Acute Kidney Injury Caused by Evans Syndrome with Systemic Lupus Erythematosus and Systemic Sclerosis

Overview

Journal Intern Med

Specialty General Medicine

Date 2020 Oct 29

PMID 33116016

Authors

Natsumi Matsuoka

Haruki Watanabe

Naoko Kurooka

Sumari Kato

Chika Higashi

Katsuyuki Tanabe

Masaru Kinomura

Nobuharu Fujii

Ken-Ei Sada

Hitoshi Sugiyama

Jun Wada

Affiliations

Soon will be listed here.

Abstract

A 65-year-old woman with systemic sclerosis and systemic lupus erythematosus developed acute kidney injury (AKI), Coombs-positive autoimmune hemolytic anemia and autoimmune thrombocytopenia; therefore, she was diagnosed with Evans syndrome (ES). Intravascular hemolysis was suggested as the cause of AKI based on the presence of acute tubular injury and trace hemosiderin deposits on the renal biopsy. The renal function, hemolytic anemia and thrombocytopenia were restored by an increased dose of glucocorticoids, hemodialysis, and plasma exchange. Although ES with severe hemolytic anemia is very rare, it is important to detect possible renal dysfunction when encountering patients with severe hemolysis.

References

Bleakly N, Fontaine M, Pate L, Sutherland S, Jeng M . Disseminated intravascular coagulation due to IgM-mediated autoimmune hemolytic anemia. Pediatr Blood Cancer. 2011; 57(2):329-31. DOI: 10.1002/pbc.23024. View

Gunawardena D, Gamakaranage G . A case of warm autoimmune haemolytic anaemia with intravascular haemolysis: a rare presentation. Ceylon Med J. 2014; 58(4):176-8. DOI: 10.4038/cmj.v58i4.6312. View

Zhang L, Wu X, Wang L, Li J, Chen H, Zhao Y . Clinical Features of Systemic Lupus Erythematosus Patients Complicated With Evans Syndrome: A Case-Control, Single Center Study. Medicine (Baltimore). 2016; 95(15):e3279. PMC: 4839809. DOI: 10.1097/MD.0000000000003279. View

Siddiqui N, Toumeh A, Yoon Y, Verghese C . Primary Evan Syndrome With Disseminated Intravascular Coagulation Suggests Progressive Immune Dysregulation and Early Immunosuppressive Intervention is Key to Improving Outcomes. Am J Ther. 2014; 23(4):e1105-7. DOI: 10.1097/MJT.0000000000000171. View

Lucchini G, Masera N, Foti G, Assali G, Perseghin P, Biagi E . A life-threatening paediatric case of acute autoimmune haemolytic anaemia (AIHA) successfully cured by plasma-exchange and combined immunosuppressive treatment. Transfus Apher Sci. 2009; 40(2):115-8. DOI: 10.1016/j.transci.2009.01.020. View

Lin H, Wu K, Zhang W, Lin G, Cai C . Evans Syndrome with Acute Kidney Injury. Arch Iran Med. 2019; 22(6):336-339. View

Gonzalez I, Rais R, Gaut J, Dehner L . Evans Syndrome Complicated by Intratubular Hemoglobin Cast Nephropathy. Case Rep Pediatr. 2017; 2017:5184587. PMC: 5661089. DOI: 10.1155/2017/5184587. View

De Sanctis L, Mandreoli M, Poggi C, Pileri S, Casanova S, Santoro A . Acute renal failure in a young woman with Fisher-Evans' syndrome. J Nephrol. 2004; 17(5):739-43. View

Cerdas-Quesada C . A life-threatening case of autoimmune hemolytic anemia successfully treated by plasma-exchange. Transfus Apher Sci. 2010; 42(3):235-7. DOI: 10.1016/j.transci.2010.03.012. View

10.

Zanella A, Barcellini W . Treatment of autoimmune hemolytic anemias. Haematologica. 2014; 99(10):1547-54. PMC: 4181250. DOI: 10.3324/haematol.2014.114561. View

11.

Fervenza F, Croatt A, Bittar C, Rosenthal D, Lager D, Leung N . Induction of heme oxygenase-1 and ferritin in the kidney in warm antibody hemolytic anemia. Am J Kidney Dis. 2008; 52(5):972-7. PMC: 2709285. DOI: 10.1053/j.ajkd.2008.07.012. View

12.

Dvanajscak Z, Walker P, Cossey L, Messias N, Boils C, Kuperman M . Hemolysis-associated hemoglobin cast nephropathy results from a range of clinicopathologic disorders. Kidney Int. 2019; 96(6):1400-1407. DOI: 10.1016/j.kint.2019.08.026. View

13.

Janatpour K, Kalmin N, Jensen H, Holland P . Clinical outcomes of ABO-incompatible RBC transfusions. Am J Clin Pathol. 2008; 129(2):276-81. DOI: 10.1309/VXY1ULAFUY6E6JT3. View

14.

Jaime-Perez J, Aguilar-Calderon P, Salazar-Cavazos L, Gomez-Almaguer D . Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018; 9:171-184. PMC: 6190623. DOI: 10.2147/JBM.S176144. View

15.

Hussain S, Qureshi A, Kazi J . Renal involvement in paroxysmal nocturnal hemoglobinuria. Nephron Clin Pract. 2013; 123(1-2):28-35. DOI: 10.1159/000351345. View

16.

Mammen C, Bissonnette M, Matsell D . Acute kidney injury in children with sickle cell disease-compounding a chronic problem. Pediatr Nephrol. 2017; 32(8):1287-1291. DOI: 10.1007/s00467-017-3650-3. View

17.

Asakura H, Takahashi H, Uchiyama T, Eguchi Y, Okamoto K, Kawasugi K . Proposal for new diagnostic criteria for DIC from the Japanese Society on Thrombosis and Hemostasis. Thromb J. 2016; 14:42. PMC: 5039801. DOI: 10.1186/s12959-016-0117-x. View

18.

Villegas A, Nunez R, Gaya A, Cuevas-Ruiz M, Bosch J, Carral A . Presence of acute and chronic renal failure in patients with paroxysmal nocturnal hemoglobinuria: results of a retrospective analysis from the Spanish PNH Registry. Ann Hematol. 2017; 96(10):1727-1733. DOI: 10.1007/s00277-017-3059-x. View

19.

Schaer D, Vinchi F, Ingoglia G, Tolosano E, Buehler P . Haptoglobin, hemopexin, and related defense pathways-basic science, clinical perspectives, and drug development. Front Physiol. 2014; 5:415. PMC: 4211382. DOI: 10.3389/fphys.2014.00415. View

20.

Yamanishi Y, Yamana S, Ishioka S, Yamakido M . Development of ischemic colitis and scleroderma renal crisis following methylprednisolone pulse therapy for progressive systemic sclerosis. Intern Med. 1996; 35(7):583-6. DOI: 10.2169/internalmedicine.35.583. View