Abstract: FR-PO575

Scleroderma Renal Crisis Mimicking Rapidly Progressive Glomerulonephritis

Session Information

Category: Hypertension

  • 1106 Hypertension: Clinical and Translational - Secondary Causes

Authors

  • Al-Sheyyab, Ahmed, Vanderbilt University Medical Center, Franklin, Tennessee, United States
  • Beltran Melgarejo, Diego Andres, Vanderbilt University Medical Center, Franklin, Tennessee, United States
  • Fissell, Rachel B., Vanderbilt University Medical Center, Franklin, Tennessee, United States
  • Dwyer, Jamie P., Vanderbilt University Medical Center, Franklin, Tennessee, United States
Background

Thrombotic microangiopathies are a group of disorders which overlap in their clinical features. We describe a case with ambivalent presentation, as it highlights some of the differentiating clinical features and the impact of treatment choices on the patient’s outcome.

Methods

A 78 year-old man presented with edema of the lower extremities and hands. Clinic evaluation revealed serum creatinine 2.2 mg/dL(baseline 0.7mg/dL), new hypertension(BP 200/103), anemia (hemoglobin 9.1mg/dL) and thrombocytopenia (platelets 69 x103/mcL). Additional testing showed microscopic hematuria with proteinuria (protein:creatinine ratio 0.65), LDH 512U/L, haptoglobin <8 mg/dL, ADAMTS13 of moderate activity, and peripheral smear negative for schistocytes. A complete serologic work up was unrevealing except for a positive Antinuclear Antibody (1:2560). Despite pulse steroids therapy, renal failure worsened and ultimately required hemodialysis.
Examination of the hands showed skin thickening. Captopril was started. Anti-RNA polymerase III antibodies were positive. Kidney biopsy was consistent with the clinical diagnosis of scleroderma renal crisis(SRC).

Conclusion

SRC can present similarly to Rapidly Progressive Glomerulonephritis(RPGN). The renal biopsy is able to differentiate between RPGN and SRC. However, thrombocytopenia can delay the performance of kidney biopsy. Clinical suspicion followed by careful hand examination and obtaining extractable nuclear antigen antibodies were key steps in revealing the diagnosis.
Early diagnosis of scleroderma is critical to initiate timely therapy. Previous studies showed dramatic improvement of mortality with angiotensin-converting-enzyme inhibitors.

In SRC, careful clinical examination for signs of scleroderma and appropriate serologic testing can guide early therapy. The astute clinician requires a high index of suspicion to make the diagnosis of SRC.