RESUMO
We herein report a patient with systemic lupus erythematosus (SLE) and neuropsychiatric SLE (NPSLE), who had been misdiagnosed with schizophrenia for a long time and presented with pancytopenia. Brain magnetic resonance imaging revealed sporadic punctate hyperintense areas in the cerebral white matter. Single-photon emission computed tomography revealed a clear decrease in blood flow from the parietotemporal association area to the temporal lobe. NPSLE is a serious organ complication that significantly worsens the SLE prognosis. NPSLE symptoms are diverse and difficult to diagnose and differentiate from those of other neuropsychiatric disorders, especially in an early onset.
RESUMO
BACKGROUND: Amphiregulin (AREG) is a ligand of epidermal growth factor receptor (EGFR), which plays an important role in injury-induced kidney fibrosis. However, the clinical significance of serum soluble AREG in chronic kidney disease (CKD) is unclear. In this study, we elucidated the clinical significance of serum soluble AREG in CKD by analyzing the association of serum soluble AREG levels with renal function and other clinical parameters in patients with CKD. METHODS: In total, 418 Japanese patients with CKD were enrolled, and serum samples were collected for the determination of soluble AREG and creatinine (Cr) levels, and other clinical parameters. Additionally, these parameters were evaluated after 2 and 3 years. Moreover, immunohistochemical assay was performed ate AREG expression in the kidney tissues of patients with CKD. RESULTS: Soluble AREG levels were positively correlated with serum Cr (p < 0.0001). Notably, initial AREG levels were positively correlated with changes in renal function (ΔCr) after 2 (p < 0.0001) and 3 years (P = 0.048). Additionally, soluble AREG levels were significantly higher (p < 0.05) in patients with diabetic nephropathy or primary hypertension. Moreover, AREG was highly expressed in renal tubular cells in patients with advanced CKD, but only weakly expressed in patients with preserved renal function. CONCLUSION: Serum soluble AREG levels were significantly correlated with renal function, and changes in renal function after 2 and 3 years, indicating that serum soluble AREG levels might serve as a biomarker of renal function and renal prognosis in CKD.
Assuntos
Anfirregulina , Creatinina , Insuficiência Renal Crônica , Humanos , Anfirregulina/sangue , Insuficiência Renal Crônica/sangue , Insuficiência Renal Crônica/diagnóstico , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Creatinina/sangue , Biomarcadores/sangue , Taxa de Filtração Glomerular , Rim/fisiopatologia , Rim/metabolismo , Rim/patologia , Adulto , Nefropatias Diabéticas/sangue , Nefropatias Diabéticas/diagnóstico , Hipertensão , Relevância ClínicaRESUMO
Vascular endothelial growth factor inhibitors and checkpoint inhibitors are effective treatments for solid tumors. These new classes of anti-cancer agents frequently cause kidney-related side effects. Although their anti-cancer effects may be enhanced when used in combination, the severity of their kidney-related side effects is unknown. We herein report the first case of thrombotic microangiopathy and mesangial proliferative glomerulonephritis caused by combined treatment with atezolizumab and bevacizumab in a 74-year-old man with hepatocellular carcinoma. The combination therapy was discontinued and replaced with intravenous methylprednisolone followed by oral prednisolone. Subsequently, the urinary protein excretion levels declined.
Assuntos
Carcinoma Hepatocelular , Glomerulonefrite , Neoplasias Hepáticas , Microangiopatias Trombóticas , Masculino , Humanos , Idoso , Bevacizumab/efeitos adversos , Fator A de Crescimento do Endotélio Vascular , Microangiopatias Trombóticas/tratamento farmacológico , Glomerulonefrite/induzido quimicamente , Glomerulonefrite/tratamento farmacológico , Carcinoma Hepatocelular/tratamento farmacológico , Neoplasias Hepáticas/tratamento farmacológicoRESUMO
Systemic capillary leak syndrome is a rare and life-threatening disorder, characterized by recurrent episodes of unexplained hypotension, hemoconcentration, and hypoalbuminemia. This condition is caused by leakage of plasma and proteins into the extravascular space and can be classified as either idiopathic or secondary. Secondary systemic capillary leak syndrome can result from cancer, infections, medications, or surgery. Systemic capillary leak syndrome frequently develops as a side effect of denileukin diftitox treatment of refractory cutaneous T-cell lymphoma. However, the pathophysiology of this disease is not well understood. Herein, we report a case of denileukin diftitox-induced systemic capillary leak syndrome.
Assuntos
Injúria Renal Aguda , Síndrome de Vazamento Capilar , Neoplasias Cutâneas , Humanos , Síndrome de Vazamento Capilar/diagnóstico , Síndrome de Vazamento Capilar/tratamento farmacológico , Síndrome de Vazamento Capilar/induzido quimicamente , Interleucina-2/efeitos adversos , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia , Injúria Renal Aguda/induzido quimicamente , Injúria Renal Aguda/complicaçõesAssuntos
Linfoma de Célula do Manto , Nocardiose , Nocardia , Adulto , Bortezomib/efeitos adversos , Humanos , Linfoma de Célula do Manto/complicações , Linfoma de Célula do Manto/tratamento farmacológico , Linfoma de Célula do Manto/patologia , Nocardiose/diagnóstico , Nocardiose/tratamento farmacológico , Rituximab/efeitos adversosAssuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Humanos , Dermatomiosite/complicações , Dermatomiosite/diagnóstico , Dermatomiosite/tratamento farmacológico , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Autoanticorpos , Helicase IFIH1 Induzida por Interferon , Progressão da DoençaRESUMO
Lumbar hernias are extremely rare. The posterolateral abdominal wall has two susceptible areas - the superior (Grynfeltt-Lesshaft's triangle) and the inferior (Petit's triangle) lumbar triangles - that cause superior and inferior lumbar hernias, respectively. We herein report a 67-year-old woman with nephrotic-range proteinuria caused by primary superior lumbar hernia. Superior lumbar hernias should be considered as a differential disease causing massive orthostatic proteinuria in adults. The present case highlights the importance of considering lumbar hernia in patients with flank swelling and the potential complications that may result from a missed diagnosis.
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Parede Abdominal , Adulto , Idoso , Feminino , Hérnia , Humanos , Região Lombossacral , Proteinúria/complicaçõesRESUMO
A 58-year-old woman with a history of Sjogren's syndrome was admitted to our hospital with cough, decreased right lung breath sounds and arthralgia in both thumbs. Chest computed tomography showed consolidation with air bronchogram in the right lung. Levels of anti-cyclic citrullinated peptide antibody and rheumatoid factor levels were significantly elevated. She was diagnosed with rheumatoid arthritis induced by bacterial organizing pneumonia. Treatment with salazosulfapyridine was added for rheumatoid arthritis and arthralgia gradually improved. This case highlights that respiratory infections could lead to anti-cyclic citrullinated peptide antibody-positive rheumatoid arthritis in patients with Sjogren's syndrome.
Assuntos
Artrite Reumatoide , Pneumonia , Síndrome de Sjogren , Artralgia , Artrite Reumatoide/complicações , Autoanticorpos , Feminino , Humanos , Pessoa de Meia-Idade , Peptídeos Cíclicos , Síndrome de Sjogren/complicaçõesAssuntos
Anticorpos , Hiperplasia do Linfonodo Gigante , Glomerulonefrite Membranosa , Interleucina-6 , Síndrome Nefrótica , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/diagnóstico , Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Glomerulonefrite Membranosa/complicações , Glomerulonefrite Membranosa/diagnóstico , Glomerulonefrite Membranosa/tratamento farmacológico , Humanos , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/etiologiaRESUMO
BACKGROUND: Zeb2, a zinc finger E-box-binding homeobox transcription factor, regulates transforming growth factor (TGF)-ß signaling pathway. However, its role in the pathogenesis of acute kidney injury (AKI) and AKI-to-chronic kidney disease (CKD) transition is unclear. METHODS: We evaluated Zeb2 function in a bilateral renal ischemia-reperfusion injury (IRI)-induced AKI model using proximal tubule-specific Zeb2 conditional knockout (Zeb2-cKO) and wild-type (WT) mice, and in renal biopsy samples. RESULTS: In Zeb2-cKO mice, the levels of plasma creatinine and blood urea nitrogen post-IRI were significantly lower than that in WT mice. Immunohistological analysis revealed mild tubular injury, reduced neutrophil infiltration, fewer fibrotic changes and reduced expression of fibrotic proteins [collagen type IV, α-smooth muscle actin (α-SMA), fibronectin and connective tissue growth factor (CTGF)], at 3-14 days post-IRI. Zeb2 expression was upregulated in proximal tubular cells post-IRI in WT mice. Zeb2 siRNA transfection reduced TGF-ß-stimulated mRNA and protein expression of collagen type IV, α-SMA, fibronectin and CTGF in cultured renal tubular cells. Patients with AKI-to-CKD transition exhibited high Zeb2 expression in renal tubules, as revealed by renal biopsy. Hypoxia and CoCl2-treatment upregulated Zeb2 promoter activity and mRNA and protein expression in cultured renal tubular epithelial cells, suggesting a regulatory role for hypoxia. CONCLUSIONS: Zeb2 was upregulated in renal tissues in both mice and humans with AKI. Zeb2 regulates fibrotic pathways in the pathogenesis of AKI and AKI-to-CKD transition. Therefore, inhibition of Zeb2 could be a potential therapeutic strategy for AKI.
Assuntos
Injúria Renal Aguda/patologia , Traumatismo por Reperfusão/complicações , Homeobox 2 de Ligação a E-box com Dedos de Zinco/genética , Injúria Renal Aguda/etiologia , Injúria Renal Aguda/genética , Injúria Renal Aguda/metabolismo , Animais , Fibrose , Humanos , Rim/patologia , Camundongos , Camundongos Endogâmicos C57BL , Camundongos Knockout , Traumatismo por Reperfusão/genética , Homeobox 2 de Ligação a E-box com Dedos de Zinco/metabolismoRESUMO
We herein report the first case of lupus-related protein-losing enteropathy associated with pseudo-pseudo Meigs' syndrome. Lupus-related protein-losing enteropathy and pseudo-pseudo Meigs' syndrome are extremely rare complications in patients with systemic lupus erythematosus, Both have a similar clinical course characterized by producing marked ascites, and respond to steroids in typical cases. However, in our case, steroid monotherapy was inadequate and the addition of hydroxychloroquine was effective for their treatment. Furthermore, no reports have previously confirmed elevated CA 125 levels with lupus-related protein-losing enteropathy or increased 99mTc-HSA activity with pseudo-pseudo Meigs' syndrome. In addition, we are the first to report an evaluation of the histopathology of lupus-related protein-losing enteropathy. Previously reported cases have been described as being caused by either pseudo-Meigs's syndrome or lupus-related protein-losing enteropathy as the cause of the rare pathology that causes marked pleural effusion and ascites in patients with systemic lupus erythematosus, but it has not been evaluated whether the other is co-occurring. Our case highlights that there is a potential case of overlapping lupus-related protein-losing enteropathy and pseudo-Pseudo-Meigs's syndrome. Furthermore, it is possible that patients with marked ascites with elevated CA 125 levels were mistakenly diagnosed with Meigs's syndrome or pseudo-Meigs's syndrome associated with malignant or benign ovarian tumors and underwent surgery. Clinicians should not forget SLE with pseudo-Pseudo-Meigs's syndrome as one of the differential diagnoses for marked ascites with elevated CA 125 levels.