Nierenbeteiligung bei thrombotischer Mikroangiopathie

ZUSAMMENFASSUNG

Der Begriff thrombotische Mikroangiopathie (TMA) fasst eine Gruppe von Krankheiten zusammen, bei denen sich durch unterschiedliche Mechanismen Mikrothromben im Kapillarbett und in den kleinen Gefäßen bilden. Diese lösen eine mechanische Hämolyse mit Anämie und (Verbrauchs-)Thrombopenie aus. Die Mikrozirkulation wird beeinträchtigt und es kommt zu einer Ischämie mit Funktionsstörung der betroffenen Organe, inklusive der Niere. Das zentrale Nervensystem, Herz, Lunge und Gastrointestinaltrakt können ebenso beteiligt sein. Die häufigste TMA im Kindesalter ist das Shiga-Toxin assoziierte hämolytisch-urämische Syndrom (STEC-HUS). Das atypische hämolytisch-urämische Syndrom (aHUS) und die thrombotisch-thrombozytopenische Purpura (TTP) sind in dieser Altersgruppe wesentlich seltener. Bei Erwachsenen treten mit zunehmendem Alter überwiegend sekundäre TMA-Formen auf.

Publikationsverlauf

Artikel online veröffentlicht:
24. Juli 2024

• Literatur

• 1 Campistol JM, Arias M, Ariceta G. et al An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia 2015; 35: 421-447
• 2 Goodship THJ, Cook HT, Fakhouri F. et al Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a „Kidney Disease: Improving Global Outcomes“ (KDIGO) Controversies Conference. Kidney Int 2017; 91: 539-551
• 3 Lusco MA, Fogo AB, Najafian B. et al AJKD Atlas of Renal Pathology: Thrombotic Microangiopathy. Am J Kidney Dis 2016; 68: e33-e34
• 4 Bhutani G, Leung N, Said SM. et al The prevalence and clinical outcomes of microangiopathic hemolytic anemia in patients with biopsy-proven renal thrombotic microangiopathy. Am J Hematol 2022; 97: E426-E429
• 5 Genest DS, Patriquin CJ, Licht C. et al Renal Thrombotic Microangiopathy: A Review. Am J Kidney Dis 2023; 81: 591-605
• 6 Wu Y, Lu C, Pan N. et al Serum lactate dehydrogenase activities as systems biomarkers for 48 types of human diseases. Sci Rep 2021; 11: 12997
• 7 Kremer Hovinga JA, Coppo P, Lämmle B. et al Thrombotic thrombocytopenic purpura. Nat Rev Dis Primer 2017; 03: 17020
• 8 Joly BS, Coppo P, Veyradier A. Thrombotic thrombocytopenic purpura. Blood 2017; 129: 2836-2846
• 9 Chiasakul T, Cuker A. Clinical and laboratory diagnosis of TTP: an integrated approach. Hematol Am Soc Hematol Educ Program 2018; 2018: 530-538
• 10 Bendapudi PK, Hurwitz S, Fry A. et al Derivation and external validation of the PLASMIC score for rapid assessment of adults with thrombotic microangiopathies: a cohort study. Lancet Haematol 2017; 04: e157-e164
• 11 Veyradier A. Von Willebrand Factor--A New Target for TTP Treatment?. N Engl J Med 2016; 374: 583-585
• 12 Page EE, Kremer Hovinga JA, Terrell DR. et al Rituximab reduces risk for relapse in patients with thrombotic thrombocytopenic purpura. Blood 2016; 127: 3092-3094
• 13 Scully M, Cataland SR, Peyvandi F. et al Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura. N Engl J Med 2019; 380: 335-346
• 14 Völker LA, Kaufeld J, Miesbach W. et al ADAMTS13 and VWF activities guide individualized caplacizumab treatment in patients with aTTP. Blood Adv 2020; 04: 3093-3101
• 15 George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med 2014; 371: 654-666
• 16 Kielstein JT, Beutel G, Fleig S. et al Best supportive care and therapeutic plasma exchange with or without eculizumab in Shiga-toxin-producing E. coli O104:H4 induced haemolytic-uraemic syndrome: an analysis of the German STEC-HUS registry. Nephrol Dial Transplant 2012; 27: 3807-3815
• 17 Wijnsma KL, van Bommel SAM, van der Velden T. et al Fecal diagnostics in combination with serology: best test to establish STEC-HUS. Pediatr Nephrol Berl Ger 2016; 31: 2163-2170
• 18 Sheerin NS, Glover E. Haemolytic uremic syndrome: diagnosis and management. F1000Res 2019; 08: F1000 Faculty Rev-1690
• 19 Noris M, Remuzzi G. Hemolytic uremic syndrome. J Am Soc Nephrol 2005; 16: 1035-1050
• 20 Garnier A, Brochard K, Kwon T. et al Efficacy and Safety of Eculizumab in Pediatric Patients Affected by Shiga Toxin-Related Hemolytic and Uremic Syndrome: A Randomized, Placebo-Controlled Trial. J Am Soc Nephrol 2023; 34: 1561-1573
• 21 Brocklebank V, Wood KM, Kavanagh D. Thrombotic Microangiopathy and the Kidney. Clin J Am Soc Nephrol 2018; 13: 300-317
• 22 Legendre CM, Licht C, Muus P. et al Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med 2013; 368: 2169-2181
• 23 Brocklebank V, Walsh PR, Smith-Jackson K. et al Atypical hemolytic uremic syndrome in the era of terminal complement inhibition: an observational cohort study. Blood 2023; 142: 1371-1386
• 24 Brocklebank V, Kumar G, Howie AJ. et al Long-term outcomes and response to treatment in diacylglycerol kinase epsilon nephropathy. Kidney Int 2020; 97: 1260-1274
• 25 Brown MA, Magee LA, Kenny LC. et al The hypertensive disorders of pregnancy: ISSHP classification, diagnosis & management recommendations for international practice. Pregnancy Hypertens 2018; 13: 291-310
• 26 Zeisler H, Llurba E, Chantraine F. et al Predictive Value of the sFlt-1:PlGF Ratio in Women with Suspected Preeclampsia. N Engl J Med 2016; 374: 13-22
• 27 Fakhouri F, Scully M, Provôt F. et al Management of thrombotic microangiopathy in pregnancy and postpartum: report from an international working group. Blood 2020; 136: 2103-2117
• 28 Werion A, Storms P, Zizi Y. et al Epidemiology, Outcomes, and Complement Gene Variants in Secondary Thrombotic Microangiopathies. Clin J Am Soc Nephrol 2023; 18: 881-891
• 29 Garcia D, Erkan D. Diagnosis and Management of the Antiphospholipid Syndrome. N Engl J Med 2018; 378: 2010-2021
• 30 Woodworth TG, Suliman YA, Li W. et al Scleroderma renal crisis and renal involvement in systemic sclerosis. Nat Rev Nephrol 2016; 12: 678-691
• 31 Martis N, Jamme M, Bagnis-Isnard C. et al Systemic autoimmune disorders associated with thrombotic microangiopathy: A cross-sectional analysis from the French National TMA registry: Systemic autoimmune disease-associated TMA. Eur J Intern Med 2021; 93: 78-86
• 32 Martins M, Bridoux F, Goujon JM. et al Complement Activation and Thrombotic Microangiopathy Associated With Monoclonal Gammopathy: A National French Case Series. Am J Kidney Dis 2022; 80: 341-352
• 33 Ravindran A, Go RS, Fervenza FC. et al Thrombotic microangiopathy associated with monoclonal gammopathy. Kidney Int 2017; 91: 691-698
• 34 Tran PN, Tran M-H. Cobalamin deficiency presenting with thrombotic microangiopathy (TMA) features: A systematic review. Transfus Apher Sci 2018; 57: 102-106
• 35 Beck BB, van Spronsen F, Diepstra A. et al Renal thrombotic microangiopathy in patients with cblC defect: review of an under-recognized entity. Pediatr Nephrol 2017; 32: 733-741
• 36 Li A, Makar RS, Hurwitz S. et al Treatment with or without plasma exchange for patients with acquired thrombotic microangiopathy not associated with severe ADAMTS13 deficiency: a propensity score-matched study. Transfusion 2016; 56: 2069-2077
• 37 Walle JV, Delmas Y, Ardissino G. et al Improved renal recovery in patients with atypical hemolytic uremic syndrome following rapid initiation of eculizumab treatment. J Nephrol 2017; 30: 127-134
• 38 Mazzierli T, Allegretta F, Maffini E. et al Drug-induced thrombotic microangiopathy: An updated review of causative drugs, pathophysiology, and management. Front Pharmacol 2022; 13: 1088031
• 39 Thoreau B, von Tokarski F, Bauvois A. et al Infection in Patients with Suspected Thrombotic Microangiopathy Based on Clinical Presentation. Clin J Am Soc Nephrol 2021; 16: 1355-1364
• 40 Adelborg K, Larsen JB, Hvas A-M. Disseminated intravascular coagulation: epidemiology, biomarkers, and management. Br J Haematol 2021; 192: 803-818