title: Renal biopsy findings predicting outcome in scleroderma renal crisis.
abstract: Scleroderma renal crisis is irreversible in some patients despite aggressive treatment. This study was designed to identify pathologic prognostic features in scleroderma renal crisis. We retrospectively reviewed the pathology and the clinical records of 17 patients who underwent kidney biopsies during scleroderma renal crisis (group A, recovered renal function [n = 7]; group B, remained in renal failure or died [n = 10]). Multiple histologic features were assessed semiquantitatively (0-3) or as percentages. C4d staining of peritubular capillaries and small vessels was assessed semiquantitatively (0-3) in patients with scleroderma (n = 11), normotensive (n = 10), and hypertensive (n = 12) nonscleroderma native kidney controls. The percentage of thrombosed vessels (25.1 +/- 21.0 versus 5.6 +/- 12.3, P = .045) and the severity of glomerular ischemic collapse (2.9 +/- 0.3 versus 1.4 +/- 0.8, P = .001) were significantly higher in group B than in group A. Also, group B patients tended to have more severe acute tubular injury and vascular fibrinoid changes. The peritubular capillary C4d score in patients with scleroderma, normotensive controls, and hypertensive controls were 1.1 +/- 0.9, 0.3 +/- 0.7, and 0.3 +/- 0.5, respectively (P = .018, scleroderma versus other controls). Small vessel C4d score was higher in scleroderma compared to normotensive but not hypertensive controls. Within scleroderma samples, a significantly higher peritubular capillary C4d score (1.6 +/- 0.7 versus 0.3 +/- 0.5, P = .024) but not small vessel score was found in group B compared to group A. This tended to be associated with peritubular capillary leukocyte margination. Vascular thrombosis, severe glomerular ischemic collapse, and peritubular capillary C4d deposits in scleroderma renal crisis kidney biopsies correlated with increased risk of failure to recover renal function.
authors.affiliation: Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA 15213, USA.
authors.forename: Ibrahim
authors.initials: I
authors.lastname: Batal
authors.forename: Robyn T
authors.initials: RT
authors.lastname: Domsic
authors.forename: Aaron
authors.initials: A
authors.lastname: Shafer
authors.forename: Thomas A
authors.initials: TA
authors.lastname: Medsger
authors.forename: Lawrence P
authors.initials: LP
authors.lastname: Kiss
authors.forename: Parmjeet
authors.initials: P
authors.lastname: Randhawa
authors.forename: Sheldon
authors.initials: S
authors.lastname: Bastacky
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D002196:Capillaries / Q000378:metabolism / Q000473:pathology; D015935:Complement C4b / Q000378:metabolism; D005260:Female; D006801:Humans; D007511:Ischemia / Q000209:etiology / Q000473:pathology; D007678:Kidney Glomerulus / Q000098:blood supply / Q000473:pathology; D007684:Kidney Tubules / Q000098:blood supply / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010446:Peptide Fragments / Q000378:metabolism; D011237:Predictive Value of Tests; D011379:Prognosis; D051437:Renal Insufficiency / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D015996:Survival Rate; D013927:Thrombosis / Q000209:etiology / Q000473:pathology; D055815:Young Adult
chemical_list: D010446:Peptide Fragments; D015935:Complement C4b; C032261:complement C4d