RESUMEN
Amyloidosis is a rare cause of inherited kidney disease, with most variants responsible for prominent glomerular involvement. In this issue, Kmochová et al. reported the first description of autosomal dominant medullary amyloidosis due to apolipoprotein A4 variants, resulting in slowly progressive chronic kidney disease with minimal proteinuria. Combining next-generation sequencing with histopathological studies incorporating Congo red staining and mass spectrometry should be considered in the diagnostic workup of hereditary tubulointerstitial disorders not identified after routine genetic testing.
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Amiloidosis , Nefritis Intersticial , Insuficiencia Renal Crónica , Humanos , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/genética , Nefritis Intersticial/complicaciones , Amiloidosis/diagnóstico , Amiloidosis/genética , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/genética , Pruebas GenéticasRESUMEN
BACKGROUND: Recent studies have focused on immune checkpoint inhibitors. Renal complications associated with the use of immune checkpoint inhibitors are uncommon compared with other immune-related adverse events. Acute interstitial nephritis accounts for most of these renal complications, with nephrotic syndrome quite rare. We herein report a case of nephrotic syndrome associated with immune checkpoint inhibitors that was more severe than that in previous cases. By comparing this case with previous reports, the possible reasons for the particular severity of this case are discussed. CASE PRESENTATION: A 75-year-old man developed nephrotic syndrome with acute kidney injury after the first combination therapy of nivolumab and ipilimumab for malignant pleural mesothelioma. The results of a kidney biopsy indicated minimal change disease with mild atherosclerosis, acute interstitial nephritis, and fusion of nearly all podocyte foot processes. Nivolumab and ipilimumab therapy were stopped, and treatment with corticosteroids was initiated. We investigated previously reported cases of nephrotic syndrome using immune checkpoint inhibitors. Seventeen cases of immune checkpoint inhibitor-related nephrotic syndrome, including ours, have been reported. Two of the 17 patients with immune checkpoint inhibitor-related nephrotic syndrome required hemodialysis treatment for acute kidney injury. Unlike many previously reported cases, the present patient was administered two different immune checkpoint inhibitors, which may be one of the reasons for the development of severe nephrotic syndrome. CONCLUSIONS: In addition to previously reported risk factors, immune checkpoint inhibitor combination therapy can exacerbate nephrotic syndrome compared to immune checkpoint inhibitor monotherapy.
Asunto(s)
Lesión Renal Aguda , Antineoplásicos Inmunológicos , Nefritis Intersticial , Síndrome Nefrótico , Masculino , Humanos , Anciano , Nivolumab/efectos adversos , Ipilimumab/efectos adversos , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Síndrome Nefrótico/inducido químicamente , Síndrome Nefrótico/tratamiento farmacológico , Antineoplásicos Inmunológicos/efectos adversos , Lesión Renal Aguda/inducido químicamente , Lesión Renal Aguda/terapia , Lesión Renal Aguda/complicaciones , Nefritis Intersticial/inducido químicamente , Nefritis Intersticial/complicacionesRESUMEN
BACKGROUND: IgG4-related disease (IgG4-RD) is a fibroinflammatory disease that affects multiple organs, including the pancreas, lacrimal glands, salivary glands, periaortic/retroperitoneum, and kidney. Interstitial nephritis is a typical renal disorder associated with IgG4-RD, but membranous nephropathy is also seen in some cases. CASE PRESENTATION: Herein we report on the case of a 77-year-old male patient with nephrotic syndrome and IgG4-related lung disease. His serum phospholipase A2 receptor (PLA2R) antibody was positive. His renal biopsy specimen was also positive for PLA2R. The renal biopsy specimen showed membranous nephropathy with equal IgG3 and IgG4 immunofluorescence staining and no interstitial nephritis, suggesting IgG4-RD manifesting as membranous nephropathy. CONCLUSIONS: Nephrotic syndrome caused by membranous nephropathy is sometimes associated with IgG4-RD. In such cases, even if serum PLA2R antibody is positive, it should be considered that the membranous nephropathy may be secondary to IgG4-RD.
Asunto(s)
Glomerulonefritis Membranosa , Enfermedad Relacionada con Inmunoglobulina G4 , Nefritis Intersticial , Síndrome Nefrótico , Masculino , Humanos , Anciano , Glomerulonefritis Membranosa/complicaciones , Glomerulonefritis Membranosa/diagnóstico , Receptores de Fosfolipasa A2 , Enfermedad Relacionada con Inmunoglobulina G4/complicaciones , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Síndrome Nefrótico/complicaciones , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Inmunoglobulina G , AutoanticuerposRESUMEN
IgG4-related kidney disease (IgG4-RKD) encompasses all forms of kidney disease that are part of IgG4-related disease (IgG4-RD). First recognized as IgG4-related tubulointerstitial nephritis (IgG4-TIN), and then IgG4-related membranous glomerulonephritis (IgG4-MGN), we now recognize additional patterns of interstitial nephritis, glomerular disease, and vascular disease that can be seen as part of IgG4-RKD. The clinical presentation is variable and can include acute or chronic kidney injury, proteinuria or nephrotic syndrome, mass lesion(s), and obstruction. While usually associated with other organ involvement by IgG4-RD, kidney-alone involvement is present in approximately 20 % of IgG4-RKD. Compared to IgG4-RD overall, patients with IgG4-RKD are more likely to show increased serum IgG4 or IgG, and more likely to have hypocomplementemia. In this review, we extensively cover other types of autoimmune and plasma cell-rich interstitial nephritis, mass forming inflammatory diseases of the kidney, and other mimics of IgG4-TIN, in particular ANCA-associated disease.
Asunto(s)
Glomerulonefritis Membranosa , Enfermedad Relacionada con Inmunoglobulina G4 , Nefritis Intersticial , Humanos , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Enfermedad Relacionada con Inmunoglobulina G4/patología , Diagnóstico Diferencial , Riñón/patología , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/complicaciones , Nefritis Intersticial/patología , Glomerulonefritis Membranosa/diagnóstico , Glomerulonefritis Membranosa/patología , Inmunoglobulina GRESUMEN
PURPOSE: Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uncommon disease. We present a confirmed case of TINU syndrome, and a systematic review of epidemiological characteristics, clinical manifestations, management, and outcomes in Chinese patients. METHODS: A systematic search was carried out using defined terms and updated up to September 2022, in PubMed, Web of Science, Wanfang, CNKI, and VIP, to identify reported cases of TINU in China, according to PRISMA guidelines. RESULTS: An 18-year-old boy presented with elevated serum creatinine and 24-h urine protein level of > 2 g. Inspection result revealed acute tubulointerstitial nephritis, and bilateral uveitis. The patient was diagnosed with TINU syndrome and received treatment with methylprednisolone sodium succinate, which resulted in a significant decrease in creatinine and urinary protein levels. Systematic review identified 35 publications that met the inclusion criteria. A total of 71 cases were included in this article, of which 70 were from publications and 1 was from our hospital. The median age at onset was 42 years and was significantly lower in males than females (P < 0.05). The symptoms of uveitis often occurred after kidney injury (54%) and most uveitis was anterior (55%) and bilateral (75%). Among the 51 patients who were followed up for more than 6 months, 24 had recurrent ocular symptoms or progression to chronic uveitis. Twenty patients experienced chronic or progressive kidney disease. CONCLUSION: TINU syndrome is prone to misdiagnosis because kidney damage may not occur simultaneously with uveitis. The incidence of kidney sequelae in children is lower than that in adults, and glucocorticoids are the preferred treatment. INPLASY REGISTRATION NUMBER: INPLASY202350050.
Asunto(s)
Nefritis Intersticial , Uveítis , Adolescente , Adulto , Femenino , Humanos , Masculino , China/epidemiología , Glucocorticoides/uso terapéutico , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/tratamiento farmacológico , Nefritis Intersticial/complicaciones , Uveítis/diagnóstico , Uveítis/tratamiento farmacológico , Uveítis/complicacionesRESUMEN
INTRODUCTION: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and IgG4-related disease (IgG4-RD) are distinct immune disorders with overlapping clinical and laboratory features. While ANCA positivity excludes IgG4-RD in the 2019 ACR/EULAR classification, this criterion is not uniformly applied, and AAV can form inflammatory masses in various organs and show increase in IgG4 + plasma cells, similar to IgG4-RD. CASE DIAGNOSIS/TREATMENT: A 5-year-old female with history of orbital mass diagnosed as IgG4-RD presents with acute kidney injury. She has a myeloperoxidase ANCA, and kidney biopsy shows pauci-immune crescentic glomerulonephritis and acute tubulointerstitial nephritis with increased IgG4 + plasma cells and tubular basement membrane (TBM) deposits. CONCLUSION: In isolation, TBM deposits and increased IgG4 + plasma cells are suggestive of IgG4-RD. In the context of a positive ANCA and pauci-immune crescentic glomerulonephritis, however, increased IgG4 + plasma cells due to AAV are favored. In cases with features of IgG4-RD, ANCA positivity suggests an alternate diagnosis of AAV to be more likely.
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Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Glomerulonefritis , Enfermedad Relacionada con Inmunoglobulina G4 , Nefritis Intersticial , Seudotumor Orbitario , Femenino , Humanos , Preescolar , Anticuerpos Anticitoplasma de Neutrófilos , Seudotumor Orbitario/patología , Enfermedad Relacionada con Inmunoglobulina G4/diagnóstico , Riñón/patología , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Inmunoglobulina G , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnósticoRESUMEN
Antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) predominantly affects small vessels. Almost all AAV patients are positive for myeloperoxidase- or proteinase 3-ANCA, and ANCA plays a crucial role in the pathogenesis of AAV. We herein report an ANCA-negative AAV patient with pauci-immune necrotizing glomerulonephritis and plasma cell-rich tubulointerstitial nephritis who was complicated with pleuritis and digital ischemia. ANCA-negative AAV is a rare clinical entity that is difficult to diagnose, and pleuritis and digital ischemia are rare manifestations of AAV. An early diagnosis and appropriate treatment are important, as any delay in the diagnosis may worsen the prognosis.
Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos , Glomerulonefritis , Nefritis Intersticial , Pleuresia , Humanos , Autoanticuerpos , Anticuerpos Anticitoplasma de Neutrófilos , Células Plasmáticas/patología , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnóstico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/complicaciones , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Pleuresia/complicaciones , Isquemia/complicaciones , PeroxidasaRESUMEN
Sporadic cases of apolipoprotein A-IV medullary amyloidosis have been reported. Here we describe five families found to have autosomal dominant medullary amyloidosis due to two different pathogenic APOA4 variants. A large family with autosomal dominant chronic kidney disease (CKD) and bland urinary sediment underwent whole genome sequencing with identification of a chr11:116692578 G>C (hg19) variant encoding the missense mutation p.L66V of the ApoA4 protein. We identified two other distantly related families from our registry with the same variant and two other distantly related families with a chr11:116693454 C>T (hg19) variant encoding the missense mutation p.D33N. Both mutations are unique to affected families, evolutionarily conserved and predicted to expand the amyloidogenic hotspot in the ApoA4 structure. Clinically affected individuals suffered from CKD with a bland urinary sediment and a mean age for kidney failure of 64.5 years. Genotyping identified 48 genetically affected individuals; 44 individuals had an estimated glomerular filtration rate (eGFR) under 60 ml/min/1.73 m2, including all 25 individuals with kidney failure. Significantly, 11 of 14 genetically unaffected individuals had an eGFR over 60 ml/min/1.73 m2. Fifteen genetically affected individuals presented with higher plasma ApoA4 concentrations. Kidney pathologic specimens from four individuals revealed amyloid deposits limited to the medulla, with the mutated ApoA4 identified by mass-spectrometry as the predominant amyloid constituent in all three available biopsies. Thus, ApoA4 mutations can cause autosomal dominant medullary amyloidosis, with marked amyloid deposition limited to the kidney medulla and presenting with autosomal dominant CKD with a bland urinary sediment. Diagnosis relies on a careful family history, APOA4 sequencing and pathologic studies.
Asunto(s)
Amiloidosis , Apolipoproteínas A , Nefritis Intersticial , Insuficiencia Renal Crónica , Humanos , Persona de Mediana Edad , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/genética , Nefritis Intersticial/complicaciones , Mutación , Insuficiencia Renal Crónica/diagnóstico , Insuficiencia Renal Crónica/genética , Insuficiencia Renal Crónica/complicacionesRESUMEN
OBJECTIVES: BK polyomavirus-associated nephropathy is a clinicopathological entity that negatively affects graft function in kidney transplant recipients. We compared the efficacy of leflunomide and cidofovir to treat BK polyomavirus-associated nephropathy in pediatric kidney transplant recipients. MATERIALS AND METHODS: Medical records of pediatric recipients with BK viremia for the period 2004 through 2019 were reviewed retrospectively, and patients diagnosed with BK polyomavirusassociated nephro-pathy were included in the study. A serum BK virus level above 104 copies/mL was accepted as BK viremia. We defined BK polyomavirusassociated nephropathy as detection of BK virus SV40 antigen on immunochemistry staining of renal graft tissue accompanied by signs of tubulointerstitial nephritis or elevated serum creatinine in addition to BK viremia. RESULTS: Of 304 kidney transplant recipients, 53 had persistent BK viremia; 36 of these patients (61.1% male) were included in the study with the diagnosis of BK polyomavirus-associated nephropathy. Twelve patients (33.3%) received cidofovir, and 14 (38.8%) received leflunomide. Results were similar between the cidofovir and leflunomide groups for serum creatinine level at last follow-up (0.91 ± 0.29 vs 0.94 ± 0.37 mg/dL, respectively; P = .843) and graft failure rate (8.3% vs 14.2%, respectively; P = .632). Graft failure was observed in 8.3% of patients with BK polyomavirus-associated nephropathy. CONCLUSIONS: Leflunomide and cidofovir showed similar efficacy for treatment of BK polyomavirus-associated nephropathy.
Asunto(s)
Virus BK , Enfermedades Renales , Trasplante de Riñón , Nefritis Intersticial , Infecciones por Polyomavirus , Infecciones Tumorales por Virus , Humanos , Masculino , Niño , Femenino , Leflunamida/efectos adversos , Cidofovir/efectos adversos , Trasplante de Riñón/efectos adversos , Viremia/diagnóstico , Estudios Retrospectivos , Creatinina , Infecciones Tumorales por Virus/diagnóstico , Infecciones Tumorales por Virus/tratamiento farmacológico , Enfermedades Renales/diagnóstico , Enfermedades Renales/tratamiento farmacológico , Enfermedades Renales/cirugía , Nefritis Intersticial/complicaciones , Infecciones por Polyomavirus/diagnóstico , Infecciones por Polyomavirus/tratamiento farmacológico , Receptores de TrasplantesRESUMEN
BACKGROUND: Sarcoidosis affects multiple organs and exhibits diverse clinical manifestations. Although tubulointerstitial nephritis is a known feature of renal involvement, necrotizing vasculitis is rare. Furthermore, prostate involvement with urinary retention is unusual in patients with sarcoidosis. Here, we report a case of systemic sarcoidosis with a rare combination of manifestations and different acute kidney injuries. CASE PRESENTATION: A 66-year-old man developed sudden urinary retention and fever. He was diagnosed with prostatitis and admitted to our hospital. An indwelling urethral catheter was inserted, and antimicrobial therapy was initiated; however, the prostatitis was refractory. Computed tomography revealed enlarged mediastinal lymph nodes. Analysis of transbronchoscopic lymph node and prostate biopsies showed epithelioid cell granulomas, suggesting systemic sarcoidosis. During the clinical course, the serum creatinine level rapidly increased to 2.36 mg/dL without oliguria. A kidney biopsy revealed tubulointerstitial injury with moderate lymphohistiocytic infiltration and small-vessel vasculitis in the interstitium. Following oral administration of 60 mg/day prednisolone, the patient's renal function immediately improved, and urinary retention did not recur. CONCLUSIONS: To the best of our knowledge, this is the first reported case of sarcoidosis with two unusual complications. Given its clinical course and pathology, this case is clinically valuable.
Asunto(s)
Nefritis Intersticial , Prostatitis , Sarcoidosis , Retención Urinaria , Vasculitis , Masculino , Humanos , Anciano , Próstata/patología , Prostatitis/complicaciones , Retención Urinaria/complicaciones , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/tratamiento farmacológico , Sarcoidosis/diagnóstico , Sarcoidosis/diagnóstico por imagen , Granuloma/complicaciones , Granuloma/diagnóstico por imagen , Vasculitis/complicaciones , Progresión de la EnfermedadRESUMEN
Tubulointerstitial nephritis is a common cause of acute renal failure, in two thirds of cases it is associated with drugs (mostly antimicrobials and NSAIDs), in 5-10% of cases it is associated with infections (bacterial/viral/parasitic), in 5-10% of cases it is idiopathic (this is the case of the TINU syndrome characterized by interstitial nephritis and bilateral uveitis, and the anti-glomerular basal membrane antibody syndrome), and finally in 10% of cases it is associated with systemic diseases (sarcoidosis, by Sjogren, LES). The pathogenesis is based on a cell-mediated immune response and in most cases removing the causative agent is the gold standard of therapy. However, a percentage of patients, in a variable range from 30% to 70% of cases, do not fully recover renal function, due to the rapid transformation of the interstitial cell infiltrate into vast areas of fibrosis. Clozapine is a second generation atypical antipsycothic usually used for the treatment of schizophrenia resistant to other types of treatment; it can cause severe adverse effects among which the best known is a severe and potentially fatal neutropenia, furthermore a series of uncommon adverse events are recognized including hepatitis, pancreatitis, vasculitis. Cases of acute interstitial tubular nephritis associated with the use of clozapine have been described in the literature, although this complication is rare. Medical personnel using this drug need to be aware of this potential and serious side effect. We describe the case of a 48-year-old man who developed acute renal failure after initiation of clozapine.
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Lesión Renal Aguda , Clozapina , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos , Nefritis Intersticial , Uveítis , Masculino , Humanos , Persona de Mediana Edad , Clozapina/efectos adversos , Uveítis/inducido químicamente , Uveítis/complicaciones , Uveítis/tratamiento farmacológico , Nefritis Intersticial/inducido químicamente , Nefritis Intersticial/complicaciones , Nefritis Intersticial/patología , Efectos Colaterales y Reacciones Adversas Relacionados con Medicamentos/complicaciones , Lesión Renal Aguda/etiologíaRESUMEN
Acute interstitial nephritis (AIN) is a cause of acute kidney injury and characterized by an inflammation of the tubulointerstitial space, leading to a decline in kidney function. Multiple etiologies can cause AIN including medications, autoimmune diseases and infections. A multiplicity of drugs is associated with AIN, while antibiotics (especially beta-lactams), proton-pump inhibitors (PPI) and non-steroidal anti-inflammatory agents (NSAIDs) are the most common. The pathognomonic triad of exanthema, fever and eosinophilia is rarely present in AIN patients. Treatment of medication-associated AIN is based upon the discontinuation of the provoking drug. Glucocorticoids can be considered in severe cases.Nephropathia epidemica (NE) is a disease caused by an infection with the Puumula-virus (PUUV) in northern and central Europe. Small rodents (mostly mice) are the host of the virus accountable for a rising number of infections during spring and summer. It is causing a syndrome consisting of AIN, fever and often thrombocytopenia. There is a good chance of complete recovery of kidney function following NE.
Asunto(s)
Lesión Renal Aguda , Infecciones por Hantavirus , Nefritis Intersticial , Humanos , Animales , Ratones , Nefritis Intersticial/complicaciones , Lesión Renal Aguda/etiología , Lesión Renal Aguda/inducido químicamente , Antibacterianos/uso terapéutico , Fiebre , Infecciones por Hantavirus/diagnóstico , Infecciones por Hantavirus/complicacionesRESUMEN
BACKGROUND: Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uveits characterized by complications of idiopathic acute tubulointerstitial nephritis, and most cases present only anterior uveitis. We report a case of TINU syndrome in which the presence of choroiditis was revealed by multimodal imaging. CASE PRESENTATION: A 12-year-old male visited our hospital with a 6-day history of ocular pain and hyperemia. Conjunctival and ciliary injections, 1 + flare and 3 + cells of anterior chamber inflammation with mutton fat keratic precipitates were observed in both eyes (OU), together with redness and swelling of the optic disc OU. Laboratory tests showed slightly high levels of soluble IL-2R and serum ß2 microglobulin and markedly high levels of urinary ß2 microglobulin. The diagnosis of probable TINU syndrome was established on the basis of bilateral uveitis and urinalysis results in accordance with a clinical criteria of tubulointerstitial nephritis. With treatment with oral prednisolone (PSL) at 20 mg/day, ocular findings improved, and the dose of PSL was gradually reduced and withdrawn 6 months later. However, 1 month later from the withdrawal, ocular inflammation recurred with the presence of retinal exudates and snowball vitreous opacities in the peripheral retina OU. Fluorescein angiography showed leakages from peripheral retinal vessels and staining corresponding to retinal exudates. Indocyanine green angiography showed hypofluorescent dots scattered over the ocular fundus. Optical coherence tomography revealed the presence of choroidal thickening. Laser speckle flowgraphy color map showed a relatively cooler color. Findings from these multimodal images indicated the presence of subclinical choroiditis; therefore, oral PSL was administered again, and ocular inflammatory findings were improved. CONCLUSIONS: TINU syndrome can exhibit subclinical choroiditis detected with multimodal imaging. Further studies are necessary to determine the frequency of subclinical choroiditis in TINU syndrome.
Asunto(s)
Coroiditis , Nefritis Intersticial , Papiledema , Uveítis , Masculino , Humanos , Niño , Uveítis/complicaciones , Uveítis/diagnóstico , Uveítis/tratamiento farmacológico , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/tratamiento farmacológico , Prednisolona/uso terapéutico , Retina , Coroiditis/complicaciones , Coroiditis/diagnóstico , Coroiditis/tratamiento farmacológico , Inflamación/tratamiento farmacológicoRESUMEN
OBJECTIVES: The incidence and prevalence of inflammatory bowel disease (IBD), mainly including ulcerative colitis (UC) and Crohn's disease (CD), are increasing globally. We aimed to evaluate the potential association between IBD and nephrolithiasis, tubulointerstitial nephritis, and chronic kidney disease (CKD). METHODS: Data of hospitalized adults ≥20 years of age were extracted from the U.S. National Inpatient Sample (NIS) during 2016-2018. Patients with UC, CD, or CKD were identified through the International Classification of Diseases, Tenth Revision (ICD-10) codes. Propensity score matching (PSM) analysis (1:1) was conducted to balance the characteristics between groups. Logistic regression analyses were performed to determine the relationships between UC or CD and kidney conditions. RESULTS: Three cohorts were included for analysis after PSM analysis. Cohorts 1, 2 and 3 contained 235 262 subjects (117 631 with CD or without IBD), 140 856 subjects (70 428 with UC or without IBD), and 139 098 subjects (69 549 with CD or UC), respectively. Multivariate analysis revealed that compared to non-IBD individuals, CD patients were significantly associated with greater odds for nephrolithiasis (adjusted odds ratio [aOR] 2.25, 95% confidence interval [CI] 2.08-2.43), tubulointerstitial nephritis (aOR 1.31, 95% CI 1.24-1.38), CKD at any stage (aOR 1.28, 95% CI 1.24-1.32), and moderate-to-severe CKD (aOR 1.22, 95% CI 1.17-1.26), while UC was associated with a higher rate of nephrolithiasis. Compared to UC, CD was associated with higher odds for all such kidney conditions. CONCLUSIONS: Patients with CD are more likely to have nephrolithiasis, tubulointerstitial nephritis, CKD at any stage, and moderate-to-severe CKD compared to non-IBD individuals.
Asunto(s)
Colitis Ulcerosa , Enfermedad de Crohn , Enfermedades Inflamatorias del Intestino , Nefritis Intersticial , Nefrolitiasis , Insuficiencia Renal Crónica , Adulto , Humanos , Pacientes Internos , Puntaje de Propensión , Estudios Retrospectivos , Enfermedades Inflamatorias del Intestino/complicaciones , Colitis Ulcerosa/complicaciones , Enfermedad de Crohn/complicaciones , Enfermedad de Crohn/epidemiología , Insuficiencia Renal Crónica/etiología , Insuficiencia Renal Crónica/complicaciones , Nefrolitiasis/epidemiología , Nefrolitiasis/complicaciones , Nefritis Intersticial/epidemiología , Nefritis Intersticial/complicacionesRESUMEN
BACKGROUND: Renal tubular acidosis is the principal clinical feature associated with tubulointerstitial nephritis in patients with primary Sjögren's syndrome. Renal tubular dysfunction due to interstitial nephritis has been considered the underlying pathophysiology connecting renal tubular acidosis and primary Sjögren's syndrome. However, the detailed mechanisms underlying the pathophysiology of renal tubular acidosis in primary Sjögren's syndrome is not fully understood. CASE PRESENTATION: A 30-year-old woman was admitted with complaints of weakness in the extremities. The patient was hospitalized thirteen years earlier for similar issues and was diagnosed with hypokalemic paralysis due to distal renal tubular acidosis with primary Sjögren's syndrome. This diagnosis was based on a positive Schirmer's test. Besides, anti-Sjögren's syndrome-related antigen A was also detected. Laboratory tests indicated distal RTA; however, a renal biopsy showed no obvious interstitial nephritis. Laboratory tests conducted during the second admission indicated distal renal tubular acidosis. Therefore, a renal biopsy was performed again, which revealed interstitial nephritis. Histological analysis of acid-base transporters revealed the absence of vacuolar type H+-ATPases in the collecting duct. The vacuolar type H+-ATPase was also absent in the past renal biopsy, suggesting that the alteration in acid-base transporters is independent of interstitial nephritis. CONCLUSIONS: This case study demonstrates that vacuolar-type H+-ATPases are associated with distal renal tubular acidosis, and distal renal tubular acidosis precedes interstitial nephritis in patients with primary Sjögren's syndrome.
Asunto(s)
Acidosis Tubular Renal , Hipopotasemia , Nefritis Intersticial , Síndrome de Sjögren , ATPasas de Translocación de Protón Vacuolares , Femenino , Humanos , Adulto , Acidosis Tubular Renal/complicaciones , Acidosis Tubular Renal/diagnóstico , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Síndrome de Sjögren/complicaciones , Síndrome de Sjögren/diagnóstico , Parálisis/complicaciones , Hipopotasemia/etiología , Proteínas de Transporte de Membrana , AnticuerposRESUMEN
Renal transplant recipients are at increased risk for the development of a malignant neoplasm. Polyomavirus-associated urothelial carcinoma is a rare tumor that occurs in renal transplant recipients, with approximately 41 cases reported since 2002. It accounts for 27-31% of all post-transplant urothelial carcinomas and develops at an average of 8.5 years after transplantation. Histologically, it shows high-grade urothelial carcinoma (95.1%) with a high frequency of glandular differentiation and micropapillary structures (58.5%) and positive immunohistochemistry for polyomavirus large T antigen, p53 (92.9%), and p16 (100%). We encountered a case of BK polyomavirus (BKPyV)-associated urothelial carcinoma of the bladder diagnosed 54 months after kidney transplantation. Histologically, it was a high-grade urothelial carcinoma with micropapillary features, and immunohistochemically, it was diffusely positive for polyomavirus large T antigen, p16, and p53. BKPyV DNA and mRNA for BKPyV large T antigen have been identified in tissues using real-time polymerase chain reaction. The same sequence of the BKPyV VP1 genome hypervariable region was detected in both transplanted kidney tissue with polyomavirus nephropathy and urothelial carcinoma tissue, suggesting that polyomavirus-associated urothelial carcinoma developed in a background of persistent polyomavirus nephropathy. This case showed typical histological features and was detected and treated at an earlier stage than has been reported. It is important to keep in mind that polyomavirus-associated urothelial carcinoma can develop early after transplantation and might be associated with polyomavirus nephropathy. Because of its rapidly progressive nature, careful follow-up with urine cytology and cystoscopy is necessary. We report this case with a literature review.
Asunto(s)
Virus BK , Carcinoma de Células Transicionales , Trasplante de Riñón , Nefritis Intersticial , Infecciones por Polyomavirus , Neoplasias de la Vejiga Urinaria , Humanos , Trasplante de Riñón/efectos adversos , Carcinoma de Células Transicionales/complicaciones , Virus BK/genética , Vejiga Urinaria/patología , Proteína p53 Supresora de Tumor , Nefritis Intersticial/complicaciones , Infecciones por Polyomavirus/complicaciones , Antígenos Virales de Tumores , Receptores de TrasplantesRESUMEN
BACKGROUND: Tubulointerstitial nephritis with IgM-positive plasma cells (IgMPC-TIN) is a newer disease about which there are many unclear points. Glucocorticoid therapy is effective in many cases of IgMPC-TIN; however, relapse during glucocorticoid tapering has been reported. Relapse and its treatment are poorly defined. CASE PRESENTATION: Case 1 was a 61-year-old man with renal dysfunction and proteinuria. Tubulointerstitial nephritis and IgM-positive plasma cells were observed in a renal biopsy. He was diagnosed with IgMPC-TIN accompanied by Fanconi syndrome and distal renal tubular acidosis (d-RTA). Prednisolone (PSL; 30 mg daily, 0.45 mg/kg/day) treatment was highly effective, and PSL was gradually tapered and discontinued after 1 year. However, 1 month after PSL discontinuation, therapeutic markers were elevated. Therefore, PSL (10 mg daily, 0.15 mg/kg/day) was administered, and the markers indicated improvement. Case 2 was a 43-year-old woman referred for renal dysfunction and proteinuria. Laboratory data revealed that she had primary biliary cholangitis (PBC), d-RTA, and Fanconi syndrome. A renal biopsy showed accumulation of IgM-positive plasma cells in the tubulointerstitium without any glomerular changes. A diagnosis of IgMPC-TIN was made and the patient was started on PSL (35 mg daily, 0.6 mg/kg/day). Therapeutic markers decreased immediately and PSL was discontinued after 1 year. Three months later, the proteinuria and Fanconi syndrome worsened. PSL treatment was restarted (20 mg daily, 0.35 mg/kg/day) and markers indicated improvement. Case 3 was a 45-year-old woman with renal dysfunction and proteinuria. Tubulointerstitial nephritis and IgM-positive plasma cells were observed in a renal biopsy. The patient had PBC, Sjögren syndrome, d-RTA, and Fanconi syndrome, and the diagnosis of IgMPC-TIN was made. The patient was started on PSL (30 mg daily, 0.4 mg/kg/day) and disease markers decreased immediately. However, when PSL was tapered to 15 mg daily (0.2 mg/kg/day), the patient's serum IgM levels increased; therefore, we maintained the PSL at 15 mg daily (0.2 mg/kg/day). CONCLUSION: We report three cases of relapsed IgMPC-TIN associated with reduction or discontinuation of glucocorticoid therapy. In these cases, elevation of serum IgM preceded that of other markers such as urinary ß2-microglobulin, proteinuria, and glycosuria. We recommend monitoring serum IgM levels while tapering glucocorticoids; a maintenance dose of glucocorticoid should be considered if relapse is suspected or anticipated.
Asunto(s)
Acidosis Tubular Renal , Síndrome de Fanconi , Glucocorticoides , Nefritis Intersticial , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Acidosis Tubular Renal/diagnóstico , Síndrome de Fanconi/complicaciones , Glucocorticoides/uso terapéutico , Inmunoglobulina M/sangre , Nefritis Intersticial/diagnóstico , Nefritis Intersticial/tratamiento farmacológico , Nefritis Intersticial/complicaciones , Células Plasmáticas , Proteinuria/tratamiento farmacológico , RecurrenciaRESUMEN
INTRODUCTION: Spanish Lavender is an herbal from the lavender family and is widely used among people for the belief that it cures various diseases. Acute interstitial nephritis (AIN) is one of the common causes of acute kidney injury (AKI). Although drugs are the most common cause of AIN, the frequency of reporting AIN cases due to various herbals has been increasing in recent years. CASE PRESENTATION: We present a 24-year-old male patient who developed AKI after consuming Spanish lavender tea to treat upper respiratory tract infection symptoms and was diagnosed with AIN. AIM AND DISCUSSION: With this case report, we wanted to explain the fact that medicinal herbs, which are used frequently and carelessly today, can have serious consequences, as in acute interstitial nephritis associated with Spanish lavender.
Asunto(s)
Lesión Renal Aguda , Criminales , Lavandula , Nefritis Intersticial , Masculino , Humanos , Adulto Joven , Adulto , Riñón , Nefritis Intersticial/inducido químicamente , Nefritis Intersticial/complicaciones , Nefritis Intersticial/diagnóstico , Lesión Renal Aguda/inducido químicamente , Lesión Renal Aguda/complicaciones , Té/efectos adversosRESUMEN
The field of nephrology has been slow in moving beyond the utilization of creatinine as an indicator for chronic kidney disease and acute kidney injury (AKI). Early diagnosis and establishment of etiology, in particular, are important for treatment of AKI. In the setting of hospital-acquired AKI, tubular injury is more common, but acute interstitial nephritis (AIN) has a more treatable etiology. However, it is likely that AIN is under- or misdiagnosed due to current strategies that largely rely on clinical gestalt. In this issue of the JCI, Moledina and colleagues made an elegant case for the chemokine called C-X-C motif ligand 9 (CXCL9) as a biomarker of AIN. The authors used urine proteomics and tissue transcriptomics in patients with and without AIN to identify CXCL9 as a promising, noninvasive, diagnostic biomarker of AIN. These results have clinical implications that should catalyze future research and clinical trials in this space.