Abstract: SA-PO212
Anti-C5 Antibody for Thrombotic Microangiopathy in Scleroderma Renal Crisis
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 103 AKI: Mechanisms
Authors
- Machado, Shana M., UT Health Science Center, Houston, Houston, Texas, United States
- Kala, Jaya, UT Health Science Center, Houston, Houston, Texas, United States
Introduction
Scleroderma renal crisis(SRC),presenting as malignant hypertension, acute kidney injury(AKI), thrombocytopenia and microangiopathic hemolytic anemia,is a major complication of systemic sclerosis. Seen more common in patients treated with glucocorticoids. Prognosis remains severe despite treatment with angiotensin converting enzyme inhibitor(ACEI). Studies suggest that complement activation trigger thrombotic microangiopathy(TMA) in SRC. We report a case of SRC with TMA treated with anti-C5 antibody
Case Description
51 yr-old female with hypertension,hypothyroidism,dyspnea due to interstitial lung disease(ILD), without kidney disease, presented with worsening vision and severe headache. She was on myfortic and prednisone for the past 8 months for ILD. Her computed tomography scan revealed frontal cortical subarachnoid hemorrhage and bilateral posterior parietal subcortical hypo densities. For her high blood pressures she was started on Cardene drip. Hemodialysis was started for uremic symptoms with creatinine of 4.06 mg/dl and blood urea nitrogen 92 mg/dl. Laboratory evaluation with platelets 161,000/cmm, LDH 800U/L, hemoglobin10.5g/dl and haptoglobin undetectable, few schistiocytes, normal ADAMTS13activity, AKI and hypertensive emergency indicated possibility of TMA, likely atypical hemolytic uremic syndrome. Rheumatological evaluation of ILD revealed nucleolar pattern ANA and high PMSL antibody indicating NSIP with PM-Scl overlap without skin involvement. There was high suspicion of SRC, possibly precipitated with the use of glucocorticoids. Despite captopril she did not show any signs of renal recovery. Renal biopsy done revealed presence of acute TMA with arterial, arteriolar and glomerular C5b-9 staining. These findings were suggestive of the presence of complement dyregulation and therefore decision to start anti-C5 antibody(ecluzimab) was made. The patient’s platelets count recovered with 3 doses of ecluzimab. The patient is currently on the maintenance dose of ecluzimab. She has improved renal functions, requiring dialysis once a week. Her genetic testing is awaited
Discussion
Scleroderma renal crisis have high incidence of end stage renal disease despite use of ACEI. Since, complement activation has been described in autoimmune disorders including SRC, we considered the use of anti-C5 antibody in the treatment of TMA in our patient to achieve decreased dialysis dependence.