Background
Primary biliary cholangitis (PBC) may progress to cirrhosis and clinically significant portal hypertension (CSPH). This study assesses different features of CSPH and their distinct ...prognostic impact regarding decompensation and survival in patients with PBC.
Methods
Patients with PBC were identified during a database query of our digital patient reporting system.
Results
A total of 333 PBC patients (mean age 54.3 years, 86.8% females, median follow-up 5.8 years) were retrospectively assessed and 127 (38.1%) showed features of CSPH: 63 (18.9%) developed varices, 98 (29.4%) splenomegaly, 62 (18.6%) ascites and 20 (15.7%) experienced acute variceal bleeding. Splenomegaly, portosystemic collaterals and esophageal varices were associated with an increased 5-year (5Y) risk of decompensation (15.0%, 17.8% and 20.9%, respectively). Patients without advanced chronic liver disease (ACLD) had a similar 5Y-transplant free survival (TFS) (96.6%) compared to patients with compensated ACLD (cACLD) but without CSPH (96.9%). On the contrary, PBC patients with cACLD and CSPH (57.4%) or decompensated ACLD (dACLD) (36.4%) had significantly decreased 5Y survival rates. The combination of LSM < 15 kPa and platelets ≥ 150G/L indicated a negligible risk for decompensation (5Y 0.0%) and for mortality (5Y 0.0%). Overall, 44 (13.2%) patients died, with 18 (40.9%) deaths attributed to CSPH-related complications.
Conclusion
In PBC, features of CSPH may occur early and indicate an increased risk for subsequent decompensation and mortality. Hence, regular screening and on-time treatment for CSPH is crucial. Combining LSM and platelets serves as a valuable preliminary assessment, as LSM < 15 kPa and platelets ≥ 150G/L indicate an excellent long-term outcome.
Liver cirrhosis accounts for considerable morbidity and mortality worldwide and late presentation limits therapeutic options. We aimed to assess characteristics of patients with liver cirrhosis at ...the time of first presentation and during their clinical course. 476 patients were included (alcohol-related liver disease, ALD: 211, 44.3%; viral hepatitis: 163, 34.2%). Of these, 106 patients (22.3%) and 160 patients (33.6%) presented already with Child-Pugh C and MELD >15, respectively, and decompensation events were registered in 50% (238 patients) at baseline, and even in 75.4% of ALD patients. Half of the patients with cirrhosis had decompensated cirrhosis at presentation. This calls for increased awareness and strategies for earlier diagnosis of chronic liver disease and cirrhosis.
Celotno besedilo
Dostopno za:
DOBA, IZUM, KILJ, NUK, PILJ, PNG, SAZU, SIK, UILJ, UKNU, UL, UM, UPUK
Objectives
Porto-sinusoidal vascular disorder (PSVD) is a recently defined vascular liver disease. Since diagnosis remains challenging, we aimed to evaluate radiological features that are distinct ...between PSVD and cirrhosis.
Methods
Clinical, laboratory, and radiological parameters (CT/MRI) of patients with histologically-confirmed PSVD vs. cirrhosis vs. non-cirrhotic parenchymal liver disease were retrospectively evaluated.
Results
Sixty-three PSVD, 155 cirrhosis, and 41 non-cirrhotic patients were included. As compared to cirrhosis, PSVD patients were younger and had lower HVPG, liver stiffness, and MELD. Routine clinical and imaging findings indicative of portal hypertension were similarly common. Intrahepatic portal tract abnormalities (49% vs. 15%;
p
< 0.001), FNH-like lesions (30% vs. 1%;
p
< 0.001), and abnormal liver morphology defined as peripheral parenchymal atrophy and compensatory hypertrophy of central segments (32% vs. 7%;
p
< 0.001) were significantly more common in PSVD patients. Hypertrophy of segment I (70% vs. 84%;
p
= 0.019), atrophy of segment IV (24% vs. 47%;
p
= 0.001), and nodular liver surface (22% vs. 89%;
p
< 0.001) were more common in patients with cirrhosis. In patients with gadoxetic acid–enhanced MRI, we identified the distinct imaging feature of “periportal hyperintensity” in the hepatobiliary phase (HBP) in 42% of patients with PSVD (14/33) vs. 1% in cirrhosis (1/95) vs. 0% in non-cirrhotic controls (0/41);
p
< 0.001).
Conclusions
Diagnosis of PSVD must be considered in younger patients presenting with clinical features of portal hypertension, portal tract abnormalities, and FNH-like lesions on CT/MRI. ‘Periportal hyperintensity’ in the HBP of gadoxetic acid–enhanced MRI was identified as a specific radiological feature of PSVD.
Key Points
• Cross-sectional imaging can provide essential information to identify patients with porto-sinusoidal vascular disorder (PSVD).
• Intrahepatic portal tract abnormalities, FNH-like lesions, and abnormal liver morphology are common in PSVD patients.
• Periportal hyperintensity on the hepatobiliary phase of gadoxetic acid–enhanced MRI seems to be specific for patients with PSVD.
Zusammenfassung
Autoimmun mediierte Lebererkrankungen, wie die Autoimmunhepatitis (AIH), die primär biliäre Cholangitis (PBC) und die primär sklerosierende Cholangitis (PSC), zählen aufgrund ihrer ...niedrigen Prävalenz zu den seltenen Lebererkrankungen. In diesem Kollektiv besteht die diagnostische Herausforderung vor allem darin, betroffene Patient*innen unter der Vielzahl der häufig vorkommenden Hepatopathien zu identifizieren und in weiterer Folge einer Therapie zuzuführen, um die Krankheitsprogression in Richtung Leberzirrhose zu unterbinden. Im Kontext der Autoimmunhepatitis steht primär die Therapie mit Kortikosteroiden und Immunsuppressiva, wie z. B. mit Azathioprin, im Vordergrund. Im Gegensatz dazu basiert die Therapie der PBC und PSC primär auf der Etablierung von Ursodeoxycholsäure, die bei einem Großteil der PBC-Patient*innen eine gute Wirkung erzielt, wohingegen sich die therapeutischen Erfolge bei PSC-Patient*innen meist in Grenzen halten. Etwa ein Drittel aller Patient*innen mit autoimmunen Lebererkrankungen präsentiert sich bereits bei Erstdiagnose mit einer begleitenden Leberzirrhose und ggf. einer klinisch signifikanten portalen Hypertension (CSPH), sodass neben der ätiologischen Therapie auch an die Etablierung pfortaderdrucksenkender Maßnahmen gedacht werden muss, wie zum Beispiel durch Verschreibung nichtselektiver Betablocker. Weiters empfiehlt sich eine sechsmonatliche sonographische Kontrolle zum regelmäßigen HCC-Screening. Mit Ausnahme der PSC haben die hier angeführten Erkrankungen unter adäquater Therapie eine gute Langzeitprognose.
LINKED CONTENT This article is linked to Burghart et al papers. To view these articles, visit https://doi.org/10.1111/apt.18060 and https://doi.org/10.1111/apt.18153
The safety and efficacy of sodium‐glucose cotransporter 2 inhibitors in posttransplantation diabetes mellitus is unknown. We converted stable kidney transplant patients to 10 mg empagliflozin, aiming ...at replacing their insulin therapy (<40 IU/d). N = 14 participants (the required sample size) completed the study visits through 4 weeks and N = 8 through 12 months. Oral glucose tolerance test (OGTT)–derived 2‐hour glucose (primary end point) increased from 232 ± 82 mg/dL (baseline) to 273 ± 116 mg/dL (4 weeks, P = .06) and to 251 ± 71 mg/dL (12 months, P = .41). Self‐monitored blood glucose and hemoglobin A1c were also clinically inferior with empagliflozin monotherapy, such that insulin was reinstituted in 3 of 8 remaining participants. Five participants (2 of them dropouts) vs nine of 24 matched reference patients developed bacterial urinary tract infections (P = .81). In empagliflozin‐treated participants, oral glucose insulin sensitivity decreased and beta‐cell glucose sensitivity increased at the 4‐week and 12‐month OGTTs. Estimated glomerular filtration rate and bioimpedance spectroscopy‐derived extracellular and total body fluid volumes decreased by 4 weeks, but recovered. All participants lost body weight. No participant developed ketoacidosis; 1 patient developed balanitis. In conclusion, although limited by sample size and therefore preliminary, these results suggest that empagliflozin can safely be used as add‐on therapy, if posttransplant diabetes patients are monitored closely (NCT03113110).
Empagliflozin appears safe, but exerts a weak antihyperglycemic effect, suggesting that the drug should be used as add‐on therapy under close medical supervision in patients with stable post–kidney transplantation diabetes mellitus.
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