Abstract: SA-PO1025

Resistant Edema – Management of Chronic Systemic Capillary Leak Syndrome with IVIG

Session Information

Category: Nephrology Education

  • 1302 Fellows and Residents Case Reports

Authors

  • Mullane, Ryan, University of Nebraska Medical Center, Omaha, Nebraska, United States
  • Langewisch, Eric D, University of Nebraska Medical Center, Omaha, Nebraska, United States
  • Plumb, Troy J., University of Nebraska Medical Center, Omaha, Nebraska, United States
Background

Systemic capillary leak syndrome (SCLS) is a rare, poorly understood disorder characterized by increased vascular permeability. It presents with intermittent acute episodes of profound capillary leak that may result in hypotension/shock. There are few reported cases of chronic SCLS, which is characterized by persistent refractory edema and pleural effusions. We present a case of chronic SCLS where ongoing treatment with intravenous immunoglobulin (IVIG) has resulted in a marked and sustained improvement in the signs and symptoms of the capillary leak syndrome.

Methods

A 54-year-old man presented with new onset lower extremity edema, which initially responded to oral diuretics. However, his edema later worsened with the development of upper extremity edema, bilateral pleural effusions and a total fluid weight gain of 20 kg which was refractory to diuretics. He required ultrafiltration for fluid removal in addition to recurrent thoracentesis for his pleural effusions. Evaluation revealed hypoalbuminemia and an IgG kappa monoclonal gammopathy, but bone marrow biopsy was normal. Cardiac, renal, hepatic and lymphatic evaluations failed to disclose an etiology for his edema. Initial therapy with theophylline was discontinued due to significant chest discomfort. Initiation of IVIG 1 g/kg every 4 weeks for a period of 4 months and then maintenance therapy every 6 weeks has resulted in a significant and persistent improvement over the past 18 months.

Conclusion

Systemic capillary leak syndrome presents with recurrent episodes of generalized edema, hypotension and hypoalbuminemia. Although the pathogenesis is unknown, IVIG decreases the occurrence of episodes in the acute form of SCLS. Few cases of the chronic form of SCLS are reported in the literature with prior treatments including theophylline, terbutaline and steroids. There have been no prior reports of IVIG therapy for chronic SCLS. Our patient had significant and durable improvement of refractory edema related to chronic systemic capillary leak syndrome with IVIG. This case demonstrates that IVIG may be an effective treatment modality for the chronic form of this disorder.