Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa

Naidu, Gsrsnk; Kopp, Chirag Rajkumar; Sharma, Vikas; Singhal, Manphool; Pinto, Benzeeta; Dhir, Varun; Nada, Ritambhra; Minz, Ranjana W.; Jain, Sanjay; Sharma, Aman

doi:10.1007/s00296-021-04867-7

Cited by 5 publications

(4 citation statements)

References 16 publications

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“…Currently, the American College of Rheumatology (ACR) criteria published in 1990 are still in use to classify PAN 207 . However, new criteria are under investigation with the aim to increase sensitivity and specificity 208,209 . Over 60 disease‐associated mutations have been identified in all domains of ADA2, affecting its catalytic activity, protein dimerization, and secretion.…”

Section: Medium‐vessel Vasculitismentioning

confidence: 99%

“…207 However, new criteria are under investigation with the aim to increase sensitivity and specificity. 208,209 Over 60 disease-associated mutations have been identified in all domains of ADA2, affecting its catalytic activity, protein dimerization, and secretion. ADA2 is highly expressed in myeloid cells but its role in vasculitis pathogenesis has not yet been elucidated.…”

Section: Polyarteritis Nodosa: Neutrophils and Aneurysm As Potential ...mentioning

confidence: 99%

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The neutrophil: A key resourceful agent in immune‐mediated vasculitis

Aymonnier

Amsler

Lamprecht

et al. 2022

Immunological Reviews

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Neutrophils are no longer considered to be a highly differentiated and uniform population whose functions are solely confined to antimicrobial activities. This former restricted view of neutrophils as fixed effectors and that lack of plasticity was partly due to their characteristic short lifespan in circulation and very low transcriptional activity. 1-3 However, there has been increasing appreciation of the plasticity of neutrophils with the ongoing categorization of their subsets, including myeloid-derived suppressor cells and lowdensity neutrophils. Newly recognized neutrophil functions include the ability to communicate and modulate with almost all immune

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Section: Medium‐vessel Vasculitismentioning

confidence: 99%

Section: Polyarteritis Nodosa: Neutrophils and Aneurysm As Potential ...mentioning

confidence: 99%

The neutrophil: A key resourceful agent in immune‐mediated vasculitis

Aymonnier

Amsler

Lamprecht

et al. 2022

Immunological Reviews

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“…Estos criterios tienen una sensibilidad y especificidad para el diagnóstico del 82 y 87 por ciento respectivamente. Naidu et al, 2021) -Laboratorio: No existen pruebas de laboratorios específicas de diagnóstico de PAN, se realizan de rutina, hemograma, enzimas musculares, estudios de función hepática y renal, serologías de hepatitis B y C, uroanálisis De Virgilio et al, 2016, Iglesias et al, 1986. La elevación de la velocidad de sedimentación globular (VSG), y proteína C reactiva (PCR) son lo más frecuente, pero no aportan un valor significativo diagnóstico, ni descarta la enfermedad (Michelitti, 2022); es valioso reducir el diagnóstico diferencial por lo que se pueden hacer adicionales (Halabi et al, 2021) como: Anticuerpos anticitoplasma de neutrófilos (ANCA), anticuerpos antinucleares (ANA), anticuerpos anti-Ro, anticuerpos anti-La, anticuerpos anti ribonucleoproteína (Anti RNP), anticuerpos anti Smith, (Fett, 2022) componentes de complemento (C3 y C4), crioglobulinas, electroforesis en suero y orina, (Helfgott y Bhattacharyya, 2022;Schnappauf et al, 2021).…”

Section: Introductionunclassified

“…Una manera que se ha evidenciado para diferenciarla de otras vasculitis es mediante el resultado de ANCA séricos negativos. (Chercoles y Fong, 2016;Helfgott y Bhattacharyya, 2022;Zahoor et al, 2022;Gaviria et al, 2020, Naidu et al, 2021 -Imágenes: Angiorresonancia, Angiotomografía y/o Angiografía en las que se pueden observar dilataciones o estrechamientos de las arterias afectadas (Munera-Campos et al, 2020;Gabsi et al, 2021) como lo es la presencia de micro aneurismas, los cuales son distintivos de la PAN. (Wuelan, 2022) -Biopsia: se observa vasculitis leucocitoclástica de arterias de mediano calibre, necrosis de las paredes del vaso, asociado a marcada inflamación perilesional (Jennette, 2013;Sociedad Argentina de Reumatología, 2022), la biopsia debe centrarse sobre un nódulo sensible o una lesión purpúrica (Michelitti, 2022).…”

Section: Introductionunclassified

Poliarteritis nodosa. A propósito de un caso

Moreno,

Bustamante,

Neira

2023

GICOS

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La poliarteritis nodosa es una vasculitis necrotizante de rara presentación que afecta vasos de mediano y pequeño calibre, que puede afectar desde un órgano hasta sistemas del cuerpo humano, por lo tanto, su presentación clínica es variable y su etiología es predominantemente idiopática. Respecto a su diagnóstico, se deben descartar otras patologías y correlacionar la clínica, las imágenes diagnósticas y paraclínicos disponibles, con el fin de brindar una terapia adecuada y oportuna, teniendo en cuenta que la terapia farmacológica ayuda a disminuir el daño y la progresión más no es una cura. Se presenta el caso de un paciente de 46 años con poliarteritis nodosa cutánea rápidamente progresiva que llevó a necrosis de los dedos de las 4 extremidades, se logró estabilizar y detener su progresión mediante el reconocimiento y manejo farmacológico con glucocorticoides y citotóxicos, sin embargo, requirió amputación.

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A Narrative Review of the Neurological Manifestations of Human Adenosine Deaminase 2 Deficiency

Dzhus,

Ehlers,

Wouters

et al. 2023

J Clin Immunol

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Deficiency of human adenosine deaminase type 2 (DADA2) is a complex systemic autoinflammatory disorder characterized by vasculopathy, immune dysregulation, and hematologic abnormalities. The most notable neurological manifestations of DADA2 are strokes that can manifest with various neurological symptoms and are potentially fatal. However, neurological presentations can be diverse. We here present a review of the neurological manifestations of DADA2 to increase clinical awareness of DADA2 as the underlying diagnosis. We reviewed all published cases of DADA2 from 1 January 2014 until 19 July 2022 found via PubMed. A total of 129 articles describing the clinical features of DADA2 were included in the analysis. Six hundred twenty-eight patients diagnosed with DADA2 were included in the review. 50.3% of patients had at least signs of one reported neurological event, which was the initial or sole manifestation in 5.7% and 0.6%, respectively. 77.5% of patients with neurological manifestations had at least signs of one cerebrovascular accident, with lacunar strokes being the most common and 35.9% of them having multiple stroke episodes. There is a remarkable predilection for the brain stem and deep gray matter, with 37.3% and 41.6% of ischemic strokes, respectively. Other neurological involvement included neuropathies, focal neurological deficits, ophthalmological findings, convulsions, and headaches. In summary, neurological manifestations affect a significant proportion of patients with DADA2, and the phenotype is broad. Neurological manifestations can be the first and single manifestation of DADA2. Therefore, stroke, encephalitis, posterior reversible encephalopathy syndrome, mononeuropathy and polyneuropathy, and Behçet’s disease-like presentations should prompt the neurologist to exclude DADA2, especially but not only in childhood.

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Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa

Cited by 5 publications

References 16 publications

The neutrophil: A key resourceful agent in immune‐mediated vasculitis

The neutrophil: A key resourceful agent in immune‐mediated vasculitis

Poliarteritis nodosa. A propósito de un caso

A Narrative Review of the Neurological Manifestations of Human Adenosine Deaminase 2 Deficiency

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