The term vasculitis is used to describe a heterogenous group of rare disease characterised by immune-mediated damage to the blood vessels resulting in downstream ischaemia, tissue injury and end-organ damage. The classification of vasculitis is based upon the size of the vessels typically affected, which results in the broad range of clinical features associated with the different forms of vasculitis. Advances in treatment of vasculitis from the emergence of corticosteroids to the modern application of targeted immunomodulatory treatments have resulted in lower mortality associated with vasculitis but it is still associated with high healthcare resources and disease-related morbidity. Vasculitis is typically managed by rheumatologists working alongside a broad multi-disciplinary team with input from a range of medical and surgical specialties, reflecting its truly multisystem nature. Vasculitis will often present on general medical wards, with diagnosis sometimes delayed as more common infective and malignant diseases are excluded. General physicians require an understanding of the clinical features of vasculitis and have knowledge of the relevant practical approaches to investigation and management of suspected vasculitis. The present review shall provide an over-arching summary of the different forms of vasculitis and a practical approach to investigation and management aimed at the general physician.

Cholesterol embolism causes various organ dysfunctions, including skin, kidney, and gastrointestinal tract dysfunction, as well as immunological abnormalities, such as hypocomplementemia and eosinophilia. However, only a few cases of vasculitis accompanied by cholesterol embolism have been reported.

We present the case of an 82-year-old man with cholesterol embolism who also developed small-vessel vasculitis of the skin and muscles. The patient had a persistent fever, and blood tests showed eosinophilia and hypocomplementemia. Two months later, the patient developed a skin rash and myalgia in the thighs. Magnetic resonance imaging of the thighs revealed diffuse intramuscular hyperintensities on T2-weighted images and short tau inversion recovery sequences in the hamstrings and quadriceps femoris. Histological findings of the skin and muscle revealed small-vessel vasculitis, and random skin biopsy revealed cholesterol embolism. We diagnosed the patient with cholesterol embolism accompanied by small-vessel vasculitis of the skin and femoral muscles. Methylprednisolone was administered intravenously, and oral prednisolone was initiated. Muscle tenderness improved rapidly after the initiation of glucocorticoid therapy. However, he developed superior mesenteric artery embolization and died.

Our case demonstrates that cholesterol embolism can be accompanied by small-vessel vasculitis of the skin and muscles.

Bartonella species are fastidious, intracellular bacteria responsible for an expanding array of human pathologies. Most are considered to be transmitted by direct inoculation with infected bodily fluids from a mammalian reservoir species or vector-transmitted through a variety of arthropod species and their excrement. However, there are mounting reports of infection in the absence of documented animal or vector contact. A variety of Bartonella species have been documented in conditions affecting both the peripheral and central nervous systems. More common conditions, including neuroretinitis, are often associated with Bartonella henselae. However, Bartonella quintana, the agent of trench fever, as well as emerging pathogens related to rodent reservoir species, B. grahamii and B. elizabethae, have also been documented. Encephalitis and encephalopathy, also most often associated with B. henselae, have been reported with B. quintana, B. washoensis (ground squirrels) and B. vinsonii subsp. vinsonii (voles) infections. Bartonella infections have also been associated with peripheral neuropathies, such as cranial nerve paresis and neuropathic pain, including infection with less commonly encountered species such as Bartonella koehlerae. Recently, molecular diagnostic testing revealed that DNA from Bartonella spp. was found to be more prevalent in blood of patients with neuropsychiatric disorders such as schizophrenia and psychoses compared to healthy controls.

A systematic literature search was conducted on PubMed, Google Scholar and Web of Science. Search terms included Bartonella and specific neurological conditions and focused on peer-reviewed case reports published after 2012 pursuant to a prior review, with limited exceptions for conditions not previously covered. Published diagnostic testing, serology, molecular testing or pathology, were necessary for inclusion, except for one case which had clinical and epidemiological evidence consistent with diagnosis along with follow-up.

Neurobartonelloses included neuralgic amyotrophy, complex regional pain syndrome, chronic inflammatory demyelinating polyneuropathy, cranial nerve paralysis, Guillain-Barré syndrome, peripheral vasculitic polyneuropathy, acute transverse myelopathy, neuroretinitis, encephalitis/encephalopathy, cerebral vasculitis/aneurysm and neuropsychiatric conditions.

The breadth of reported symptoms and clinical syndromes associated with an increasing number of Bartonella species continues to expand. Increased clinical awareness of this important zoonotic pathogen is necessary to advance One Health among the medical and veterinary communities.

Vasculitis is characterized by the inflammation of blood vessels. Vasculitides refers to the different forms of vasculitis, often classified according to the size of the blood vessel that is involved. Vasculitis may occur as a primary process or secondary to many systemic diseases. This topic provides an overview of the clinical features, diagnosis, and classification of the different forms of vasculitides.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a rare heterogeneous disease in which treatment must be initiated early to prevent irreversible organ damage and death. There are several diseases that can mimic AAV, even in the presence of positive ANCA serology and/or histological evidence of vasculitis, as demonstrated in this case series. We reflect on the diagnostic approach of patients with AAV and provide an overview of AAV-mimicking diseases that can be considered in patients with atypical disease presentation or course.

Polyarteritis nodosa (PAN) is a rare autoimmune disease that affects medium-sized arteries and causes inflammation and damage to the blood vessel walls. Testicular pain is an uncommon symptom of PAN but can occur in rare cases. This specific symptom may be useful in diagnosing older patients with limited tissue access because of their vulnerability and high risk for biopsy complications. We report the case of a 78-year-old male patient with progressive fatigue and walking difficulty. After ruling out various forms of vasculitis and malignancy, we diagnosed the patient with PAN and intensively treated him with rituximab, which successfully cured his symptoms. This case report highlights the importance of intensively ruling out possible diagnoses mimicking vasculitis and treating vasculitis with a tentative diagnosis of PAN in older patients in rural hospitals. The progressive clinical course of vasculitis may devastate older patients' activities of daily living (ADLs). PAN may particularly affect older patients with possible hepatitis B infections. Thus, shared decision-making and prompt intensive treatment should be considered.

To provide a comprehensive overview of the spectrum of large and medium vessel vasculitis in adults with primary vasculitides, arthritides, connective tissue, and fibroinflammatory diseases as well as vasculitis mimics, for an efficient differential diagnosis and initial diagnostic approach.

Imaging has had a tremendous impact on the diagnosis of medium to large vessel vasculitis, now often replacing histopathologic confirmation and identifying new disease manifestations (e.g., intracranial disease in giant cell arteritis; vascular manifestations of IgG4-related disease). Novel diseases or syndromes involving blood vessels have been described (e.g., VEXAS-Syndrome with polychondritis). The use of the terms "medium" or "large" vessel varies considerably between medical specialties. The differential diagnosis of large and medium vessel vasculitis is becoming increasingly complex as new entities or disease manifestations of known inflammatory rheumatic diseases are regularly identified. A more precise and widely recognized definition of the vessel sizes would make future research more comparable.

A 2-stage therapeutic approach is now applied as standard-of-care to treat ANCA-associated vasculitides (AAVs): first, glucocorticoids (GCs) combined with cyclophosphamide (CYC) or rituximab (RTX) to induce remission, then relapse prevention with remission-maintenance therapy. Nonetheless, a substantial risk of relapse persists.

The authors provide an overview of the current state of maintenance therapies, and discuss new strategies recommended to prevent and treat relapses, focusing on granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA).

For remission-induction after GPA or MPA relapse with organ-threatening manifestations, reintroduction or intensification of the GC dose in combination with CYC or RTX cycle is recommended; we prefer RTX in light of its superior responses obtained in patients with relapsing disease. Rapid tapering of GCs has been shown not to alter AAV evolution while decreasing the risk of serious infections. In contrast, for non-severe, active MPA, we recommend GCs alone as first-line therapy. For patients whose MPA remains uncontrolled by GCs alone, immunosuppressant adjunction can be a GC-sparing option or to counter GC intolerance. Once remission is achieved, we recommend prolonged maintenance therapy with preemptive low-dose (500 mg) RTX infusion biannually.

Purpura is common in pediatric patients, mostly diagnosed as IgA-related vasculitis (Henoch-Schönlein purpura), idiopathic thrombocytopenic purpura (ITP), and thrombotic thrombocytopenic purpura (TTP). However, in some cases, for example, cases with dermatitis artefacta, it could puzzle a physician or pediatrician for a long time, with great challenges in diagnosis.

We present the case of a 13-year-old boy with recurrent painful purpura on both upper limbs. The physical exam was unremarkable, except for right blepharoptosis and scars from burns. The diagnostic tests were normal. Through repeated communication, the patient was finally diagnosed as having dermatitis artefacta, accompanied by underlying psychological problems.

Before dermatitis artefacta was diagnosed, we spent a lot of money and effort on the diagnosis. Therefore, in order to determine the diagnosis as soon as possible and save on unnecessary medical expenses, we propose a rapid process for the diagnosis of purpura of dermatitis artefacta in children.

The pediatric vasculitides are a relatively uncommon and heterogeneous group of disorders characterized by vessel inflammation, often with cardiothoracic involvement. Diagnosis and monitoring are often clinically challenging because of the nonspecific symptoms and laboratory markers. Thus, imaging has assumed increasing importance for early detection of disease activity, extent and complications as well as long-term monitoring pre- and post-treatment. Herein, we review the major pediatric vasculitides with frequent chest manifestations, including Takayasu arteritis, Kawasaki disease, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, Behçet disease and potential mimics. We highlight key clinical features and management considerations, emphasizing the central role of imaging.