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Immunoglobulin A (IgA) dominant postinfectious glomerulonephritis (IgA PIGN) is a distinct clinical entity increasingly recognized in adult. It usually presents with reduced glomerular filtration rate, heavy proteinuria, and has unfavorable prognosis. Immunofluorescence study of renal biopsy specimens have IgA as dominant or codominant antibody. We...
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Background/aims:
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e commonest primary glomerular disease worldwide. Studies on its prevalence in Brazil are however scarce. Databases and clinical records from 10 reference centres were retrospectively reviewed. Clinical and laboratory features at the moment of the biopsy were retrieved (age, gender, presence of hematuria, serum creatini...
Background/Aims
Metabolic syndrome (MetS) may have an independent impact on the development of chronic kidney disease. This study examines the prevalence of MetS in subjects with IgA glomerulonephritis (IgAGN) and its impact on disease progression in a retrospective fashion.
Patients and Methods
Altogether, 174 subjects (104 males) were examined 1...
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Citations
... Less commonly, Immunoglobulin A (IgA) deposits may be present (1,2). In this case study, we present a patient with a history of Staphylococcus epidermidis infection, a past medical history of diabetes mellitus, and histopathological findings of APIGN with IgA deposition confirming a worse prognosis than primary IgA nephropathy (3)(4)(5). ...
Introduction
Acute postinfectious glomerulonephritis (APIGN) is an immunological glomerular disease that is an important health issue in developing countries. The incidence remains high in developing countries with a male-to-female ratio of 2:1 and age predominantly above 50 years. In this case study, we present a patient with a history of Staphylococcus epidermidis infection, a past medical history of diabetes mellitus, and histopathological findings of APIGN with Immunoglobulin A (IgA) deposition.
Methods
A 58-year-old male presented to the emergency room with a 6-day history of severe low back pain. Three days later, the patient developed fever, chills, abdominal pain in the upper quadrant and a subsequent lower limb cellulitis. Various immunological tests, imaging studies, and kidney biopsy were performed to arrive at a diagnosis.
Results
Following the diagnosis and treatment of Cholangitis and Staphylococcus epidermidis , further investigation led to a diagnosis of IgA-dominant APIGN. IgA-dominant APIGN was treated with antibiotics, renin-angiotensin-aldosterone system inhibitors and steroids, and the patient was discharged from the hospital.
Conclusion
In developing countries, APIGN is a relatively common presentation of kidney damage due to acute kidney injury and nephritic syndrome. IgA-dominant APIGN is a rare but increasingly recognized morphological variant in which IgA is the sole or dominant immunoglobulin. This unique presentation and multidisciplinary approach for diagnosing and treating IgA-dominant APIGN need to be considered and understood by healthcare professionals to better help these patients. Further investigation is needed to understand the best treatment of this IgA-dominant APIGN presentation and its prognosis.
... It is characterized by proliferative glomerulonephritis seen in light microscopy (LM) with dominant or codominant mesangial and glomerular capillary wall deposits of IgA detected by immunofluorescence (IF) in Open Access *Correspondence: abdallasagher@yahoo.com Saghar et al. BMC Nephrology (2022) 23:333 combination with hump-like deposits obtained by electron microscopy (EM) [1]. The Arkana laboratory database gathered more than 80,000 kidney biopsies and identified about 160 cases of IgA PIGN. ...
... Features supporting the diagnosis of IgA PIGN over IgA nephropathy include manifestation at an older age, acute kidney failure, a documented staphylococcal infection and low serum complement (C3). Histologic features of IgA PIGN comprise endocapillary hypercellularity, a strong infiltration of neutrophils, a marked deposition of C3 compared to IgA, and characteristic subepithelial humps visible on electron microscopy [1,2]. ...
Background
Immunoglobulin A dominant postinfectious glomerulonephritis (IgA PIGN) is a unique medical entity that is rare in the paediatric population. It usually presents with severe renal failure, heavy proteinuria, hypertension, and hypocomplementemia and frequently has an unfavourable prognosis. IgA PIGN generally occurs in association with staphylococcal infections and diabetes mellitus in adult patients. Other pathogens include Escherichia coli and Streptococcus sp. Immunofluorescence studies of kidney biopsy samples show IgA as dominant or codominant antibody.
Case presentation
We encountered a 3-year-old girl with IgA PIGN presenting with acute renal failure, oedema, hypertension, and heavy proteinuria of 7955 mg/g creatinine. Renal biopsy specimens showed diffuse glomerular endocapillary hypercellularity with prominent neutrophil and monocyte infiltration on light microscopy. Strong deposits of IgA and C3 were observed along the glomerular basement membranes and the mesangium by immunofluorescence microscopy, and electron microscopy revealed the presence of subepithelial humps.
The patient was managed with steroid (and probatory antibiotic) therapy and is now undergoing follow-up, with a significant improvement 6 months after the initial presentation (glomerular filtration rate (GFR) and cystatin C clearance rate of 165 ml/min/1.73m² and 106 ml/min/1.73m², respectively). No signs of bacterial infection were detectable.
Conclusion
This variant of IgA PIGN must be distinguished from other clinical entities, especially IgA nephropathy (mesangial IgA deposits) and postinfectious glomerulonephritis (C3, IgG and occasional IgM capillary loop deposits with or without mesangial distribution), since patients with IgA PIGN may require steroid treatment in addition to antibiotic therapy. Differential diagnosis should also include C3 glomerulopathy.
IgA PIGN is a recently identified disease entity that generally manifests in adult patients with both IgA and C3 mesangial and glomerular capillary wall deposits. We present a biopsy-proven case of IgA PIGN that manifested in a patient at an exceptionally young age and that has had a good clinical outcome. To the best of our knowledge, this is the youngest IgA PIGN patient reported thus far.
... Reports of the IgAdominant postinfectious glomerulonephritis have gradually increased in recent years, and many cases have an atypical presentation, making it difficult to differentiate the disease from primary IgA nephropathy, where the prognosis differs considerably. Most of these research were case reports, retrospective reviews or case series in a single center, and the sample size was often small, leading it hard to fully explain the clinicopathological characteristics and prognosis of the disease and the differentiation from similar diseases [5][6][7][8][9][10][11][12]. Therefore, indepth research of the clinicopathological features and prognosis of IgA-dominant postinfectious glomerulonephritis has an important clinical significance. ...
Background
The clinicopathological and prognostic features of IgA-dominant postinfectious glomerulonephritis and its difference from the primary IgA nephropathy remains to be investigated.
Methods
The clinical and pathological data of 6542 patients who underwent renal biopsy from 2009 to 2020 in our hospital were reviewed and 50 patients who met the selection criteria of IgA-dominant postinfectious glomerulonephritis were enrolled to conduct a retrospective and observational single-center study. The selection criteria were: meet the characteristics of IgA dominance or codominance in immunofluorescence, and conform to 3 of the following 5 criteria: 1.Clinical or laboratory evidence show that there is infection before or at the onset of glomerulonephritis; 2.The level of serum complement decreased; 3.Renal pathology is consistent with endocapillary proliferative glomerulonephritis; 4. Glomerular immunofluorescence staining showed complement C3 dominance or codominance; 5. Hump-like subepithelial immune complex deposition was observed under electron microscopy. According to age, sex, renal function (estimated glomerular filtration rate, eGFR) and follow-up time, the control group was constructed with 1:3 matched cases of primary IgA nephropathy. The clinicopathological and prognostic differences between the two groups were analyzed.
Results
The most common histological pattern of IgA-dominant postinfectious glomerulonephritis was acute endocapillary proliferative glomerulonephritis and exudative glomerulonephritis. Immunofluorescence showed mainly IgA deposition or IgA deposition only, mainly deposited in the mesangial area (deposition rate 100 %), with typical C3 high-intensity staining (intensity++~+++), mainly deposited in the mesangial area (deposition rate 92.0 %). The fluorescence intensity of kappa is usually not weaker than lambda. The probability of the appearance of typical hump-like electron deposition under electron microscopy is low. Compared to primary IgA nephropathy, patients with IgA-dominant postinfectious glomerulonephritis had higher proportion of crescents ( p = 0. 005) and endocapillary hypercellularity ( p < 0.001) in pathological manifestations. Using serum creatinine level doubled of the baseline or reached end-stage renal disease as the endpoint, the prognosis of IgA-dominant postinfectious glomerulonephritis patients was worse than that of primary IgA nephropathy patients ( p = 0.013).
Conclusions
The clinicopathological features of patients with IgA-dominant postinfectious glomerulonephritis was different from that of primary IgA nephropathy, and the prognosis was worse.
... The most common organisms for PIGN are Staphylococcus and gram-negative organisms. IgA PIGN patients present with acute kidney injury with severe proteinuria, and most of them have chronic renal disease [7]. ...
Post-infectious glomerulonephritis (PIGN) is an injury to glomerules mediated by the immune response after infection, and it is commonly seen in children. However, the elderly with immunocompromised conditions are at higher risk of developing PIGN after a recent infection. A 74-year-old female presented to the ER with a history of severe, sharp, on and off left flank pain for two days. Initial laboratory results workup were suggestive of acute kidney injury with no obvious reason. Dialysis was required as the renal function was deteriorating. Serologic test was negative for ANA (anti-neutrophil antibodies), C-ANCA (anti-neutrophil cytoplasmic antibodies), and P-ANCA (perinuclear anti-neutrophil cytoplasmic antibodies). C3 level was low, and anti-streptolysin O titer was high. Renal biopsy was performed. With reference to the clinical and histological examination, she was diagnosed with PIGN and diabetic nephropathy. After six months, the renal function was improving gradually until hemodialysis was stopped, and the PermcathTM (Medtronic) was removed with a creatinine level of 120 µmol/L. The elderly diabetic female developed PIGN with crescents that required dialysis, and dialysis was stopped after six months with good prognosis. Since PIGN is a very rare entity, the suspicion of PIGN in the elderly with acute kidney injury should be raised after a history of upper respiratory tract or skin infection.
... The initial description by Nasr et al. reported diabetic nephropathy in all biopsies. Nevertheless, the association between diabetes and IRGN-IgA is inconstant, reported in 8 to 55% of patients in previous studies and in 44% of patients in our study [10,12,[16][17][18][22][23][24]. As seen since the first report, Staphylococcus represents the most frequent germ (78% in our study, 60 to 100% in other studies) [10,12,17,18,24]. ...
Background:
Infection-related glomerulonephritis with IgA deposits (IRGN-IgA) is a rare disease but it is increasingly reported in the literature. Data regarding epidemiology and outcome are lacking, especially in Europe. We aimed to assess the clinical, pathologic and outcome data of IRGN-IgA.
Methods:
Clinical and outcome data from patients from 11 French centers over the 2007-2017 period were collected retrospectively. We reviewed pathologic patterns and immunofluorescence of renal biopsies and evaluated C4d expression in IRGN-IgA. We analyzed the correlation between histological presentation and outcome.
Results:
Twenty-seven patients (23 men, mean age: 62 ± 15 years) were included. Twenty-one (78%) had Staphylococcus aureus infection and twelve (44%) were diabetic. At the time of biopsy, 95.2% had haematuria, 48.1% had a serum creatinine level of > 4 mg/dL, and 16% had hypocomplementemia. The most common pathologic presentation included mesangial (88.9%) and endocapillary proliferative glomerulonephritis (88.9%) with interstitial fibrosis and tubular atrophy (IF/TA) (85.1%). Diffuse and global glomerular C4d expression was found in 17.8%, mostly in biopsies with acute or subacute patterns, and was associated with a short delay between infection and renal biopsy compared to segmental and focal staining. After median follow-up of 13.2 months, 23.1% died, 46.2% had persistent renal dysfunction and 15.4% reached end-stage renal disease. Renal outcome was correlated to IF/TA severity.
Conclusions:
Infection-related glomerulonephritis with IgA deposits is usually associated with Staphylococcus infections and mainly affects adult men. This entity has a poor prognosis which is correlated to interstitial fibrosis and tubular atrophy severity.
... [3,[6][7][8][9][10] Previous reports suggest that IgA-dominant glomerulonephritis is common in elderly diabetic individuals and male predominance. [11] In our series, all but [15] Features that favor IgA-dominant IRGN over IgAN include initial presentation in older age or in a diabetic patient, acute renal failure, intercurrent culture-documented staphylococcal infection, hypocomplementemia, diffuse glomerular endocapillary hypercellularity with prominent neutrophil infiltration on LM, stronger IF staining for C3 than IgA, and the presence of subepithelial humps on EM. [16,17] Finally, stronger mesangial staining for lambda light chains than kappa light chains may point to IgAN. Both these entities need to be differentiated as prognosis and treatment outcomes vary. ...
IgA-dominant infection-related glomerulonephritis (IRGN) is a distinct morphologic variant of IRGN, characterized by dominant or codominant glomerular deposits of IgA, mostly in elderly and patients with diabetes. More cases are being reported in recent times due to increased awareness of the disease entity and increased rate of Staphylococcus infection. It usually presents as rapidly progressive renal failure with proteinuria, and treatment guidelines for this disease entity are not well defined. We report here 12 cases of IgA-dominant IRGN seen over a period of 5 years from a single center. Clinical features, biopsy findings, treatment, and outcomes were analyzed. Out of 12 patients, eight were males. The mean age of presentation was 52.4 ± 21 years. Skin was the most common site of infection seen in six patients. Gross hematuria was seen in 4 patients and 11 had nephrotic proteinuria. Eleven had low serum C3. Only two patients had diabetes. Methicillin-resistant Staphylococcus aureus (MRSA) was the most common organism isolated in six patients. Most common histopathology was crescentic glomerulonephritis seen in seven patients, followed by endocapillary proliferation in three and diffuse proliferative glomerulonephritis in two. Hemodialysis was done in eight patients and six patients received steroid therapy. End-stage renal disease developed in three patients, chronic kidney disease in three, and three patients died due to sepsis. Various infections including MRSA and Escherichia coli were associated with IgA-dominant IRGN both in patients with diabetes and nondiabetics. Suspicion and recognition of the disease is important as it has therapeutic and prognostic implications. © 2017 Indian Journal of Nephrology | Published by Wolters Kluwer - Medknow.
Background
Infection-related glomerulonephritis with IgA deposits (IRGN-IgA) is being more widely recognized but the precise epidemiology and outcome is lacking, particularly in Europe. We aimed to assess clinical, pathologic and outcome data of IRGN-IgA.
Methods
Clinical and outcome data from patients from 11 French centers over the 2007-2017 period were retrospectively collected. We reviewed pathologic patterns and immunofluorescence of renal biopsies and evaluated C4d expression in IRGN-IgA. We analyzed correlation between histological presentation and outcome using the Chi square test (qualitative data) and Kruskal-Wallis test (quantitative data).
Results
Twenty-seven patients (23 men, mean age: 62 ± 15 years) were included. Most of them had a Staphylococcus aureus infection (77.8%) and 44.4% were diabetic. At the time of biopsy, 95.2% had haematuria, 48.1% had a serum creatinine >4 mg/dL, and 16% had a hypocomplementemia. The most common pathologic presentation included mesangial (88.9%) and endocapillary proliferative glomerulonephritis (88.9%) with interstitial fibrosis with tubular atrophy (IF/TA) (85.1%). Diffuse and global glomerular C4d expression, found in 17.8% of the cases, was most frequently observed in biopsies with acute or subacute pattern and associated with a shorter delay between infection and renal biopsy compared to segmental and focal staining. After a median follow-up of 13.2 months, 23.1% died, 46.2% had persistent renal dysfunction and 15.4% reached end-stage renal disease. Renal outcome was correlated to IF/TA severity.
Conclusions
Infection-related glomerulonephritis with IgA deposits is usually associated with Staphyloccus infections and mainly affects adult men. This entity has a poor prognosis which is correlated to interstitial fibrosis and tubular atrophy severity.
We report a case of IgA-dominant postinfectious glomerulonephritis in a 49-yearold man presenting with acute kidney injury, nephrotic range proteinuria and hematuria. He suffered from ischemic heart disease, cardiac insufficiency, mitral regurgitation, tricuspid insufficiency, septal aneurysm and hypertension. Renal biopsy revealed segmental and focal endocapillary and mesangial hypercellularity, and thickening of the glomerular capillary wall. Immunofluorescence showed co-dominant strong coarse granular immunostaining of IgA, IgG and C3 mainly along the glomerular capillary wall. On electron microscopy some large subepithelial hump-shaped deposits were present. In summary, this case demonstrates the presence of a broad spectrum of glomerular histological findings in postinfectious glomerulonephritis. © 2016, Versalius University Medical Publisher. All rights reserved.
IgA-dominant infection-associated glomerulonephritis (IgA-dominant IAGN) is a unique form of glomerulonephritis. There are numerous case reports in the literature. However, the risk factors, treatment approach, and outcomes of the disease are not clearly characterized.
We completed a pooled analysis based on published literature. Clinical features, laboratory findings, and histopathological changes were analyzed. A logistic regression model was employed to identify the determinants of disease outcome, for example, end-stage renal disease (ESRD) or death.
Seventy-eight patients with IgA-dominant IAGN from 28 reports were analyzed. All of these patients showed granular IgA deposits predominantly along the glomerular peripheral capillary walls using immunofluorescence and majority showed subepithelial 'hump-shaped' electron-dense deposits using electron microscopy. The majority of patients had hematuria (76/78), proteinuria (75/78), acute kidney injury (AKI) (66/78) and hypocomplementemia (43/75) without a previous history of renal disease. All of the patients had clinical infections at the time of presentation. Skin infections (19/78) and visceral abscesses (15/78) were frequently encountered, and staphylococcus was the most common pathogen. After treatment with antibiotics and/or supportive therapy, the renal function of 42 patients (54.5%) improved, 9 patients (11.7%) had persistent renal dysfunction, 15 patients (19.5%) progressed to ESRD, and 11 patients (14.3%) died. A multivariate regression analysis revealed that age (odds ratio [OR], 30.71; 95% confidence interval [CI], 2.53-373.07; p = 0.007) and diabetes mellitus (DM) (OR, 16.65; 95% CI, 1.18-235.84; p = 0.038) were independent risk factors for ESRD or death.
IgA-dominant IAGN has unique clinicopathological manifestations and treatment responses. Age and DM are independent risk factors associated with an unfavorable prognosis for IgA-dominant IAGN. © 2015 S. Karger AG, Basel.
