Case reportPlasma separation and anion adsorption transiently relieve intractable pruritus in primary biliary cirrhosis
Introduction
Pruritus is a common complication of cholestatic liver diseases, particularly of primary biliary cirrhosis (PBC) [1]. It may dramatically impair the patient’s quality of life by limiting normal activities, causing sleep deprivation and even suicidal sensations [2]. The pathogenesis of cholestasis-associated pruritus remains poorly understood and may be multifactorial. Peripherally acting pruritogens and altered central neurotransmission have been implicated as causing pruritus in cholestasis [3]. Current therapeutic interventions for cholestatic pruritus are thus directed towards elimination or inactivation of putative peripheral pruritogens by anion exchange resins such as cholestyramine and colestipol or enzyme-inducing agents such as rifampicin or modulation of altered central neurotransmission by opioid or serotonin receptor antagonists [3]. In patients who do not respond to medical therapies, invasive procedures, such as nasobiliary drainage or partial external diversion of bile [4], [5], [6], [7], ileal diversion [8], hemodialysis [9], charcoal hemoperfusion [10], plasmapheresis and plasma perfusion [11], [12], [13], and extracorporeal albumin dialysis [14], [15], have been tried in small numbers of patients.
The present pilot study reports on the effect of plasma separation and anion adsorption via a peripheral vein in patients with PBC suffering from severe, otherwise intractable pruritus.
Section snippets
Patients
Three patients with clinical, biochemical, and histological characteristics of primary biliary cirrhosis (Table 1) and intractable pruritus defined by severity of pruritus ⩾7 on a scale between 0 and 10 on at least 4 of 7 days despite treatment with cholestyramine (⩾8 g/day), rifampicin (300–600 mg/day), and naltrexone (50 mg/day), each for >4 weeks, were included in the pilot study. All patients had undergone nasobiliary drainage of bile at least once in the past [7].
Plasma separation and anion adsorption
Plasma separation and anion
Safety
Plasma separation and anion adsorption were well tolerated by all patients. No adverse effects were reported. No significant hemodynamic abnormalities or bleeding events were observed during and after the treatment.
Pruritus
All three patients reported a rapid improvement of pruritus as assessed by a rating scale from 0 (no pruritus) to 10 (unbearable or maximal imaginable pruritus) (Fig. 1B). The reduction in pruritus over the first week was 80% in patient 1, 21% in patient 2, and 37% in patient 3 (mean
Discussion
In this pilot study, we show that severe intractable pruritus in patients with PBC was successfully treated with plasma separation and anion adsorption through anion exchange resin columns. The treatment was well tolerated and no adverse effects were observed. All patients reported a rapid, but temporary attenuation of pruritus intensity paralleled by an improvement in fatigue and quality of life suggesting that pruritus is a symptom, which contributes to fatigue and reduced quality of life in
Acknowledgement
The supply of columns for 2 of 3 patients by Diamed Medizintechnik GmbH (Cologne, Germany) is gratefully acknowledged.
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Cited by (41)
Primary biliary cirrhosis
2015, The LancetCitation Excerpt :Plasmapheresis can be considered when pruritus is refractory to medical therapy. Cholestatic pruritus should always respond to plasmapheresis, with duration of benefit ranging from weeks to years.98 The expense, invasiveness, and inconvenience of plasmapheresis make this procedure a last resort.
Severe cholestatic hepatitis revealing a DRESS syndrome
2013, Revue de Medecine InternePathophysiology and current management of pruritus in liver disease
2011, Clinics and Research in Hepatology and GastroenterologyCitation Excerpt :As experimental drug therapies propofol [129], lidocaine [130], phenobarbital [126], flumecinol [131], stanozolol [132], ondansentrone [107], dronabinol [133] and butorphanol [134] have been used in the past with variable success. Furthermore, invasive procedures such as plasmapharesis [135], molecular adsorbent recirculating system therapy [136–138], plasma separation/anion absorption [139], transcutaneous and nasobiliary drainage [24,25] have been beneficial in severe, otherwise, untreatable cholestatic pruritus in case series. For these experimental approaches, the reader is referred to reference [5].
Treatment of resistant pruritus from cholestasis with albumin dialysis: Combined analysis of patients from three centers
2010, Journal of HepatologyCitation Excerpt :In patients who do not respond to medical therapies, different invasive procedures, such as hemodialysis [11], charcoal hemoperfusion [12], and plasmapheresis [13–15] have been used with positive, although uncertain results, due to the small number of patients evaluated. More recently, the therapeutic effects of various methods of extracorporeal albumin dialysis [16–28], mainly the molecular adsorbent recirculating system (MARS), have been published [16–27]. However, from these case reports or short series (the highest has included seven patients) [21], it is difficult to ascertain the actual efficacy of the procedure in patients with cholestatic pruritus.
Systematic review: efficacy of therapies for cholestatic pruritus
2023, Therapeutic Advances in Gastroenterology