title: Oxalate nephropathy in systemic sclerosis: Case series and review of the literature.
abstract: OBJECTIVE
  To increase awareness of oxalate nephropathy as a cause of acute kidney injury (AKI) among systemic sclerosis patients with small intestinal dysmotility and malabsorption, and to prompt consideration of dietary modification and early treatment of predisposing causes of oxalate nephropathy in this population.
  
  
  METHODS
  Two cases of biopsy-proven oxalate nephropathy were identified among systemic sclerosis patients in the course of direct clinical care. Subsequently, a retrospective search of the Johns Hopkins Pathology databases identified a third patient with systemic sclerosis who developed oxalate nephropathy.
  
  
  RESULTS
  Among the three patients with qualifying biopsies, all three had systemic sclerosis with lower gastrointestinal involvement. All three presented with diarrhea, malabsorption, and AKI. In two of the three patients, diarrhea was present for at least 2 years before the development of AKI; in the third, incidental oxalate nephropathy was noted 3 years before she developed AKI and extensive oxalate nephropathy in the setting of a prolonged mycobacterium avium-intracellulare enteritis. In the first case, oxalate crystals were present by urinalysis months before diagnosis by biopsy; in the second, hyperoxaluria was diagnosed by urine collection immediately after; and in the third, oxalate crystals had been noted incidentally on post-transplant renal biopsy 3 years before the development of fulminant oxalate nephropathy. All three patients died within a year after diagnosis.
  
  
  CONCLUSIONS
  Patients with systemic sclerosis and bowel dysmotility associated with chronic diarrhea and malabsorption may be at risk for an associated oxalate nephropathy. Regular screening of systemic sclerosis patients with small bowel malabsorption syndromes through routine urinalysis or 24-h urine oxalate collection, should be considered. Further studies defining the prevalence of this complication in systemic sclerosis, the benefit of dietary modification on hyperoxaluria, the effect of treating small intestinal bowel overgrowth with antibiotics, and the effectiveness of probiotics, calcium supplements, or magnesium supplements to prevent hyperoxaluria-associated renal disease in these patients, are warranted.
authors.affiliation: Department of Medicine, Johns Hopkins University School of Medicine, 5200 Eastern Ave, Mason F. Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224.
authors.forename: Colin B
authors.initials: CB
authors.lastname: Ligon
authors.affiliation: Department of Medicine, Johns Hopkins University School of Medicine, 5200 Eastern Ave, Mason F. Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224.
authors.forename: Laura K
authors.initials: LK
authors.lastname: Hummers
authors.affiliation: Department of Medicine, Johns Hopkins University School of Medicine, 5200 Eastern Ave, Mason F. Lord Building, Center Tower, Suite 4100, Baltimore, MD 21224. Electronic address: zmcmaha1@jhmi.edu.
authors.forename: Zsuzsanna H
authors.initials: ZH
authors.lastname: McMahan
mesh_terms: D058186:Acute Kidney Injury / Q000150:complications* / Q000652:urine; D000368:Aged; D005260:Female; D006801:Humans; D006959:Hyperoxaluria / Q000150:complications* / Q000652:urine; D008875:Middle Aged; D019815:Oxalic Acid / Q000652:urine*; D012595:Scleroderma, Systemic / Q000150:complications* / Q000652:urine
chemical_list: D019815:Oxalic Acid
keywords: Acute kidney injury; Calcium oxalate; Chronic kidney disease; Gastrointestinal disease; Malabsorption; Oxalate nephropathy; Scleroderma; Scleroderma renal crisis; Small intestinal bacterial overgrowth; Systemic sclerosis