OBJECTIVE: Granulomatosis with polyangiitis (GPA) is an ANCA-associated vasculitis. The 2022 ACR/EULAR-endorsed classification criteria for GPA was derived using data only from adult patients. We aimed to assess the performance of the ACR/EULAR classification criteria for GPA in paediatric patients and compare it with the EULAR/Pediatric Rheumatology International Trials Organization (PRINTO)/Pediatric Rheumatology European Society (PReS)-endorsed Ankara 2008 criteria for GPA. METHODS: Retrospective data of paediatric patients with GPA in 20 centres from 9 countries were evaluated. The diagnosis of GPA was made according to the expert opinion. The sensitivity, specificity, positive predictive value, and negative predictive value of the criteria sets were evaluated. RESULTS: The study included 77 patients with GPA and 108 controls [IgA vasculitis (n = 44), Takayasu's arteritis (n = 20), microscopic polyangiitis (n = 16), polyarteritis nodosa (n = 14), Behçet's disease (n = 12), eosinophilic granulomatosis with polyangiitis (n = 1) and Cogan's syndrome (n = 1)] with a median age of 17.8 and 15.2 years, respectively. Among patients with GPA, constitutional symptoms (85.7%) and ENT involvement (79.2%) were the most common presentations. In the GPA group, 73 patients fulfilled the Ankara 2008 criteria and 69 the ACR/EULAR classification criteria. Sensitivities of the Ankara 2008 criteria and the ACR/EULAR classification criteria were 94.8% and 89.6%, while specificities were 95.3% and 96.3%, respectively. No significant difference was found between sensitivities and specificities of both classification criteria (P = 0.229 and P = 0.733, respectively). CONCLUSION: In children, both the ACR/EULAR and EULAR/PRINTO/PReS Ankara 2008 classification criteria for GPA perform well and similarly.

• MeSH
• Behcet Syndrome classification   diagnosis   MeSH
• Churg-Strauss Syndrome diagnosis   classification   MeSH
• Child MeSH
• Granulomatosis with Polyangiitis * classification   diagnosis   MeSH
• IgA Vasculitis diagnosis   classification   MeSH
• Humans MeSH
• Microscopic Polyangiitis classification   diagnosis   MeSH
• Adolescent MeSH
• Polyarteritis Nodosa classification   diagnosis   MeSH
• Predictive Value of Tests MeSH
• Child, Preschool MeSH
• Retrospective Studies MeSH
• Rheumatology standards   MeSH
• Sensitivity and Specificity * MeSH
• Takayasu Arteritis * classification   diagnosis   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Multicenter Study MeSH
• Comparative Study MeSH
• Geographicals
• Europe MeSH

Článek přináší přehledné informace o Takayasuově arteritidě z hlediska epidemiologie, patogeneze, klinického obrazu a diferenciální diagnostiky. Uvedena jsou klasifikační kritéria historická (1990) i současná (2022), umožňující lepší odlišení této nemoci od ostatních forem vaskulitid. V léčbě se používají kortikosteroidy, imunosupresiva, v posledních letech také moderní anticytokinová léčba (anti TNFα a IL-6) zlepšující kontrolu onemocnění. V kazuistice je demonstrován případ ženy, u níž byla diagnóza stanovena ve věku 16 let při klinickém obrazu srdečního selhávání, poruch pulzace na horních končetinách, jeden rok trvajících kolapsových stavů se systolicko-diastolickým šelestem, jehož kolísavá přítomnost byla zaznamenávána společně s dalšími nespecifickými příznaky (občasné bolesti břicha) již od dvou let života. V léčbě byla použita indukční léčba kortikosteroidy a pulsním cyklofosfamidem. V udržovací léčbě byl s úspěchem použit tocilizumab, nasazený pro intoleranci metotrexatu. Kazuistika poukazuje na skutečnost, že ačkoliv jsou vaskulitidy vzácným onemocněním, je možné se nimi setkat v běžné ambulantní praxi praktického dětského lékaře.

This article provides an overview of Takayasu arteritis in terms of epidemiology, pathogenesis, clinical picture, and differential diagnosis. Historical (1990) and current (2022) classification criteria are presented, allowing better differentiation from other forms of vasculitis. Treatment includes corticosteroids, immunosuppressants, and in recent years, modern anticytokine therapy (anti TNFα and IL-6) improving disease control. A case of Takayasu’s arteritis in a woman of childhood age is demonstrated in this case report. The diagnosis was made at the age of 16 years with a clinical picture of heart failure, upper limb pulsation disturbances, one year of collapse with systolic-diastolic murmur, whose fluctuating presence was noted together with other non-specific symptoms (intermittent abdominal pain) since the age of 2 years. Induction therapy with corticosteroids and pulse cyclophosphamide was used in the treatment. Tocilizumab, deployed for methotrexate intolerance, has been used successfully in maintenance therapy. The case report highlights the fact that although vasculitis is a rare disease, it can be encountered in the routine outpatient practice of a general pediatrician.

• MeSH
• Angiography methods   MeSH
• Biological Therapy MeSH
• Early Diagnosis MeSH
• Ultrasonography, Doppler, Duplex MeSH
• Adrenal Cortex Hormones pharmacology   therapeutic use   MeSH
• Immunosuppressive Agents pharmacology   therapeutic use   MeSH
• Humans MeSH
• Adolescent MeSH
• Positron Emission Tomography Computed Tomography MeSH
• Takayasu Arteritis diagnosis   classification   complications   physiopathology   MeSH
• Check Tag
• Humans MeSH
• Adolescent MeSH
• Female MeSH
• Publication type
• Case Reports MeSH
• Review MeSH

Aim. The studied group included patients at different age and gender with follow-up period at least 1 year. The work represents endovascular treatment of patients with different localization of the disease and according to the target lesion proper endovascular access was selected. Methods and results. The patients are divided into groups, depending on the Numano classification. Seven of studied patients were in age between 33 and 44 years, only 1 was 65 years old, 7 of them were females and 1 male. The patients were accumulated for the period of 1 year. Doppler sonography was used for screening of the patients with known Takayasu disease. Treated lesions were as follows: left subclavian artery – 3 patients, right subclavian artery – 1 patient, right common carotid artery – 1 patient, brachiocephalic trunk – 1 patient, superior mesenteric artery – 2 patients, both external iliac arteries – 1 patient, LAD – 1 patient. Endovascular access of choice were right femoral, right radial, left radial, and right brachial. The used devices were balloon-expandable stents, self-expandable stents, DES/coronary/, PTA only – DCB. Conclusions. All Takayasu patients in our report were successfully treated with implantation of different types of stents. One year results showed no significant restenosis, except in 1 patient with early restenosis until 6th month.

• MeSH
• Angiography MeSH
• Angioplasty, Balloon methods   instrumentation   MeSH
• Adult MeSH
• Humans MeSH
• Constriction, Pathologic diagnostic imaging   surgery   MeSH
• Stents MeSH
• Takayasu Arteritis * diagnostic imaging   epidemiology   surgery   classification   MeSH
• Ultrasonography, Doppler instrumentation   MeSH
• Treatment Outcome MeSH
• Check Tag
• Adult MeSH
• Humans MeSH
• Female MeSH
• Publication type
• Case Reports MeSH
• Geographicals
• Bulgaria MeSH

Termínem aortitis označujeme zánět aortální stěny. Jedná se o nepříliš časté onemocnění, jehož podkladem bývají zpravidla vaskulitidy velkých a středních tepen. Méně častou příčinou jsou infekce. V článku se zabýváme etiologií, klinickými projevy, diagnostikou a léčbou známých forem onemocnění.

The term aortitis is used to describe an inflammation of the aortic wall. The disease is rather rare and is mostly caused by vasculitis of medium-sized and large arteries. Infections represent a less frequent cause. The paper discusses aetiology, clinical manifestation, diagnosis and therapy of the most common forms of the disease.

• Keywords
• periaortitis, vaskulitis, diagnostika FDG-PET,
• MeSH
• Anti-Bacterial Agents administration & dosage   adverse effects   therapeutic use   MeSH
• Aortitis diagnosis   etiology   classification   MeSH
• Arteritis diagnosis   etiology   classification   MeSH
• Echocardiography methods   utilization   MeSH
• Fever of Unknown Origin diagnosis   complications   MeSH
• Adrenal Cortex Hormones administration & dosage   adverse effects   therapeutic use   MeSH
• Cardiovascular Surgical Procedures methods   utilization   MeSH
• Disease Attributes MeSH
• Humans MeSH
• Giant Cell Arteritis etiology   classification   MeSH
• Tomography, X-Ray Computed methods   utilization   MeSH
• Positron-Emission Tomography methods   utilization   MeSH
• Radiography methods   utilization   MeSH
• Takayasu Arteritis diagnosis   etiology   classification   MeSH
• Vasculitis diagnosis   etiology   classification   MeSH
• Check Tag
• Humans MeSH

• MeSH
• Humans MeSH
• Giant Cell Arteritis diagnosis   MeSH
• Polymyalgia Rheumatica diagnosis   MeSH
• Takayasu Arteritis diagnosis   classification   MeSH
• Thromboangiitis Obliterans diagnosis   MeSH
• Vasculitis diagnosis   etiology   classification   MeSH
• Check Tag
• Humans MeSH

• MeSH
• Arteritis diagnosis   classification   pathology   MeSH
• Churg-Strauss Syndrome diagnosis   classification   pathology   MeSH
• Granulomatosis with Polyangiitis diagnosis   classification   pathology   MeSH
• IgA Vasculitis diagnosis   classification   pathology   MeSH
• Humans MeSH
• Respiratory Burst diagnosis   classification   pathology   MeSH
• Takayasu Arteritis diagnosis   classification   pathology   MeSH
• Vasculitis diagnosis   classification   pathology   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Review MeSH

• MeSH
• Arteritis diagnosis   classification   MeSH
• Granulomatosis with Polyangiitis diagnosis   classification   MeSH
• Polyarteritis Nodosa diagnosis   classification   MeSH
• Takayasu Arteritis diagnosis   classification   MeSH
• Thromboangiitis Obliterans diagnosis   classification   MeSH
• Vasculitis diagnosis   classification   MeSH
• Publication type
• Review MeSH