Granulomatosis with polyangiitis (GPA) is a rare multisystem disease characterized by vasculitis affecting small vessels, resulting in the formation of necrotising granulomata, primarily affecting the lungs, the upper respiratory tract, and kidneys. Almost all patients have upper and lower respiratory involvement; up to 85% of patients with GPA develop kidney disease within two years of diagnosis. Cutaneous, neurological, and ocular manifestations are also seen with varying frequencies. However, cardiac manifestations of the disease are rare and scarcely reported in the literature. Here, we report a case of a 65-year-old female with an initial diagnosis of pulmonary aspergillosis based on the presence of septate hyphae branching at acute angles on lung biopsy and elevated serum galactomannan, who, over the following months, developed a multitude of issues such as myocardial infarction, sterile endocarditis, splenic infarction, and heart block, as well as the challenges faced in establishing a diagnosis and managing its complications.

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis characterized by inflammation and damage to the walls of small blood vessels. It typically presents with palpable purpura and can be associated with various systemic conditions. Although its etiology is diverse, LCV has been associated with systemic diseases, infections, medications, and autoimmune disorders. Here, we present a case of LCV in a patient with decompensated heart failure. A 58-year-old man presented with progressively deteriorating swelling in both his lower limbs and scrotum, a persistent dry cough associated with minor ulcerative lesions on his shins, and a patchy rash with pustules and flat reddish spots. He was hospitalized three days prior due to atrial fibrillation and rapid ventricular rate, for which he was commenced on amiodarone. This rash persisted for three days, yet he denied experiencing any discomfort or itchiness along with it. Based on his clinical picture, laboratory evaluations, and imaging findings, he was diagnosed with LCV induced by amiodarone.

Neuroimaging has a key role in identifying small-vessel vasculitis from common diseases it mimics, such as multiple sclerosis. Oftentimes, a multitude of these conditions present similarly, and thus diagnosis is difficult. To date, there is no standardized method to differentiate between these diseases. This review identifies and presents existing scoring tools that could serve as a starting point for integrating artificial intelligence/machine learning (AI/ML) into the clinical decision-making process for these rare diseases. A scoping literature review of EMBASE and MEDLINE included 114 articles to evaluate what criteria exist to diagnose small-vessel vasculitis and common mimics. This paper presents the existing criteria of small-vessel vasculitis conditions and mimics them to guide the future integration of AI/ML algorithms to aid in diagnosing these conditions, which present similarly and non-specifically.

UNASSIGNED: Data on non-infectious cryoglobulinemic vasculitis (NICV) is scarce, especially concerning the management of relapses, which are troublesome. We aimed to investigate risk factors for relapse in NICV.
UNASSIGNED: A systematic literature search of CINAHL, Embase, MEDLINE, Scopus, and the Web of Science databases was implemented until April 2023. Eligible studies included randomized control trials, observational studies, and case series with ≥4 patients. Two reviewers independently extracted data and assessed the quality of the eligible studies.
UNASSIGNED: A total of 3,724 articles were retrieved from a database search, with 27 studies meeting the inclusion criteria for review. Most studies (n = 23) detailed relapses, with the time to relapse varying between 1 and 80 months. The relapse rate was reported at 28% in Type I NICV and ranged from 22% to 60% in mixed NICV. Risk factors for relapse in NICV were identified based on the cryoglobulin subtype and correlated with clinical and immunological responses to varying treatment regimens. Type I NICV with an associated lymphoproliferative disorder exhibited a response-relapse pattern. Cutaneous and articular involvement and incomplete clinical and immunological responses to treatment, particularly corticosteroid monotherapy and occasionally rituximab, influence the risk of relapse in Type II and Type III NICV.
UNASSIGNED: Our findings underscore the significance of attaining both clinical and immunological responses and identifying risk factors for relapse in NICV. Appropriate risk stratification for NICV patients is essential for the successful implementation of effective treatment strategies.
UNASSIGNED: https://www.crd.york.ac.uk/prospero/, identifier CRD42023408140.

A 65-year-old man developed palpable purpuric papules and plaques on his lower extremities, which quickly spread to his trunk and upper extremity after being prescribed cephalexin and doxycycline in the emergency room. Here, we define the details of a textbook-like presentation of IgA vasculitis, formerly referred to as Henoch-Schönlein purpura, in an adult.

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Background: IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin, GI tract, joints, and kidneys are frequently affected and considered, central nervous system (CNS) involvement of this disease is underestimated. Methods: We provide a case report and systematically review the literature on IgAV, collecting data on the spectrum of neurological manifestations. Results: We report on a 7-year-old girl with IgAV who presented with diplopia and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic resonance imaging was consistent with posterior reversible encephalopathy syndrome (PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of the developing disease in the initial phase of PRES manifestation. Renal disease and other secondary causes for PRES were also excluded. Supportive- and steroid treatment resulted in restitution ad integrum. Reviewing the literature, we identified 28 other cases of IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema, or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES. The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias toward gender or ethnic background. Treatment regimens varied from watchful waiting to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent CNS impairment. Conclusion: Collectively, our data demonstrate that (I) severe CNS involvement such as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which hampers treatment decisions. We, therefore, conclude that clinical awareness and the collection of structured data are necessary to elucidate the pathophysiological connection of IgAV and PRES.

Leukocytoclastic vasculitis (LCV), also known as small-vessel cutaneous vasculitis, is rarely seen in the setting of staphylococcal infection without bacteremia. Here, we present a case of LCV in a 61-year-old male with chronic staphylococcal osteomyelitis without any evidence of bacteremia. The rash resolved with the treatment of osteomyelitis. As seen in this case, local infection should be considered in the differential diagnosis of LCV even when there is no evidence of bacteremia.

Anti-neutrophil cytoplasmic antibodies (ANCA) are a group of autoantibodies, predominantly IgG, involved in the pathogenesis of several autoimmune disorders, detected either through indirect immunofluorescence or enzyme-linked immunosorbent assay. By means of indirect immunofluorescence, the main patterns are C-ANCA (cytoplasmic) and P-ANCA (perinuclear), while proteinase 3 (PR3) and myeloperoxidase (MPO) represent the main autoantigens in granulomatosis with polyangiitis and microscopic polyangiitis, both belonging to the family of ANCA-associated vasculitis (AAV). While several experiments established the pathogenicity of MPO-ANCA, evidence remains elusive for PR3-ANCA and an additional target antigen, i.e. LAMP2, has been postulated with specific clinical relevance. The presence of a subset of AAV without ANCA may be explained by the presence of further target antigens or the presence of molecules in blood which make ANCA undetectable. A rise in ANCA titers is not necessarily predictive of a flare of disease in AAV if not accompanied by clinical manifestations. ANCA may develop through variable mechanisms, such as autoantigen complementarity, apoptosis impairment, neutrophil extracellular traps dysfunction and molecular mimicry. We will provide herein a comprehensive review of the available evidence on the biological mechanisms, pathogenetic role, and clinical implications of ANCA testing and disease management. Further, we will address the remaining open challenges in the field, including the role of ANCA in inflammatory bowel disease and in cocaine-induced vasculitis.