Progressive systemic sclerosis with the nephrotic syndrome and acquired factor X deficiency.

Abstract:

:A patient with progressive systemic sclerosis (PSS) involving skin, digit, esophagus, and lung developed the nephrotic syndrome and rapidly progressive renal insufficiency. Renal pathology revealed capillary collapse, cellular crescents, arteriolar hyaline deposition, and mesangial proliferation. On immunofluorescence IgM, C3, and fibrinogen were present in mesangium and capillary walls. Prebiopsy coagulation screening revealed a factor X deficiency which caused substantial prolongation of the partial thromboplastin time without an overt bleeding diathesis. The acquired factor X deficiency resolved after fresh frozen plasma and vitamin K administration, although some spontaneous improvement was noted. Nephrotic syndrome may occasionally be seen in the acute fulminant form of PSS and should not deter diagnosis of PSS.

journal_name

Am J Nephrol

authors

Eiser AR,Zilversmit R,Neff MS,Grishman E,Slifkin RF

doi

10.1159/000167055

subject

Has Abstract

pub_date

1986-01-01 00:00:00

pages

61-5

issue

1

eissn

0250-8095

issn

1421-9670

journal_volume

6

pub_type

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