Nephron
First Department of Pathology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Published: April 2024
Tubulointerstitial nephritis with uveitis syndrome is a rare, immune-mediated entity, characterized by oculo-renal inflammation. Diagnosis requires the exclusion of all other causes of tubulointerstitial nephritis (TIN). We present 6 patients with clinical, laboratory, and renal biopsy findings denotative of tubulointerstitial nephritis with uveitis syndrome. All our patients experienced ocular and renal manifestations, defined by bilateral uveitis and photosensitivity, along with a decline of renal function. In some patients, increased serum creatinine was accompanied by non-nephrotic range proteinuria, glucosuria or "full-blown" Fanconi syndrome. The rest of the laboratory evaluation was normal apart from the presence of elevated erythrocyte sedimentation rate and increased urine β2-microglobulin, as well as normochromic, normocytic anemia in some cases. All patients underwent renal biopsy. Histochemical (PAS, Masson, silver, Congo-red) and immunohistochemical stains for immune cell populations (CD3, CD20, CD4, CD8, PGM1, CD138) and for the assessment of β2-microglobulin were conducted. Electron microscopy examination of the biopsies was also performed. Follow-up, ranging from 18 months to 10 years, was available for 4 patients. Histological evaluation revealed interstitial inflammatory infiltration consisting mainly of lymphocytes, with a T-cell predominance, along with several macrophages. Inflammation severity varied among different patients, with some showing scarce foci of immune cell clusters, while others demonstrated a dense, diffuse interstitial infiltration. Interestingly, in 2 cases, a granulomatous pattern, characterized by non-necrotic, ill-defined granulomas was detected. Tubulitis was also encountered in some patients. A divergence was noted regarding the chronicity index, with different levels of tubular atrophy, interstitial fibrosis, and global glomerulosclerosis among different cases. β2-Microglobulin immunohistochemical evaluation revealed a substantial diminishment of cytoplasmic staining in tubular epithelial cells compared to control kidneys. The most notable finding derived from electron microscopy examination was the presence, in 1 patient, of scattered granular electron-dense deposits along some tubular basement membranes. First-line treatment included steroids, supplemented in some cases by additional immunosuppressive agents. Three patients experienced a partial or complete response, while progressive renal damage was observed in a case with severe chronic lesions and persistence of inflammation-triggering factor. Our cases seem to represent progressive stages within the continuum of disease evolution. Patients with more prominent inflammation might represent a more initial state, while those with a more severe chronicity index, probably depict more advanced stages. While the predominance of T-cells predicates a cell-mediated autoimmune mechanism, as the driving force of the disease occurrence, the presence of immune complexes in more advanced stages might indicate the involvement of humoral immunity as a late event during the disease course.
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http://dx.doi.org/10.1159/000533402 | DOI Listing |
Typical renal involvement of antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is pauci-immune glomerulonephritis that presents clinically as rapidly progressive renal failure (RPRF). Here, we report an unusual presentation of myeloperoxidase (MPO)-specific ANCA with isolated involvement of the tubulointerstitium in the form of peritubular capillaritis as the sole lesion without any involvement of the glomerulus. A 52-year-old woman with no previous comorbidities presented with nonspecific symptoms such as fatigue, dysuria, and nausea for two months.
View Article and Find Full Text PDFNephrology (Carlton)
January 2025
Division of Nephrology, Department of Internal Medicine, Faculty of Medicine, Thammasat University, Pathumthani, Thailand.
The case report presents a male patient in his mid-60s with a history of hypertension, benign prostatic hyperplasia and chronic kidney disease (CKD). He presented with gradually increasing serum creatinine levels and hyperglobulinemia, leading to suspicion of multiple myeloma. However, subsequent testing revealed features consistent with systemic lupus erythematosus (SLE) and IgG4-related kidney disease (IgG4-RKD).
View Article and Find Full Text PDFJ Am Soc Nephrol
January 2025
Centre de Recherche des Cordeliers, INSERM, Sorbonne Université, Université Paris Cité, F-75006 Paris, France.
The renal tubule and collecting duct express a large number of proteins, all having putative immunoreactive motives. Therefore, all can be the target of pathogenic autoantibodies. However, autoimmune tubulopathies seem to be rare and we hypothesize that they are underdiagnosed.
View Article and Find Full Text PDFClin Kidney J
January 2025
Department of Medicine, Division of Nephrology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea.
Background: Although kidney biopsy is definitive for the diagnosis of acute interstitial nephritis (AIN) and acute tubular necrosis (ATN), its invasiveness limits its use. We aimed to identify urine biomarkers for differentiating AIN and ATN and to predict the response of patients with AIN to steroid treatment.
Methods: In this prospective cohort study, biopsy-proven ATN ( = 34) and AIN ( = 55) were included.
Int J Biol Sci
January 2025
CAMS Key Laboratory of Antiviral Drug Research, Institute of Medicinal Biotechnology, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, 100050, China.
Although therapies based on direct-acting antivirals (DAAs) effectively eradicate hepatitis C virus (HCV) in patients, there is still a high risk of liver fibrosis even after a sustained virological response. Therefore, it is of great clinical importance to understand the mechanism of potential factors that promote liver fibrosis after virological cure by treatment with DAAs. Here, we found that tubulointerstitial nephritis antigen-like 1 (TINAGL1) is significantly increased in HCV-infected hepatocytes and in the liver of patients with liver fibrosis, and that higher TINAGL1 expression persists in HCV-eradicated hepatocytes after treatment with DAAs.
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