Notfälle bei Kindern mit Granulomatose mit Polyangiitis

Zusammenfassung

Bei Kindern mit Granulomatose mit Polyangiitis (GPA) kann es bei verschiedenen Krankheitsmanifestationen zu einem medizinischen Notfall kommen. Lebensbedrohliche oder organbedrohende Manifestationen sind u. a. eine diffuse alveoläre Hämorrhagie, eine Glomerulonephritis mit Niereninsuffizienz, tracheale/bronchiale Manifestationen wie z. B. eine subglottische Stenose, ZNS-Manifestationen, kardiovaskuläre oder gastrointestinale Manifestationen. Bei diesen schweren Manifestationen muss sofort an eine GPA gedacht werden und ohne Verzögerung eine Abklärung sowie effektive Therapie erfolgen. Auch bei Kindern mit bekannter GPA können plötzlich Notfallsituationen auftreten. Nicht selten weisen unspezifische Beschwerden auf eine entsprechende Organbeteiligung hin und müssen sofort weiter untersucht werden. Der vorliegende Artikel gibt eine Übersicht über die typischen schweren, d. h. lebens- oder organbedrohenden Manifestationen bei GPA.

Schlüsselwörter

Granulomatose mit Polyangiitis - pädiatrische Notfälle - diffuse alveoläre Hämorrhagie - rapid progressive Glomerulonephritis - tracheobronchiale Stenosen

Publikationsverlauf

Artikel online veröffentlicht:
01. Dezember 2025

• Literatur

• 1 Jennette JC, Falk RJ, Bacon PA. et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 2013; 65: 1-11
• 2 Ozen S, Pistorio A, Iusan SM. et al. EULAR/PRINTO/PRES criteria for Henoch-Schonlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010; 69: 798-806
• 3 Cabral DA, Canter DL, Muscal E. et al. Comparing presenting clinical features in 48 children with microscopic polyangiitis to 183 children who have granulomatosis with polyangiitis (Wegener’s): An ARChiVe cohort study. Arthritis Rheumatol 2016; 68: 2514-2526
• 4 Sacri AS, Chambaraud T, Ranchin B. et al. Clinical characteristics and outcomes of childhood-onset ANCA-associated vasculitis: a French nationwide study. Nephrol Dial Transplant 2015; 30 ( (Suppl. 1) i104-112
• 5 Mahi SL, Bahram S, Harambat J. et al. Pediatric ANCA vasculitis: clinical presentation, treatment, and outcomes in a French retrospective study. Pediatr Nephrol 2023; 38: 2649-2658
• 6 James KE, Xiao R, Merkel PA. et al. Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis. Clin Exp Rheumatol 2017; 35 ( (Suppl. 103) 202-208
• 7 Chung SA, Langford CA, Maz M. et al. 2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis. Arthritis Rheumatol 2021; 73: 1366-1383
• 8 Hellmich B, Sanchez-Alamo B, Schirmer JH. et al. EULAR recommendations for the management of ANCA-associated vasculitis: 2022 update. Ann Rheum Dis 2024; 83: 30-47
• 9 Holle JU, Kubacki T, Aries P. et al. [Diagnosis and treatment of ANCA-associated vasculitis : S3 guideline of the German Society for Rheumatology and Clinical Immunology e. V. (DGRh) and German Society for Internal Medicine e. V. (DGIM), German Society for Nephrology e. V. (DGfN), German Society for ENT Medicine and Head and Neck Surgery e. V. (DGHNO-KHC), German Ophthalmological Society e. V. (DOG), German Society for Neurology e. V. (DGN), German Society for Pneumology and Respiratory Medicine e. V. (DGP), German Society for Pathology e. V. (DGP), German Radiological Society, Society for Medical Radiology e. V. (DRG), Federal Association of German Pathologists, Federal Kidney Association e. V., German Rheumatism League Federal Association e. V.]. Z Rheumatol 2025; 84: 1-49
• 10 Kidney Disease: Improving Global Outcomes (KDIGO) ANCA Vasculitis Work Group. KDIGO 2024 Clinical Practice Guideline for the Management of Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis. Kidney Int 2024; 105: S71-S116
• 11 Cortazar FB, Niles JL, Jayne DRW. et al. Renal recovery for patients with ANCA-associated vasculitis and low eGFR in the ADVOCATE trial of avacopan. Kidney Int Rep 2023; 8: 860-870
• 12 Chalkia A, Flossmann O, Jones R. et al. Avacopan for ANCA-associated vasculitis with hypoxic pulmonary haemorrhage. Nephrol Dial Transplant 2024; 39: 1473-1482
• 13 Falde SD, Lal A, Cartin-Ceba R. et al. Treatment of antineutrophil cytoplasmic antibody-associated vasculitis with diffuse alveolar hemorrhage with avacopan. ACR Open Rheumatol 2024; 6: 707-716
• 14 Fouka E, Drakopanagiotakis F, Steiropoulos P. Pathogenesis of pulmonary manifestations in ANCA-associated vasculitis and Goodpasture syndrome. Int J Mol Sci 2024; 25: 5278
• 15 Falde SD, Fussner LA, Tazelaar HD. et al. Proteinase 3-specific antineutrophil cytoplasmic antibody-associated vasculitis. Lancet Rheumatol 2024; 6: e314-e327
• 16 Villeneuve T, Prevot G, Pugnet G. et al. Role of bronchoscopy for respiratory involvement in granulomatosis with polyangiitis and microscopic polyangiitis. ERJ Open Res 2023; 9: 00141-2023
• 17 Morishita KA, Moorthy LN, Lubieniecka JM. et al. Early outcomes in children with antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol 2017; 69: 1470-1479
• 18 Noone DG, Twilt M, Hayes WN. et al. The new histopathologic classification of ANCA-associated GN and its association with renal outcomes in childhood. Clin J Am Soc Nephrol 2014; 9: 1684-1691
• 19 Toor KK, Chen A, Cabral DA. et al. Evaluating renal disease in pediatric-onset antineutrophil cytoplasmic antibody-associated vasculitis: disease course, outcomes, and predictors of outcome. Arthritis Rheumatol 2025; 77: 606-614
• 20 Catano J, Uzunhan Y, Paule R. et al. Presentation, diagnosis, and management of subglottic and tracheal stenosis during systemic inflammatory diseases. Chest 2022; 161: 257-265
• 21 Quinn KA, Gelbard A, Sibley C. et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology (Oxford) 2019; 58: 2203-2211
• 22 Iudici M, Pagnoux C, Quartier P. et al. Childhood- versus adult-onset ANCA-associated vasculitides: A nested, matched case-control study from the French Vasculitis Study Group Registry. Autoimmun Rev 2018; 17: 108-114
• 23 Calatroni M, Oliva E, Gianfreda D. et al. ANCA-associated vasculitis in childhood: recent advances. Ital J Pediatr 2017; 43: 46
• 24 Girard C, Charles P, Terrier B. et al. Tracheobronchial stenoses in granulomatosis with polyangiitis (Wegener’s): A report on 26 cases. Medicine (Baltimore) 2015; 94: e1088
• 25 Matsumoto K, Akiyama M, Kajio N. et al. Adolescent PR3-ANCA-positive hypertrophic pachymeningitis: A case report and review of the literature. Medicine (Baltimore) 2018; 97: e0521
• 26 Laass MW, Ziesmann J, de Laffolie J. et al. Anti-proteinase 3 antibodies as a biomarker for ulcerative colitis and primary sclerosing cholangitis in children. J Pediatr Gastroenterol Nutr 2022; 74: 463-470