title: Eculizumab for rescue of thrombotic microangiopathy in PM-Scl antibody-positive autoimmune overlap syndrome.
abstract: A 46-year-old female with interstitial lung disease presented with proximal muscle weakness, worsening hypertension, microangiopathic hemolysis, thrombocytopenia and deteriorating renal function. She had no sclerodactyly, but had abnormal capillaroscopy. She tested positive for PM-Scl antibodies, and a renal biopsy showed an acute thrombotic microangiopathy consistent with scleroderma renal crisis (SRC). She failed to respond to corticosteroids, plasmapheresis and renin-angiotensin pathway inhibitors. She recovered quickly with the anti-C5 antibody, eculizumab. She had no genetic abnormalities associated with atypical hemolytic uremic syndrome except a DNA variant of unknown significance in C3. This case suggests that eculizumab may be effective for SRC.
authors.affiliation: Division of Nephrology, Department of Internal Medicine , Carver College of Medicine, The University of Iowa , Iowa City, IA , USA ; Stead Department of Pediatrics, Carver College of Medicine , The University of Iowa , Iowa City, IA , USA ; The VA Medical Center , Iowa City, IA , USA.
authors.forename: Christie P
authors.initials: CP
authors.lastname: Thomas
authors.affiliation: Division of Nephrology, Department of Internal Medicine , Carver College of Medicine, The University of Iowa , Iowa City, IA , USA ; Stead Department of Pediatrics, Carver College of Medicine , The University of Iowa , Iowa City, IA , USA.
authors.forename: Carla M
authors.initials: CM
authors.lastname: Nester
authors.affiliation: Division of Immunology, Department of Internal Medicine , Carver College of Medicine, The University of Iowa , Iowa City, IA , USA ; Present address: St Joseph Heritage Group in Orange , Orange, CA , USA.
authors.forename: Andrew C
authors.initials: AC
authors.lastname: Phan
authors.affiliation: Division of Immunology, Department of Internal Medicine , Carver College of Medicine, The University of Iowa , Iowa City, IA , USA.
authors.forename: Manisha
authors.initials: M
authors.lastname: Sharma
authors.affiliation: Division of Immunology, Department of Internal Medicine , Carver College of Medicine, The University of Iowa , Iowa City, IA , USA ; Present address: Park Nicollet Rheumatology in Minneapolis , Minneapolis , MN , USA.
authors.forename: Amanda L
authors.initials: AL
authors.lastname: Steele
authors.affiliation: Division of Immunology, Department of Internal Medicine , Carver College of Medicine, The University of Iowa , Iowa City, IA , USA.
authors.forename: Petar S
authors.initials: PS
authors.lastname: Lenert
keywords: AKI; autoantibodies; complement; plasmapheresis; scleroderma renal crisis