Systemic Lupus Erythematosus

TL;DR. Systemic Lupus Erythematosus (SLE) is a rare, multi-system autoimmune disease in dogs and cats where the body's immune system attacks its own tissues, causing widespread inflammation, joint pain, skin lesions, and organ damage.

Systemic Lupus Erythematosus can cause painful joint inflammation and persistent fatigue in dogs.

What is it?

Systemic Lupus Erythematosus (SLE) is a multi-systemic immune-mediated disease. In a healthy pet, the immune system acts as a shield, defending the body against foreign invaders like bacteria and viruses. In a pet with SLE, this protective system malfunctions. It loses the ability to distinguish between "self" and "non-self," leading to the production of autoantibodies. These autoantibodies target the pet's own cellular components, particularly the nuclei of cells.

As described in a leading veterinary dermatology reference:

"Systemic lupus erythematosus is a multisystemic immune-mediated disease characterized by the production of a variety of autoantibodies (e. g. , ANA, rheumatoid factor, anti-RBC antibodies) that form circulating immune complexes."

These autoantibodies bind to self-antigens, creating "circulating immune complexes." These complexes travel through the bloodstream and settle in various tissues throughout the body, such as the joints, skin, kidneys, and blood vessels. Once deposited, they trigger a severe, destructive inflammatory response. Because these complexes can settle almost anywhere, SLE is often called the "great imitator." The disease can manifest in completely different ways from one pet to another, making it one of the most challenging conditions to manage in veterinary medicine. It is considered uncommon in dogs and rare in cats.

Causes & risk factors

The exact cause of SLE is complex and not fully understood, but it is widely accepted to be a combination of genetic predisposition and environmental triggers.

Genetics play a significant role in who develops the disease. Certain dog breeds are statistically more likely to develop SLE, suggesting an inherited genetic component. As noted in a leading veterinary internal medicine reference:

"Breeds that are predisposed include the German Shepherd Dog, Shetland Sheepdog, Collie, Beagle, and Poodle. Several colonies of dogs with a high predisposition toward SLE have been established, and an association with certain MHC (DLA) types exists. Other risk factors likely include environmental factors and exposure to certain infectious agents and drugs."

Environmental factors such as ultraviolet (UV) light exposure can trigger or worsen skin lesions associated with lupus. Additionally, exposure to certain infectious diseases or specific medications can stimulate the immune system in a way that initiates this abnormal, self-destructive response in genetically vulnerable pets.

Signs to watch for

Because SLE can affect multiple organ systems simultaneously or sequentially, the clinical signs can wax and wane, making them difficult to track. The symptoms are categorized by how frequently they occur in affected pets:

Cardinal Signs

• Polyarthritis: This is the most common sign of SLE. It causes inflammation in multiple joints, leading to stiffness, pain, and a "shifting-leg" lameness where the pet limps on different legs at different times.
• Fever: A persistent, fluctuating high temperature that does not respond to standard antibiotic therapy.

Common Signs

• Glomerulonephritis: Inflammation of the kidney's filtering units. This leads to protein leaking into the urine and can progress to kidney failure.
• Dermatologic lesions: Skin issues including redness, scaling, crusting, hair loss, and deep ulcers. These lesions are most common on the face, bridge of the nose, ears, and footpads, and they often worsen with exposure to sunlight.

Occasional Signs

• Polymyositis: Inflammation of the muscles, leading to generalized muscle pain, weakness, or muscle wasting.
• Anemia and Thrombocytopenia: Immune-mediated destruction of red blood cells (causing pale gums, weakness, and lethargy) or platelets (causing pinpoint bruising, nosebleeds, or blood in the stool).
• Leukopenia: A decrease in white blood cells, which can leave the pet vulnerable to secondary infections.
• Pleuritis or Pericarditis: Inflammation of the lining around the lungs or heart, which can cause chest pain, coughing, or rapid, shallow breathing.
• CNS signs: Neurological symptoms, including seizures, behavioral changes, or loss of coordination.
• Oral ulceration: Painful open sores inside the mouth or on the gums.

Rare Signs

• Laryngeal paralysis: Loss of function in the larynx, leading to voice changes, harsh breathing sounds, and airway obstruction.

Dermatologic lesions, especially around the nose and face, are common in pets with SLE.

How vets diagnose it

Diagnosing SLE is a complex process. There is no single test that can definitively confirm the disease on its own. Instead, your vet will look at the entire clinical picture, combining your pet's medical history, physical examination findings, and a series of specialized laboratory tests.

To help guide the diagnosis, veterinarians often use a points-based system of "major" and "minor" clinical signs alongside laboratory results. A leading internal medicine reference highlights the importance of this clinical picture:

"Conversely, 13 of 16 dogs with two major signs compatible with SLE had immune-mediated disease, and ANA was positive in 10 of these dogs. These results emphasize that the positive predictive value of a diagnostic test is lower in a population of animals in which the disease prevalence is low. The LE test is rarely used clinically for diagnosis of SLE because of very low sensitivity."

Your vet will likely recommend a combination of the following tests:

• Antinuclear Antibody (ANA) Titer: This is the primary screening test for SLE. It measures the level of antibodies in the blood that are targeting cell nuclei. As the reference text notes:

  "Results are reported as a titer that is the highest dilution of patient serum that causes definitive immunofluorescent nuclearstaining. Various patterns of nuclearstaining (diffuse, speckled, peripheral, and nucleolar) can be identified, but the clinical significance of the various staining patterns is still under investigation in dogs and cats. Measurement of ANA antibodies is sensitive for diagnosis..."

• Lupus Erythematosus (LE) Cell Test: Though historically used, this test is rarely performed today due to its low sensitivity and the superiority of the ANA test.
• Complete Blood Count (CBC) and Chemistry Profile: These tests help identify cytopenias (like anemia or low platelets) and assess organ function, particularly the liver and kidneys.
• Urinalysis and Urine Protein/Creatinine (UPC) Ratio: This is crucial for detecting protein loss in the urine, which is an early indicator of kidney damage (glomerulonephritis).
• Synovial Fluid Analysis: If your pet has swollen joints, your vet will collect fluid from multiple joints to check for sterile inflammation and rule out infectious arthritis.
• Skin or Renal Biopsy: Taking a small tissue sample from active skin lesions or the kidneys can confirm characteristic immune-complex deposition under a microscope.

Your vet will tailor this diagnostic plan to your pet's specific symptoms:

"Not all testing listed above is necessary in all cases. Specific diagnostic test will depend on the individual case presentation and geographic location."

Treatment options

The primary goal of treating SLE is to suppress the overactive immune system, reduce inflammation, and manage pain. Treatment is typically divided into first-line and second-line therapies.

First-Line Therapy

• Glucocorticoids (Corticosteroids): High doses of prednisone or prednisolone are the cornerstone of initial treatment. These medications rapidly suppress the immune response and reduce systemic inflammation. Once the disease is in remission, your vet will slowly and carefully taper the dose to the lowest effective level to minimize long-term side effects.

Second-Line Therapy

If glucocorticoids alone are not controlling the disease, or if the side effects are too severe, your vet will add secondary immunosuppressive agents:

• Azathioprine: A purine antagonist immunosuppressant commonly used in dogs to help lower the required steroid dose. It is generally avoided in cats due to severe bone marrow toxicity.
• Cyclosporine: A targeted immunosuppressive drug that can help control the immune response with fewer systemic side effects than steroids.
• Cyclophosphamide: An alkylating agent used occasionally in severe, life-threatening cases to rapidly halt the immune attack.
• Levamisole: Historically used as an immunomodulator to help balance the immune system, though it is less commonly utilized in modern protocols.

Prognosis

The long-term prognosis for pets with SLE is guarded to poor. While some pets can achieve temporary remission, relapses are common and often require lifelong, carefully monitored immunosuppressive therapy.

The prognosis depends heavily on which organs are affected. Pets with severe kidney damage (glomerulonephritis) or severe blood cell deficiencies face a much more difficult road and a shorter life expectancy. Because SLE is exceptionally rare in cats, long-term prognosis data in this species is limited, and guidance is largely extrapolated from canine cases.

Prevention

Because SLE is an inherited, complex autoimmune disease, there is no known way to prevent it from developing.

To reduce the prevalence of this disease, dogs from breeds with a known genetic predisposition (such as German Shepherds, Collies, and Shetland Sheepdogs) should not be bred if they or their close relatives have been diagnosed with SLE or other systemic autoimmune conditions.

For pets already diagnosed with SLE, you can help prevent flare-ups by avoiding known triggers. Keep your pet out of direct, intense sunlight, and consult your veterinarian before administering any new medications or vaccinations, as these can sometimes stimulate a relapse.

When to call your vet

If your pet has been diagnosed with SLE or is undergoing diagnostic testing, you must monitor them closely for sudden changes.

Contact your veterinarian immediately if you observe any of the following emergency red flags:

• Sudden weakness, collapse, or extremely pale gums (signs of severe anemia).
• Unexplained bruising, pinpoint red spots on the skin or gums, or active bleeding (signs of dangerously low platelets).
• Difficulty breathing, rapid breathing, or a harsh, raspy sound when inhaling (signs of fluid around the lungs or laryngeal paralysis).
• A sudden return of a high fever or severe, painful joint stiffness.
• Frequent vomiting, a sudden loss of appetite, or changes in urination habits (signs of worsening kidney damage).

For specific breeds

If you own a German Shepherd Dog, Shetland Sheepdog, Collie, Beagle, or Poodle, it is important to be aware of this condition. While SLE remains uncommon overall, these breeds carry a higher genetic risk. If your dog develops a mysterious, shifting lameness, a persistent fever, or crusting skin sores on their nose or face, ensure your veterinarian is aware of their breed risk so that diagnostic testing for autoimmune diseases can begin promptly.

Sources

• Internal Medicine 5th, pages 1438, 1467, 1468.
• Small-Animal-Dermatology-A-Color-Atlas-and-Therapeutic-Guide, page 260.