IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?IgA Nephropathy with Thrombotic microangiopathy: Is this secondary thrombotic microangiopathy or IgA nephropathy-triggered atypical Hemolytic Uremic Syndrome?

Diniz, Hugo; Bandeira, Maria de Fátima Santos; Nunes, Ana; Besteiro, Bruno; Coimbra, J; Gomes, Filipa; Sampaio, Susana

doi:10.32932/pjnh.2020.01.050

Cited by 3 publications

(3 citation statements)

References 36 publications

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“…Disorders such as antineutrophil cytoplasmic antibodies-associated vasculitis, IgA vasculitis, and membranous nephropathy may be associated with TMA [1]. The findings of TMA can be observed in over half of patients with IgAN [8,9]. Also, microbial factors contribute to secondary IgAN by triggering the generation of pathogenic IgA and the deposition of IgA in the mesangium.…”

Section: Discussionmentioning

confidence: 99%

Diarrhea and Acute Tubular Necrosis Mimicking Hemolytic Uremic Syndrome in a Man With Immunoglobulin A (IgA) Nephropathy

Saibaba

Selvaraj

Viswanathan

et al. 2021

Cureus

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Immunoglobulin A (IgA) nephropathy is a heterogeneous disease with variable clinical presentations ranging from asymptomatic hematuria to advanced renal failure. A young male diagnosed with IgA vasculitis (skin, joints, and gastrointestinal) one month ago and placed on oral steroids presented with acute diarrhea, hemolytic anemia, renal failure (non-dialysis requiring), altered sensorium, and thrombocytopenia. The stool was found to be positive for Shiga toxin. He improved with methylprednisolone pulse alone, and renal biopsy showed acute tubular injury.

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Section: Discussionmentioning

confidence: 99%

Diarrhea and Acute Tubular Necrosis Mimicking Hemolytic Uremic Syndrome in a Man With Immunoglobulin A (IgA) Nephropathy

Saibaba

Selvaraj

Viswanathan

et al. 2021

Cureus

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“…TMA presents as the formation of small thrombosis in the renal microvasculature, often observed in IgAN through renal biopsy (16). Renal biopsy is necessary for identifying TMA changes in IgAN (17).…”

Section: Clinical Features and Diagnosismentioning

confidence: 99%

Thrombotic microangiopathy in IgA nephropathy: pathophysiology, histopathology, and treatment perspectives

Rastegar-Kashkouli,

Jafari,

Shahrokh

et al. 2024

J Nephropathol

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The current narrative review study aims to provide an overview of thrombotic microangiopathy (TMA) in immunoglobulin A nephropathy (IgAN), with a particular emphasis on its pathophysiology, histopathology, and treatment options. The prevalence and clinical significance of TMA in IgAN may vary across different populations. Estimates suggest that TMA events occur in 2-50% of patients with IgAN. Endothelial injury is a key factor in TMA development in IgAN, triggered by immune complex deposition, complement activation, and potentially hypertension. TMA in IgAN correlates with vascular lesions, including arterial intimal sclerosis, arteriolar lumen reduction, and smooth muscle hypertrophy. Notably, patients with TMA show more intense deposition of C4, C3d, and C5b-9 complements. Treatment involves blood pressure management, immunosuppression, and targeted therapies such as eculizumab.

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“…Therefore; this study may not necessarily reflect the degree of association between these two entities and the clinical behavior of such conditions in the pediatric age group, apart from being an extensive and well-designed one with valuable results [ 24 ]. Furthermore, the coincidence of atypical hemolytic uremic syndrome with IgA nephropathy was described in two different case reports, one of which was in a pediatric patient [ 25 , 26 ]. Therefore, a comprehensive understanding of this phenomenon in pediatrics requires further studies describing the pathogenesis, the extent of association, clinical behavior, and the overall outcome with dedication for the pediatric age group.…”

Section: Microvascular Injury (Thrombotic Microangiopathy)mentioning

confidence: 99%

Histological Features of IgA Nephropathy in Pediatrics and the Magnitude of the Disease in Saudi Children

Zahrani

2023

International Journal of Pediatrics

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Objectives. This review addresses the microscopic features of immunoglobulin A nephropathy (IgA nephropathy), its prognostic variables in children, and measures to which extent these features and variables differ from adults. Furthermore, it describes the extent of this disease process among children in Saudi Arabia and the rest of the Arab countries and compares it with the data from the West and the Far East. Method. All the original work described the histological features of pediatric IgA nephropathy, and studies involved in developing the prognostic classification of IgA nephropathy, Oxford Classification, were reviewed. Moreover, the studies describing the crescent prevalence and outcome in pediatric IgA nephropathy in addition to thrombotic microangiopathy association were studied. National studies describing the prevalence of pediatric IgA nephropathy and pediatric crescentic glomerulonephritis were tracked with an overview of the regional data from the rest of the Arab world. Results. IgA nephropathy in children showed more glomerular proliferative changes and less glomerular vascular and tubule-interstitial chronic injury compared to adults. The reference study that described the association between thrombotic microangiopathy and IgA nephropathy did not include the pediatric age group. Moreover, it was found that the data from the Middle East was not encountered in developing the original and updated IgA nephropathy Oxford Classification. Furthermore, the prevalence of IgA nephropathy in children is described in the regional literature, but its histological features were not well detailed. Finally, the percentage of crescentic glomerulonephritis (GN) due to IgA nephropathy is less in our country compared to the West and concords with the Far East findings. Conclusion. A well-designed regional study addressing IgA nephropathy in Middle East children with a focus on histological features, association with crescent, and thrombotic microangiopathy and challenging the validity of the updated IgA nephropathy Oxford Classification is recommended.

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Cited by 3 publications

References 36 publications

Diarrhea and Acute Tubular Necrosis Mimicking Hemolytic Uremic Syndrome in a Man With Immunoglobulin A (IgA) Nephropathy

Diarrhea and Acute Tubular Necrosis Mimicking Hemolytic Uremic Syndrome in a Man With Immunoglobulin A (IgA) Nephropathy

Thrombotic microangiopathy in IgA nephropathy: pathophysiology, histopathology, and treatment perspectives

Histological Features of IgA Nephropathy in Pediatrics and the Magnitude of the Disease in Saudi Children

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