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Shar-Pei Autoinflammatory Disease, SPAID (Familial Shar-Pei Fever) in Dogs

Occurrence: very rare
Location of illness: General/Whole body

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

Shar-Pei Autoinflammatory Disease (SPAID), also known as Familial Shar-Pei Fever, is a genetically determined disease that occurs in Shar-Pei dogs. It is characterized by recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. fever spikes and inflammatory reactions that can affect various organ systems.

The most important facts at a glance

Shar-Pei Autoinflammatory Disease (SPAID) is a genetically determined disease that occurs in Shar-Pei dogs and is characterized by a dysregulation of the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body.. This leads to spontaneous inflammatory reactions without recognizable infectionAn infection is the colonization and multiplication of pathogens such as bacteria, viruses, fungi, or parasites in an organism, which often, but not always, leads to a disease.. A mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. in the Hyaluronan Synthase 2 gene (HAS2) is associated with SPAID and causes excessive hyaluronic acid production, which contributes to both the characteristic wrinkled skin and inflammation. The disease is hereditary and shows an autosomal dominant or incompletely dominant inheritance pattern. Symptoms include fever, anorexiaAnorexia describes the loss of appetite or the lack of desire to eat. Although often associated with anorexia nervosa, an eating disorder, anorexia can also be caused by other medical and psychological factors., joint swelling, and breathing problems. 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 by clinical assessment, genetic testing, and exclusion of other diseases. Treatments focus on symptom control with anti-inflammatory drugsAnti-inflammatory drugs are medications that reduce inflammation. They can be divided into nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids and are used in dogs and cats to reduce Inflammatory conditions and Pain. and immunomodulatoryImmunomodulatory describes the ability of a substance to modify the reaction of the immune system. Immunomodulatory drugs can either stimulate the immune system to fight infections more effectively or suppress it to prevent overreactions. therapies. Kidney involvement requires special care. PreventionPrevention includes measures taken to prevent diseases or injuries. In veterinary medicine, this includes vaccinations, parasite control, and health monitoring. through genetic testing in breeding can help minimize spread. Research aims to understand the molecular mechanisms and develop better diagnostic and therapeutic approaches. Environmental factors and nutrition are also being investigated to clarify their role in disease management. The long-term 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. varies, depending on the severity of the symptoms and the response to treatment.

Causes

The scientific basis of SPAID lies in a dysregulation of the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body., which leads to an excessive inflammatory reaction in the body. This disease belongs to the group of autoinflammatory diseases, which are characterized by spontaneous inflammatory reactions without recognizable infectionAn infection is the colonization and multiplication of pathogens such as bacteria, viruses, fungi, or parasites in an organism, which often, but not always, leads to a disease. or autoimmunity.

Genetic studies have shown that SPAID is associated with a mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. in the Hyaluronan Synthase 2 gene (HAS2). This mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. leads to an overproduction of hyaluronic acid, which causes characteristic wrinkled skin in Shar-Pei dogs and can also contribute to the development of inflammatory reactions.

The exact mechanism of how this genetic mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. leads to the clinical symptoms of SPAID is not yet fully understood. However, research suggests that excessive hyaluronic acid production can put the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body. into a state of chronicChronic refers to a condition or disease that is long-lasting or permanent. Chronic diseases often develop slowly and can lead to persistent or recurring health problems over time. activation, which explains the recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. fever spikes and inflammatory symptoms.

The genetic basis of SPAID means that the disease has a hereditary component. It is known that affected dogs can inherit the mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. from one or both parents, suggesting an autosomal dominant or incompletely dominant inheritance pattern. This 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. makes it difficult to eliminate the disease through simple breeding practices.

Symptoms

Very common symptoms:

Common symptoms:

Rare symptoms:

Dehydration

The symptoms of SPAID are diverse and can vary in severity. A characteristic feature of the disease are recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. fever spikes, which often occur for no apparent reason and can last for several hours to days. These fever spikes are usually associated with restlessness, reduced appetite, and general malaise.

In addition to fever, affected dogs may have swelling in the area of the hock or muzzle, which is known as “muzzle edemaAn edema is a buildup of fluid in the body’s tissues, leading to Swelling. Edema can be localized or generalized and is often a sign of an underlying condition.”. These swellings are painful and can impair the dog’s mobility. In severe cases, lameness can occur.

ChronicChronic refers to a condition or disease that is long-lasting or permanent. Chronic diseases often develop slowly and can lead to persistent or recurring health problems over time. inflammatory conditions can also affect other organ systems. These include kidney damage, which can lead to chronicChronic refers to a condition or disease that is long-lasting or permanent. Chronic diseases often develop slowly and can lead to persistent or recurring health problems over time. kidney disease. This kidney damage is often a result of the persistentPersistent means lasting or continuing for an extended period. In veterinary medicine, it can refer to chronic diseases or symptoms that do not subside. inflammatory reaction and can lead to kidney failure over time.

Other possible symptoms include skin changes, recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. gastrointestinalGastrointestinal refers to the gastrointestinal tract, a system that extends from the oral cavity to the anus and is responsible for the digestion of food and the absorption of nutrients. problems such as vomiting and diarrheaDiarrhea refers to the condition of frequent, watery bowel movements, which can be a sign of infections, digestive disorders, or other health problems., and in some cases respiratoryRespiratory refers to breathing or the process of breathing. Respiratory diseases affect the respiratory tract and can impair the ability to breathe effectively. distress. The range and combination of symptoms can vary from dog to dog, which can make 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. difficult.

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 SPAID is based on a combination of clinical symptoms, genetic tests, and the exclusion of other diseases. An important step in 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 the careful recording of the dog’s medical 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. to determine whether recurrent fever spikes and other typical symptoms are present.

Genetic tests can help identify the mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. in the HAS2 gene that is associated with SPAID. These tests are particularly useful for confirming a hereditary component and securing 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.. However, it is important to note that not all dogs with the genetic mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. develop clinical symptoms, which can complicate the interpretation of test results.

Additional diagnostic procedures may include blood tests to measure inflammatory markers and monitor kidney function. In some cases, imaging procedures 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 X-rays may be necessary to assess organ damage and rule out other possible causes.

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 SPAID often requires interdisciplinary collaboration between veterinarians, geneticists and possibly immunologists to enable a comprehensive assessment and plan the best possible treatment.

Therapy

The treatment of SPAID primarily focuses on controlling the symptoms and reducing the inflammatory reactions. Since it is a genetic disease, there is currently no cure, but early and appropriate therapeutic intervention can help improve the quality of life of the affected dog.

The use of anti-inflammatory drugsAnti-inflammatory drugs are medications that reduce inflammation. They can be divided into nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids and are used in dogs and cats to reduce Inflammatory conditions and Pain. such as non-steroidalNon-steroidal refers to medications or substances that are not steroids and often have anti-inflammatory, analgesic, or antipyretic effects. They are used in dogs and cats to treat Pain and Inflammatory conditions. anti-inflammatory drugsAnti-inflammatory drugs are medications that reduce inflammation. They can be divided into nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids and are used in dogs and cats to reduce Inflammatory conditions and Pain. (NSAIDsNSAID (non-steroidal anti-inflammatory drug) stands for non-steroidal anti-inflammatory drugs, a class of medications used to relieve pain, reduce inflammatory conditions, and lower fever without using steroidal agents. They are a commonly used pain reliever in veterinary medicine.) or corticosteroidsCorticosteroids are a class of steroid hormones that occur naturally in the body and can also be produced synthetically. They have anti-inflammatory and immunosuppressive properties and are used in veterinary medicine to treat a variety of conditions. can help control acuteAcute refers to a condition that occurs suddenly and is usually of short duration. In a medical context, it describes symptoms or illnesses that can quickly become serious and require immediate attention. inflammation and fever spikes. In some cases, immunomodulatoryImmunomodulatory describes the ability of a substance to modify the reaction of the immune system. Immunomodulatory drugs can either stimulate the immune system to fight infections more effectively or suppress it to prevent overreactions. drugs may be needed to regulate the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body. and reduce the frequency of flare-ups.

For dogs with kidney involvement, careful monitoring and treatment of kidney function is crucial. This may include dietary changes, fluid 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., and specific medications to support kidney function.

In addition to drug 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., supportiveSupportive refers to supportive treatments that aim to alleviate symptoms and improve quality of life without directly treating the underlying cause of the disease. care, including a balanced diet, stress reduction, and regular veterinary monitoring, can help promote the stability of the state of health and detect possible complications early.

Prognosis and follow-up care

The prognosis for dogs with SPAID varies depending on the severity of the disease 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 appropriate treatment, many dogs can lead a relatively normal life, although they may require regular medication and be subject to lifelong monitoring.

Dogs with kidney involvement tend to have a poorer prognosis, especially if the kidney damage is progressing. Timely treatment and regular monitoring of kidney function are crucial to 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 and maintain quality of life.

Severe cases of SPAID that do not respond well to treatment or are associated with significant complications may have a limited life expectancy. In such cases, close collaboration with the veterinarian is important to determine the best possible care and 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. options.

Prevention

Since SPAID is a genetically determined disease, preventionPrevention includes measures taken to prevent diseases or injuries. In veterinary medicine, this includes vaccinations, parasite control, and health monitoring. is primarily focused on breeding practices. Breeders should use genetic tests to identify dogs with the mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. in the HAS2 gene and avoid mating two carrier dogs with each other. This can help reduce the risk of producing affected offspring.

Educating breeders and dog owners about the disease, its symptoms, and the importance of genetic testing is crucial to minimize the spread of the disease. Responsible breeding practices and careful selectionSelection refers to the process of natural or artificial selection of specific individuals for reproduction, based on desired genetic traits. In breeding, this is applied to promote specific characteristics in dogs and cats. of breeding animals are essential steps to reduce the incidenceIncidence refers to the number of new cases of a disease or condition that occur within a specific time period in a specific population group. It provides information about the risk of contracting the disease. of SPAID.

For dogs that are already affected, the preventionPrevention includes measures taken to prevent diseases or injuries. In veterinary medicine, this includes vaccinations, parasite control, and health monitoring. of flare-ups and complications can be achieved through consistent medical monitoring and adapted care. This includes regular veterinary examinations, an adapted diet and the avoidance of stressful situations that could triggerA trigger is a stimulus that elicits a specific reaction or state. In veterinary medicine, this can refer to emotional responses, such as fear or aggression, or medical conditions, such as seizures in dogs and cats. inflammatory reactions.

Outlook on current research

Research into Shar-Pei Autoinflammatory Disease (SPAID), also known as Familial Shar-Pei Fever, is a rapidly evolving field that focuses on the genetic and immunological aspects of this disease. The disease is a hereditary autoinflammatory disease that typically occurs in Shar-Pei dogs and manifests as recurrent fever and swelling of the joints or muzzle. Scientists have discovered that a genetic mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. associated with the so-called “fever gene” (Hyaluronan Synthase 2, HAS2) can be linked to the development of the disease.

A major focus of current research is understanding the role of hyaluronic acid, a major component of connective tissue, the production of which is regulated by HAS2. Overproduction of this substance could lead to the inflammatory processes observed in SPAID. Researchers are working to decipher the exact molecular mechanisms that lead to the clinical symptoms. It is being investigated how this excess of hyaluronic acid leads to a dysregulation of the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body., which ultimately leads to the recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. fever spikes and inflammation.

Another research approach is to identify genetic markers that could enable 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. of the disease. By analyzing the 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. of Shar-Pei dogs, researchers hope to develop breeding control strategies to 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.. In the long term, this could also lead to targeted 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. approaches that target the genetic causes of the disease.

In addition, research is being conducted into how environmental factors and nutrition can influence the severity and frequency of flare-ups. Some studies suggest that certain feed additives or diets could alleviate the symptoms by having anti-inflammatory effects. However, research has not yet progressed far enough to make general recommendations.

In therapeutic research, the use of drugs that can modulate the immune responseAn immune response is the immune system's reaction to foreign substances (antigens), such as microorganisms or proteins. It involves a series of processes aimed at recognizing, fighting, and eliminating these antigens. to alleviate the inflammatory symptoms is being investigated. Both conventional drugs such as non-steroidalNon-steroidal refers to medications or substances that are not steroids and often have anti-inflammatory, analgesic, or antipyretic effects. They are used in dogs and cats to treat Pain and Inflammatory conditions. anti-inflammatory drugsAnti-inflammatory drugs are medications that reduce inflammation. They can be divided into nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids and are used in dogs and cats to reduce Inflammatory conditions and Pain. and novel biological therapies are being explored. The challenge is to develop treatments that effectively control symptoms without suppressing the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body. too much, which could increase the risk of infections.

In summary, research into SPAID is at a dynamic stage in which both genetic and therapeutic advances are being made. The hope is that these findings could benefit not only Shar-Pei dogs, but also other dog breeds and possibly even humans with similar autoinflammatory diseases.

Frequently asked questions (FAQs)

What is SPAID? SPAID, also known as Familial Shar-Pei Fever, is a genetic disease in Shar-Pei dogs that is characterized by recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. fever and inflammation. The disease affects the immune systemThe immune system is the body's defense system against infectious agents such as bacteria, viruses, fungi, and parasites. It comprises a complex network of organs, cells, and molecules that work together to protect the body. and leads to an excessive production of hyaluronic acid, which leads to the typical symptoms.
What symptoms are typical of SPAID? Typical symptoms of SPAID include recurrentRecurrent refers to a disease that repeatedly returns or worsens after a temporary remission or improvement. fever spikes, swelling of the joints, especially the hocks, and sometimes swelling of the muzzle or face. In some cases, there may also be reduced mobility and pain.
How is SPAID diagnosed? 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 SPAID is usually made through a combination of clinical examination, anamnesis and the exclusion of other diseases. A genetic test can be performed to identify the specific mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. in the HAS2 gene that is associated with the disease.
Is there a cure for SPAID? There is currently no cure for SPAID. Treatment focuses on relieving symptoms and preventing complications. Anti-inflammatory drugsAnti-inflammatory drugs are medications that reduce inflammation. They can be divided into nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids and are used in dogs and cats to reduce Inflammatory conditions and Pain. and pain relievers can be used for this purpose.
Can all Shar-Pei dogs develop SPAID? Not all Shar-Pei dogs develop SPAID, but the breed has 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. for the disease. Dogs that carry the specific genetic mutationA mutation is a permanent change in the DNA sequence (genetic material) of an organism. In dogs and cats, mutations can lead to genetic diseases or desirable changes in certain traits. have a higher risk of developing symptoms.
How can I help my dog with SPAID? To help a dog with SPAID, it is important to carry out regular veterinary examinations and monitor the symptoms. An adapted diet and the avoidance of 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. can also be helpful. Talk to your veterinarian about appropriate treatment strategies.
Is SPAID a life-threatening disease? In severe cases, SPAID can lead to complications that can be life-threatening, such as kidney failureRenal failure is a condition in which the kidneys can no longer adequately perform their function of filtering and excreting waste products from the blood. It can be acute or chronic and requires immediate medical treatment.. However, 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 appropriate treatment can help improve the dog’s quality of life and avoid complications.
Are there preventive measures against SPAID? Since SPAID is genetically determined, there are no specific preventive measures that can completely prevent the disease. However, selecting breeding animals without the genetic mutation could help reduce the incidenceIncidence refers to the number of new cases of a disease or condition that occur within a specific time period in a specific population group. It provides information about the risk of contracting the disease. of the disease in the long term.
What role does nutrition play in controlling SPAID? Nutrition can play a role in controlling the symptoms of SPAID. A balanced diet with anti-inflammatory properties can help reduce the frequency and severity of flare-ups. Talk to your veterinarian about suitable feed and dietary supplements.
Can SPAID also occur in other dog breeds? SPAID is specific to the Shar-Pei breed, although similar autoinflammatory diseases can also occur in other breeds. However, the genetic and clinical characteristics may be different.