Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working‐Age Adult Populations In the United States

Panupattanapong, Sirada; Stwalley, Dustin; White, Andrew J.; Olsen, Margaret A.; French, Anthony R.; Hartman, Mary E.

doi:10.1002/art.40577

Cited by 72 publications

(66 citation statements)

References 46 publications

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“…The prevalence of AAV in that study was 42.1 (29.6 to 54.6) per 100 000 37. Similar estimates of annual incidence of GPA were reported in a large US health insurance claims database (1.3 (1.2 to 13.4) per 100 000),38 as well as the UK General Practice Research Database (0.8 (0.8 to 0.9) per 100 000) 39…”

Section: Incidence and Prevalence Of Aavsupporting

confidence: 80%

Management of ANCA associated vasculitis

Wallace

Miloslavsky

2020

BMJ

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Anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) is a small to medium vessel vasculitis associated with excess morbidity and mortality. This review explores how management of AAV has evolved over the past two decades with pivotal randomized controlled trials shaping the management of induction and maintenance of remission. Contemporary AAV care is characterized by approaches that minimize the cumulative exposure to cyclophosphamide and glucocorticoids, increasingly use rituximab for remission induction and maintenance, and consider therapies with less toxicity (for example, methotrexate, mycophenolate mofetil) for manifestations of AAV that do not threaten organ function or survival. Simultaneously, improvements in outcomes, such as renal and overall survival, have been observed. Additional trials and observational studies evaluating the comparative effectiveness of agents for AAV in various patient subgroups are needed. Prospective studies are necessary to assess the effect of psychosocial interventions on patient reported outcomes in AAV. Despite the expanding array of treatments for AAV, little guidance on how to personalize AAV care is available to physicians.

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Section: Incidence and Prevalence Of Aavsupporting

confidence: 80%

Management of ANCA associated vasculitis

Wallace

Miloslavsky

2020

BMJ

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“…The incidence of paediatric-onset GPA has between reported to range from 0.03 to 3.2 per year, and delayed diagnosis is commonly reported due to the broad range of symptoms 2. The most recent data from the USA of over 5000 patients with GPA reported the incidence in the paediatric population of approximately 1.8 cases/million person-years, which constituted approximately 4% of all cases of GPA 3. There tends to be an adolescent, female and Caucasian predominance in paediatric GPA 4…”

Section: Discussionmentioning

confidence: 99%

Coughing up clues: 16-year-old girl with acute haemoptysis

2020

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A 16-year-old previously well girl presented with bilateral painful lower leg swelling and non-pruritic blanching rash across her torso and upper and lower limbs. These symptoms started after commencing amoxicillin for presumed tonsillitis. She was diagnosed with serum sickness-like illness and started on non-steroidal agents. The rash and painful leg swelling improved over the next 48 hours. However, she subsequently developed fevers, cough and new-onset haemoptysis.She continued to deteriorate with increasing amounts of haemoptysis, work of breathing and escalating respiratory support requirements. Serial chest radiographs showed worsening lung consolidation and enlarging pleural effusion. A CT chest revealed extensive bilateral lung consolidation, most likely pulmonary haemorrhage. Subsequent investigations showed positive classic antineutrophil cytoplasmic antibody, confirming the diagnosis of granulomatosis with polyangiitis.

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“…Most are associated with the respiratory tract but any body system may be affected. The condition occurs over a wide age range with slight female predilection and almost all cases occur in Caucasians [ 17 ].…”

Section: Reactive Lesionsmentioning

confidence: 99%

Lumps and Bumps of the Gingiva: A Pathological Miscellany

Brierley

Crane

Hunter

2019

Head and Neck Pathol

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Lesions of the gingivae are amongst the commonest lesions seen in patients and the vast majority are reactive hyperplasias, related to a number of chronic irritant stimuli. However, there are a number of entities that have a predilection for the gingivae, which are much less common in other parts of the oral cavity. The purpose of this paper is to discuss the clinical and histological differential diagnoses when presented with a lump on the gingivae, including the approach to diagnosis and diagnostic pitfalls.

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Epidemiology and Outcomes of Granulomatosis With Polyangiitis in Pediatric and Working‐Age Adult Populations In the United States

Abstract: This study represents the largest cohort of GPA reported to date. Pediatric GPA patients experienced more frequent hospitalizations and were more vulnerable to hematologic complications than non-elderly adult patients.

Cited by 72 publications

References 46 publications

Management of ANCA associated vasculitis

Management of ANCA associated vasculitis

Coughing up clues: 16-year-old girl with acute haemoptysis

Lumps and Bumps of the Gingiva: A Pathological Miscellany

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