Pauci‐immune glomerulonephritis: the <scp>ANCA</scp>‐negative side of the coin

Ramirez, Giuseppe A.; Mason, Justin C.; Tombetti, Enrico

doi:10.1111/1756-185x.12836

Cited by 3 publications

(3 citation statements)

References 18 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is an antineutrophil cytoplasmic antibodies- (ANCA-) associated vasculitis (AAV) that pathogenically lies at the crossroads between asthma, small vessel inflammation, and eosinophil-mediated tissue injury [ 274 , 275 ]. This complex scenario, combined with the low prevalence of EGPA in the general population [ 276 ], has delayed our understanding of the pathophysiological mechanisms underlying the disease and the development of (a) reliable tools for the diagnosis and (b) novel therapeutic options, especially in comparison with other AAVs [ 80 , 277 – 280 ]. Of even higher concern, there is still also a lack of universal consensus on the nosologic classification of EGPA.…”

Section: Eosinophils In Immune-mediated Diseasesmentioning

confidence: 99%

Eosinophils from Physiology to Disease: A Comprehensive Review

Ramirez

Yacoub

Ripa

et al. 2018

BioMed Research International

Self Cite

224

198

View full text Add to dashboard Cite

Despite being the second least represented granulocyte subpopulation in the circulating blood, eosinophils are receiving a growing interest from the scientific community, due to their complex pathophysiological role in a broad range of local and systemic inflammatory diseases as well as in cancer and thrombosis. Eosinophils are crucial for the control of parasitic infections, but increasing evidence suggests that they are also involved in vital defensive tasks against bacterial and viral pathogens including HIV. On the other side of the coin, eosinophil potential to provide a strong defensive response against invading microbes through the release of a large array of compounds can prove toxic to the host tissues and dysregulate haemostasis. Increasing knowledge of eosinophil biological behaviour is leading to major changes in established paradigms for the classification and diagnosis of several allergic and autoimmune diseases and has paved the way to a “golden age” of eosinophil-targeted agents. In this review, we provide a comprehensive update on the pathophysiological role of eosinophils in host defence, inflammation, and cancer and discuss potential clinical implications in light of recent therapeutic advances.

show abstract

Section: Eosinophils In Immune-mediated Diseasesmentioning

confidence: 99%

Eosinophils from Physiology to Disease: A Comprehensive Review

Ramirez

Yacoub

Ripa

et al. 2018

BioMed Research International

Self Cite

224

198

View full text Add to dashboard Cite

show abstract

“…Retrospective analysis has reported that the etiology of ANCA negative CrGN is not entirely clear, but the pathogenesis is thought to be due to neutrophil infiltration. Although the idea of the existence of AECA and other autoantibodies with close characteristics of ANCA cannot be ruled out [1,3,13]. Literature also notes a relationship between inflammatory cytokines like interleukins (IL6) and ANCA negative vasculitis [14].…”

Section: Discussionmentioning

confidence: 99%

“…ANCA positivity has been correlated with clinical manifestations, risk of flares, and even treatment responsiveness in addition to being a diagnostic marker and is thought to be responsible for the pathogenesis of GPA until cases of ANCA negative GPA have been reported [2,3]. Indirect immunofluorescence detects two types of ANCA, diffuse cytoplasmic (c-ANCA) and perinuclear/nuclear (p-ANCA).…”

Section: Introductionmentioning

confidence: 99%

When Anti-Neutrophil Cytoplasmic Antibody Fails: A Case of Anti-Neutrophil Cytoplasmic Antibody Negative Granulomatosis With Polyangiitis

Gangireddy¹,

Kanderi²,

Gomez³

et al. 2020

Cureus

View full text Add to dashboard Cite

Granulomatosis with polyangiitis (GPA) is a vasculitis of small and medium-sized vessels and presents with varying signs and symptoms. It includes upper and lower airway manifestations and glomerulonephritis with a positive antineutrophil cytoplasmic antibody (ANCA) in serology in 90% of cases. However, about 10% of cases with GPA can have negative serology, often resulting in a diagnostic delay. Obtaining a tissue pathology is needed to confirm GPA. Here we present a 77-year-old male who presented with generalized weakness and loss of appetite and was found to have glomerulonephritis and bilateral opacities in the lungs with a negative ANCA. He was diagnosed with ANCA negative granulomatosis with polyangiitis after a renal biopsy revealed necrotizing inflammation with crescent formation. He was successfully treated with systemic glucocorticoids and rituximab. In conclusion, prompt diagnosis and treatment of ANCA negative vasculitis are required to decrease mortality.

show abstract