This document describes a study that evaluated liver fibrosis in 20 patients with systemic sclerosis using transient elastography (Fibroscan) and APRI score. The median liver stiffness score was 5.32 kPa, with only one patient having a score over 7.5 kPa indicating significant fibrosis. The median APRI score was 0.29. There was no significant correlation found between APRI score and liver stiffness. The study suggests liver fibrosis may be less common in systemic sclerosis patients without clinical signs of liver disease compared to other conditions, and non-invasive methods like transient elastography can help assess fibrosis risk.

1. Evaluation of liver fibrosis with
transient elastography (Fibroscan) and
APRI score, in patients with
Systemic Sclerosis
Alejandro Muñoz Jiménez.
Rheumatology Unit.
Valme University Hospital (Seville, Spain).
alejandrogaleno@hotmail.com

3. Apologies for my English level…

5. 1. Introduction.
2. Material and Methods
3. Results
4. Conclusions

6. 1. Introduction Systemic sclerosis (SSc) is a complex and rare autoimmune
disease characterized by vascular damage and sclerosis
2. Material and Methods within the skin and internal organs.
Gastrointestinal involvement.
3. Results Pulmonary involvement.
Renal disease.
4. Conclusions Cardiac disease.
Genitourinary.
The occurrence and extent of primary liver damage in SSc
patients have not been adequately studied.

7. 1. Introduction Liver disease found in association with scleroderma was first
described by Milbradt in 1934.
2. Material and Methods
Liver disease has not been considered a significant feature
3. Results of scleroderma and, in a large series, a higher prevalence of
liver disease was found in the control populations.
4. Conclusions

8. 1. Introduction In a review of 727 patients with scleroderma only 8 (1.1%)
had hepatic involvement.
2. Material and Methods
If this uncommon complication occurs, it usually occurs 10
3. Results to 15 years after the onset of scleroderma.
4. Conclusions

9. 1. Introduction However, in a retrospective review of postmortem findings
in scleroderma, hepatomegaly and cirrhosis were both more
2. Material and Methods common in a matched control group.
3. Results In a prospective assessment of the extent of visceral
involvement in scleroderma 16/31 (52%) patients were
4. Conclusions shown to have abnormal liver function tests or lengthened
prothrombin times.

10. 1. Introduction In general the liver and biliary system are not affected by
scleroderma.
2. Material and Methods
However, AUTOINMUNE HEPATITIS AND PRIMARY BILIARY
3. Results CIRRHOSIS are associated with the limited form of
scleroderma.
4. Conclusions

15. 1. Introduction The relationship between primary sclerosing cholangitis
(PSC) and scleroderma is extremely rare (only one case reported
2. Material and Methods of PSC in scleroderma)
3. Results
4. Conclusions

16. 1. Introduction TRANSIENT ELASTOGRAPHY (FibroScan; Echosens; Paris,
France).
2. Material and Methods
Transient Elastography (TE) is a novel and non-invasive
3. Results technique for the evaluation of fibrosis in chronic liver
disease (mainly in hepatitis C virus-infected patients).
4. Conclusions
Sensitivity can be higher than 90% for diagnosis cirrhosis.
It is not widely used in practice because the results are
operator-dependent and the performance has been shown
to be inferior to clinical examination and laboratory tests.

17. 1. Introduction An ultrasound transducer probe is mounted on the axis of a
vibrator.
2. Material and Methods
Vibrations of mild amplitude an low frequency are
3. Results transmitted by the transducer, inducing a elastic shear wave
that propagates through the underlying tissues.
4. Conclusions
Pulse-echo ultrasound acquisitions are then used to follow
the propagation of the shear wave and the measure of the
velocity, which are directly related to:
The stiffness of the tissue
The speed of the shear wave spreading.

18. 1. Introduction In a recent prospective study involving 183 patients with
chronic hepatitis C, this group assessed the performance of
2. Material and Methods TE in comparison with liver biopsy, FibroTest and APRI score.
3. Results The combination (TE and FibroTest) offered the best
diagnostic performance.
4. Conclusions

19. 1. Introduction LIMITS OF TE:
2. Material and Methods Obesity .
3. Results Ascites.
4. Conclusions

20. 1. Introduction APRI (AST platelet ratio index) SCORE
2. Material and Methods Easy (ASPARTATE AMINOTRANSFERASE and PLATELET
COUNT).
3. Results Upper Limit of the Normal range (ULN) of AST.
low cost
4. Conclusions Serological marker
Satisfactory sensitivity and specificity
High predictive value

22. 1. Introduction
OUR AIM
2. Material and Methods was to study the frequency of liver stiffness detected with TE in
patients with SSc and the association of liver stiffness with liver
3. Results disease severity measured by the APRI score.
4. Conclusions

23. 1. Introduction. 20 patients (M/F 3/17) with a diagnosis of SSc (ACR criteria).
Median age 59 years (range: 34-79).
2. Material and Methods HBV, HVC, and anti-mitochondrial antibodies were all
negatives.
3. Results Alcohol consumption was less than 20 g/day in all cases.
An APRI < 0.5 has acceptable accuracy for excluding
4. Conclusions significant fibrosis.
APRI >0.5

24. 1. Introduction. Transient elastography (TE) was performed with the
fibroscan XL.
2. Material and Methods
Liver stiffness scores were expressed as kilopascals (Kpa).
3. Results
For the diagnosis of fibrosis, a cut-off value of TE >7.5 kpa
4. Conclusions has been proposed.
TE >7.5 kpa

25. 1. Introduction. Median liver stiffness score was 5.32 Kpa (range: 3.1-9.4).
2. Material and Methods A patient had a value of 9.4 Kpa with an ultrasound pattern
compatible with liver stiffness and an APRI value of 0.18
3. Results (Only this patient had a TE ≥ 7.5 Kpa).
4. Conclusions The median APRI value was 0.29 (range: 0.16-0.58).
The patient with the APRI value of 0.58 had a liver stiffness
score of 0.31 Kpa.
There were no significant statistical correlations between
APRI score and liver stiffness (the Spearman´s rho was of
0.11).

26. 1. Introduction. Low frecuency of significant liver fibrosis (1/20 in our study) has
been demonstrated with non-invasive methods in SSc patients with
2. Material and Methods no clinical evidence of hepatic disease, as the liver could be less
prone to SSc-specific pathophysiologic events.
3. Results
The prognosis of chronic liver disease is directly related to the
4. Conclusions development of fibrosis.
At present, liver biopsy is considered as the “gold standard”
technique for the assessment of liver fibrosis, but it has several
limitations (complications in 3/1000 cases).
On the contrary, TE is a no-invasive method to asses liver fibrosis.
Further research will be needed to define advantages and
disadvantages of this technique in this study field (Liver fibrosis/SSc)

27. Acknowledgements
 Rheumatology Unit. Valme University Hospital (Seville,
Spain). Dr. J.L. Marenco and S. Rodríguez.
 Infectious Disease Unit. Valme University Hospital
(Seville, Spain).
 Biostatistics Group. Valme University Hospital (Sevilla,
Spain).
 Dra. Loreto Carmona. University Professor of the
University Camilo José Cela.

28. Thanks…