Secondary sclerosing cholangitis in critically ill patients has a poor outcome but lower tumour incidence than primary sclerosing cholangitis

Kirstein, Martha M.; Book, Thorsten; Manns, Michael P.; Hahn, Thomas von; Voigtländer, Torsten

doi:10.1177/2050640620924274

Cited by 14 publications

(18 citation statements)

References 32 publications

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“…In general, the prognosis of SSC is usually worse than PSC, but the propensity for disease progression is heavily dependent on the underlying etiology of SSC and whether it can be effectively addressed [ 15 ]. For instance, SSC related to IgG4 disease is highly responsive to steroid therapy [ 16 ], whereas SSC occurring in the setting of critical illness is often irreversible and rapidly progressive [ 17 ]. Similar to PSC, end-stage SSC results in biliary cirrhosis which is only curable with liver transplantation [ 18 ].…”

Section: Secondary Sclerosing Cholangitis (Ssc)mentioning

confidence: 99%

Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis

et al. 2022

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Sclerosing cholangitis is a chronic cholestatic disease characterized by stricturing, beading, and obliterative fibrosis of the bile ducts. Sclerosing cholangitis is considered primary (PSC) if no underlying etiology is identified or secondary (SSC) if related to another identifiable cause. In this article, we will review the clinical features, pathogenesis, diagnosis, and imaging findings of PSC and SSC, with an emphasis on features that may aid in the distinction of these entities. We will also discuss various etiologies of SSC including recurrent pyogenic cholangitis, other infectious etiologies, ischemic damage, toxic insults, and immunologic, congenital, and miscellaneous causes, highlighting the unique imaging findings and clinical context of each diagnosis. Graphical abstract

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Section: Secondary Sclerosing Cholangitis (Ssc)mentioning

confidence: 99%

Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis

et al. 2022

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“…Histological findings of liver biopsy, radiological imaging, ultrasound testing, Doppler ultrasound, computed tomography (CT), MRI, and ERCP [ 5 , 22 ], and elastography for evaluation of liver stiffness. ERCP is considered in order to conclude the diagnosis in most cases, and it is the gold standard diagnostic test for SSC-CIP [ 16 , 17 ].…”

Section: Discussionmentioning

confidence: 99%

“…Ten of 11 patients were discharged from the hospital after liver transplantation; however, 2 patients died within 4 and 8 months due to sepsis, and 1 patient died 1.5 months after liver transplantation due to shock after a rupture of a hepatic artery aneurysm. In the non-traumatic SC-CIP group, sepsis was the main cause of death [ 22 ]. Patients who survived the first 8 months after liver transplantation had a good long-term survival prognosis.…”

Section: Discussionmentioning

confidence: 99%

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Meta-analysis and systematic review of liver transplantation as an ultimate treatment option for secondary sclerosing cholangitis

Gadour¹,

Hassan²

2022

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Introduction Secondary sclerosing cholangitis (SSC) is a chronic cholestatic biliary disease, characterized by inflammation, obliterative fibrosis of the bile ducts, stricture formation, and progressive destruction of the biliary tree, which leads to biliary cirrhosis. In recent years, the development of secondary sclerosing cholangitis in critically ill patients (SSC-CIP) has increasingly been perceived as a separate disease entity. Aim To perform a systematic review and meta-analysis of secondary sclerosing cholangitis and ischaemic cholangiopathy in post organ transplant patients and intensive care unit (ICU). Material and methods A comprehensive search strategy using the PubMed, Biosis, and EMBASE databases was designed to retrieve relevant clinical data from the published literature up to 2020. Demographic characteristics, laboratory, transplantation, mortality rate, and follow-up data undergoing liver transplantation were extracted from the inclusion studies. We used DerSimonian–Laird random-effects meta-analysis. Analysis was carried out using R statistical software version 4.02. Results A total of 862 patients with SSC-CIP were extracted from 16 studies. Eighteen studies were searched for the meta-analysis, out of which 16 studies were eligible for the meta-analysis and 2 were excluded. A proportion meta-analysis was performed on liver transplant patients with SSC-CIP and on mortality rate. Significant results were found (Prop = 0.30, 95% CI: 0.12–0.49, p < 0.01), with high heterogeneity among the studies ( I 2 = 98%, p < 0.01) and (Prop = 0.45; 95% CI: 0.35–0.56 with I 2 = 80, p < 0.01), respectively. No indication of publication bias, as confirmed by the funnel plot and the risk of bias in the included studies, shows that the study reporting is adequate to judge that no major or minor sources of bias are likely to influence results. Conclusions The systematic review and meta-analysis show that liver transplantation is a valid option for patients with SSC-CIP, with excellent long-term outcome and improvement of quality of life.

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“…In the case of SCC, therapeutic options are limited to liver transplantations (LT) while patients not undergoing transplantation show significantly reduced survival compared with patients with primary sclerosing cholangitis (idiopathic form mostly associated with inflammatory bowel disease). Sclerosing cholangitis in critically ill patients is, therefore, associated with rapid disease progression and poor outcome with estimated mortality rates after 6 months being higher than 50% (15,34). In the case of severely burned patients, liver transplantation is frequently not possible because of open wounds and ongoing infections.…”

Section: Discussionmentioning

confidence: 99%

Technical Note: Novel Use of CytoSorb™ Haemadsorption to Provide Wound Healing Support in Case of Severe Burn Trauma via Reduction of Hyperbilirubinaemia

et al. 2021

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Hyperbilirubinaemia has been shown to compromise wound healing in severely burned patients. The therapy options for patients with impairment of wound healing and subsequent severe liver dysfunction are limited. A novel extracorporeal treatment, CytoSorb® (CytoSorbents Corp, USA), is a whole blood adsorber composed of highly biocompatible and porous polystyrene divinylbenzene copolymer beads covered in a polyvinylpyrrolidone coating. It is capable of extracting mainly hydrophobic middle-sized (up to 55 kDa) molecules from blood via size exclusion, including cytokines and bilirubin. We performed therapy with CytoSorb® on a severely burned (48% Total Body Surface Area-TBSA) patient with secondary sclerosing cholangitis (SCC) to promote the wound healing process by reducing bilirubin concentrations and to bridge the time to spontaneous liver regeneration or eventually to liver transplantation after two skin transplantations had failed to provide wound closure. In the first 6 days the cartridge was changed on a daily basis and later after every 2–4 days. The therapy with six adsorbers decreased a total bilirubin concentration from 14.02 to 4.29 mg/dl. By maintaining a stable bilirubin concentration under 5 mg/dl, debridement of abdomen and upper extremities with autologous skin grafting and, 4 weeks later, autologous skin grafting of the back from scrotum and lower extremities were performed successfully. After wound healing had been achieved, the CytoSorb therapy was discontinued after 57 days and 27 adsorber changes. CytoSorb therapy can be a promising support of wound and skin graft healing in patients with severe burns and liver dysfunction due to a significant reduction of total bilirubin concentration.

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Secondary sclerosing cholangitis in critically ill patients has a poor outcome but lower tumour incidence than primary sclerosing cholangitis

Cited by 14 publications

References 32 publications

Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis

Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis

Meta-analysis and systematic review of liver transplantation as an ultimate treatment option for secondary sclerosing cholangitis

Technical Note: Novel Use of CytoSorb™ Haemadsorption to Provide Wound Healing Support in Case of Severe Burn Trauma via Reduction of Hyperbilirubinaemia

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