ABSTRACT
We analyzed the association between the renal arterial resistive index (RI) and the histologic features of lupus nephritis. All consecutive patients with systemic lupus erythematosus (SLE) who required a kidney biopsy were enrolled. The study protocol included ultrasonographic assessment to measure the RI and kidney biopsy (International Society of Nephrology/Renal Pathology Society classification). A RI > 0.7 was considered pathologic. Patients with non-renal SLE and healthy patients were studied as control groups. We enrolled 42 patients with renal SLE, 10 with non-renal SLE and 14 healthy patients: their mean (±standard deviation) RI values were 0.64 ± 0.08, 0.60 ± 0.04 and 0.59 ± 0.01, respectively (p = not significant). RIs > 0.7 were recorded only in patients with renal SLE (5/42, 11.9%). The percentage of patients with a pathologic RI was significantly higher in class IV nephritis in comparison with other classes (p < 0.009). In conclusion, we found a significant correlation between pathologic RI and class IV nephritis, suggesting a role for RI as a severity marker.
Subject(s)
Kidney/diagnostic imaging , Kidney/ultrastructure , Lupus Nephritis/diagnostic imaging , Vascular Resistance/physiology , Adult , Biopsy/methods , Female , Follow-Up Studies , Humans , Kidney/pathology , Lupus Nephritis/pathology , Male , Reproducibility of Results , UltrasonographyABSTRACT
INTRODUCTION: Familial amyloid polyneuropathy is characterized by transthyretin (TTR) deposition in various tissues, including the kidneys. While deposition induces organ dysfunction, renal involvement in TTR-related amyloidosis could manifest from proteinuria to end-stage kidney failure. As proteinuria is considered result of glomerular filtration barrier injury we investigated whether TTR deposition affects either glomerular basement membrane (GBM) or podocytes. MATERIALS AND METHODS: Immunohistochemistry, immunoblot and gene expression studies for nephrin, podocin and WT1 were run on renal tissue from human-TTRV30M transgenic mice hemizygous or homozygous for heat shock factor one (Hsf-1). Transmission electron microscopy was used for evaluation of podocyte foot process width (PFW) and GBM thickness in Hsf-1 hemizygous mice with or without TTRV30M or amyloid deposition. RESULTS: Glomeruli of hsf-1 hemizygous transgenic mice showed lower nephrin and podocin protein levels but an increased podocyte number when compared to Hsf-1 homozygous transgenic mice. Nephrin, podocin and WT1 gene expression levels were unaffected by the Hsf-1 carrier status. TTRV30M deposition was associated with increased PFW and GBM thickness. CONCLUSIONS: Under the effect of Hsf-1 hemizygosity, TTRV30M deposition has deleterious effects on GBM thickness, PFW and slit diaphragm composition, without affecting nephrin and podocin gene expression.
Subject(s)
Amyloid Neuropathies, Familial/metabolism , Amyloid/metabolism , DNA-Binding Proteins/metabolism , Intracellular Signaling Peptides and Proteins/metabolism , Membrane Proteins/metabolism , Podocytes/metabolism , Prealbumin/metabolism , Transcription Factors/metabolism , WT1 Proteins/metabolism , Amyloid/genetics , Amyloid Neuropathies, Familial/genetics , Amyloid Neuropathies, Familial/pathology , Animals , Biomarkers/metabolism , DNA-Binding Proteins/genetics , Disease Models, Animal , Gene Expression , Glomerular Basement Membrane/metabolism , Glomerular Basement Membrane/pathology , Heat Shock Transcription Factors , Hemizygote , Homozygote , Humans , Intracellular Signaling Peptides and Proteins/genetics , Membrane Proteins/genetics , Mice , Mice, Transgenic , Podocytes/pathology , Prealbumin/genetics , Transcription Factors/genetics , WT1 Proteins/geneticsABSTRACT
We report the case of a young man, affected by rheumatoid arthritis who developed a rapid-onset short-of-breath, hemoptysis, and severe weakness, about 2 weeks after the administration of leflunomide. Chest radiography showed central bilateral opacities and pleural effusion as confirmed by the high-resolution computed tomography that demonstrated diffuse ground-glass and interlobular septal thickening as well. On admission at the Emergency Department, a microhematuria and a severe anemia were also documented. On the basis of the clinico-radiologic presentation, a pulmonary hemorrhage was likely to occur; so to clarify the origin of this process, a complete serologic examination was performed but all the antibodies were negative. Finally a renal biopsy was performed and it showed a pauci-immunologic glomerulonephritis and the bronchioloalveolar lavage confirmed the diffuse alveolar hemorrhage. In conclusion, the diagnosis of leflunomide-pulmonary-renal syndrome was rendered. The treatment with leflunomide was suspended; the conditions of the patient gradually improved and he became completely asymptomatic 1 week later.
