case_images: PMC10959543_01
case_images: a Sclerodactyly. b Sclerodermic facies.
case_images: cnd-2024-0014-0001-537873_F01.jpg
case_images: PMC10959543_01_cnd-2024-0014-0001-537873_F01.jpg
case_images: F1
case_images: She received a consultation from a general practitioner for a 2-year clinical presentation characterized by asthenia, adynamia, thick cardboard-like skin, sclerodactyly (Fig. 1), limited ability to open her mouth, and a 10-kg weight loss associated with dry eye and mouth with no other manifestations.
case_images: Serum creatinine and proteinuria/creatinine ratio (PrU(/CrU) evaluation.
case_images: cnd-2024-0014-0001-537873_F02.jpg
case_images: PMC10959543_01_cnd-2024-0014-0001-537873_F02.jpg
case_images: F2
case_images: The 3-month serology work-up presented elevated levels of serum creatinine (5.5 mg/dL) and urea (122 mg/dL) and a urinary protein-to-creatinine ratio (PrU/CrU) of 4.9 g/g (Fig. 2).
case_images: Renal histopathology. a Extracapillary glomerulonephritis with active fibrocellular crescent stained with periodic acid Schiff, PAS at x40. b Narrowing of the arterial diameter with marked fibrotic changes in the intima, PAS at x40. c Foci of interstitial inflammatory infiltrate of mononuclear cells (plasma cells) extending to some walls. PAS at x40. d Foci of interstitial inflammatory infiltrate of mononuclear cells (plasma cells) and tubular atrophy evaluated with H&E at x40. e Focal acute tubular injury with intratubular blood debris evaluated with methenamine silver stain at x40. f C3-negative, immunofluorescence, and the rest of the immunoreagents. g Electron microscopy, photomicrograph at x5,000 magnification, the capillary loops with folded and healed segments are observed, there is preservation of podocyte processes, there is no evidence of electron-dense material that suggests immune complexes, in the urinary space there are also few cells of the parietal epithelium.
case_images: cnd-2024-0014-0001-537873_F03.jpg
case_images: PMC10959543_01_cnd-2024-0014-0001-537873_F03.jpg
case_images: F3
case_images: After the initiation of hemodialysis, a kidney biopsy was performed, in which extracapillary glomerulonephritis with fibrous predominance (EUVAS-Berden sclerotic class), active tubulointerstitial nephritis rich in plasma cells, focal tubular injury, and narrowing of the arterial diameter with marked fibrotic changes in the intima were identified (Fig. 3 a-j).