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

Apologies for my English level…

1. Introduction.

2. Material and Methods

3. Results

4. Conclusions

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

Systemic sclerosis (SSc) is a complex and rare autoimmune disease characterized by vascular damage and sclerosis within the skin and internal organs.

Gastrointestinal involvement. Pulmonary involvement. Renal disease. Cardiac disease. Genitourinary.

The occurrence and extent of primary liver damage in SSc patients have not been adequately studied.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

Liver disease found in association with scleroderma was first described by Milbradt in 1934. Liver disease has not been considered a significant feature of scleroderma and, in a large series, a higher prevalence of liver disease was found in the control populations.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

In a review of 727 patients with scleroderma only 8 (1.1%) had hepatic involvement. If this uncommon complication occurs, it usually occurs 10 to 15 years after the onset of scleroderma.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

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

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

In general the liver and biliary system are not affected by scleroderma. However, AUTOINMUNE HEPATITIS AND PRIMARY BILIARY CIRRHOSIS are associated with the limited form of scleroderma.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

The relationship between primary sclerosing cholangitis (PSC) and scleroderma is extremely rare (only one case reported

of PSC in scleroderma)

TRANSIENT ELASTOGRAPHY (FibroScan; Echosens; Paris, France).

Transient Elastography (TE) is a novel and non-invasive technique for the evaluation of fibrosis in chronic liver disease (mainly in hepatitis C virus-infected patients). 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.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

An ultrasound transducer probe is mounted on the axis of a vibrator. Vibrations of mild amplitude an low frequency are transmitted by the transducer, inducing a elastic shear wave that propagates through the underlying tissues. 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.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

In a recent prospective study involving 183 patients with chronic hepatitis C, this group assessed the performance of TE in comparison with liver biopsy, FibroTest and APRI score. The combination (TE and FibroTest) offered the best diagnostic performance.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

LIMITS OF TE: Obesity . Ascites.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

APRI (AST platelet ratio index) SCORE

Easy (ASPARTATE AMINOTRANSFERASE and PLATELET COUNT). Upper Limit of the Normal range (ULN) of AST. low cost Serological marker Satisfactory sensitivity and specificity High predictive value

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

OUR AIM was to study the frequency of liver stiffness detected with TE in patients with SSc and the association of liver stiffness with liver

disease severity measured by the APRI score.

1. Introduction

2. Material and Methods

3. Results

4. Conclusions

1. Introduction.

2. Material and Methods

3. Results

4. Conclusions

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

APRI >0.5

1. Introduction.

2. Material and Methods

3. Results

4. Conclusions

Transient elastography (TE) was performed with the fibroscan XL. Liver stiffness scores were expressed as kilopascals (Kpa). For the diagnosis of fibrosis, a cut-off value of TE >7.5 kpa has been proposed.

TE >7.5 kpa

1. Introduction.

2. Material and Methods

3. Results

4. Conclusions

Median liver stiffness score was 5.32 Kpa (range: 3.1-9.4). A patient had a value of 9.4 Kpa with an ultrasound pattern compatible with liver stiffness and an APRI value of 0.18 (Only this patient had a TE ≥ 7.5 Kpa). 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).

1. Introduction.

2. Material and Methods

3. Results

4. Conclusions

Low frecuency of significant liver fibrosis (1/20 in our study) has been demonstrated with non-invasive methods in SSc patients with no clinical evidence of hepatic disease, as the liver could be less prone to SSc-specific pathophysiologic events.

The prognosis of chronic liver disease is directly related to the 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)

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.

Thanks…