Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic granulomatosis with polyangiitis (EGPA): a possible relationship

Cao, Mercedes; Ferreiro, Tamara; Leite, Bruna N.; Pita, Francisco José Castro; Bolaños, Luis; Valdés, Francisco; Alonso, Ángel; Vázquez, Eduardo; Mosquera, Juan Miguel; Trigás, María; Rodríguez, Santiago

doi:10.1007/s13730-017-0251-8

Cited by 6 publications

(3 citation statements)

References 32 publications

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“…HES has not been hitherto identified as one of the aHUS triggers. However, there have been a few reports of the association; for example, aHUS with a coincidental finding of eosinophilia (but not HES), aHUS with Churg Strauss syndrome (eosinophilic granulomatosis with polyangiitis), HES followed by renal TMA (but not aHUS), and HES followed by thrombotic thrombocytopenic purpura [2,[9][10][11][12]. Three cases of HES followed by renal TMA might be aHUS, especially since one case had low C3 [12].…”

Section: Discussionmentioning

confidence: 99%

Complement Factor I Gene Variant in an Atypical Hemolytic Uremic Syndrome Triggered by Hypereosinophilia Syndrome

Banjongjit,

Kittanamongkolchai,

Kanjanabuch

2023

Nephron

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Atypical hemolytic uremic syndrome (aHUS) is a condition characterized by acute kidney injury (AKI), thrombocytopenia, and microangiopathic hemolytic anemia secondary to complement pathway dysregulation. Several triggers have been identified as causing aHUS in genetically susceptible patients; however, hypereosinophilia syndrome (HES)-triggered aHUS has not been reported. In this article, we present a case of aHUS presented with generalized urticarial rashes and angioedema. The initial investigations revealed hypereosinophilia (maximal absolute eosinophil count of 6,840 cells/µL) with normal bone-marrow analyses; hence, idiopathic HES was diagnosed. During hospitalization, the patient developed convulsion, stuporous, and full-blown thrombotic microangiopathy (TMA), with AKI requiring temporary hemodialysis. A kidney biopsy confirmed the existence of renal TMA. Next-generation sequencing of the coding regions of aHUS-related genes was performed, revealing an underlying complement factor I (CFI) deficiency, a heterozygous variant p.P64L of <i>CFI</i> gene. The patient was successfully treated with high-dose steroids and extended duration of plasmapheresis.

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Section: Discussionmentioning

confidence: 99%

Complement Factor I Gene Variant in an Atypical Hemolytic Uremic Syndrome Triggered by Hypereosinophilia Syndrome

Banjongjit,

Kittanamongkolchai,

Kanjanabuch

2023

Nephron

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“…Thrombocytopenia is not a symptom of EGPA, but it is mentioned in some isolated reports. 7 The causes may be related to thrombotic microangiopathies, 8 hemolytic uremic syndrome, 9 heparin-associated thrombocytopenia, or thrombosis. 7 Thrombocytopenia due to Dengue virus was ruled out in our patient despite his living in endemic areas.…”

Section: Discussionmentioning

confidence: 99%

A fatal eosinophilic granulomatosis with polyangiitis case presenting intracerebral hemorrhage and thrombocytopenia

Nguyen¹,

Trần²,

Nguyen³

et al. 2023

Journal of Allergy and Clinical Immunology: Global

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“…Eight reported cases of TMA with PBE were diagnosed as HES [17,102,103,104,105,106]. Recently, one case of atypical HUS with AIN and PBE was reported and the first two case reports of concomitant atypical HUS and EGPA were published [107,108]. Activated eosinophils are known to favor thrombosis by various mechanisms.…”

Section: Eosinophilia In Kidney Diseasementioning

confidence: 99%

Eosinophilia and Kidney Disease: More than Just an Incidental Finding?

Gauckler

Smıth

Mayer

et al. 2018

JCM

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Peripheral blood eosinophilia (PBE), defined as 500 eosinophils or above per microliter (µL) blood, is a condition that is not uncommon but often neglected in the management of patients with chronic kidney disease (CKD), acute kidney injury (AKI), or patients on renal replacement therapy (RRT). The nature of PBE in the context of kidney diseases is predominantly secondary or reactive and has to be distinguished from primary eosinophilic disorders. Nonetheless, the finding of persistent PBE can be a useful clue for the differential diagnosis of underdiagnosed entities and overlapping syndromes, such as eosinophilic granulomatosis with polyangiitis (EGPA), IgG4-related disease (IgG4-RD), acute interstitial nephritis (AIN), or the hypereosinophilic syndrome (HES). For patients on RRT, PBE may be an indicator for bio-incompatibility of the dialysis material, acute allograft rejection, or Strongyloides hyperinfection. In a subset of patients with EGPA, eosinophils might even be the driving force in disease pathogenesis. This improved understanding is already being used to facilitate novel therapeutic options. Mepolizumab has been licensed for the management of EGPA and is applied with the aim to abrogate the underlying immunologic process by blocking interleukin-5. The current article provides an overview of different renal pathologies that are associated with PBE. Further scientific effort is required to understand the exact role and function of eosinophils in these disorders which may pave the way to improved interdisciplinary management of such patients.

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Two cases of atypical hemolytic uremic syndrome (aHUS) and eosinophilic granulomatosis with polyangiitis (EGPA): a possible relationship

Cited by 6 publications

References 32 publications

Complement Factor I Gene Variant in an Atypical Hemolytic Uremic Syndrome Triggered by Hypereosinophilia Syndrome

Complement Factor I Gene Variant in an Atypical Hemolytic Uremic Syndrome Triggered by Hypereosinophilia Syndrome

A fatal eosinophilic granulomatosis with polyangiitis case presenting intracerebral hemorrhage and thrombocytopenia

Eosinophilia and Kidney Disease: More than Just an Incidental Finding?

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