Primary biliary cholangitis PBC

Primary biliary cholangitis PBC information

Primary biliary cholangitis, use to be called primary biliary Cirrhosis (PBC) also called Hanot Syndrome.

It is a chronic liver disease, where the body destroys the cells lining the bile ducts in the liver. They are called the intrahepatic bile ducts. If left untreated it can lead to scarring of the liver, which can cause liver failure.

Symptoms include abdominal pain, fatigue, itchiness and jaundice of the skin.  Jaundice occurs when PBC is severe as the liver is so damaged that it's normal function is impaired. Many people with PBC have no symptoms and it is discovered due to abnormal results on routine liver blood tests.
It has been estimated that PBC is one of the most common autoimmune diseases, affecting nearly 1 in 1000 women over the age of 40. NORD
Diagnosis of PBC requires the presence of:
  • High alkaline phosphatase (ALP, a liver blood test) along with
  • Positive anti-mitochondrial antibody (+AMA)
If AMA testing is negative, then a patient would need a liver biopsy because a number of diseases cause high ALP.
It is thought that PBC is most common in Northern Europe and North America, although it is found in people all over the world. PBC Foundation
PBC Foundation UK The PBC Foundation is the only UK organisation that specifically deals with PBC. They serve members in over 65 countries around the world and run support meetings and a helpline.
PBCer's The PBCers mission is to offer education and support to Primary Biliary Cholangitis patients, family members and friends, and to raise funds to help research the causes and cure for PBC. 
Primary biliary cholangitis PBC



Rheumatoid arthritis and Sjögren's syndrome linked to higher risk of carpal tunnel syndrome

Rheumatoid arthritis Sjögren's syndrome linked to  carpal tunnel syndrome

Newswise - People with two autoimmune diseases, rheumatoid arthritis (RA) and Sjögren’s syndrome, are at an increased risk for developing carpal tunnel syndrome (CTS), and should be screened for CTS and made aware of this risk, according to a new study presented this week at the Association of Academic Physiatrists Annual Meeting in Puerto Rico.

The relationship between CTS and autoimmune diseases is still unclear. Although RA is a known risk factor for developing CTS, physicians still do not know its exact role. CTS involves compression of the median nerve through the wrist at the carpal tunnel, and its symptoms include pain, tingling, and numbness in the fingers. To find out more about the relationship between CTS and common autoimmune diseases, researchers at the Taipei Veterans General Hospital Department of Physical Medicine and Rehabilitation conducted a cohort study focusing on autoimmune rheumatic disease (ARD) and inflammatory bowel disease (IBD) patients.
“Patients with autoimmune diseases may be considered with variable conditions, including the involvement of peripheral nerve system. Yet, there were only suspicions regarding ARD/IBD and CTS due to lack of large-scale cohort study to verify. This study provides the epidemiological evidence to support the correlation. Carpal tunnel syndrome is a disease that may affect health-related quality of life. Early diagnosis when managing patients with RA and Sjögren’s syndrome with adequate early health education and treatment could decrease the influence of CTS,” said Po-Cheng Hsu, MD, the study’s co-author.
The study’s findings suggest that people with Sjögren’s disease and RA have an increased risk of CTS compared to the general population, and screening for CTS in patients with these autoimmune diseases may be warranted.

“These findings provide information that clinical practitioners and patients may keep in mind: the classical symptoms of (numbness, tingling and paresthesia may be related to CTS,” said Dr. Hsu.


Increased prevalence of Sjogren's syndrome in high level chromium soils

Increased prevalence of Sjogren's syndrome in high level chromium soils

Three areas of Taiwan, Changhua, Taichung, and Nantou, were found to have the highest prevalence of Sjogren’s syndrome and the highest levels of chromium in the soil.

Chromium is a naturally occurring heavy metal often used in industrial processes. It is known to cause health problems in humans. The main cause of chromium in high levels in the soil is from industrial sources.
The following report is from ScienceDirect: Increased prevalence of Sjogren's syndrome in where soils contain high levels of chromium.


Farm soil levels of chromium was strongly correlated with people's serum chromium levels. •

Sjogren syndrome (SS) prevalence was 31 per 105 people, 5.59 for men and 55.01 for women. •

The highest SS prevalence (53 per 105) was located in an area where farm soils contain the highest amounts of chromium.

The SS prevalence is significantly increased in areas with high soil levels of chromium.


Previously, we showed that farm soil levels of chromium were strongly correlated with people's serum chromium levels and an increase (3.6 fold) in both the incidence and prevalence of sicca syndrome in areas where farm soil chromium was high.

Because Sjogren's syndrome (SS) is the major disease causing a dry mouth and dry eyes, we aimed in the study to investigate whether these areas with high soil chromium have a high SS prevalence.
We used a database from the authority in charge of catastrophic illness certificates.
Heavy metal concentrations in farm soils were retrieved from nationwide surveys.
We used spatial regression models to study the relationships between the SS prevalence and soil metal concentrations.

There were 11,220 people, 1165 men and 10,055 women who received a SS certificate from 2000 to 2011. The SS prevalence was 31 per 105 people, 5.59 for men and 55.01 for women.
The highest SS prevalence in Taiwan was located in an area where farm soils contain the highest amounts of chromium. In contrast, other types of heavy metal did not show such a strong association.
In conclusion, the SS prevalence is significantly increased in areas where soils contain high levels of chromium. Chromium is likely a risk for SS.



Personality, depression and anxiety in primary Sjogren's syndrome

Personality, depression and anxiety in primary Sjogren's syndrome
Personality, depression and anxiety in primary Sjogren's syndrome - Association with sociodemographic factors and comorbidity.

Mood, sleeping and several neuro-psychologic domains such as cognition are affected by pSS. Specifically, difficulties with attention, focusing, memory and new learning are commonly reported problems
Moreover, the personality traits of pSS patients can potentially interact with the subjective dryness symptoms and treatment outcome. Psychological factors may influence the ability of patients to cope with fatigue and its consequences including negative cognitions such as catastrophizing, avoidance of psychical activity and lack of social support or overprotection.


Patients with primary Sjögren's Syndrome (pSS) have diminished health quality and fatigue, arthralgia along with dryness of the mouth and eyes have major impact on their psychological and social aspects of life. 
The purpose of this study was to determine psychological features of patients with pSS. 
We analyzed personality, depression and anxiety of patients with primary Sjögren's Syndrome (pSS) in comparison with patients with rheumatoid arthritis (RA) and healthy controls (HC) and assessed their association with sociodemographic factors and comorbidity.


In 105 pSS patients (mean age 51.34 years, mean disease duration 5.98 years), 52 RA patients (mean age 51.37 years, mean disease duration 8.10 years) and 54 HC (mean age 51.35 years) clinical and sociodemographic characteristics were determined and results analyzed. 
At enrollment patients and controls completed the Revisited NEO Personality Inventory Five-Factor model (NEO-PI-R), the Zung Self-Rating Depression Scale and the Zung Self-Rating Anxiety Scale. Statistical analyses were performed using SPSS [Version 16.0]. The relative size of the effect was assessed based on standardized estimates of effect size (d).


Patients with pSS, similarly to RA patients had higher scores of Neuroticism and lower scores of Extraversion and Openness for experience  compared to HC. 
There was no significant differences between pSS group and HC in the depression. However, patients with pSS had higher anxiety in comparison to HC.

We confirmed that pSS patients have psychological profiles and levels of anxiety different to healthy subjects. 
In our study, patients with pSS were emotionally unstable, introverted, and more anxious than healthy controls. 
Education and satisfaction with family relationships were significant predictors for psychological characteristics of patients, independently of clinical diagnosis. 
The better understanding of personality dimensions in patients with pSS may provide adequate help by professionals in overcoming adaptation problems which have been observed in these patients.

See Full report on this study 


Diagnosis, treatment, and follow-up of autoimmune hepatitis

Autoimmune Hepatitis Clinical Practice Guidelines (2019)

Diagnosis, treatment, and follow-up of autoimmune hepatitis

The guidelines on the diagnosis, treatment, and follow-up of autoimmune hepatitis were released on January 1, 2019, by the Hellenic Association for the Study of the Liver.

Clinical Characteristics

Autoimmune hepatitis (AIH) should be considered a possibility in any individual with acute or chronic hepatitis, particularly in those with high immunoglobulin G (IgG) levels.
Untreated AIH carries high morbidity and mortality rates, so early and accurate diagnosis is mandatory.
Screening for concomitant autoimmune diseases can be considered in patients with AIH (particularly autoimmune thyroiditis), as AIH is associated with various other autoimmune-mediated conditions.
Cirrhosis should be suspected upon AIH diagnosis, since nearly one-third of adults and half of children with AIH are at the stage of cirrhosis at AIH diagnosis.
The presentation of acute AIH can take one of two clinical forms, as follows:
  • Acute worsening of previously undiagnosed or misdiagnosed AIH
  • Original acute-onset AIH without chronic lesions on liver histology
AIH can be classified as either (1) AIH-1, antinuclear (ANA), smooth muscle (SMA), and/or soluble liver antigens/liver pancreas antibody (anti-SLA/LP)–positive or (2) AIH-2, anti-liver/kidney microsomal antibody type-1 (anti-LKM1), anti-liver/kidney microsomal antibody type-3 (anti-LKM3), and/or liver cytosol type-1 antigen (anti-LC1)–positive.


Patients with AIH-related cirrhosis should undergo ultrasonography every 6 months to monitor for hepatocellular carcinoma (HCC).


All children with AIH should undergo magnetic resonance cholangiopancreatography (MRCP) at minimum to rule out autoimmune sclerosing cholangitis.
Patients who have AIH with cholestatic features should undergo testing for primary biliary cholangitis (PBC).
Interface hepatitis, hepatocyte rosetting, and emperipolesis strongly support but do not confirm AIH.
In daily clinical practice, the 2008 simplified score should be used for AIH diagnosis.
The 1999 revised score can help diagnose difficult AIH cases, since it incorporates treatment response as an important parameter.
Diagnosis of AIH-PBC and AIH–primary sclerosing cholangitis (PSC) variants should not be based on diagnostic scores, but they may be used cautiously in the diagnosis of childhood AIH and acute or fulminant disease.


The goal of AIH therapy is to achieve complete biochemical and histological remission to prevent disease progression.



Just 20 minutes of exercise to reduce inflammation

Just 20 minutes of exercise to reduce inflammation
As little as 20 minutes of exercise could have anti-inflammatory effects, according to this study.

Research, published in the journal Brain, Behavior and Immunity, investigates the benefits of 20-minute exercise sessions on the body's immune system.

Researchers from the University of California-San Diego School of Medicine - led by Suzi Hong, Ph.D., from the Department of Psychiatry and the Department of Family Medicine and Public Health - hypothesized that exercise would improve the body's anti-inflammatory response by activating the sympathetic nervous system.
The sympathetic nervous system helps to increase heart rate, blood pressure, and breathing rate. Physical exercise activates this system to help the body keep up.
During this time, the body releases hormones such as epinephrine and norepinephrine into the bloodstream, which activate the adrenergic receptors of immune cells.

Analyzing the body's immune response to exercise

More specifically, the researchers tested the hypothesis that a single 20-minute session of exercise would be enough to trigger sympathoadrenergic activation, which, in turn, would suppress the production of monocytic cytokines.
Monocytes are a type of white blood cell, or immune cell, that help to fight off bacteria and infections. Cytokines are a type of protein that help other cells to become so-called effector cells, which, in turn, kill off cancerous or infected cells.
TNF is one of these cytokines. TNF can induce cell differentiation and proliferation, but also cell death, including cancerous ones. TNF also has pro-inflammatory properties, which help the body to bring its inflammatory cells to the site of the injury, creating an immunological response.
Inflammation is a necessary part of the body's immune response, but too much inflammation can lead to disease. Chronic inflammation may contribute to diabetesobesityceliac diseasearthritisfibromyalgia, or bowel diseases such as Crohn's disease or ulcerative colitis.
To test their hypothesis, the researchers asked 47 participants to walk on a treadmill for 20 minutes at an intensity rate adjusted to suit each individual's fitness level. Hong and team took blood samples from the participants both before and immediately after the exercise sessions.

As little as 20 minutes of exercise reduces inflammation

The results revealed that a 20-minute session of moderate exercise can have anti-inflammatory effects.
The study confirmed the researchers' hypothesis. Exercise did seem to produce an anti-inflammatory cellular response, which could be seen in the reduction of the cytokine TNF.
"Our study shows a workout session does not actually have to be intense to have anti-inflammatory effects. Twenty minutes to half an hour of moderate exercise, including fast walking, appears to be sufficient. Feeling like a workout needs to be at a peak exertion level for a long duration can intimidate those who suffer from chronic inflammatory diseases and could greatly benefit from physical activity." - Suzi Hong


What is the lymph system?

What is the lymph system?

Our bodies have a network of lymph vessels and lymph nodes. (Lymph is pronounced limf.) This network is a part of the body’s immune system. It collects fluid, waste material, and other things (like viruses and bacteria) that are in the body tissues, outside the bloodstream.

Lymph vessels are a lot like the veins that collect and carry blood through the body. But instead of carrying blood, these vessels carry the clear watery fluid called lymph.
What is the lymph system?
Lymph fluid flows out from capillary walls to bathe the body’s tissue cells. It carries oxygen and other nutrients to the cells, and carries away waste products like carbon dioxide (CO2) that flow out of the cells. Lymph fluid also contains white blood cells, which help fight infections.
Lymph fluid would build up and cause swelling if it were not drained in some way. That’s the role of the lymph vessels. Lymph vessels draw up the lymph fluid from around the cells to send it towards the chest. There, lymph fluid collects into a large vessel that drains into a blood vessel near the heart.

Lymph nodes and what they do

Lymph vessels route lymph fluid through nodes throughout the body. Lymph nodes are small structures that work as filters for harmful substances. They contain immune cells that can help fight infection by attacking and destroying germs that are carried in through the lymph fluid.
There are hundreds of lymph nodes throughout the body. Each lymph node filters the fluid and substances picked up by the vessels that lead to it. Lymph fluid from the fingers, for instance, works its way toward the chest, joining fluid from the arm. This fluid may filter through lymph nodes at the elbow, or those under the arm. Fluid from the head, scalp, and face flows down through lymph nodes in the neck. Some lymph nodes are deep inside the body, such as between the lungs or around the bowel, to filter fluid in those areas. The lymph fluid slowly flows in from all around the body, making its way back to the chest. At the end of its journey, the filtered fluid, salts, and proteins are dumped back into the bloodstream.

Swollen lymph nodes

When there’s a problem, such as infection, injury, or cancer, the node or the group of lymph nodes in that area may swell or enlarge as they work to filter out the “bad” cells. This may be called lymphadenopathy (LIMF-ad-uh-NOP-uh-thee). Swollen lymph nodes tell you that something is not right, but other symptoms help pinpoint the problem. For instance, ear pain, fever, and enlarged lymph nodes near your ear are clues that you may have an ear infection or cold.
Some areas where lymph nodes commonly swell are in the neck, groin, and underarms. In most cases, only one area of nodes swells at a time. When more than one area of lymph nodes is swollen it’s called generalized lymphadenopathy. Some infections (such as strep throat and chicken pox), certain medicines, immune system diseases, and cancers like lymphoma and leukemia can cause this kind of swelling. The health care provider will look for more information to figure out the cause of the swelling. Lymph node swelling is often caused by something other than cancer.


Innovative approaches in the treatment of multiple sclerosis

Innovative approaches in the treatment of multiple sclerosis
By Will Frostick, Senior Associate Consultant Will Frostick delves into the MS treatment landscape and explores some of the cutting-edge treatments on the horizon.

Innovative approaches in the treatment of multiple sclerosis. Multiple Sclerosis (MS) is a chronic autoimmune disease that affects the central nervous system (CNS), leading to progressive and permanent disability.

It is estimated that 2,500,000 people across the world suffer from MS. Click to Tweet

The disease impacts everyone differently, with the symptoms ranging from fatigue and vision problems to mental health issues and mobility problems, along with issues with speech and swallowing, and loss of bladder function (to name a few). Sadly, the age of onset is typically 20-40 years and MS is the leading cause of nontraumatic neurologic disability in young people.

The pathogenesis of MS consists of two key components: focal inflammatory demyelination, and neurodegeneration. Nerve function is dependent on the ability of electrical signals to flow through the wire-like axons of neurons, which are wrapped in and insulated by myelin sheaths. In the central nervous system (CNS, which includes the brain, optic nerves and spinal cord), myelin extends from octopus-like arms of oligodendrocytes. This myelination supports neuronal signal conductance and the survival of the neurons themselves.

However, in MS, lymphocytes mount an auto-immune attack on myelin, leading to damage of the myelin sheath. The demyelinated neurons can then no longer function properly and begin to atrophy and degenerate, causing the symptoms of MS.2 As damage can occur anywhere in the CNS, symptoms are highly varied.

There are two main clinical presentations of MS – Relapsing Refractory MS (RRMS) and Progressive MS (PMS).

In RRMS symptoms are intermittent but relapses gradually become more frequent, severe and longer-lasting as the disease progresses. However, PMS is characterised by a progressive worsening of symptoms between flareups, without remission. Pathophysiologically, the disease courses of both types overlap. As a result, patients can transition between the types of MS, making monitoring and treating the disease more challenging.

For example, PMS is characterised by focal inflammatory lesions (an area of damage or scaring in the CNS, also referred to as plaques) in the early stages only, with more diffuse damage becoming common later on. Focal inflammatory lesions are also a feature throughout RRMS, and it is also possible for RRMS patients to show progressive symptoms in the later stages of the disease.



Is sonoelastography a helpful method of evaluation to diagnose Sjögren's syndrome?

Is sonoelastography a helpful method of evaluation to diagnose Sjögren's syndrome?

Sonoelastography is a diagnostic ultrasound technique that provides a noninvasive means of estimating soft tissue elasticity and stiffness. Within the last decade, sonoelastography has gained an increasing amount of attention because it is a noninvasive way of estimating tissue stiffness.

The study, “Is sonoelastography a helpful method of evaluation to diagnose Sjögren’s syndrome?,” was published in the International Journal of Rheumatic Diseases.

At this point in time Sjogren's Syndrome is diagnosed by looking at the eye and mouth symptoms and damage, by evaluating levels of autoantibodies in the blood, and tissue biopsy analysis. A less-invasive, quicker and low-cost procedure that accurately identifies patients affected with Sjögren’s syndrome is needed.

A new sonoelastography technique – virtual touch tissue quantification of acoustic radiation force impulses (ARFI) – offers a promising method for measuring tissue rigidity. 

The aim of this study was to assess the usefulness of ARFI for diagnosing Sjögren's syndrome (SS).


Acoustic radiation force impulses sonoelastography can assist diagnosis of SS, and is a non‐invasive and fast method of detecting pathological changes to the parotid and submandibular glands.
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