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

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

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 recurrent 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 system. This leads to spontaneous inflammatory reactions without recognizable infection. A mutation 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, anorexia, joint swelling, and breathing problems. Diagnosis is made by clinical assessment, genetic testing, and exclusion of other diseases. Treatments focus on symptom control with anti-inflammatory drugs and immunomodulatory therapies. Kidney involvement requires special care. Prevention 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 prognosis 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 system, 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 infection or autoimmunity.

Genetic studies have shown that SPAID is associated with a mutation in the Hyaluronan Synthase 2 gene (HAS2). This mutation 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 mutation leads to the clinical symptoms of SPAID is not yet fully understood. However, research suggests that excessive hyaluronic acid production can put the immune system into a state of chronic activation, which explains the recurrent 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 mutation from one or both parents, suggesting an autosomal dominant or incompletely dominant inheritance pattern. This genetic predisposition 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 recurrent 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 edema”. These swellings are painful and can impair the dog’s mobility. In severe cases, lameness can occur.

Chronic inflammatory conditions can also affect other organ systems. These include kidney damage, which can lead to chronic kidney disease. This kidney damage is often a result of the persistent inflammatory reaction and can lead to kidney failure over time.

Other possible symptoms include skin changes, recurrent gastrointestinal problems such as vomiting and diarrhea, and in some cases respiratory distress. The range and combination of symptoms can vary from dog to dog, which can make diagnosis difficult.

Diagnosis

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

Genetic tests can help identify the mutation in the HAS2 gene that is associated with SPAID. These tests are particularly useful for confirming a hereditary component and securing the diagnosis. However, it is important to note that not all dogs with the genetic mutation 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 ultrasound or X-rays may be necessary to assess organ damage and rule out other possible causes.

The diagnosis 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 drugs such as non-steroidal anti-inflammatory drugs (NSAIDs) or corticosteroids can help control acute inflammation and fever spikes. In some cases, immunomodulatory drugs may be needed to regulate the immune system 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 therapy, and specific medications to support kidney function.

In addition to drug therapy, supportive 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 diagnosis 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 progression 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 therapy options.

Prevention

Since SPAID is a genetically determined disease, prevention is primarily focused on breeding practices. Breeders should use genetic tests to identify dogs with the mutation 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 selection of breeding animals are essential steps to reduce the incidence of SPAID.

For dogs that are already affected, the prevention 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 trigger 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 mutation 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 system, which ultimately leads to the recurrent fever spikes and inflammation.

Another research approach is to identify genetic markers that could enable early diagnosis of the disease. By analyzing the genetic predisposition of Shar-Pei dogs, researchers hope to develop breeding control strategies to reduce the spread of the disease in the population. In the long term, this could also lead to targeted therapy 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 response to alleviate the inflammatory symptoms is being investigated. Both conventional drugs such as non-steroidal anti-inflammatory drugs and novel biological therapies are being explored. The challenge is to develop treatments that effectively control symptoms without suppressing the immune system 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 recurrent fever and inflammation. The disease affects the immune system 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 recurrent 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 diagnosis 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 mutation 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 drugs 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 predisposition for the disease. Dogs that carry the specific genetic mutation 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 stress 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 failure. However, early diagnosis 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 incidence 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.