Home » Diseases » In dogs » Copper Storage Hepatopathy, Wilson’s Disease (Copper Storage Disease of the Liver)

Copper Storage Hepatopathy, Wilson’s Disease (Copper Storage Disease of the Liver) in Dogs

Occurrence: very rare
Location of illness: Abdomen/pelvis

Author: Dr. med. vet. Gisa Löwe | Last modified: 06.11.2025

Copper storage hepatopathy, also known as Wilson’s disease in dogs, is a genetic disorder characterized by the abnormal accumulation of copper in the liver. This leads to liver damage and, if left untreated, can lead to serious health problems.

The most important facts at a glance

Copper storage hepatopathy, also known as Wilson’s disease in dogs, is a genetically determined disease that occurs primarily in certain breeds such as Bedlington Terriers, West Highland White Terriers, and Doberman Pinschers. These dogs have difficulty excreting copper from the liver, which leads to an accumulation. The excess amounts of copper cause oxidative stressStress refers to the state of an organism reacting to external stimuli or demands. In veterinary medicine, stress can have physical or psychological effects on animals, impacting their health and well-being. and cell damage in the liver, which can lead to Inflammatory conditions and fibrosisFibrosis is the formation of connective tissue in the body in response to injury or long-term inflammation, which can lead to scarring. In dogs and cats, fibrosis can occur in various organs such as the lungs, liver, or heart and impair their function.. Symptoms of the disease include loss of appetite, Vomiting, DiarrheaDiarrhea refers to the condition of frequent, watery bowel movements, which can be a sign of infections, digestive disorders, or other health problems., lethargy, and jaundice. In severe cases, neurological symptoms and coagulopathies can occur.

The diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients. is made through clinical examinations, laboratory analyses, and imaging procedures. A liver biopsyA biopsy is a medical procedure in which a small amount of tissue is removed from the body to be examined under a microscope for signs of disease. Biopsies can help diagnose cancer and other conditions. is often necessary to directly determine the copper content. Treatment includes a low-copper diet and medications that promote copper excretion. Antioxidants can help protect the liver. The prognosisThe prognosis is the prediction of the likely course and outcome of a disease based on the general state of health, the nature of the disease, and the response to treatment. It can provide information on how a disease is expected to develop. depends on the stage of the disease at diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients.; early detection improves the chances of a good outcome.

PreventionPrevention includes measures taken to prevent diseases or injuries. In veterinary medicine, this includes vaccinations, parasite control, and health monitoring. focuses on genetic testing in breeding to identify and exclude affected dogs. For dogs with a genetic predispositionA predisposition is an innate inclination or susceptibility of an organism to certain diseases or conditions. In dogs and cats, there may be a genetic predisposition to certain conditions such as hip dysplasia or certain heart diseases., a low-copper diet is crucial. Regular veterinary examinations are important to intervene early if signs of the disease appear. A comprehensive approach that considers both genetic and environmental factors is crucial to minimize the risk of the disease.

Causes

The disease is genetic and often occurs in certain dog breeds, including Bedlington Terriers, West Highland White Terriers, and Doberman Pinschers. These breeds have genetic predispositions that impair their ability to eliminate copper from the liver.

Normally, copper absorbed through food is stored in the liver and used or excreted from the body as needed. In dogs with copper storage hepatopathy, this mechanism is disrupted, causing copper to accumulate in the liver. This accumulation leads to oxidative stressStress refers to the state of an organism reacting to external stimuli or demands. In veterinary medicine, stress can have physical or psychological effects on animals, impacting their health and well-being. and cell damage, which can eventually lead to liver inflammation and fibrosisFibrosis is the formation of connective tissue in the body in response to injury or long-term inflammation, which can lead to scarring. In dogs and cats, fibrosis can occur in various organs such as the lungs, liver, or heart and impair their function..

The genetic basis of the disease varies depending on the breed. In Bedlington Terriers, a specific genetic trait has been identified that is responsible for the accumulation of copper. In other breeds, the genetic basis is not yet fully understood, but there is evidence that multipleMultiple means manifold or repeated and is used to describe conditions or events that occur in multiple instances or at different locations. In veterinary medicine, this may indicate multiple lesions, tumors, or other pathological changes. genes may be involved.

Environmental factors can also play a role in the severity of the disease. A copper-rich diet can worsen the disease, especially in genetically predisposed dogs. Therefore, managing copper intake is an important aspect of disease control.

Symptoms

Very common symptoms:

Common symptoms:

Rare symptoms:

The symptoms of copper storage hepatopathy can vary, depending on the stage of the disease and the severity of copper accumulation. The most common signs include loss of appetite, weight loss, lethargy, and Vomiting. These symptoms are non-specific and can easily be confused with other liver diseases.

In the advanced stages, dogs may develop jaundice, which is characterized by a yellowing of the skin and mucous membranes. This is a sign that the liver is severely compromised and can no longer effectively process bilirubinBilirubin is a yellow pigment that is produced when hemoglobin from red blood cells is broken down. It is further processed in the liver and then excreted via the bile. Elevated bilirubin levels may indicate liver dysfunction or impaired bile excretion., a waste product of hemoglobinhemoglobin is an iron-containing protein in red blood cells responsible for transporting oxygen from the lungs to the body tissues and carbon dioxide back to the lungs. breakdown.

Neurological symptoms can also occur if liver function is severely impaired. These can include Behavioral changes, coordination problems, and, in severe cases, seizures. These symptoms are often indicative of advanced liver disease and require immediate veterinary attention.

Diagnosis

The diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients. of copper storage hepatopathy requires a combination of clinical examinations, laboratory tests, and imaging procedures. A thorough historyThe medical history is the systematic collection of a patient's medical history through questioning. It includes information about previous illnesses, treatments, allergies, and lifestyle habits. and physical examination are the first step in making a presumptive diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients.. The veterinarian will ask about the dog’s specific symptoms and breed.

Blood tests are crucial to assess liver function. Elevated liver enzymesEnzymes are biological catalysts that accelerate specific chemical reactions in the body without being consumed. They play a crucial role in virtually all physiological processes., bilirubinBilirubin is a yellow pigment that is produced when hemoglobin from red blood cells is broken down. It is further processed in the liver and then excreted via the bile. Elevated bilirubin levels may indicate liver dysfunction or impaired bile excretion., and specific copper levels can provide clues to the disease. However, definitive evidence of copper accumulation can only be obtained through a liver biopsyA biopsy is a medical procedure in which a small amount of tissue is removed from the body to be examined under a microscope for signs of disease. Biopsies can help diagnose cancer and other conditions.. In this invasiveInvasive describes medical procedures or pathogens that enter the body or penetrate tissue. In medicine, invasive procedures can range from minimally invasive techniques to open surgery. method, a small sample of liver tissue is taken and examined for copper content.

Imaging techniques such as ultrasoundUltrasound refers to sound waves that are above the hearing threshold of the human ear. In medicine, ultrasound is used for diagnostic imaging procedures to visualize internal organs, tissues, and blood flow. or CT scans can be helpful in assessing the condition of the liver and ruling out other possible causes of the symptoms. These methods are non-invasive and provide valuable information about the structure of the liver.

Therapy

The treatment of copper storage hepatopathy aims to reduce copper accumulation in the liver and control symptoms. A special low-copper diet is often the first step. This diet helps to prevent further accumulation of copper and slow the progressionProgression refers to the advancement or worsening of a disease over time. It describes an increase in the severity or extent of the disease symptoms. of the disease.

Medications that promote the excretion of copper are also an essential part of the therapyTherapy refers to the treatment of diseases or disorders with the aim of alleviating symptoms, promoting healing, or improving quality of life. Therapies can be medicinal, surgical, or through other medical interventions.. Penicillamine and trientine are chelating agents that help remove copper from the body. However, these medications can have side effects and must be dosed under veterinary supervision.

Antioxidants such as vitaminVitamins are organic compounds that are necessary in small amounts for the normal growth, development, and physiological function of the body. Vitamins must mostly be obtained through food, as the body cannot produce them or not in sufficient quantities. E and S-adenosylmethionine (SAMe) can also be considered to reduce oxidative stressStress refers to the state of an organism reacting to external stimuli or demands. In veterinary medicine, stress can have physical or psychological effects on animals, impacting their health and well-being. in the liver. These supplements can help protect liver cells from further damage.

In severe cases where liver function is severely impaired, supportiveSupportive refers to supportive treatments that aim to alleviate symptoms and improve quality of life without directly treating the underlying cause of the disease. therapyTherapy refers to the treatment of diseases or disorders with the aim of alleviating symptoms, promoting healing, or improving quality of life. Therapies can be medicinal, surgical, or through other medical interventions. may be necessary. This may include the administration of fluids and electrolytesElectrolytes are minerals in the body that carry an electric charge and perform vital functions such as regulating fluid balance, acid-base balance, and nerve and muscle activity. The main electrolytes include sodium, potassium, chloride, and magnesium., as well as the treatment of complications such as infections or neurological disorders.

Prognosis and follow-up care

The prognosis for dogs with copper storage hepatopathy depends on various factors, including the stage of the disease at the time of diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients., the breed, and the response to treatment. With early diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients. and adequate therapyTherapy refers to the treatment of diseases or disorders with the aim of alleviating symptoms, promoting healing, or improving quality of life. Therapies can be medicinal, surgical, or through other medical interventions., the prognosisThe prognosis is the prediction of the likely course and outcome of a disease based on the general state of health, the nature of the disease, and the response to treatment. It can provide information on how a disease is expected to develop. can be favorable, and many dogs can achieve a good quality of life.

However, dogs diagnosed in the advanced stages of the disease have a poorer prognosisThe prognosis is the prediction of the likely course and outcome of a disease based on the general state of health, the nature of the disease, and the response to treatment. It can provide information on how a disease is expected to develop.. The liver damage may be irreversible, and treatment often focuses on alleviating symptoms and improving quality of life.

Long-term follow-up and regular veterinary check-ups are crucial to monitor the progressionProgression refers to the advancement or worsening of a disease over time. It describes an increase in the severity or extent of the disease symptoms. of the disease and adjust treatment accordingly. With proper care, many dogs can lead a relatively normal life despite the diagnosisA diagnosis is the identification of a disease or condition by its symptoms and/or results from diagnostic tests. It is the first step in the management and treatment of patients..

Prevention

Because copper storage hepatopathy is genetic, the focus of preventionPrevention includes measures taken to prevent diseases or injuries. In veterinary medicine, this includes vaccinations, parasite control, and health monitoring. is on responsible breeding practices. Breeders should conduct genetic tests to identify affected dogs and exclude them from breeding. This can help reduce the spread of the disease in the populationIn veterinary medicine, the term population refers to a group of animals of a particular species living in a specific geographic area or environment..

For dogs that already have a genetic predispositionA predisposition is an innate inclination or susceptibility of an organism to certain diseases or conditions. In dogs and cats, there may be a genetic predisposition to certain conditions such as hip dysplasia or certain heart diseases., controlling copper intake is crucial. A low-copper diet can help prevent the accumulation of copper in the liver. Veterinarians can recommend special diets tailored to the dog’s needs.

Regular veterinary examinations are important to detect early signs of the disease. Blood tests and, if necessary, liver biopsies can help monitor liver function and intervene early if abnormalities are detected.

Overall, preventionPrevention includes measures taken to prevent diseases or injuries. In veterinary medicine, this includes vaccinations, parasite control, and health monitoring. requires a holistic approach that considers both genetic and environmental factors. By understanding the disease and working with a veterinarian, dog owners can help minimize the risk of developing copper storage hepatopathy.