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AUTOIMMUNE
DISEASES Note for Pet Owners:
This information is provided by
Provet for educational purposes only.
You should seek the advice of
your veterinarian if your pet is ill as only he or she can correctly advise
on the diagnosis and recommend the treatment that is most appropriate for
your pet.
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Description
In nature animals have evolved a sophisticated defence system that produces
glycoproteins called immunoglobulins or antibodies in response to chemicals (antigens) on
the surface of organisms (eg bacteria, viruses) or other substances (eg
toxins) that gain access to the body. The antibodies chemically bind with the
antigens - an initial step in the process to remove them from the body.
Under normal circumstances the body's immune system recognises tissues and
cells that are part of itself, and the immune system will only produce
antibodies against foreign cells. However, sometimes the controlling mechanisms
fail and the immune system does produce antibodies which
attack the animals own body tissues. These are called autoantibodies and
the disease that results is called an autoimmune disease.
Autoantibodies may be formed against specific organs or specific types of
cell (eg blood cells), or they may be non-organ specific. In the last category
antibodies are often produced which act against nuclear material within the nucleus
of cells and these are called antinuclear antibodies (ANAs). ANAs are commonly
found in
some forms of autoimmune disease eg systemic lupus erythematosus (SLE)
Cause
Th e cause of autoimmune diseases is the production of autoantibodies against
the animals own tissues. The underlying reasons for these disorders occurring are complex and not
fully understood.Infectious agents are thought to be the initiating factor in
some of these disorders eg Systemic Lupus Erythematosus (SLE)
Breed Occurrence
There are several reports of possible increased incidence of autoimmune diseases
in some breeds and sexes :
- Cocker Spaniels,Old English Sheepdogs and Poodles may be predisposed to
develop autoimmune haemolytic anaemia
- Poodles are over-represented in reports of autoimmune thrombocytopenia
- Female dogs more often develop autoimmune haemolytic anaemia, and
autoimmune thrombocytopenia than males
- Poodles and German Shepherds may be predisposed to develop Systemic Lupus
Erythematosus
- Toy breeds of dog may be predisposed to develop rheumatoid arthritis
Signs
Th e common signs of autoimmune disease in animals depend upon the target
organ(s) for the autoantibodies :Adrenal gland
- Some cases of canine hypoadrenocorticism (Addisons
disease) are due to autoantibody production against the adrenal
glands.
- Signs include :
- Muscle weakness - the dog collapses during exercise
- Depression
- Sudden collapse and shock
- Kidney failure
- Inappetance
- Diarrhoea - sometimes contains blood
- Abdominal pain
- Increased thirst (polydipsia)
- Increased urine production (polyuria)
- Weight loss
Blood
- Autoimmune haemolytic anaemia (dogs, cats (rare -
often secondary to leukaemia), cattle (very rare), horses (rare - usually
secondary eg abscesses). It can occur with autoimmune thrombocytopenia.
- Due to autoantibodies against the animals own red blood cells
causing them to disrupt (called haemolysis)
- Coombs test is positive in these patients
- Anaemia may be present on routine haematological examination
- Free haemoglobin is present in blood and urine
- The animal may develop jaundice (accumulation of yellow pigment in
tissues)
- Affected animals are lethargic
- Extremities (ears, feet, nose and tail) may become blue (cyanosed)
or reddened, swollen, ulcerated and crusts may form.
- Enlargement of the spleen may occur
- Enlargement of lymph nodes may occur
- Sometimes autoimmune haemolytic anaemia has a primary cause eg it
can be induced by drugs
- Autoimmune thrombocytopenia (dogs, cats
(rare), horses. It can be secondary to systemic lupus erythematosus - see
below). It can occur with autoimmune haemolytic anaemia
- Due to autoantibodies against platelets which are important in blood
clotting.
- Haemorrhages into the skin (small petechial haemorrhages, larger
ecchymotic haemorrhages (patches) or sometimes massive haemorrhages -
nose bleeds or internal bleeding into the abdomen or chest )
- Systemic lupus erythematosus (SLE) - see below
Musculoskeletal System
- Myasthenia gravis (dogs and cats)
- The acquired form of this disease is associated with the production
of autoantibodies against acetylcholine receptors on muscle. Signs
include :
- Poor exercise tolerance
- Muscle weakness
- Difficulty eating and swallowing
- Regurgitation of food
- Dilation of the oesophagus (megaoesophagus)
- Rheumatoid arthritis - Autoantibodies
against IgG are associated with rheumatoid arthritis the signs of
which are :
- Lameness
- Swollen joints - often affects the same joints in the left and right
limbs.
- Restricted range of movement in joints, and in advanced cases there
may be no movement in the joint
- In some cases the joint dislocates because local ligaments rupture,
and so there is an excessive range of movement
- Crepitus (a grating sensation and clicking or cracking sound) is
present when the joints are manipulated
- High body temperature
- Inappetance
- Systemic lupus erythematosus (SLE) - see
Multiple organ autoimmune diseases below.
Skin
- Bullous Autoimmune Skin Disease
- Pemphigus vulgaris (dogs and cats)
- Erosions or ulcers around the mucocutaneous junctions (mouth,
nails)
- The lesions discharge serum and crusts form over the ulcers
- Depression
- Inappetance
- Death
- Pemphigus foliaceus (most
common canine and equine autoimmune skin disease, also reported in
cats and goats)
- Small swellings under the surface of the skin (bullae or
pustules)
- Crusting
- Scale (scurf or dandruff) formation
- Loss of hair (alopecia)
- Black pigmentation of the skin (hyperpigmentation)
- Sloughing of the foot pads
- Itchiness (pruritus)
- Usually affects the head and nose, but can be generalised
- Pemphigus vegetans (rare- dog))
- Pustules
- Crusting
- Papilloma formation
- Can be generalised or in the groin area
- Pemphigus erythematosus (dog and cat)
- Discharging sores
- Crusting
- Itchiness (pruritus)
- Affects mainly the skin around the eyes, the ears and on the
bridge of the nose.
- The disease gets worse if the skin is exposed to sunlight
- Bullous pemphigoid (dog and horse)
- Erosions or ulcers around the mucocutaneous junctions (eg lips
and mouth),
the ears or the groin
- The lesions discharge serum and crusts form over the ulcers
- Depression
- Inappetance
- High body temperature - due to secondary infection
Thyroid
- Autoantibodies against thyroglobulin (and occasionally against thyroid
hormone T3) are associated with lymphocytic
thyroiditis - the main cause of hypothyroidism
in dogs. Signs of
hypothyroidism include :
- Bilateral hair loss (alopecia)
- Thinning of the skin
- Lethargy
- Black pigmentation of the skin
- Scurf (dandruff)
- Lethargy
- Obesity
- Cold intolerance - seeks warm places to lie down
- Reproductive problems.
- Fat deposits in the cornea of the eye (lipidosis)
- Dry eye (keratitis sicca)
- Diarrheoa
- Vomiting
- Constipation
- Slow heart rate (bradycardia)
- Muscle weakness
Multiple Organ Autoimmune Diseases
- Systemic lupus erythematosus
(SLE) - (dogs and
cats)
- Arthritis (rheumatoid) in several joints - see above - most common
presenting sign
- Skin disease - second most common presenting sign
- Bilaterally symmetrical loss of hair and production of scurf
(scale or dandruff)
- Ulcerations and crusting form in severe cases - often
affecting the ears, feet and head
- High body temperature - does not respond to antibiotics, does
respond to corticosteroids (present in over 50% of cases)
- Inappetance
- Anaemia - causes lethargy. Positive Coombs test.
- Thrombocytopenia (low platelet count - less than 50,000 per cubic
mm - present in about 30% of cases) - may lead to haemorrhages
- Neutropenia - low white cell count (neutrophils) (present in up to
50% of cases)
- Kidney disease - glomerulonephritis (in about 25% of cases) -
protein leaks into urine.
- Weight loss
- High total serum protein
Complications
Multiple organ system involvement is common and this can make diagnosis
difficult.
Diagnosis
Diagnosis depends upon tests to identify the circulating antibodies, or
antibody-antigen complexes that form and other tests specific to the organ
involved ;
Adrenal gland
- Adrenal insufficiency (Addisons disease)
- Indirect immunofluorescence tests
- Low blood
cortisol concentrations
(below 35 nmol/l)
Low plasma sodium concentration (less than 135 mmol/l)
High plasma potassium concentration (over 5.5 mmol/l)
High blood calcium concentrations (in about 50% of cases)
High blood urea and creatinine (about 75% of cases)
Poor ACTH stimulation test
Histopathological examination of adrenal biopsy
Blood
Musculoskeletal system
Multiple organ systems
Thyroid
- Lymphocytic thyroiditis
- Clinical signs
- Low thyroid hormone (thyroxine - T4) concentrations in the blood
- Histopathological examination of thyroid biopsy
- Detection of antithyroglobulin antibodies in plasma
Treatment
Treatment depends upon the organs involved . The aim is usually to reduce the
immune response, however it should be remembered that this will also reduce the
animals resistance to infection, so secondary complications may occur. Adrenal
gland
- Addisons disease
- Emergency treatment to replace fluids and electrolytes
- Long term treat with mineralocorticoids eg fludrocortisone acetate at
0.1mg/kg body weight per day AND glucocorticoids eg prednisolone at
0.1-0.3mg/kg body weight per day
Blood
- Autoimmune haemolytic anaemia
- Immunosuppressive drugs eg
- High dose corticosteroids - prednisone or prednisolone at 2mg/kg
body weight twice daily by mouth
- Cyclophosphamide - in acute, severe cases - 1.5-2.5mg/kg body
weight daily by mouth
- Blood transfusion - may be beneficial but controversial as haemolysis
(disruption of the red cells) may occur
- Removal of the spleen (splenectomy)
- Treat any primary cause eg remove drug therapy if it is a drug-induced
problem
- Autoimmune thrombocytopenia
- Immunosuppressive drugs :
- High dose corticosteroids (see above)
- Vincristine - 0.01-0.025mg/kg body weight intravenously every 7
days until platelet counts are normal
- Cyclophosphamide - in severe cases - see above
- Blood transfusion - severe cases
- Splenectomy - if recurrent or non-responsive
- Treat any primary cause eg drug-induced problem
Musculoskeletal system
- Myasthenia gravis
- Cholinesterase inhibitors eg pyridostigmine bromide at 10-60mg three
times daily
- Corticosteroids eg prednisone at 2-3mg/kg body weight per day
- Rheumatoid arthritis
- Aspirin - for its anti-inflammatory/analgesic effects - but not if SLE
or thrombocytopenia are present
- High dose corticosteroids (with/without aspirin) - see above
- Cytotoxic drugs :
- Azathioprine - 2mg/kg body weight daily or on alternate days or in
combination with cyclophosphamide
- Cyclophosphamide - 2 mg/kg body weight daily for 4 days per week
- Gold salt therapy - 1mg.kg body weight of sodium aurothiomalate every
week
- Systemic lupus erythematosus - see below
- Enteropathic arthritis
- Polyarthritis associated with inflammatory bowel disease has been
reported to occur in dogs
Multiple organ autoimmune disease
- Systemic lupus erythematosus (SLE)
- High dose corticosteroids - see above
- Azathioprine - for long term treatment
- Cyclophosphamide - see above
- Vincristine - if thrombocytopenia is present - see above
Skin
- Bullous autoimmune skin diseases
- Drugs to suppress the immune response. Combinations of these are often
given together :
- Corticosteroids - eg prednisone or prednisolone 1-2mg/kg body
weight by mouth twice daily
- Cytotoxic drugs
- azathioprine 2mg/kg body weight daily or on alternate days
OR
- cyclophosphamide 2 mg/kg body weight daily for 4 days per week
- Gold salt therapy - 1mg.kg body weight of sodium aurothiomalate
every week
- Megestrol acetate (cats only)
Avoid exposure to sunlight (cases of pemphigus erythematosus)
Thyroid
- Lymphocytic thyroiditis
- Synthetic thyroid hormone (T4) replacement therapy
Prognosis
In autoimmune diseases the prognosis is always guarded with only fair to poor
chances of long term survival without treatment. However some respond well to
therapy. Some examples are:
- Autoimmune thrombocytopenia - prognosis is good if treated aggressively
- Bullous autoimmune skin diseases - can often go into remission and be
controlled with drugs.
- Canine hypothyroidism due to lymphocytic thyroiditis can often be
controlled well with thyroid hormone replacement therapy
- Myasthenia gravis - prognosis is guarded. Some can undergo remission and
be controlled, others progress and die
- Systemic lupus erythematosis - prognosis is fair
Last Updated : March 2008
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