Pulmonary Vasculitis

Lally, Lindsay; Spiera, Robert

doi:10.1016/j.rdc.2015.01.004

Cited by 34 publications

(13 citation statements)

References 83 publications

(91 reference statements)

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“…ANCA-associated vasculitis (AVV) is a life-threatening condition and patients typically sustain renal and pulmonary injury ( Holguin et al, 2008 , Lally and Spiera, 2015 , Cid, 2012 ). It has further confirmed that the impaired endothelial function contributes to the development and progression of diverse vascular, inflammatory, and pulmonary hemorrhage ( Verma et al, 2003 , Dzau et al, 2002 ).…”

Section: Discussionmentioning

confidence: 99%

Enho Mutations Causing Low Adropin: A Possible Pathomechanism of MPO-ANCA Associated Lung Injury

et al. 2016

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BackgroundMyeloperoxidase (MPO) anti-neutrophil cytoplasm autoantibody (ANCA)-associated vasculitis commonly causes life-threatening pulmonary alveolar hemorrhage or fibrosis. Only a limited number of candidate gene variants have been explored, but hitherto, are not widely confirmed. In the present study, we investigated the importance of energy homeostasis associated gene (Enho) mutations and adropin deficiency in the development of MPO-ANCA associated lung injury.MethodsWe analyzed the peripheral blood mononuclear cells from 152 unrelated patients and 220 population-matched healthy individuals for genetic variations in Enho. Functional studies with adropin knockout (AdrKO) on C57BL/6J mice were also performed.FindingsSequencing revealed six patients with p.Ser43Thr and that five patients shared Cys56Trp amino acid substitution in Enho. Serum concentration of adropin was significantly lower in patients than that of the healthy subjects (P < 0.0001), especially those with Enho mutations. In vivo, homo- and heterozygous carriers of the null adropin allele exhibited MPO-ANCA associated pulmonary alveolar hemorrhage as compared to wild-type mice. AdrKO mice exhibit reduced eNOS (Ser1177) and Akt1 (Ser473) phosphorylation and loss of Treg cells.InterpretationOur findings indicate that the presence of Enho mutations or adropin-deficiency is a probable molecular basis for the initial events triggered in MPO-ANCA associated lung injury.

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Section: Discussionmentioning

confidence: 99%

Enho Mutations Causing Low Adropin: A Possible Pathomechanism of MPO-ANCA Associated Lung Injury

et al. 2016

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“…El retorno hemorrágico seriado del lavado broncoalveolar, confirma el diagnóstico de HAD. La presencia de hemosiderofagos >20% es compatible con este diagnóstico, pero tiene una especificidad limitada para determinar la causa de la enfermedad [9] . Se diferencian tres entidades, según hallazgos histopatológicos: capilaritis pulmonar, hemorragia alveolar blanda y daño alveolar difuso, siendo la primera la más común [3,10,11] .…”

Section: Discussionunclassified

Hemorragia alveolar por montelukast: reporte de caso

2020

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La hemorragia alveolar difusa es un hallazgo clínico patológico, consecuencia de una lesión en la microcirculación pulmonar que se presenta con sintomatología inespecífica e incluso en pacientes asintomáticos. Se diferencian tres diagnósticos histopatológicos, siendo la capilaritis el más común y secundario a patologías autoinmunes. Se presenta el caso de un paciente, con hallazgo imagenológico y en lavado broncoalveolar, compatible con hemorragia alveolar difusa en quien se descartó como causa, enfermedades autoinmunes e infecciosas; durante la anamnesis, se identificó inicio reciente de montelukast, con relación temporal, respuesta a suspensión, cambio imagenológico y escalas de probabilidad de reacción adversa de Naranjo que apoya el diagnóstico.

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“…atherosclerosis), pulmonary hypertension (PH), or post-stenotic dilatation from pulmonic valve stenosis. 10 , 11 Systemic vasculitides may involve the large pulmonary arteries leading to PAA, 12 but this is rarely an isolated finding without evidence of vasculitis elsewhere.…”

Section: Discussionmentioning

confidence: 99%

Isolated large vessel pulmonary vasculitis leading to pulmonary artery aneurysm formation: a case report and literature review

Moghaddam

Dehghan

et al. 2018

Pulm. circ.

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Pulmonary artery (PA) vasculitis occurs in association with primary vasculitides—in particular, giant cell arteritis, Takayasu’s arteritis, or Behçet’s disease—or secondary vasculitis as a result of infections or malignancy. However, PA vasculitis in isolation and with concomitant aneurysmal dilation is an unusual finding. We present a rare case of PA aneurysm secondary to isolated PA vasculitis in an asymptomatic patient with no features of systemic vasculitis. This case highlights one of the first cases of PA vasculitis managed with surgical resection alone.

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Pulmonary Vasculitis

Cited by 34 publications

References 83 publications

Enho Mutations Causing Low Adropin: A Possible Pathomechanism of MPO-ANCA Associated Lung Injury

Enho Mutations Causing Low Adropin: A Possible Pathomechanism of MPO-ANCA Associated Lung Injury

Hemorragia alveolar por montelukast: reporte de caso

Isolated large vessel pulmonary vasculitis leading to pulmonary artery aneurysm formation: a case report and literature review

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