Weimaraner Immunodeficiency Syndrome in Dogs

Weimaraner immunodeficiency syndrome seems to be an inherited condition as it has been found predominantly in some breeds like Weimaraners, Miniature Dachshunds and Cavalier King Charles Spaniels and in certain situations and health conditions, in other breeds like German Shepherds, Rottweilers, Miniature Pinschers and Irish Wolfhounds, to name a few.   When this disease is primary, it is generally fatal to the host.

Weimaraner immunodeficiency syndrome is an immunodeficiency in which the host suffers from lower than normal levels of concentration of serum IgG as well as frequently IgM and IgA  (proteins found in plasma and other body fluids) in addition to neutrophil (a type of white blood cell) functional defects.

Young Weimaraner dogs enjoy a certain amount of protection from this immunodeficiency as long as they are nursing and receiving maternal colostral immunoglobulin.  After the loss of this maternal protection, the symptoms listed below may begin to present.  This is not a complete list and they will vary from dog to dog:

• Lethargy
• Anorexia
• Fever
• Vomiting
• Diarrhea
• Bone or joint pain
• Lymphadenopathy (chronic swelling of the lymph nodes)
• UTI (urinary tract infection)
• Recurrent or severe pyoderma (skin eruptions which display pustules or the formation of pus)
• Chronic and recurrent inflammation of various tissues which include bowels, skin and central nervous system 

Types  

The only types of Weimaraner immunodeficiency syndrome in dogs are those which refer to the absence of or low levels of concentration of the serum IgG, IgM and IgA proteins and neutrophils which have been found to be abnormal in the laboratory blood testing.  This is a multi-system infectious disease which presents around 12 to 15 weeks of age in the young dog and can affect any or all of the following systems in his body:

• Skin (pyoderma)
• Gut (diarrhea)
• Joints (hypertrophic osteodystrophy)
• Bone (osteomyelitis)
• Lung (pneumonia)
• Central nervous system (seizures, behavioral abnormality)
• Eyes (conjunctivitis)

While the exact pathogenesis (the origination and development of the disease) is not fully known, there are two schools of thought:

• The inability of the dog’s body to produce normal levels of immunoglobulin, especially IgG and IgA which can cause the inability of the dog’s system to mount adequate “protective” titers in response to vaccine antigens
• Or, a basic functional defect in neutrophils (a specific type of white blood cell) which results in the inability of the body to destroy pathogens involved in infections 

The evidence is growing that links the initial stages of the disease with the vaccination of multicomponent, modified live viral vaccines - the thought being that administration of this type of vaccine may be a trigger for the disease process.  Additionally, inherited immunodeficiencies in dogs are considered rare but some other causes in adult animals which might be related to drug therapy, chronic neoplastic or infectious diseases are more often seen.

The diagnosis of this disease, which was first noted in Melbourne, Australia in 1984 and has been found, not only in Australia, but also in Europe, USA and Israel, is based on dog breed, age, clinical signs and diagnostic tests and procedure results.  Some of the diagnostic testing which might be suggested by your veterinary professional include radiography (x-ray), bacterial culture, biopsy and endoscopy.  Necropsies which have been done historically on dogs who have died or have been euthanized show widespread neutrophil (a type of white blood cell) inflammatory lesions, with bone marrow showing active granulopoiesis (a type of white blood cell which contains microscopic granules).

Additionally, reactive amyloidosis, which is abnormal deposits of protein in the organs or bodily system, has been found in dogs with severe Weimaraner immunodeficiency syndrome.  Your veterinary professional will need your canine’s complete history in addition to the physical examination and the above mentioned testing and imaging to determine the definitive diagnosis, and then to develop an appropriate treatment plan to initiate as soon as possible.

The treatment plan recommended by your veterinary professional will likely include steps and medications designed to treat the symptoms and clinical signs being suffered by your pet.  Some treatments may include antibiotics for any secondary bacterial infections from which your pet may be suffering.  Some vets have used glucocorticoids successfully to treat the symptoms and clinical signs and your vet may wish to utilize glucocorticoid therapy for your pet.  

NSAIDs (non-steroidal anti-inflammatory medications) have also been shown to be effective in treating the symptoms and clinical signs, and accordingly,  your vet may wish to utilize this class of medications in addition to or in lieu of the glucocorticoids.  Since there is no cure for this condition, supportive care possibly consisting of fluids, rest and close monitoring may also be part of the treatment plan as well.  For most dogs in whom this condition is primary, the prognosis is poor with high morbidity rates.

While primary congenital inherited immunodeficiencies in dogs is considered rather rare, there are causes, as noted above, which can affect adult dogs which are seen more often.  If you have a dog suffering from Weimaraner immunodeficiency syndrome, and you have been told by your veterinary professional that it is likely an inherited trait, don’t be surprised if your vet advises you that it is not something that needs to be passed along to offspring in the breeding process.  This inherited trait will only serve to reduce the purity of the breed.  

Your afflicted pet will need close monitoring even after this episode has resolved because the root cause of this episode is still alive and well in the inherited immunodeficiency which still exists.  While this condition may be treatable if it is not inherited, the inherited variety of it carries with it a poor prognosis for your beloved pet.  The disease is nearly always fatal for those dogs having the inherited variety, but take heart because there are some protective measures which can be taken to extend the life of your pet.   These measures will be explained in detail by your veterinary professional.