ASN's Mission

ASN leads the fight to prevent, treat, and cure kidney diseases throughout the world by educating health professionals and scientists, advancing research and innovation, communicating new knowledge, and advocating for the highest quality care for patients.

learn more

Contact ASN

1401 H St, NW, Ste 900, Washington, DC 20005

email@asn-online.org

202-640-4660

The Latest on Twitter

Kidney Week

Abstract: PO1512

C2 Deficiency Associated with Severe Recurrent ANA-Negative Lupus and Microangiopathy

Session Information

Category: Glomerular Diseases

  • 1202 Glomerular Diseases: Immunology and Inflammation

Authors

  • Llanos, Maria, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, United States
  • Marino, Daniel, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, United States
  • Menon, Sanjay K., NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, United States
  • Neelakantappa, Kotresha, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, United States
  • Malekan, Michael, NewYork-Presbyterian Brooklyn Methodist Hospital, Brooklyn, New York, United States
Introduction

Deficiencies in the classical complement pathway have been associated with the development of systemic lupus erythematosus (SLE) and lupus-like disease in 10-20% of affected patients. In fact, SLE patients deficient in classical complement present at an earlier age, with severe manifestations and a worse prognosis. Several mechanisms have been described to explain these immune phenomena including impaired clearance of immune complexes, impaired handling of apoptotic cells, or changes in regulation of cytokines. Here, we present a case of a young female with ANA-negative lupus presenting with dyspnea and acute renal failure responsive to immunosuppressive therapy found to have C2 Complement deficiency.

Case Description

44 year old female with sickle cell trait, ANA-negative SLE, ESRD due to biopsy-proven class IV lupus nephritis briefly requiring HD, pulmonary HTN, presented with signs of fluid overload, acute on chronic renal failure in the setting of malignant hypertension. Labs revealed thrombocytopenia of 52 with a creatinine of 2.66 (prior 1.61), hypocomplementemia and undetectable CH50 levels concerning for acute flare of SLE and an underlying functional complement deficiency. Low haptoglobin and peripheral smear with schistocytes raised concerns for microangiopathy. Hospital course was complicated by encephalopathy and possible CNS involvement of lupus. Renal biopsy confirmed chronic sclerosing immune complex glomerulonephritis with minimal activity. The patient responded well to high dose corticosteroids, plasma exchange and mycophenolate mofetil. Final serology confirmed persistent C2 complement deficiency.

Discussion

There is a well-studied link between immune-complex mediated disease and complement deficiency. Of these, C2 deficiency is the most common. In our case, a C2 deficiency was found in the setting of ANA-negative SLE with severe clinical manifestations. Our case raises the question of whether testing to exclude underlying complement deficiency is particularly indicated in patients with ANA-negative SLE. It also remains to be seen whether clinical manifestations of microangiopathy are prevalent in these patients, and whether therapeutics targeting complement may be effective in their treatment.