Staffan Wahlin

Karolinska Institutet | KI · Department of Medicine, Huddinge

Objective To estimate the incidence, prevalence, and outcomes of patients with diagnosed alpha‐1‐antitrypsin deficiency (AATD) in Sweden, 2002–2020. Study design and setting The Swedish National Patient Registry was utilized to identify patients with a first diagnosis of AATD between 2002 and 2020. Each patient was matched with up to 10 comparator...

Introduction Erythropoietic protoporphyria and X-linked protoporphyria, collectively referred to as the protoporphyrias, result in accumulation of protoporphyrin IX, causing severe cutaneous pain and, in a minority of patients, liver failure. Approximately half of patients with protoporphyria have microcytic hypochromic anemia, which may be related...

Background and Aims A limited number of drugs are used as standard or alternative therapies in autoimmune hepatitis (AIH). No specific-recommendations are available for patients failing to respond to these therapies. We analyzed the efficacy and safety of infliximab in patients with AIH. Approach and Results We performed a retrospective study of 4...

The porphyrias are a heterogeneous group of metabolic disorders that result from defects in heme synthesis. The metabolic defects are present in all cells, but symptoms are mainly cutaneous or related to neuropathy. The porphyrias are highly relevant to hepatologists since patients can present with symptoms and complications that require liver tran...

Background and aims Soluble liver antigen/liver pancreas antibodies (anti-SLA/LP) are specific markers for autoimmune hepatitis (AIH) that have been associated with a distinct clinical phenotype and a more aggressive form of AIH. We prospectively evaluated the frequency and clinical significance of anti-SLA/LP in Turkish patients with AIH. Materia...

Acute intermittent porphyria (AIP) is an inherited metabolic disorder associated with complications including kidney failure and hepatocellular carcinoma, probably caused by elevations in the porphyrin precursors porphobilinogen (PBG) and delta-aminolevulinic acid (ALA). This study explored differences in modern biomarkers for renal and hepatic dam...

Acute intermittent porphyria (AIP) is a rare hereditary metabolic disease characterized by acute attacks and accumulation of the porphyrin precursors 5‐aminolevulinic acid (ALA) and porphobilinogen (PBG). Patients with AIP have a high risk of primary liver cancer (PLC). We aimed to assess the association between porphyrin precursor excretion and th...

Synthesis of plasma proteins is an important function of the liver that has sparsely been investigated by modern techniques in patients with advanced chronic liver disease (CLD). Twenty-eight well-characterized patients with CLD under evaluation for liver transplantation were included. Albumin and fibrinogen synthesis rates were measured by the flo...

Current knowledge of pregnancy and perinatal outcomes in women with acute hepatic porphyria (AHP) is largely based on biochemical disease models, case reports and case series. We performed a nationwide, registered-based cohort study to investigate the association between maternal AHP and the risk of adverse pregnancy and perinatal outcomes. All wom...

We read with interest this review by Ramai et al. [...]

The acute hepatic porphyrias (AHP) are associated with long-term complications such as primary liver cancer, hypertension and chronic kidney disease. Data on other related comorbidities are scarce. In this register-based, matched cohort study, we assessed the risks of non-hepatic cancers, cardiovascular diseases, renal diseases, psychiatric disorde...

Background Data regarding outcome of Coronavirus disease 2019 (COVID-19) in vaccinated patients with autoimmune hepatitis (AIH) are lacking. We evaluated the outcome of COVID-19 in AIH patients who received at least one dose of Pfizer- BioNTech (BNT162b2), Moderna (mRNA-1273) or AstraZeneca (ChAdOx1-S) vaccine. Patients and methods We performed a...

Objectives Primary biliary cholangitis (PBC) is an autoimmune liver disease that may progress into liver cirrhosis. Ursodeoxycholic acid (UDCA) is known to prevent or delay the disease progression, but little is known about work incapacity in PBC patients. We aimed to compare clinical outcomes (transplantation-free survival; cirrhosis development)...

LINKED CONTENT This article is linked to Téllez et al papers. To view these articles, visit https://doi.org/10.1111/apt.16926

Background and aims: Available epidemiological data on hepatocellular carcinoma (HCC) originate mainly from centre-based or disease-specific cohorts and may not reflect the general population. This population-based register study presents the incidence, aetiologies, treatments, survival, and differences related to sex or socioeconomic status in pa...

Background: Current risk estimates for hepatocellular carcinoma (HCC) in individuals with cirrhosis vary between studies. The risk has mostly been evaluated for single etiologies separately. Objectives: We examined the risk of HCC in Swedish outpatients with a new diagnosis of cirrhosis, aiming to identify subgroups with a particularly high risk...

Background& aims: A few case reports of autoimmune hepatitis like liver injury have been reported after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination. We evaluated clinical features, treatment response and outcomes of liver injury following SARS-CoV-2 vaccination in a large case series. Approach& results: We collected...

Background: The acute hepatic porphyrias (AHP) are associated with a risk of primary liver cancer (PLC), but risk estimates are unclear, and what AHP characteristics that predict PLC risk are unknown. In this register-based, matched cohort study, we assessed the PLC risk in relation to biochemical and clinical porphyria severity, genotype, age, an...

Background We investigated associations between baseline use of immunosuppressive drugs and severity of Coronavirus Disease 2019 (COVID-19) in autoimmune hepatitis (AIH). Patients and methods Data of AIH patients with laboratory confirmed COVID-19 were retrospectively collected from 15 countries. The outcomes of AIH patients who were on immunosupp...

Background: Health literacy is increasingly recognized as an essential determinant for the health of the population. Liver patients report perceived stigma to be a considerable problem. Little is however known about liver health literacy in the general population and to what extent liver disease is considered stigmatizing in comparison with other...

Background: Data regarding outcome of Coronavirus disease 2019 (COVID-19) in patients with autoimmune hepatitis (AIH) are lacking. Patients and methods: We performed a retrospective study on AIH patients with COVID-19 from 34 centres in Europe and the Americas. We analyzed factors associated with severe COVID-19 outcomes defined as the need for...

Recurrent attacks of acute intermittent porphyria (AIP) result in poor quality of life and significant risks of morbidity and mortality. Liver transplantation (LT) offers cure but published data on outcome after LT are limited. We aimed to assess the pre‐transplant characteristics, complications and outcomes for patients transplanted for AIP. Data...

Abstract Objectives Autoimmune hepatitis (AIH) is a life-long liver disease for most patients. Telehealth may be an alternative way to follow these patients remotely. We aimed to evaluate the feasibility of telehealth in the management of patients with AIH. The COVID-19 outbreak during the study period provided an opportunity to evaluate any pandem...

Background The prevalence and clinical significance of extrahepatic autoimmune diseases (EHAID) have not been evaluated in a large cohort of primary biliary cholangitis (PBC). Methods The medical records of 1554 patients with PBC from 20 international centres were retrospectively reviewed. Development of decompensated cirrhosis (ascites, variceal...

Background/aims: Autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) are the 3 main autoimmune liver diseases (AILDs). The epidemiology of AILD in Turkey is not known. To determine the scientific status, we performed a scientometric analysis of AILD-related original articles that originated from...

Objective There is no consensus on how to estimate energy requirements after liver transplantation (LT). The aim of this study was to compare measured resting energy expenditure (mREE) with predictive equations and fixed factors and to evaluate whether clinical variables were associated with REE. Methods During the period 2011–2018, REE measured w...

Available epidemiological data on primary biliary cholangitis (PBC) in Sweden originate from regional studies in the 1980s and may not reflect modern day PBC. We aimed to estimate incidence and prevalence, survival and death causes, and gender differences in PBC. We used international classification of disease (ICD) codes to identify patients with...

Background and aims: Patients with chronic liver disease often experience symptoms that affect their ability to eat. These symptoms can contribute to weight loss and malnutrition. We aimed to examine the prevalence of nutrition impact symptoms (NIS) in patients with chronic liver disease, and to investigate the relationships between NIS, malnutrit...

Aim: Doxorubicin-eluting beads transarterial chemoembolization (DEB-TACE) is reported to improve survival and tolerability when compared with conventional lipiodol-TACE (cTACE) for the treatment of hepatocellular carcinoma (HCC). The aim of this study was to evaluate tolerability and long-term survival in patients treated with cTACE or DEB-TACE in...

Introduction: Risk stratification based on biochemical variables is a useful tool for monitoring ursodeoxycholic acid (UDCA)-treated patients with primary biliary cholangitis (PBC). Several UDCA response criteria and scoring systems have been proposed for risk prediction in PBC, but these have not been validated in large external cohorts. Methods...

Background and Aims Nonalcoholic fatty liver disease (NAFLD) is a growing cause of hepatocellular carcinoma (HCC). In NAFLD, HCC occurs more commonly in the absence of cirrhosis compared with other liver diseases; yet, patients with non‐cirrhotic NAFLD‐HCC are poorly characterized. Here, we characterized a large cohort of HCC cases and assessed the...

Objectives: The aim of this study was to perform intermethod comparisons between the following three measures of muscle mass depletion in patients eligible for liver transplantation: 1) fat-free mass index (FFMI) measured by dual-energy x-ray absorptiometry (DXA), 2) appendicular skeletal muscle mass index (ASMI) measured by DXA, and 3) skeletal m...

Porphyrias are inherited diseases with low penetrance affecting the heme biosynthesis pathway. Acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP) together constitute the acute hepatic porphyrias (AHP). These diseases have been identified as risk factors for primary liver cancers (PLC), mainly hepatocell...

Background We studied the efficacy and safety of mycophenolate mofetil (MMF) and tacrolimus as second-line therapy in pediatric patients with autoimmune hepatitis (AIH) who were intolerant or non-responders to standard therapy (corticosteroid and azathioprine). Patients and Methods We performed a retrospective study of data from 13 centers in Euro...

Pruritus is a common complication of cholestatic liver diseases. Inhibition of the ileal bile acid transporter (IBAT/ASBT) may emerge as treatment option. Our aim was to assess tolerability and effect on pruritus of the selective IBAT inhibitor A4250 in patients with primary biliary cholangitis (PBC). Ten patients with PBC and bile acid sequestrant...

Posthepatectomy liver failure (PHLF) represents the single most important cause of postoperative mortality after major liver resection, yet no effective treatment option is available. Extracorporeal liver support devices might be helpful, but systematic studies are lacking. Accordingly, we aimed to assess the safety and feasibility of the Molecular...

Background: Acute intermittent porphyria (AIP) is an inherited disorder of heme metabolism characterized by life-threatening acute neurovisceral attacks due to the induction of hepatic δ-aminolevulinic acid synthase 1 (ALAS1) associated with hydroxymethylbilane synthase (HMBS) deficiency. So far, the treatment of choice is hemin which represses AL...

Background: Beyond available guidelines, therapy of autoimmune hepatitis (AIH) shows wide variation among physicians. We compared two regimens for treatment naive AIH: one recommended protocol with an initial prednisolone dose of 30 mg/day and our own 40 mg/day prednisolone with a slow dose tapering protocol. We analyzed the safety, response rates...

The severity of liver disease is assessed by scoring systems, which include the conventional coagulation test prothrombin time-the international normalized ratio (PT-INR). However, PT-INR is not predictive of bleeding in liver disease and thromboelastometry (ROTEM) has been suggested to give a better overview of the coagulation system in these pati...

Background Predniso(lo)ne, alone or in combination with azathioprine, is the standard of care (SOC) therapy for autoimmune hepatitis (AIH). However, the SOC therapy is poorly tolerated or does not control disease activity in up to 20% of patients. We assessed the efficacy of mycophenolate mofetil (MMF) and tacrolimus as second-line therapy for pati...

Background/aim: Antimitochondrial antibody (AMA) positivity is the serological marker of primary biliary cholangitis (PBC), but can also be sporadically detected in autoimmune hepatitis (AIH). Little is known about the clinical significance of AMA in AIH. Patients and methods: We recruited 47 AMA-positive AIH cases from several centres and compa...

Background: It is challenging to identify malnutrition, which is a risk factor for poor outcome in patients with liver cirrhosis. In the present study, we aimed to investigate the prevalence of malnutrition among patients listed for liver transplantation, as assessed by different methods, and also to relate dual-energy X-ray absorptiometry (DXA) t...

Background Higher than normal plasma glutamine concentration at admission to an intensive care unit is associated with an unfavorable outcome. Very high plasma glutamine levels are sometimes seen in both acute and chronic liver failure. We aimed to systematically explore the relation between different types of liver failure and plasma glutamine con...

There are no validated noninvasive markers of liver fibrosis in autoimmune hepatitis (AIH). An activated renin-angiotensin system (RAS) and its key element angiotensin-converting enzyme (ACE) have been implicated in the pathogenesis of hepatic fibrogenesis. We aimed to study the assumed role of activated RAS in the fibrogenic process and whether th...

Aim: To study the impact of molecular adsorbent recirculating system (MARS) treatment on the outcome of patients with post-hepatectomy liver failure (PHLF). Patients and methods: Records of all patients treated with MARS due to PHLF between January 2003 and December 2013 at the intensive care units of Karolinska University Hospital (KH), Stockholm,...

Background: Despite advances in the understanding of the pathophysiological basis of autoimmune hepatitis (AIH), it is still difficult to delineate the mechanisms involved in progression from hepatic inflammation toward fibrosis. Our aim was to study serum concentrations of NO in AIH of different histological severity and possible effects of immun...

Background Acute intermittent porphyria (AIP) is an autosomal dominant disorder characterized byinsufficient porphobilinogen deaminase (PBGD) activity. When hepatic heme synthesis isinduced, porphobilinogen (PBG) and 5-aminolevulinic acid (ALA) accumulate, which causesclinical symptoms such as abdominal pain, neuropathy and psychiatric disturbances...

Background & aims: Primary biliary cirrhosis (PBC) may present in all decades of life, also in childbearing age. Data on maternal and fetal outcome is limited. We aimed to investigate the impact of pregnancy and childbirth on the disease course and possible effects of PBC on fetal outcome. Methods: Retrospective study of local cases and a compac...

Primary biliary cirrhosis (PBC)-autoimmune hepatitis (AIH) overlap syndrome is used to describe the coexistence of both diseases, with either a sequential or a simultaneous presentation in the same patient. Available studies have focused on the simultaneous form, whereas there is limited information on sequential PBC-AIH. We carried out a retrospec...

Chapter summary Liver transplantation is an established treatment option in both AIP and EPP. Severe AIP is a good indication for liver transplantation, but definite indications have not been established. Transplantation leads to a phenotypic cure, thereby improving both morbidity and mortality. It has shown excellent results if done before neuropa...

For patients with primary biliary cirrhosis (PBC) with features of autoimmune hepatitis (AIH), treatment with ursodeoxycholic acid (UDCA), alone or in combination with immunosuppression, is controversial. Little is known about the factors associated with initial response to therapy or outcome. We performed a retrospective analysis of treatment stra...

Painful photosensitivity is characteristic of erythropoietic protoporphyria (EPP). In women, symptoms may be affected by menstrual cycle and pregnancy but very little is known about maternal and fetal outcome. To investigate the impact of menstruation, pregnancy and breast-feeding on photosensitivity and possible effects of EPP on maternal, fetal a...

Background De-novo or reactivated autoimmune hepatitis (AIH) has been reported during or a short time after administration of interferon (IFN) in patients treated for hepatitis C (HCV). Reports on AIH during long-term follow-up after IFN treatment are scarce.Patients and methodsPatients diagnosed with both HCV and AIH were identified in clinical da...

Background De-novo or reactivated autoimmune hepatitis (AIH) has been reported during or a short time after administration of interferon (IFN) in patients treated for hepatitis C (HCV). Reports on AIH during long-term follow-up after IFN treatment are scarce. Patients and methods Patients diagnosed with both HCV and AIH were identified in clinical...

Background De-novo or reactivated autoimmune hepatitis (AIH) has been reported during or a short time after administration of interferon (IFN) in patients treated for hepatitis C (HCV). Reports on AIH during long-term follow-up after IFN treatment are scarce. Patients and methods Patients diagnosed with both HCV and AIH were identified in clinical...

Background De-novo or reactivated autoimmune hepatitis (AIH) has been reported during or a short time after administration of interferon (IFN) in patients treated for hepatitis C (HCV). Reports on AIH during long-term follow-up after IFN treatment are scarce. Patients and methods Patients diagnosed with both HCV and AIH were identified in clinical...

Background: The diagnosis of autoimmune hepatitis (AIH) is already difficult, and that of AIH with chronic viral hepatitis including hepatitis B (HBV) or hepatitis C (HCV) is even more challenging. To date, only a few case-based studies have described this association. Aim: The aim was to retrospectively assess diagnostic difficulties, therapeut...

Antibodies to soluble liver antigen (anti-SLA) are specific serological markers of autoimmune hepatitis (AIH). The clinical significance and frequency of anti-SLA have never been reported among AIH patients from Italy and Turkey. To retrospectively assess the estimated prevalence, sensitivity, specificity and clinical significance of anti-SLA in AI...

Background/aims: Previous studies have indicated a high risk of hepatocellular carcinoma in acute hepatic porphyrias. In this retrospective study we present the incidence of primary liver cancer and clinical characteristics in a cohort of 179 acute porphyria patients above the age of 50 years. Methods: Twenty-three cases with primary liver cance...

Objective Development of ascites in patients with liver cirrhosis is an ominous sign with a poor outcome. A liver transplantation must be considered, and it then becomes important to know if there are any factors indicating a worsened prognosis. Material and methods We used official registers for a follow-up study of at least 5 years considering t...

To assess the prevalence of concurrent extrahepatic autoimmune diseases in patients with autoimmune hepatitis (AIH)/primary biliary cirrhosis (PBC) overlap syndrome and applicability of the 'mosaic of autoimmunity' in these patients. The medical data of 71 AIH/PBC overlap patients were evaluated for associated autoimmune diseases. In the study popu...

The inherited disease protoporphyria is characterized by acute dermal phototoxicity, due to accumulation of the porphyrin metabolite protoporphyrin. Protoporphyria impairs life quality and working capabilities seriously, as only complete avoidance of sunlight exposure prevents protoporphyria-related dermal pain. Afamelanotide induces skin tanning a...

Liver transplantation is an established lifesaving treatment for patients with severe protoporphyric liver disease, but disease recurrence in the graft occurs for the majority of recipients. Severe burn injuries may occur when protective light filters are not used with surgical luminaires. Motor neuropathy with an unclear pathogenesis is a frequent...

To investigate the demographic, clinical, biochemical and genotypic features of patients with erythropoietic protoporphyria (EPP) in a Swedish cohort. Cross-sectional questionnaire, biochemical and genetic study. Sweden. Fifty-one Swedish individuals known in 2008 to have EPP confirmed by molecular diagnosis. There were no exclusion criteria; all p...

We aimed to investigate the characteristics of autoimmune liver disease (AILD) developed in patients with systemic lupus erythematosus (SLE), including autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC) and the AIH/PBC overlap syndrome. We also evaluated the accuracy of diagnostic criteria and scoring systems for AILD in SLE. A retrospecti...

The American Journal of Gastroenterology is published by Nature Publishing Group (NPG) on behalf of the American College of Gastroenterology (ACG). Ranked the #1 clinical journal covering gastroenterology and hepatology*, The American Journal of Gastroenterology (AJG) provides practical and professional support for clinicians dealing with the gastr...