A kidney disease where IgA antibodies accumulate in the kidneys, causing inflammation and damage over time.
✦ AI summary
IgA nephropathy (IgAN) is a kidney disease where deposits of a specific antibody form (galactose-deficient IgA1) build up in the kidney and trigger immune complexes that damage kidney tissue, and researchers are working to better define exactly which IgA forms are most harmful [8][12]. Diagnosis currently usually needs a kidney biopsy, but scientists are developing blood tests to detect this abnormal IgA more sensitively, which could help avoid biopsies in the future [3]. Research is also looking at biomarkers to track disease activity and progression, such as blood lipid ratios linked to kidney scarring [1], urine markers that may reflect treatment response and relapse [7], and gene-expression patterns in kidney tissue tied to inflammation severity [10]. Some patients with IgAN also have overlapping conditions, like a podocyte (filtering cell)-related antibody sometimes found alongside minimal change disease, which is linked to different treatment responses and relapse patterns [5][6]; and in rare cases, IgAN has appeared alongside other genetic immune conditions [2]. Beyond lab findings, patients report symptoms like persistent, aching loin (flank) pain that aff
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Atypical Wiskott-Aldrich syndrome presenting with normal platelet volume and end-stage renal disease: from misdiagnosis as ITP to combined transplantation.
We report a 30-year-old male with a novel WAS mutation (c.252C > A, p.F84L) who was misdiagnosed with immune thrombocytopenia (ITP) for years. Despite persistent thrombocytopenia, his mean platelet volume remained normal, an atypical feature of Wiskott-Aldrich syndrome (WAS). His course was complica…
The relationship between the non-high-density lipoprotein cholesterol to high-density lipoprotein cholesterol ratio and tubular atrophy/interstitial fibrosis in patients with IgA nephropathy.
Background: The tubular atrophy/interstitial fibrosis (T) lesions of the Oxford Classification is a key prognostic determinant in IgA nephropathy (IgAN). The non-high-density lipoprotein cholesterol to high-density lipoprotein cholesterol ratio (NHHR) is an emerging lipid marker, but its link with r…
Evaluation of clinicopathological features of renal biopsies in non-lupus rheumatic diseases: a university hospital experience.
Renal involvement is a common condition in rheumatologic diseases. The aim of this study was to evaluate the clinical presentations and histopathological results in patients diagnosed with non-lupus rheumatic diseases who underwent renal biopsy. The records of 900 renal biopsies performed at our hos…
Circulating Antinephrin Antibodies in Adult Chinese Patients With IgAN and Nephrotic-Range Proteinuria.
Antinephrin autoantibodies are detectable in podocytopathies such as minimal change disease (MCD) and primary focal segmental glomerulosclerosis (FSGS); however, their prevalence and clinical relevance in IgA nephropathy (IgAN) with nephrotic-range proteinuria (NRP) and nephrotic syndrome (NS) are u…
Ultrasensitive Immunoassay Using a Novel Galactose-Deficient IgA1 Antibody and Its Clinical Application in the Diagnosis of IgAN.
IgA nephropathy (IgAN) diagnosis remains dependent on invasive renal biopsy because currently available serologic biomarkers, including galactose-deficient IgA1 (Gd-IgA1), have limited diagnostic performance. This study aimed to develop and evaluate a high-sensitivity time-resolved fluorescence immu…
Anti-CD20 Therapies and Antinephrin Autoantibodies in IgA Nephropathy With Minimal Change Disease.
In IgA nephropathy (IgAN) with minimal change disease (MCD) (MCD-IgAN), the efficacy of anti-CD20 monoclonal antibodies (mAbs) and the profile of antinephrin antibodies remain poorly understood. We evaluated the efficacy of anti-CD20 mAbs or combined short-course low-dose steroids (anti-CD20 mAbs gr…
Characterization of Patient-Reported Loin Pain in IgAN.
Loin pain is a frequently reported symptom in IgA nephropathy (IgAN), but its significance is unclear. No known data exists regarding its prevalence, characteristics, or its impact on daily life, resulting in little support being offered for its management. A UK-wide mixed-methods study was conducte…
The mechanism of intestinal IgA class switching regulated by TRIM21 through down-regulation of AID in IgA nephropathy.
Aberrant mucosal IgA production is central to IgA nephropathy (IgAN), yet the regulatory mechanisms remain poorly understood. TRIM21 has been identified as a key gene in IgAN, but its functional contribution to disease pathogenesis is unclear. In this study, we investigated whether TRIM21 regulates…