J Korean Med Sci.  2006 Dec;21(6):1121-1123. 10.3346/jkms.2006.21.6.1121.

A Case of Renal Crisis in a Korean Scleroderma Patient with Anti-RNA polymerase I and III Antibodies

• Kang EH
• Im CH
• Kim SH
• Chung JR
• Lee EY
• Kim DJ
• Lee EB
• Song YW

• KMID: 1713132
• DOI: http://doi.org/10.3346/jkms.2006.21.6.1121

Abstract

Scleroderma (SSc) renal crisis has been reported to be associated with anti-RNA polymerase I and III (RNAP I/III) antibodies in Caucasians and the Japanese. However, no report is available for Korean SSc patients. Here, we describe the case of a 65-yr-old female SSc patient who developed renal crisis and whose serum contained anti-RNAP I/III antibodies. She was finally diagnosed as having diffuse cutaneous SSc based on skin thickening proximal to the elbows and knees. Sudden hypertension, oliguria, and pulmonary edema were features of her renal crisis. Despite the use of captopril and adequate blood pressure control, her renal function deteriorated. Subsequent renal biopsy findings showed severe fibrinoid necrosis with luminal obliteration in interlobar arteries and arterioles consistent with SSc renal crisis. Serum anti-RNAP I/III antibodies were detected by radioimmunoprecipitation. This is the first report of a renal crisis in a Korean SSc patient with RNAP I/III antibodies.

Keyword

Scleroderma, Systemic; Autoantibodies; RNA Polymerase III; RNA Polymerase I

MeSH Terms

Scleroderma, Systemic/*complications/enzymology/*immunology
RNA Polymerase III/*immunology
RNA Polymerase I/*immunology
Korea
Kidney Failure, Acute/*complications/enzymology/*immunology
Humans
Female
Critical Care
Autoantibodies/*blood
Aged

Figure

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  Fig. 1 Renal biopsy shows fibrinoid necrosis and intraluminal obliteration of the interlobar artery.

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  Fig. 2 Radioisotope labeled subunits of RNAP I (190 and 126 kDa) and III (155 and 138 kDa) were precipitated by the patient serum. The serum of an SSc patient with antibodies to all three RNAPs (I, II, III) was used as a reference.

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