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Hwang, Yoon, Kang, Lee, Lee, Lee, and Park: The Primary Biliary Cirrhosis Presenting with Raynaud's Disease and Digital Necrosis
Case

J Rheum Dis 2011;18(1):50-54.

Published online: 31 March 2011

DOI: https://doi.org/10.4078/jrd.2011.18.1.50

The Primary Biliary Cirrhosis Presenting with Raynaud's Disease and Digital Necrosis

Jenie Yoonoo Hwang, Hwang Yoon, Jong Sik Kang, Sang Ah Lee, Woo Je Lee, Ki-Up Lee, Joong Yeol Park

Department of Internal Medicine, University of Ulsan College of Medicine, Seoul Asan Medical Center, Seoul, Korea

Received 7 September 201028 September 2010       Accepted 13 October 2010

Copyright © 2011 The Korean College of Rheumatology

Abstract

Necrosis of the upper extremity occurs rarely in type 2 diabetic patients compared to lower extremity necrosis. We report a 69-year-old woman with type 2 diabetes mellitus who presented with necrosis of the left 5 th finger tip. The patient had primary biliary cirrhosis accompanied by necrosis of the fingertip due to severe Raynaud's phenomenon. Primary biliary cirrhosis (PBC) is a typical autoimmune disease, which can in rare cases be accompanied by autoimmune symptoms including sicca symptom and Raynaud's phenomenon. Furthermore, autoimmune diseases, such as systemic sclerosis, rheumatoid arthritis and undifferentiated connective tissue disease (UCTD) can be associated. Although every type of vascular etiology should be considered as a cause of digital necrosis, Raynaud's phenomenon is usually not considered in diabetes. We report this case of finger tip necrosis due to severe Raynaud's phenomenon accompanied by PBC and UCTD in a diabetic patient.

Keywords: Primary biliary cirrhosis, Undifferentiated connective tissue disease, Raynaud's phenomenon, Necrosis, Fingers, Diabetes mellitus

References

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Figure 1.
Computed tomography of the upper abdomen (A) showed cirrhosis of the liver and spleno-megaly. An angiogram of the left hand (B) revealed slightly decreased blood flow in the palmar branch of the left ulnar artery with smooth tapering, which suggests a normal variant of palmar circulation. (C) The patient's left 5 th finger tip was swollen and showed necrotic changes.
jrd-18-50f1.tif
Table 1.
Laboratory findings on the admission and 2 years prior to admission
Test 2 yrs PTA Admission Reference value
Rheumatoid factor (IU/ml) 15.2 16.1 0∼15
ANA titer 1 : 640 discrete speckled type 1 : 640 discrete speckled type <1 : 40
Anti-SSA (Ro) (S/C ratio) Negative Negative Negative
Anti-SSB (La) Negative Negative Negative
Anti-Mitochondrial Ab. Not done 1 : 160 Negative
Anti-Smooth Muscle Ab. Negative Negative Negative
Anti-LKM 1 Ab. Not done Negative Negative
Anti-Scl-70 Ab. Negative Negative <1 : 2
HBsAg Negative Negative Negative
HCV RNA (IU/mL) <12 <12 <12
PT (%) 106.1 86.1 70∼140
Albumin (g/dL) 3.4 3.1 3.3∼5.2
AST (IU/L) 119 146 0∼40
ALT (IU/L) 25 94 0∼40
ALP (IU/L) 317 213 40∼120
r-GT (IU/L) 451 223 8∼35
ANCA Negative Negative <1 : 20 negative
Total bilirubin (mg/dL) 0.9 0.9 0.2∼1.2
Anti-RNP Ab. Negative Negative Negative
HbA1c (%) 6.3 6.1 4∼6.4
IgG (mg/dL) Not done 1,690 <1,600
8 hr microablumin (ug/min) 1.3 3.2 0∼18
Anticardiolipin IgG (GPL/mL) 36 Not done <21
Anticardiolipin IgM (MPL/mL) 25 Not done <21

PTA, prior to admission; PT, prothrombin time; Anti-LKM 1 Ab, anti-liver/kidney microsome 1 antibody

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