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Article ; Online: Peri- and Post-operative Evaluation and Management of Atypical Hemolytic Uremic Syndrome (aHUS) in Kidney Transplantation.

Java, Anuja

2020 Volume 27, Issue 2, Page(s) 128–137

Abstract: Atypical hemolytic uremic syndrome (aHUS) is a severe thrombotic microangiopathy characterized by over-activation of the alternative complement pathway. The etiology of the dysregulated complement system is commonly a genetic variant in one or more ... ...

Abstract	Atypical hemolytic uremic syndrome (aHUS) is a severe thrombotic microangiopathy characterized by over-activation of the alternative complement pathway. The etiology of the dysregulated complement system is commonly a genetic variant in one or more complement proteins as identified in ∼ 60%-70% patients. The risk of recurrence after a kidney transplantation is high and depends on the underlying complement abnormality. For a long time, kidney transplantation was contraindicated in these patients because of the high rate of recurrence and subsequent allograft loss. Over the past decade, advancements in the understanding of etiopathogenesis of aHUS and approval of the anti-complement drug, eculizumab, have allowed for successful kidney transplantation in these patients. All patients with ESRD due to aHUS should undergo screening for complement genetic variants. Patients in whom a genetic variant is not identified or in whom a genetic variant of uncertain significance is identified should undergo further testing to determine etiology of disease. This review aims to shed light on the diagnostic and therapeutic considerations in patients with aHUS preceding and following kidney transplantation.
Language	English
Publishing date	2020-06-19
Publishing country	United States
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ISSN	1548-5609 ; 1548-5595
ISSN (online)	1548-5609
ISSN	1548-5595
DOI	10.1053/j.ackd.2019.11.003
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Article: The Role of Complement in Autoimmune Disease-Associated Thrombotic Microangiopathy and the Potential for Therapeutics.

Java, Anuja / Kim, Alfred H J

The Journal of rheumatology

2023 Volume 50, Issue 6, Page(s) 730–740

Abstract: The complement system is a tightly regulated, cascading protein network representing a key component linking the innate and humoral immune systems. However, if misdirected or dysregulated, it can be similarly damaging to host-tissue. The role of ... ...

Abstract	The complement system is a tightly regulated, cascading protein network representing a key component linking the innate and humoral immune systems. However, if misdirected or dysregulated, it can be similarly damaging to host-tissue. The role of complement dysregulation on vascular endothelial cells has been well established in atypical hemolytic uremic syndrome (aHUS), a thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and target organ injury. Yet, a great deal of complexity exists around the role of complement in TMA associated with other diseases. A further complicating factor is the cross-talk between complement, neutrophils, and coagulation pathways in the pathophysiology of TMA. Advancements in the understanding of the etiopathogenesis of aHUS paved the way for the successful development of anticomplement therapies (complement C5 inhibitors), which have revolutionized the treatment of aHUS. Therefore, a clearer understanding of the role of the complement system in TMA associated with other conditions will help to identify patients who would benefit from these therapies. This review aims to provide an assessment of the nature and extent of complement involvement in TMA associated with autoimmune diseases such as systemic lupus erythematosus, antiphospholipid syndrome, and scleroderma renal crisis. Defining the role of complement in TMA in these conditions will help to guide timely diagnosis and management.
MeSH term(s)	Humans ; Endothelial Cells/pathology ; Thrombotic Microangiopathies/complications ; Complement System Proteins ; Lupus Erythematosus, Systemic/complications ; Atypical Hemolytic Uremic Syndrome/complications ; Atypical Hemolytic Uremic Syndrome/pathology ; Complement C5
Chemical Substances	Complement System Proteins (9007-36-7) ; Complement C5
Language	English
Publishing date	2023-01-15
Publishing country	Canada
Document type	Journal Article ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	194928-7
ISSN	1499-2752 ; 0315-162X
ISSN (online)	1499-2752
ISSN	0315-162X
DOI	10.3899/jrheum.220752
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Article ; Online: Mutations in atypical hemolytic uremic syndrome provide evidence for the role of calcium in complement factor I.

Java, Anuja / Atkinson, John / Hu, Zheng / Pozzi, Nicola

Blood

2023 Volume 142, Issue 6, Page(s) 607–610

Abstract: Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy. Genetic variants in complement proteins are found in ≈60% of patients. Of these patients, ≈15% carry mutations in complement factor I (CFI). Factor I (FI) is a multidomain ... ...

Abstract	Atypical hemolytic uremic syndrome (aHUS) is a rare thrombotic microangiopathy. Genetic variants in complement proteins are found in ≈60% of patients. Of these patients, ≈15% carry mutations in complement factor I (CFI). Factor I (FI) is a multidomain serine protease that cleaves and thereby inactivates C3b and C4b in the presence of cofactor proteins. Crystal structures have shown that FI possesses 2 calcium-binding domains, low-density lipoprotein receptor class A (LDLRA) 1 and LDLRA2. Yet, the role of calcium in FI is unknown. We determined that 9 genetic variants identified in aHUS (N151S, G162D, G188A, V230E, A240G, G243R, C247G, A258T, and Q260D) cluster around the calcium-binding site of LDLRA1. Using site-directed mutagenesis, we established that the synthesis of all, except A258T, was impaired, implying defective protein folding, perhaps due to loss of calcium binding. To further explore this possibility, we generated 12 alanine mutants that coordinate with the calcium in LDLRA1 and LDLRA2 (K239A, D242A, I244A, D246A, D252A, E253A, Y276A, N279A, E281A, D283A, D289A, and D290A) and are expected to perturb calcium binding. Except for K239A and Y276A, none of the mutants was secreted. These observations suggest that calcium ions play key structural and functional roles in FI.
MeSH term(s)	Humans ; Atypical Hemolytic Uremic Syndrome/genetics ; Calcium ; Complement Factor I/genetics ; Complement Factor I/chemistry ; Complement Factor I/metabolism ; Complement System Proteins ; Mutation
Chemical Substances	Calcium (SY7Q814VUP) ; Complement Factor I (EC 3.4.21.45) ; Complement System Proteins (9007-36-7) ; CFI protein, human (EC 3.4.21.45)
Language	English
Publishing date	2023-04-19
Publishing country	United States
Document type	Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
ZDB-ID	80069-7
ISSN	1528-0020 ; 0006-4971
ISSN (online)	1528-0020
ISSN	0006-4971
DOI	10.1182/blood.2022019361
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Zs.MO 364: Show issues

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Article ; Online: Post-Transplant Thrombotic Microangiopathy due to a Pathogenic Mutation in Complement Factor I in a Patient With Membranous Nephropathy: Case Report and Review of Literature.

Saleem, Maryam / Shaikh, Sana / Hu, Zheng / Pozzi, Nicola / Java, Anuja

Frontiers in immunology

2022 Volume 13, Page(s) 909503

Abstract: Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ injury occurring due to endothelial cell damage and microthrombi formation in small vessels. TMA is primary when a genetic or acquired ... ...

Abstract	Thrombotic microangiopathy (TMA) is characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ injury occurring due to endothelial cell damage and microthrombi formation in small vessels. TMA is primary when a genetic or acquired defect is identified, as in atypical hemolytic uremic syndrome (aHUS) or secondary when occurring in the context of another disease process such as infection, autoimmune disease, malignancy or drugs. Differentiating between a primary complement-mediated process and one triggered by secondary factors is critical to initiate timely treatment but can be challenging for clinicians, especially after a kidney transplant due to presence of multiple confounding factors. Similarly, primary membranous nephropathy is an immune-mediated glomerular disease associated with circulating autoantibodies (directed against the M-type phospholipase A2 receptor (PLA2R) in 70% cases) while secondary membranous nephropathy is associated with infections, drugs, cancer, or other autoimmune diseases. Complement activation has also been proposed as a possible mechanism in the etiopathogenesis of primary membranous nephropathy; however, despite complement being a potentially common link, aHUS and primary membranous nephropathy have not been reported together. Herein we describe a case of aHUS due to a pathogenic mutation in complement factor I that developed after a kidney transplant in a patient with an underlying diagnosis of PLA2R antibody associated-membranous nephropathy. We highlight how a systematic and comprehensive analysis helped to define the etiology of aHUS, establish mechanism of disease, and facilitated timely treatment with eculizumab that led to recovery of his kidney function. Nonetheless, ongoing anti-complement therapy did not prevent recurrence of membranous nephropathy in the allograft. To our knowledge, this is the first report of a patient with primary membranous nephropathy and aHUS after a kidney transplant.
MeSH term(s)	Atypical Hemolytic Uremic Syndrome/diagnosis ; Atypical Hemolytic Uremic Syndrome/drug therapy ; Atypical Hemolytic Uremic Syndrome/genetics ; Complement Factor I/genetics ; Complement System Proteins/genetics ; Glomerulonephritis, Membranous/genetics ; Humans ; Mutation ; Thrombotic Microangiopathies/genetics
Chemical Substances	Complement System Proteins (9007-36-7) ; Complement Factor I (EC 3.4.21.45)
Language	English
Publishing date	2022-05-26
Publishing country	Switzerland
Document type	Case Reports ; Review ; Research Support, Non-U.S. Gov't
ZDB-ID	2606827-8
ISSN	1664-3224 ; 1664-3224
ISSN (online)	1664-3224
ISSN	1664-3224
DOI	10.3389/fimmu.2022.909503
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Article: Rare variants in genes coding for components of the terminal pathway of the complement system in preeclampsia.

Lokki, A / Triebwasser, Michael / Daly, Emma / Cohort, Finnpec / Kurki, Mrtja / Perola, Markus / Auro, Kirsi / Salmon, Jane / Java, Anuja / Daly, Mark / Atkinson, John / Laivuori, Hannele / Meri, Seppo

Research square

2024

Abstract: Preeclampsia is a common multifactorial disease of pregnancy. Dysregulation of the complement activation is among emerging candidates responsible for disease pathogenesis. In a targeted exomic sequencing study we identified 14 variants within nine genes ... ...

Abstract	Preeclampsia is a common multifactorial disease of pregnancy. Dysregulation of the complement activation is among emerging candidates responsible for disease pathogenesis. In a targeted exomic sequencing study we identified 14 variants within nine genes coding for components of the membrane attack complex (MAC, C5b-9) that are associated with preeclampsia. We found two rare missense variants in the
Language	English
Publishing date	2024-04-02
Publishing country	United States
Document type	Preprint
DOI	10.21203/rs.3.rs-4121735/v1
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Article ; Online: Differentiating Hemolysis, Elevated Liver Enzymes, and Low Platelet Count Syndrome and Atypical Hemolytic Uremic Syndrome in the Postpartum Period.

Burwick, Richard M / Moyle, Kimberly / Java, Anuja / Gupta, Megha

Hypertension (Dallas, Tex. : 1979)

2021 Volume 78, Issue 3, Page(s) 760–768

Abstract: Figure: see text]. ...

Abstract	[Figure: see text].
MeSH term(s)	Adult ; Atypical Hemolytic Uremic Syndrome/blood ; Atypical Hemolytic Uremic Syndrome/diagnosis ; Creatinine/blood ; Diagnosis, Differential ; Female ; HELLP Syndrome/blood ; HELLP Syndrome/diagnosis ; Humans ; L-Lactate Dehydrogenase/blood ; Platelet Count ; Postpartum Period ; Pregnancy ; Young Adult
Chemical Substances	Creatinine (AYI8EX34EU) ; L-Lactate Dehydrogenase (EC 1.1.1.27)
Language	English
Publishing date	2021-07-19
Publishing country	United States
Document type	Journal Article
ZDB-ID	423736-5
ISSN	1524-4563 ; 0194-911X ; 0362-4323
ISSN (online)	1524-4563
ISSN	0194-911X ; 0362-4323
DOI	10.1161/HYPERTENSIONAHA.121.17311
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Article ; Online: Rare Dysfunctional

Seddon, Johanna M / Rosner, Bernard / De, Dikha / Huan, Tianxiao / Java, Anuja / Atkinson, John

Ophthalmology science

2022 Volume 3, Issue 2, Page(s) 100265

Abstract: Purpose: To evaluate associations between rare dysfunctional : Design: Prospective, longitudinal study.: Participants: Patients aged 55 to 80 years at baseline identifying as White with non-AAMD in 1 or both eyes at baseline were included. Follow- ... ...

Abstract	Purpose: To evaluate associations between rare dysfunctional Design: Prospective, longitudinal study. Participants: Patients aged 55 to 80 years at baseline identifying as White with non-AAMD in 1 or both eyes at baseline were included. Follow-up grades were assigned as early, intermediate, or AAMD (GA or NV). Methods: Analyses were performed using the Seddon Longitudinal Cohort Study (N = 2116 subjects, 3901 eyes, and mean follow-up of 8.3 years) and the Age-Related Eye Disease Study (N = 2837 subjects, 5200 eyes, and mean follow-up of 9.2 years). Main outcome measures: Progression to AAMD, GA, or NV. Results: In the prospective cohort of 4953 subjects (9101 eyes with non-AAMD at baseline), 1% were type 1 rare Conclusions: Results suggest that carriers of rare dysfunctional type 1 Financial disclosures: Proprietary or commercial disclosure may be found after the references.
Language	English
Publishing date	2022-12-30
Publishing country	Netherlands
Document type	Journal Article
ISSN	2666-9145
ISSN (online)	2666-9145
DOI	10.1016/j.xops.2022.100265
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Article ; Online: Show Me CKDintercept Initiative: A Collective Impact Approach to Improve Population Health in Missouri.

Laue, Katelyn / Schultz, Megan / Talbot-Montgomery, Elizabeth / Garrick, Alexandra / Java, Anuja / Corbett, Christine / Lammert, Dana M / Rogers, JoAnna / Davis, Kathleen / Malhotra, Kunal / Philipneri, Marie / Kimbel, Mary Ann / Mustafa, Reem A / Hardesty, Valerie

Mayo Clinic proceedings. Innovations, quality & outcomes

2024 Volume 8, Issue 1, Page(s) 82–96

Abstract: Ninety percent of people with chronic kidney disease (CKD) remain undiagnosed, most people at risk do not receive guideline-concordant testing, and disparities of care and outcomes exist across all stages of the disease. To improve CKD diagnosis and ... ...

Abstract	Ninety percent of people with chronic kidney disease (CKD) remain undiagnosed, most people at risk do not receive guideline-concordant testing, and disparities of care and outcomes exist across all stages of the disease. To improve CKD diagnosis and management across primary care, the National Kidney Foundation launched a collective impact (CI) initiative known as Show Me CKDintercept. The initiative was implemented in Missouri, USA from January 2021 to June 2022, using a data strategy, stakeholder engagement and relationship mapping, learning in action working groups (LAWG), and a virtual leadership summit. The Reach, Effectiveness, Adoption, Implementation, and Maintenance framework was used to evaluate success. The initiative united 159 stakeholders from 81 organizations (Reach) to create an urgency for change and engage new CKD champions (Effectiveness). The adoption resulted in 53% of participants committed to advancing the roadmap (Adoption). Short-term results reported success in laying a foundation for CI across Missouri. The long-term success of the CI initiative in addressing the public health burden of kidney disease remains to be determined. The project reported the potential use of a CI initiative to build leadership consensus to drive measurable public health improvements nationwide.
Language	English
Publishing date	2024-01-12
Publishing country	Netherlands
Document type	Journal Article
ISSN	2542-4548
ISSN (online)	2542-4548
DOI	10.1016/j.mayocpiqo.2023.12.004
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Article: Clinicopathologic Implications of Complement Genetic Variants in Kidney Transplantation.

Ren, Zhen / Perkins, Stephen J / Love-Gregory, Latisha / Atkinson, John P / Java, Anuja

Frontiers in medicine

2021 Volume 8, Page(s) 775280

Abstract: Genetic testing has uncovered rare variants in complement proteins associated with thrombotic microangiopathy (TMA) and C3 glomerulopathy (C3G). Approximately 50% are classified as variants of uncertain significance (VUS). Clinical risk assessment of ... ...

Abstract	Genetic testing has uncovered rare variants in complement proteins associated with thrombotic microangiopathy (TMA) and C3 glomerulopathy (C3G). Approximately 50% are classified as variants of uncertain significance (VUS). Clinical risk assessment of patients carrying a VUS remains challenging primarily due to a lack of functional information, especially in the context of multiple confounding factors in the setting of kidney transplantation. Our objective was to evaluate the clinicopathologic significance of genetic variants in TMA and C3G in a kidney transplant cohort. We used whole exome next-generation sequencing to analyze complement genes in 76 patients, comprising 60 patients with a TMA and 16 with C3G. Ten variants in complement factor H (
Language	English
Publishing date	2021-11-29
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	2775999-4
ISSN	2296-858X
ISSN	2296-858X
DOI	10.3389/fmed.2021.775280
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Article ; Online: Treatment preference and quality of life impact: ravulizumab vs eculizumab for atypical hemolytic uremic syndrome.

Mauch, Teri J / Chladek, Michael R / Cataland, Spero / Chaturvedi, Shruti / Dixon, Bradley P / Garlo, Katherine / Gasteyger, Christoph / Java, Anuja / Leguizamo, Jorge / Lloyd-Price, Lucy / Pham, Tan P / Symonds, Tara / Tomazos, Ioannis / Wang, Yan

Journal of comparative effectiveness research

2023 Volume 12, Issue 9, Page(s) e230036

Abstract: Aim: ...

Abstract	Aim:
MeSH term(s)	Adult ; Humans ; Child ; Middle Aged ; Atypical Hemolytic Uremic Syndrome/drug therapy ; Atypical Hemolytic Uremic Syndrome/chemically induced ; Quality of Life ; Antibodies, Monoclonal, Humanized ; Complement Inactivating Agents/therapeutic use ; Complement Inactivating Agents/adverse effects
Chemical Substances	ravulizumab (C3VX249T6L) ; eculizumab (A3ULP0F556) ; Antibodies, Monoclonal, Humanized ; Complement Inactivating Agents
Language	English
Publishing date	2023-07-29
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	2669725-7
ISSN	2042-6313 ; 2042-6305
ISSN (online)	2042-6313
ISSN	2042-6305
DOI	10.57264/cer-2023-0036
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