European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides – the SHARE initiative

Graeff, Nienke de; Groot, Noortje; Brogan, Paul A.; Özen, Seza; Avčin, Tadej; Bader‐Meunier, Brigitte; Doležalová, Pavla; Feldman, Brian M.; Koné‐Paut, Isabelle; Lahdenne, Pekka; Marks, Stephen D.; McCann, Liza; Pilkington, Clarissa; Ravelli, Angelo; Royen, Annet van; Uziel, Yosef; Vastert, Bas Sj; Wulffraat, Nico; Kamphuis, Sylvia; Beresford, Michael W.

doi:10.1093/rheumatology/keaa057

Cited by 34 publications

(63 citation statements)

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“…Induction regimens typically include high dose corticosteroids, intravenous cyclophosphamide, and therapeutic plasmapheresis. Recommended first-line maintenance options are azathioprine, methotrexate and mycophenolate mofelil [5].…”

Section: Discussionmentioning

confidence: 99%

“…Serological tests are extremely helpful when making a diagnosis, but ultimately it must be based on the results of renal biopsy. Treatment with corticosteroids, immunosuppressive agents, and, in severe cases, plasmapheresis has considerably improved the prognosis [5,6]. Here we have reported the case of a 14year-old girl with an overlap syndrome of SLE and AAV and reviewed her kidney biopsy finding, the differential diagnosis, diagnosis as well as the management of the disease.…”

Section: Introductionmentioning

confidence: 99%

“…The pulmonary lesion in ANCAassociated vasculitis is DAH or focal inflammatory vasculitis. In GPA, there may also be lesions in the upper airways (tracheitis, sinusitis and otitis), necrotic pulmonary lesions, and hilar lymphadenopathy[5]. Inflammatory markers such as erythrocyte sedimentation rate and C-reactive protein levels are also elevated.…”

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confidence: 99%

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Microscopic Polyangitis in Pediatric Systemic Lupus Erythematosus: A Unique Presentation of Pulmonary - Renal Syndrome and Case Report of an Overlap Syndrome

Zhang

Yin

Mao

et al. 2020

Preprint

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Background: Secondary vasculitis is encountered in about one third of all cases of systemic lupus erythematosus (SLE). Skin is most commonly involved in lupus-related small vasculitis. Although antineutrophil cytoplasmic autoantibodies (ANCA) associated vasculitis (AAV) is relatively uncommon, it can be the most dangerous manifestation associated with high mortality. SLE and AAV are separate diseases with different pathophysiologies and an overlap syndrome has only been reported few times in previous literature. Case Presentation: We present a unique case of a pediatric patient of pulmonary-renal syndrome, presenting with pulmonary alveolar hemorrhage and rapidly progressive glomerulonephritis. Serological and biopsy findings were suggestive of SLE and AAV occurring simultaneously. Renal biopsy demonstrated necrotizing and crescentic glomerulonephritis, superimposed on diffuse segmental proliferative lupus glomerulonephritis class IV. Conclusions: Presentations of autoimmune diseases and vasculitis can be multi-systemic. Considering overlap syndromes, especially in patients with underlying connective tissue disease or systemic vasculitis, is vital for prompt therapy and prevention of morbidity in this population.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Microscopic Polyangitis in Pediatric Systemic Lupus Erythematosus: A Unique Presentation of Pulmonary - Renal Syndrome and Case Report of an Overlap Syndrome

Zhang

Yin

Mao

et al. 2020

Preprint

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“…The pathophysiology of TA starts with inflammation, and endothelial damage usually leads to thickening of the wall, thrombus formation, stenotic and occlusive lesions, while the destruction of the muscular and elastic layers originates -dilation and aneurysms. These lesions often result in organiccellular dysfunction secondary to ischemia [6,8].…”

Section: Discussionmentioning

confidence: 99%

“…It is more prevalent in Central and South America, Africa, India, and the Far East [4]. In a recent study, the mortality rate of TA was estimated to be 2.5 × 100,000 inhabitants due to the association of this arteritis with premature deaths [5,6].…”

Section: Introductionmentioning

confidence: 99%

Takayasu Arteritis in Chronic Migraine

Pabon

2019

J Med Cases

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Takayasu arteritis (TA) is a systemic granulomatous vasculitis of large vessels with low incidence and nonspecific symptoms, and late diagnosis and management lead to complications such as stroke, acute myocardial infarction or peripheral ischemia. This case illustrates the complexity of TA diagnosis because its symptomatology is frequently mistaken as chronic migraine. Therefore, without a syndromic approach, it is more likely to increase comorbidity and progression of the disease. This paper deacribes a 25-year-old woman with chronic migraine for 8 years, with recurrent admissions to emergency service, and specialist outpatient consultations. The patient has a history of fugax amaurosis, hypertension and claudicating chest pain that required treatment with corticoid, hydroxychloroquine, methotrexate and neflunomide, due to a stenosis of 50-70% of the left carotid artery and thickening of the walls of the distal thoracic aorta and the proximal abdominal aorta, corresponding to TA, with improvement of its subsequent symptomatology to treatment for rheumatology.

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Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

Morishita

Wagner‐Weiner

Yen

et al. 2022

Arthritis Care & Research

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for the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Antineutrophil Cytoplasmic Antibody-Associated Vasculitis WorkgroupObjective. There is no standardized approach to the treatment of pediatric antineutrophil cytoplasmic antibodyassociated vasculitis (AAV). Because of the rarity of pediatric AAV, randomized trials have not been feasible. The present study of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) was undertaken to establish consensus treatment plans (CTPs) for severe pediatric AAV to enable the future study of comparative effectiveness and safety.Methods. A workgroup of CARRA members (rheumatologists and nephrologists) formed the AAV Workgroup. This group performed a literature review on existing evidence-based treatments and guidelines for the management of AAV. They determined that the target population for CTP development was patients <18 years of age with new-onset granulomatosis with polyangiitis (GPA), microscopic polyangiitis, or renal-limited AAV (eosinophilic GPA was excluded), with presentation confined to those with severe disease (i.e., organ-or life-threatening). Face-to-face consensus conferences employed nominal group techniques to identify treatment strategies for remission induction and remission maintenance, data elements to be systematically collected, and outcomes to be measured over time.Results. The pediatric AAV Workgroup developed 2 CTPs for each of the remission induction and remission maintenance of severe AAV. A glucocorticoid-weaning regimen for induction and maintenance, a core data set, and outcome measures were also defined. A random sample of CARRA membership voted acceptance of the CTPs for remission induction and remission maintenance, with a 94% (75 of 80) and 98% (78 of 80) approval rate, respectively.Conclusion. Consensus methodology established standardized CTPs for treating severe pediatric AAV. These CTPs were in principle accepted by CARRA-wide membership for the evaluation of pragmatic comparative effectiveness in a long-term registry.

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European consensus-based recommendations for the diagnosis and treatment of rare paediatric vasculitides – the SHARE initiative

Cited by 34 publications

References 0 publications

Microscopic Polyangitis in Pediatric Systemic Lupus Erythematosus: A Unique Presentation of Pulmonary - Renal Syndrome and Case Report of an Overlap Syndrome

Microscopic Polyangitis in Pediatric Systemic Lupus Erythematosus: A Unique Presentation of Pulmonary - Renal Syndrome and Case Report of an Overlap Syndrome

Takayasu Arteritis in Chronic Migraine

Consensus Treatment Plans for Severe Pediatric Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

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