Journal article
Eculizumab for rescue of thrombotic microangiopathy in PM-Scl antibody-positive autoimmune overlap syndrome
Clinical Kidney Journal, Vol.8(6), pp.698-701
12/2015
DOI: 10.1093/ckj/sfv101
PMCID: PMC4655803
PMID: 26613027
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.
Details
- Title: Subtitle
- Eculizumab for rescue of thrombotic microangiopathy in PM-Scl antibody-positive autoimmune overlap syndrome
- Creators
- Christie P Thomas - The University of IowaCarla M Nester - The University of IowaAndrew C Phan - Present address: St Joseph Heritage Group in OrangeManisha Sharma - Carver College of Medicine, The University of IowaAmanda L Steele - Present address: Park Nicollet Rheumatology in MinneapolisPetar S Lenert - Carver College of Medicine, The University of Iowa
- Resource Type
- Journal article
- Publication Details
- Clinical Kidney Journal, Vol.8(6), pp.698-701
- Publisher
- Oxford University Press
- DOI
- 10.1093/ckj/sfv101
- PMID
- 26613027
- PMCID
- PMC4655803
- ISSN
- 2048-8505
- eISSN
- 2048-8513
- Language
- English
- Date published
- 12/2015
- Academic Unit
- Nephrology, Dialysis and Transplantation; Stead Family Department of Pediatrics; Obstetrics and Gynecology; Internal Medicine
- Record Identifier
- 9983985904902771
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