The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey

Corral‐Gudino, Luis; González-Vázquez, Elvira; Calero-Paniagua, Ismael; Perez-Garrido, Laura; Cusácovich, Iván; Rivas-Lamazares, Alicia; Quesada-Moreno, Alba; González-Fernández, Ana; Mora-Peña, Damián; Lerma-Márquez, Jose Luis; Pino-Montes, Javier del

doi:10.1007/s00296-019-04406-5

Cited by 12 publications

(13 citation statements)

References 29 publications

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“…Treatment and prognosis of vasculitis depend on what type of vasculitis an individual patient has, but individual cases of vasculitis are often difficult to classify. 5 Our case illustrates this aspect of vasculitis. The 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides provides definitions of the types of vasculitis.…”

Section: Discussionmentioning

confidence: 71%

Vasculitis with a neuromuscular presentation and associated cancer revealed by autopsy

Nichols

Karivelil

2020

Autops. Case Rep.

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Section: Discussionmentioning

confidence: 71%

Vasculitis with a neuromuscular presentation and associated cancer revealed by autopsy

Nichols

Karivelil

2020

Autops. Case Rep.

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“…The experience acquired from large numbers of patients in single-center [ 23 – 25 ], polycenter [ 30 – 32 ], and nationwide [ 33 – 35 ] studies has clearly shown that GPA is a treacherous, necrotizing, granulomatous vasculitis of the small vessels, involving a single organ or characterized by multisystem involvement. Patients should be diagnosed as quickly as possible to avoid disease progression to severe or even life-threatening stages.…”

Section: Discussionmentioning

confidence: 99%

The eye is a common site of granulomatosis with polyangiitis. A collaborative study

et al. 2023

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Background Ocular manifestations of granulomatosis with polyangiitis (GPA) have been reported in a limited number of studies and with largely variable frequency. Here we report on the clinical, diagnostic, and therapeutic features of a cohort of 63 GPA patients, with particular regard to 22 of them with ophthalmic involvement (35%). Methods Clinical manifestations, results of immunological findings, histopathological pictures, imaging data, Birmingham Vasculitis Activity Score, therapeutic regimens, and outcomes were retrospectively analyzed. At diagnosis, in addition to a structured clinical assessment, all patients underwent a comprehensive ophthalmologic examination. Results The most frequently involved organs were kidneys, lungs, ear/nose/throat, and eyes. Ocular manifestations were bilateral in 32%. The three most commonly diagnosed ophthalmologic manifestations were scleritis (36%), retro-orbital pseudotumor or orbital mass (23%), and episcleritis (13%). Ocular and systemic involvement were simultaneously present at onset in 41% of the patients; systemic involvement was followed by ocular lesions in 36%; ocular inflammation was followed by systemic manifestations in 18%; and an orbital mass in the absence of systemic disease characterized 5%. Glucocorticoids plus cyclophosphamide and glucocorticoids plus rituximab were the combined therapies most frequently employed during remission induction and remission maintenance, respectively. Persistent ophthalmologic and extra-ocular remissions were achieved in 77 and 64% of the patients, respectively. One to three systemic relapses were diagnosed in 7 patients (31.8%). At the last follow-up, a visual outcome 20/40 or better in 31 (70%) of 44 eyes was determined. Conclusions The eye was involved in over one third of our patients with GPA. Increased awareness, early diagnosis, and multi-specialty collaboration are critical in achieving a favorable outcome of GPA.

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“…Indeed, the classification of ANCA-associated small-vessel vasculitis remains complex in actual clinical practice. As a matter of fact, nearly 50% of patients are classified according to ANCA specificities without being able to fulfill the current diagnostic criteria (9).…”

Section: Discussionmentioning

confidence: 99%

Microscopic Polyangiitis With Selective Involvement of Central and Peripheral Nervous System: A Case Report

et al. 2020

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Background: Microscopic polyangiitis (MPA) is a necrotizing vasculitis that affects predominantly small-sized vessels in many organ systems. The disease generally causes glomerulonephritis, pulmonary damage, arthritis, and neuropathy. An exclusive involvement of both central nervous system (CNS) and peripheral nervous system (PNS) is extremely rare. Case Presentation: A 62-year-old woman was admitted to our hospital with a 3 months history of right foot drop, recently complicated by intense myalgia, arthralgia, and allodynia to tactile, vibratory, and pressure stimuli. Since blood tests revealed elevated inflammatory indexes, we suspected either infectious or immune-mediated disorders. Chest radiograph, blood culture series, and echocardiogram revealed normal findings, while urinalysis showed a bacterial infection that was successfully treated. The neurophysiological findings were compatible with multiple mononeuritis, and a brain MRI evidenced ischemic lesions of both basal ganglia and thalamus. A wide-spectrum autoantibody assay revealed the presence of high-titer perinuclear anti-neutrophil cytoplasmic antibodies specific for myeloperoxidase (MPO-ANCA). According to these findings, the diagnosis of MPA was made, and the patient was successfully treated with intravenous (IV) methylprednisolone, followed by two doses of rituximab. Conclusions: An assessment of both CNS and PNS should be included in the diagnostic evaluation of MPA. The involvement of the PNS may raise the risk of a relapsing course and treatment failure, therefore it should be considered in the choice of induction and maintenance therapy.

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The complexity of classifying ANCA-associated small-vessel vasculitis in actual clinical practice: data from a multicenter retrospective survey

Cited by 12 publications

References 29 publications

Vasculitis with a neuromuscular presentation and associated cancer revealed by autopsy

Vasculitis with a neuromuscular presentation and associated cancer revealed by autopsy

The eye is a common site of granulomatosis with polyangiitis. A collaborative study

Microscopic Polyangiitis With Selective Involvement of Central and Peripheral Nervous System: A Case Report

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