ANCA associated vasculitis

Hunter, Robert W.; Welsh, Nicola; Farrah, Tariq E.; Gallacher, Peter J.; Dhaun, Neeraj

doi:10.1136/bmj.m1070

Cited by 50 publications

(45 citation statements)

References 18 publications

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“…Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a chronic inflammatory disorder (CID) that usually involves the small-sized vasculatures and has three distinct subtypes: microscopic polyangiitis (MPA), granulomatosis with polyangiitis, and eosinophilic granulomatosis with polyangiitis [1,2]. Generally, AAV involves the vessels and induces necrotizing vasculitis within the arterioles, venules, and capillaries and could present with a wide spectrum of clinical manifestations [3].…”

Section: Introductionmentioning

confidence: 99%

Atherogenic index of plasma predicts cerebrovascular accident occurrence in antineutrophil cytoplasmic antibody-associated vasculitis

et al. 2020

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Background: To investigate whether atherogenic index of plasma (AIP) at diagnosis is associated with the occurrence of cerebrovascular accident (CVA) or coronary artery disease (CAD) in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Methods: The medical records of 167 AAV patients on initial diagnosis was reviewed, and 300 healthy controls were included. AIP was calculated using the following equation: AIP = Log (triglyceride [mg/dL] / high-density lipoprotein cholesterol [mg/dL]). AAV patients were divided into two groups according to the AIP cutoff of 0.11. The event of stroke, transient ischemic attack, and cerebral hemorrhage was recorded as CVA, and CAD events consisted of either myocardial infarction and angina pectoris. CVA-and CAD-free survival rate between those with AIP ≥ 0.11 and < 0.11 were compared by the Kaplan-Meier analysis, and Cox hazard analysis was conducted to identify predictors of CVA. Results: The median age of AAV patients were 59.0 years, and 54 (32.3%) patients were male. One-hundred and fifteen (68.9%) patients had AIP < 0.11 and 52 (31.1%) had AIP ≥ 0.11. The mean Birmingham vasculitis activity score in AAV patients with AIP < 0.11 was lower than that seen in patients with AIP ≥ 0.11 (12.0 vs. 14.0, P = 0.041). AAV patients had a significantly higher AIP compared to controls (mean − 0.01 vs.-0.10, P < 0.001). During follow-up, the occurrence of CVA and CAD was observed in 16 (9.6%) and 14 (8.4%) patients, respectively. In Kaplan-Meier analysis, AAV patients with AIP ≥ 0.11 had significantly lower CVA-free survival rates than in those with AIP < 0.11 (P = 0.027), whereas there was no difference in CAD according to AIP (P = 0.390). Multivariable Cox analysis indicated that AIP ≥ 0.11 at diagnosis was the sole predictor of CVA (Hazard ratio 3.392, 95% confidence interval 1.076, 10.696, P = 0.037).

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Section: Introductionmentioning

confidence: 99%

Atherogenic index of plasma predicts cerebrovascular accident occurrence in antineutrophil cytoplasmic antibody-associated vasculitis

et al. 2020

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“…Clinicians may further attribute specific disease symptoms to more prevalent conditions such as viral pneumonia and asthma. [6][7][8] The most commonly reported prodromal symptoms of ANCA-positive vasculitides and COVID-19 were dyspnoea, fever, and cough. 4,9 In one study, the median delay from initial symptom onset to a diagnosis of ANCApositive vasculitis (or relapse) was significantly longer during the pandemic than previous cohorts.…”

Section: Discussionmentioning

confidence: 99%

Massive Alveolar Haemorrhage Presenting During the COVID-19 Pandemic

et al. 2021

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The coronavirus disease (COVID-19) pandemic has challenged healthcare systems and has resulted in complex diagnostic processes for patients with non-COVID-19 pathology. Here, we demonstrate a case of massive alveolar haemorrhage secondary to antineutrophil cytoplasmic antibody-positive vasculitis, presented to a district general hospital in the UK during the first wave of the global pandemic. This case highlights some of the difficulties clinicians may face when diagnosing life-threatening antineutrophil cytoplasmic antibody-positive vasculitis amidst the COVID-19 pandemic. The authors place emphasis on the careful interpretation of chemical biomarkers such as troponin and D-dimer when assessing patients with acute respiratory distress. They also aim to highlight the importance of CT thorax imaging when seeking an alternate diagnosis to COVID-19.

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“…In fact, when purpura and excruciating pain develop concurrently, the index of suspicion for SVV, in particular ANCA-associated vasculitis should be high. Failure to recognize systemic involvement at an early stage delays treatment, increases organ damage and decreases quality of life ( 22 ). In an international cohort study consisting of 105 patients with childhood-onset ANCA-associated vasculitis, evidence of damage was still present in over 60% of patients 12 months after diagnosis ( 23 ).…”

Section: Discussionmentioning

confidence: 99%

Case Report: Systemic Small-Vessel Vasculitis in an Adolescent With Active Ulcerative Colitis

2021

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Introduction: Small-vessel vasculitis (SVV) is a rare immunological disease that affects arterioles, capillaries and venules. It causes purpura, but can also manifest in other organs, including the gastrointestinal tract. SVV and inflammatory bowel disease (IBD) co-occur more frequently than would be expected by chance.Case description: A 16-year-old girl, who had been diagnosed with ulcerative colitis (UC) 2 years earlier at a general hospital, developed purpura, progressive abdominal pain with frequent bloody diarrhea and frontotemporal headache and swelling while on azathioprine and mesalamine maintenance therapy. Serology was positive for perinuclear antineutrophil cytoplasmic antibodies (p-ANCA) without antiprotease- or myeloperoixidase antibodies. Endoscopy revealed active left-sided UC and atypical ulcerations in the ascending colon. Biopsies of these ulcerations and of affected skin revealed leukocytoclastic vasculitis. Initially this was interpreted as an extraintestinal manifestation of UC that would subside when remission was induced, consequently infliximab was started. Over the next 3 weeks she developed severe burning pain in her right lower leg that progressed to a foot drop with numbness and the purpura progressed to bullous lesions. The diagnosis was adjusted to ANCA-associated vasculitis with involvement of skin, bowel and peripheral nerves. Infliximab was discontinued and induction treatment with high-dose prednisolone and cyclophosphamide was given until remission of SVV and UC was achieved. Subsequently, infliximab induction and maintenance was re-introduced in combination with methotrexate. Remission has been maintained successfully for over 2 years now. The foot drop only partly resolved and necessitated the use of an orthosis.Conclusion: Pediatric patients with IBD who present with purpuric skin lesions and abdominal pain should be evaluated for systemic involvement of SVV, which includes endoscopic evaluation of the gastrointestinal tract. We discuss a practical approach to the diagnosis, evaluation and management of systemic SVV with a focus on prompt recognition and early aggressive therapy to improve outcome.

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ANCA associated vasculitis

Cited by 50 publications

References 18 publications

Atherogenic index of plasma predicts cerebrovascular accident occurrence in antineutrophil cytoplasmic antibody-associated vasculitis

Atherogenic index of plasma predicts cerebrovascular accident occurrence in antineutrophil cytoplasmic antibody-associated vasculitis

Massive Alveolar Haemorrhage Presenting During the COVID-19 Pandemic

Case Report: Systemic Small-Vessel Vasculitis in an Adolescent With Active Ulcerative Colitis

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