV), IgA vasculitis (IgAV) and polyarteritis nodosa (PAN), and age- and sex-matched healthy subjects (HS) and patients with rheumatoid arthritis (RA) as control groups...

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The incidence of venous thromboembolism (VTE) in ANCA-associated vasculitis patients varies in different populations. Moreover, the risk factors for VTE in these patients are poorly described due to the small number of events. Ovid MEDLINE, EMBASE, and the Cochrane Library were searched for eligible articles. The inclusion criteria included observational studies that enrolled patients age ≥ 18 years diagnosed with ANCA-associated vasculitis. The incidence of VTE is the outcome of interest. Of 1362 citations, a total of 21 studies (n = 4422) dated from 2006 to 2019 were included in the systematic review...

A 65-year-old man, a smoker, presented to the emergency department with progressive digital ischaemia, fever and weight loss. The clinical examination revealed generalised lymphadenopathy and ischaemic changes of the right distal phalanges of the second, third and fourth fingers. He had an ultrasound-guided biopsy of the cervical lymph node, which showed histopathological findings of classic Hodgkin's lymphoma. Paraneoplastic acral vascular syndrome (PAVS) is a rare phenomenon and seen more in solid malignancies...

The complement system (CS) includes more than 50 proteins and its main function is to recognize and protect against foreign or damaged molecular components. Other homeostatic functions of CS are the elimination of apoptotic debris, neurological development, and the control of adaptive immune responses. Pathological activation plays prominent roles in the pathogenesis of most autoimmune diseases such as systemic lupus erythematosus, antiphospholipid syndrome, rheumatoid arthritis, dermatomyositis, and ANCA-associated vasculitis...

We describe the case of a child with progressive abdominal pain and rash admitted to a large tertiary children's hospital in the Chicago metropolitan area and subsequently found to have immunoglobulin A (IgA) vasculitis and coronavirus disease 2019 (COVID-19). This patient presented with abdominal pain, purpuric lesions, hematochezia, increasingly elevated D-dimer, and abnormal inflammatory markers on laboratory evaluation. To the best of our knowledge, this article describes the only reported pediatric case of COVID-19 associated with IgA vasculitis...

Pulmonary arterial hypertension (PAH) is a rare complication of ANCA-associated vasculitis (AAV). We report a 37-year-old man with PAH complicated by both AAV and SSc who presented with dyspnea, cardiac enlargement, positive myeloperoxidase (MPO)-ANCA, anti-centromere antibodies, proteinuria, and urinary casts. Elevated pulmonary arterial pressure (58/22/34 mmHg) and low PAWP (2 mmHg) were confirmed by right heart catheterization. Treatment with glucocorticoids (GC) decreased urinary protein and serum MPO-ANCA; however, PAH did not respond to GC...

Background: Antineutrophil 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 summary: A 60-year-old male patient presented to the emergency room with recent-onset dyspnoea New York Heart Association Class IV...

The systemic vasculitides are a heterogenous group of rare conditions with an incompletely understood aetiology. Any of the systemic vasculitides may cause respiratory disease, but some conditions are more likely to affect the pulmonary system, often through pulmonary infarction and diffuse alveolar haemorrhage. These conditions are often difficult to diagnose due to their rarity and significant clinical overlap with common respiratory conditions. Prompt diagnosis and management can significantly reduce morbidity and mortality...

Objective: Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiographic entity of heterogeneous etiologies having similar clinical and neuroimaging features. Pediatric data are sparse, making early diagnosis challenging, which needs a high index of suspicion. So, we conducted this study to evaluate clinico-radiological features, associated risk factors, etiology, and outcome in children. Materials and methods: This is a retrospective case series of patients, diagnosed as having PRES and followed up at a tertiary care hospital in Eastern India between September 2016 and December 2019...