Aggregation of erythrocyte sodium/lithium countertransport activity in families of patients with immunoglobulin A nephropathy

1992 ◽  
Vol 83 (2) ◽  
pp. 241-245 ◽  
Author(s):  
A. Fabbri ◽  
R. Boero ◽  
E. Degli Esposti ◽  
C. Guarena ◽  
A. Lucatello ◽  
...  

1. We evaluated the inheritance of erythrocyte Na+/Li+ countertransport activity in IgA nephropathy by assessing this parameter in 19 patients with biopsy-proven IgA nephropathy and in their 53 relatives (32 parents and 21 siblings). The possible use of erythrocyte Na+/Li+ countertransport activity as a marker of poor prognosis was also evaluated. 2. A significant correlation was found between ‘familial’ and proband Na+/Li+ countertransport activity, but not between that of spouses. 3. Mean blood pressure, although within the normal range, and Na+/Li+ countertransport activity were significantly higher in patients with proteinuria than in those without proteinuria. 4. Parents of proteinuric patients had a higher Na+/Li+ countertransport activity than parents of non-proteinuric patients. 5. In IgA nephropathy the inheritance of erythrocyte Na+/Li+ countertransport activity was preserved. Therefore genetic factors could play a role in the non-immunological progression of IgA nephropathy.

2011 ◽  
Vol 45 (10) ◽  
pp. 1284-1296 ◽  
Author(s):  
Jennifer L Rosselli ◽  
Stacey M Thacker ◽  
Julie P Karpinski ◽  
Katherine A Petkewicz

Objective: To review current literature regarding treatment options for immunoglobulin A nephropathy (IgAN). Data Sources: A MEDLINE search was performed using the terms IgA nephropathy, Berger's disease, immunoglobulin A nephropathy, angiotensin converting enzyme inhibitors, angiotensin II receptor blockers, fish oil, omega-3 fatty acids, statins. hydroxymethylglutaryl-CoA reductase Inhibitors, immunosuppressive therapy, corticosteroids, mycophenolate mofetil, cyclophosphamide, cyclosporine, azathioprine, leflunomide, antiplatelets, anticoagulants, vitamin E, infliximab, calcitriol, and intravenous immunoglobulins. A date limit was not set; however, focus was on publications from 1999 to June 2011 to review recent literature and therapeutic recommendations. Study Selection and Data Extraction: All articles in English, including studies conducted in humans, meta-analyses, review articles, guidelines, statements, and reference citations, were identified and evaluated. Data Synthesis: IgAN is the most common primary glomerulonephritis worldwide, leading to end-stage renal disease in 20–30% of patients. Evidence guiding management of IgAN has been sparse and clinical trials have not conclusively demonstrated effective treatments, largely due to suboptimal methodologies. Treatment strategies have included management of blood pressure and lipids, improvement or stabilization of kidney function, and reduction of proteinuria. This review of IgAN provides an update regarding standard and nonconventional treatment options based on recently published literature. Conclusions: Supportive therapies, including angiotensin blockade, should be considered as first-line therapy for patients with urine protein >0.5 g/day and/or blood pressure > 140/90 mmHg, Corticosteroids could be considered as add-on or monotherapy for patients with urine protein >1 g/day with preserved renal function. Conclusive data are lacking for general treatment recommendations for the use of other therapies for IgAN.


2019 ◽  
Vol 20 (24) ◽  
pp. 6199 ◽  
Author(s):  
Martina Perše ◽  
Željka Večerić-Haler

Immunoglobulin A (IgA) is the most abundant antibody isotype produced in humans, predominantly present in the mucosal areas where its main functions are the neutralization of toxins, prevention of microbial invasion across the mucosal epithelial barrier, and simultaneous maintenance of a physiologically indispensable symbiotic relationship with commensal bacteria. The process of IgA biosynthesis, interaction with receptors, and clearance can be disrupted in certain pathologies, like IgA nephropathy, which is the most common form of glomerulonephritis worldwide. This review summarizes the latest findings in the complex characteristics of the molecular structure and biological functions of IgA antibodies, offering an in-depth overview of recent advances in the understanding of biochemical, immunologic, and genetic factors important in the pathogenesis of IgA nephropathy.


Hypertension ◽  
2001 ◽  
Vol 38 (1) ◽  
pp. 81-85 ◽  
Author(s):  
Yoshio Konishi ◽  
Noriyuki Okada ◽  
Mikio Okamura ◽  
Takashi Morikawa ◽  
Michiaki Okumura ◽  
...  

Author(s):  
Loreto Gesualdo ◽  
Vincenzo Di Leo ◽  
Rosanna Coppo

Abstract The precise pathogenesis of immunoglobulin A nephropathy (IgAN) is still not clearly established but emerging evidence confirms a pivotal role for mucosal immunity. This review focuses on the key role of mucosa-associated lymphoid tissue (MALT) in promoting the onset of the disease, underlying the relationship among microbiota, genetic factors, food antigen, infections, and mucosal immune response. Finally, we evaluate potential therapies targeting microbes and mucosa hyperresponsiveness in IgAN patients.


2003 ◽  
Vol 5 (1) ◽  
pp. 44-49
Author(s):  
Hitoshi SUZUKI ◽  
Atsushi KURUSU ◽  
Toshimasa HISHIKI ◽  
Kazuhiko FUNABIKI ◽  
Satoshi HORIKOSHI ◽  
...  

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Lingzhi Wu ◽  
Di Liu ◽  
Ming Xia ◽  
Guochun Chen ◽  
Yu Liu ◽  
...  

Abstract Background Immunoglobulin A nephropathy (IgAN) is identified as mesangial IgA deposition and is usually accompanied by other immunofluorescence deposits. The impact of immunofluorescent features in IgAN patients, however, remains unclear. Methods Baseline clinicopathologic parameters and renal outcomes of 337 patients diagnosed with IgAN between January 2009 and December 2015 were analyzed. We then categorized these patients into four groups: without immunofluorescence deposits, mesangial-only, mesangial and glomerular capillary loops (GCLs), and GCLs-only. The study endpoint was end-stage kidney disease (ESKD) or a ≥ 50% decline in the estimated glomerular filtration rate (eGFR). Kaplan–Meier and Cox regression analyses were performed to calculate renal survival. Results Of the 337 IgAN patients, women comprised 57.0%. Compared to patients with IgA deposition in the mesangial-only group, patients with IgA deposition in the mesangial +GCLs group were much heavier, and exhibited higher systolic blood pressure, lower serum IgG levels, and heavier proteinuria (all P < 0.05). Patients with IgG deposition in the mesangial +GCLs group presented with higher levels of cholesterol, heavier proteinuria than IgG deposition in the mesangial-only group (both P < 0.05). Compared with the mesangial-only group exhibiting C3 deposits, patients in the mesangial +GCLs group with C3 deposition had a higher systolic blood pressure (P = 0.028). A total of 38 patients (11.3%) continued to the study endpoint after a median follow-up time of 63.5 months (range,49.8–81.4). Kaplan–Meier analysis and Cox regression analysis showed that C1q deposition in the mesangial +GCLs group predicted a poor renal prognosis. Conclusions IgA and IgG deposits in the mesangial region and GCLs were associated with more unfavorable clinical and histopathologic findings in IgAN patients. C1q deposition in the mesangial region and GCLs predicted a poor renal prognosis. However, the impact of the pattern of immunofluorescence deposits on renal outcomes remains to be proven by further investigation.


2020 ◽  
pp. 4909-4917
Author(s):  
Jonathan Barratt ◽  
John Feehally

Immunoglobulin A nephropathy (IgAN) is the commonest pattern of glomerulonephritis identified in areas of the world where renal biopsy is widely practised. It is defined pathologically by IgA deposition in the glomerular mesangium accompanied by a mesangial proliferative glomerulonephritis which may vary greatly in severity. Aetiology is uncertain, but abnormalities of IgA1 hinge-region O-glycosylation are consistently found. Clinical features—IgAN can present with (1) visible haematuria, typically in children and young adults, developing within a day or two of upper respiratory tract infection (‘synpharyngitic’); (2) asymptomatic nonvisible haematuria/proteinuria; (3) nephrotic syndrome (<5% of cases); (4) acute kidney injury (uncommon); and (5) chronic renal failure with up to 25% of patients reaching endstage renal failure within 20 years of diagnosis. Henoch–Schönlein purpura (HSP) is a small vessel systemic vasculitis characterized by small blood vessel deposition of IgA that predominantly affects the skin, joints, gut, and kidney, with nephritis that may be histologically indistinguishable from IgA nephropathy. Management—there is no treatment known to modify mesangial deposition of IgA. Treatment options are mostly directed at controlling blood pressure and limiting proteinuria through blockade of the renin–angiotensin–aldosterone axis. In the rare patient presenting with acute kidney injury in whom biopsy shows crescentic IgA nephropathy, a regimen such as those used for renal vasculitis and other forms of crescentic glomerulonephritis should be considered, for example, oral prednisolone in combination with cyclophosphamide.


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