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Abstract: FR-PO673

A Unique Case of Atypical Anti-Glomerular Basement Membrane Disease

Session Information

Category: Glomerular Diseases

  • 1303 Glomerular Diseases: Clinical‚ Outcomes‚ and Trials

Authors

  • Rawala, Muhammad, The University of Texas Medical Branch at Galveston, Galveston, Texas, United States
  • Kassem, Hania, The University of Texas Medical Branch at Galveston, Galveston, Texas, United States
  • Alhosainat, Nidal, The University of Texas Medical Branch at Galveston, Galveston, Texas, United States
  • Afrouzian, Marjan, The University of Texas Medical Branch at Galveston, Galveston, Texas, United States
Introduction

Classic anti-glomerular basement membrane (GBM) nephritis is clinically and pathologically the most aggressive form of glomerulonephritis. The binding of the autoimmune antibodies to GBM leads to crescent formation and rapidly progressive glomerulonephritis (RPGN). We present a case of a 56-year-old female with atypical features of anti-GBM disease who did not require immunosuppression.

Case Description

A 56-year-old female with a history of chronic kidney disease (CKD) stage III, diabetes mellitus type II, smoking, hypertension, chronic obstructive pulmonary disease, hepatitis C-related cirrhosis presented to the hospital with a 2-month history of worsening ascites that did not respond to diuretics. Large volume paracentesis was performed, after which the patient developed acute kidney injury, which was initially thought to be secondary to decreased effective circulating volume. However, work-up identified nephrotic range proteinuria and dysmorphic RBCs on urine microscopy. This prompted a renal biopsy for evaluation of glomerulonephritis (GN). The biopsy was reported as atypical Anti-GBM disease with proliferative GN and diabetic glomerulosclerosis. The patient’s anti-GBM antibody was negative. She was managed conservatively as creatinine stabilized around 1.2-1.4 mg/dL.

Discussion

The incidence of atypical anti-GBM is reported to be 11.8%. Our patient exhibited IgG linear staining along the glomerular and tubular basement membranes, the pathologic hallmark of the anti-GBM disease. But the clinical characteristics of our patient having nephrotic range proteinuria and mild nonprogressive worsening in kidney function, differed from the traditional presentation of pulmonary symptoms and RPGN found in anti-GBM disease. Also, similarly to other cases of atypical anti-GBM, the patient was a smoker, had a negative anti-GBM antibody, and histologically had evidence of endocapillary proliferation. The objective of the case is to delineate that anti-GBM disease can present atypically with a less aggressive phenotype, variable histologic changes, and undetectable anti-GBM antibody. This subset of patients has an overall better prognosis and may be managed conservatively.