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Abstract: TH-PO666

Cardiac Failure in Fibrillary Glomerulopathy Associated with Systemic Lupus Erythematosus

Session Information

Category: Glomerular Diseases

  • 1402 Glomerular Diseases: Clinical, Outcomes, and Trials

Authors

  • Zapata Beltrán, Carina Sandybel, Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Guerrero Gonzalez, Elisa Maria, Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Arteaga Muller, Giovanna Y., Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Olivo Gutierrez, Mara Cecilia, Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Garza Treviño, Ricardo Abraham, Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Elvir, Daniela Campos, Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Vargas Santana, Joary, Hospital Universitario José Eleuterio González , UANL, Monterrey, Mexico
  • Soto, Virgilia, Instituto Nacional de Cardiologia Ignacio Chavez, Mexico, Estado de Mexico, Mexico
Introduction

SLE involves the kidneys in a large percentage of cases, and less frequently, there is the presence of myocardial damage with a prevalence of 8-25%.

Case Description

This is a 42-year-old male with a past medical history of pleural tuberculosis diagnosed three years ago, presenting with refractory exudative left pleural effusion, dyspnea, and pleuritic pain. He also has a three-year history of type 2 diabetes mellitus. His current symptoms as the same previously additional bilateral pleural effusion (transudate), intolerance to lying down, no peripheral edema, no retinopathy on fundoscopy.
Laboratory findings included Hb of 11.7 g/dL, normo-normo anemia, eGFR by CKD-EPI of 28 mL/min/1.73m2, proteinuria of 8.9 g/24hrs with an inverted albumin/globulin ratio. Echocardiogram showed an ejection fraction of 23%. Only ANAs (1:640) and anti-U1-RNP (>200) antibodies were positive. C3 and C4 were normal.
Renal biopsy showed negative staining with Congo red, indicating glomerulonephritis due to immune complexes with a membranoproliferative and nodular pattern. Immunofluorescence revealed a diffuse, global, and granular full house pattern, electron microscopy revealed fibrillary deposits measuring 10-20 nm.

Discussion

The presence of antibodies anti-U1-RNP and the occurrence of myocarditis are related to autoimmune activity against striated muscle. Myocarditis occurs in 5% to 10% of patients with SLE and most cases are asymptomatic and it can progress to heart failure. Atypical serology, unusual clinical presentation, and nodular biopsy make the diagnosis challenging, requiring electron microscopy for the diagnosis.

The mesangium shows diffuse expansion of the matrix with predominantly global proliferation, which gives some glomeruli a hiperlobulated appearance with the presence of acellular nodules.
There are subendothelial and mesangial deposits of fibrils measuring 10-20 nm.