Spectrum of Large- and Medium-Vessel Vasculitis in Adults: Neoplastic, Infectious, Drug-Induced, Autoinflammatory, and Primary Immunodeficiency Diseases

Abstract

Purpose of Review To provide a comprehensive review of drugs and neoplastic, infectious, autoinflammatory, and immunodeficiency diseases causing medium- to large-vessel vasculitis in adults with emphasis on information essential for the initial diagnostic process. Recent Findings Entities with medium- to large-vessel vasculitis as clinical manifestations have been described recently (e.g., adenosine deaminase-2 deficiency, VEXAS-Syndrome), and vasculitis in established autoinflammatory or immunodeficiency diseases is increasingly being identified. Summary In the diagnostic process of medium- to large-vessel vasculitis in adults, a large variety of rare diseases should be included in the differential diagnosis, especially if diagnosis is made without histologic confirmation and in younger patients. Although these disorders should be considered, they will undoubtedly remain rare in daily practice.

EULAR recommendations for the use of imaging in large vessel vasculitis in clinical practice: 2023 update

“…Additional testing is also important if GCA mimickers are suspected 36. In some diseases such as granulomatosis with polyangiitis, lymphoma or amyloidosis, imaging abnormalities similar to those found in GCA have been described; relevant differential diagnoses need therefore to be excluded on clinical grounds, by laboratory exams and/or histology 37–40…”

The “Viral” Form of Polyarteritis Nodosa (PAN)—A Distinct Entity: A Case Based Review

“… Other forms of vasculitis: There are various types of vasculitis that can present with similar clinical features, such as giant cell arteritis or Takayasu arteritis. The distinction between these different forms of vasculitis is essential for the appropriate treatment, and in our case, the differential diagnosis was performed through laboratory determinations (absence of ANCA antibodies) and histopathological examination [ 6 , 8 , 9 ]. Connective tissue diseases: Conditions such as systemic lupus erythematosus (SLE), rheumatoid arthritis, and Sjögren’s syndrome can present with clinical manifestations of systemic vasculitis.…”

“…It is a chronic inflammatory condition characterized by recurrent oral and genital ulcers, skin lesions, eye inflammation, and various systemic manifestations. Although it primarily affects the mucous membranes, Behçet’s disease can also involve blood vessels, leading to vasculitis that can resemble PAN-HVB [ 6 , 8 , 9 ]. The paraclinical data excluded the presence of these conditions in our patient.…”

“… The paraclinical data excluded the presence of these conditions in our patient. Drug-induced vasculitis: Certain medications, such as certain antibiotics, anticonvulsants, and nonsteroidal anti-inflammatory drugs (NSAIDs), can cause drug-induced vasculitis that mimics the symptoms of polyarteritis nodosa [ 8 , 9 ]. Malignancies: In some cases, certain types of cancer, including lymphomas and leukemia, can have vasculitis-like symptoms.…”

New imaging strategy for large vessel vasculitis (based on the EULAR-2023 recommendations)

“…При сомнительных результатах инструментального обследования, например, когда утолщение стенки ограниченное или немного превышает нормальное значение, необходимо дополнительное исследование для подтверждения диагноза ГКА [28]. При некоторых заболеваниях, таких как гранулематоз с полиангиитом, лимфома или амилоидоз, описаны изменения, аналогичные обнаруживаемым при ГКА, поэтому соответствующие диагнозы необходимо исключить по клиническим признакам, с помощью лабораторного и/или гистологического исследования [29][30][31].…”