A case of intractable pruritus in Turner’s syndrome successfully treated with molecular adsorbent recirculating system

Abstract

Pruritus is the most disabling symptom in patients with cholestatic liver diseases, such as primary biliary cirrhosis, primary sclerosing cholangitis and drug induced cholestasis. Many drug therapies have been used for treating pruritus in these patients, such as histamine antagonists, ursodeoxycholic acid, cholestyramine, enzyme inducing agents as rifampicin or phenobarbital, for inactivation of putative peripheral pruritogens, and finally opioid antagonists as naloxone or naltrexone, which modulate altered central neurotransmission. Other methods have been tried in patients who do not respond to medical therapies. Extracorporeal albumin dialysis with the molecular adsorbent recirculating system (MARS) is used for the treatment of acute decompensation of chronic liver disease, acute liver failure, liver failure after surgery or liver transplantation [1, 2]. It has also been claimed that it may be useful in intractable pruritus in cholestatic syndromes [1, 3, 4]. We report the effect of this treatment in a patient with Turner’s syndrome and drug induced cholestasis refractory to medical therapy. A 54-year-old woman came to our attention because of intractable pruritus. She was affected by Turner’s syndrome (karyotype 45, X0),and hypothyroidism. Since the age of 23 years she had suffered of amenorrea and she had been treated with estro-progestinic therapy for many years. One year before hospitalisation, her therapy was changed into tibolone at a dose of 2.5 mg once daily. Approximately 6 months later, the patient presented itching and mild jaundice, she was admitted to the hospital and a percutaneous liver biopsy was performed; it revealed a pattern of drug induced cholestatic hepatitis. She was treated with ursodeoxycholic acid, cholestiramine and histamine antagonist, and a resolution of jaundice occurred. After an initial improvement of pruritus, later on she suffered from a severe relapse, in spite of continuing medical therapy. Six months later she was admitted to our hospital; the patient presented scratching skin lesions on arms and legs and sleep deprivation that made worse her quality of life. Our laboratory data demonstrated a normal liver function (Child-Pugh-Turcotte: A5), a moderate elevation of transaminases (GOT: 55 U/L, GPT: 51 U/L) and elevated AP: 863 U/L and gGT: 53 U/L. Bilirubin level was within normal values. Autoimmune liver disease was excluded because of negative antimytochondrian antibodies, antinuclear antibodies, antineutrophilic cytoplasm antibodies, smooth muscle antibodies, C-reactive protein, rheumatoid factor. Hepatic ultrasonography was normal. Taking into account the unsuccessful previous medical treatment we decided to treat the patient with the extracorporeal albumin dialysis (MARS). The MARS system (MARS module: Teraklin AG, Rostock, Germany) was used in combination with a standard dialysis machine. The blood flow was set to 185 ml/min, the flow in the closed albumin dialysate E. Silvagni B. Stagni L. Bolondi (&) Division of Internal Medicine, Department of Internal Medicine and Gastroenterology, University of Bologna, S.Orsola-Malpighi Hospital, Via Albertoni, 15, 40138 Bologna, Italy e-mail: bolondi@med.unibo.it