Sjögren’s Sydrome (SS)
What is it?
Sjogrens Sydrome is a very prevelant autoimmune disease that is severely under-recognized and currently has no FDA approved drug specifically for this illness.
Sjögren’s syndrome is a systemic disease in which the defining clinical features, dryness of the eyes and mouth, arise from an autoimmune process affecting the lacrimal and salivary glands. It may occur either alone or in the context of another autoimmune disease, such as rheumatoid arthritis or systemic lupus erythematosus. Sjögren’s syndrome is one of the most prevalent systemic rheumatic diseases with a unique predilection for post-menopausal women. The disease may affect the nervous system, lungs, and kidneys in addition to the exocrine glands. Chronic fatigue, joint pain, and neuropathic pain are significant sources of disability. Late complications may include blindness, dental destruction, oral candidiasis, and non-Hodgkin lymphoma.
It is estimated that one to four million people in America suffer from Sjögren’s syndrome, an autoimmune disease in which the body attacks its own healthy moisture-producing glands. Its hallmark symptoms are dry eyes and a dry mouth, which are caused by a reduction in the production of tears and saliva, respectively. Some patients with Sjögren’s may develop peripheral neuropathy.
Sjögren’s is often called a rheumatic disease, which means it is characterized by inflammation in joints, muscles, skin, or other body tissue. Sjögren’s is also considered a disorder of connective tissue, which is the framework of the body that supports organs and tissues (joints, muscles and skin). People with Sjögren’s may have features of rheumatoid arthritis, systemic lupus erythematosus, and vasculitis.
Sjögren’s progresses slowly and is often difficult to diagnose because the symptoms are similar to other diseases. The characteristic dryness may be hard to accurately detect because dryness is often a side effect of various medications used to treat other medical conditions. Researchers think Sjögren’s is caused by a combination of genetic and environmental factors. The onset of the disease may be triggered by a viral or bacterial infection.
Without proper treatment, Sjögren’s may cause significant damage to important organs such as the kidneys, liver and lungs. People with unexplained dryness in their mouth or eyes that lasts for several months should discuss their symptoms with a doctor.
Complications of Sjogrens Syndrome
- Peripheral neuropathy of Sjögren’s syndromes
What is neuropathy?
Neuropathy, which means inflammation and/or damage to the peripheral nerves, can affect patients with Sjögren’s. Neuropathy can cause various symptoms, from “numbness,” to “coldness;” in its most severe, neuropathy has been described as “burning,” “lancinating” or “feeling like my skin is on fire”. Neuropathy can also cause weakness and clumsiness.
1. Sjögren’s may uniquely target nerves which are not tested on normal nerve-conduction tests.
Neuropathy can target nerves either of larger or smaller caliber, respectively referred to as a “large-fiber” neuropathy and a “small-fiber” neuropathy. Symptoms of large-fiber neuropathy include weakness and poorly localizable numbness and are associated with abnormalities on nerve-conduction tests. In contrast, patients with small-fiber neuropathy may have symptoms of pain, burning, and prickling, even without weakness. The nerve-conduction test is only sensitive to damage in the large-fiber nerves. and does not detect abnormalities in the smallest-caliber nerves.
2. The lack of any definitive blood tests
Sjögren’s syndrome is an example of an autoimmune syndrome. In autoimmune disorders, the immune system, which normally protects the body from infection and cancers, may cause injury to the body’s own tissues. In addition to the nervous system, organs which may be targeted in Sjögren’s syndrome include the eye, the lung, the heart, the kidney, and the joints. Many patients with Sjögren’s syndrome have autoantibodies, which bind to the body’s organ tissue and cells. Some examples of autoantibodies in Sjögren’s syndrome include anti-Ro (or SS-A antibodies) and anti-La (or SS-B antibodies).
However, more than 50% of patients with neurological manifestations of Sjögren’s may not have autoantibodies. In patients who have neuropathy and compelling glandular symptoms of dry eyes and dry mouth, negative blood tests for SS-A and SS-B antibodies do not exclude the diagnosis of Sjögren’s syndrome. In the context of sicca symptoms, further diagnostic studies are warranted, including a Schirmer’s test, and a minor salivary gland biopsy.
What are other types of neuropathy which can occur in Sjogren’s syndrome?
1. Autonomic neuropathy
Sjögren’s syndrome can cause nerve damage which regulates the coordination of heartbeat, respiration, and gastric motility. This is called an “autonomic neuropathy.” Examples of symptoms include lightheadedness when standing, decreased or increased sweating, and feeling full despite eating small meals. The diagnosis of autonomic neuropathy should be made by a neuromuscular specialist.
2. Trigeminal neuralgia and glossopharyngeal neuralgia
Sjögren’s syndrome can cause numbness or burning of the face, called “trigeminal neuralgia.” Pain in the back of the throat, which may worsen while swallowing, is called “glossopharyngeal neuralgia.” Patients with trigeminal or glossopharyngeal neuralgia can have agonizing mouth and facial pain. These neuropathies may co-exist with other neuropathies in different parts of the body. For example, up to 20% of patients with a “small-fiber” neuropathy may also have trigeminal neuropathy.
Medicines which may help alleviate symptoms in small-fiber neuropathy may also have efficacy in trigeminal neuralgia. Such medications may include a class of agents which are typically used to treat seizures, and include gabapentin, topiramate, and pregabalin. In seizure disorders, paroxysmal and irregular bursts of electrical activity in brain nerves may lead to propagation of seizures. Similarly, in Sjögren’s neuropathy, irritative electrical signals produced by nerves in the skin instead of the brain, may similarly contribute to pain. Just as anti-seizure medicines can dampen electrical activity of brain cells, the dampening of electrical activity produced by pain-fibers may ameliorate burning pain. It is important to note that use of these symptomatic medications does not target the neuron-inflammation which may be contributing to neuropathy. In such cases, judicious use of immunosuppressant medications should be considered.
3. Mononeuritis multiplex
More severe patterns of weakness or clumsiness may cause weakness or paralysis of different muscles, and is called “mononeuritis multiplex”. These more cataclysmic episodes of numbness or weakness necessitate a thorough diagnostic evaluation, both by nerve-conduction tests, and often by biopsy of nerve and/or muscles. Mononeuritis multiplex occurs when there is inflammation of small blood-vessels. The muscles and nerves nourished by such blood vessels may be deprived of oxygen and nutrients, and is similar to a “stroke of the nervous system.” In such cases, ameliorating symptoms of pain is not sufficient – immunosuppressant therapy is always warranted in cases of mononeuritis multiplex.
The pace of recovery from mononeuritis multiplex can be frustratingly slow. In some cases, it may be difficult to determine, whether a slow pace of recovery is due to ongoing and ineffectively treated inflammation, or is just a manifestation of the slow process of healing and “rewiring.” In such cases, repeat nerve-conduction tests may be important. Immunosuppresant medications which may be used in the pattern of mononeuritis multiplex include cyclophosphamide, azathioprine, as well as prednisone.
CNS or Central Nervous System complications of Sjogren’s syndrome
Patients with Sjögren’s syndrome may have “myelitis,” which is inflammation of the spinal cord. Myelitis can cause weakness, numbness, and difficulty with urination and/or defecation. Myelitis can present quickly (i.e. within hours). However, symptoms due to inflammation of the spinal cord may evolve more slowly, progressing over weeks or months. This slower progression may be difficult to distinguish from Multiple Sclerosis (MS). Unfortunately, patients with myelitis and Sjögren’s syndrome can be misdiagnosed with MS. Appropriate tests, which may include spinal tap and MRI of the brain/spinal cord, can lead to diagnostic clarity. The importance of accurately distinguishing between Multiple Sclerosis and Sjögren’s syndrome is of paramount importance. MS and Sjögren’s have completely different treatments. The approved treatments for MS are ineffective for Sjögren’s syndrome, and may actually lead to “flares” or worsening of Sjögren’s syndrome.
Patients with Sjögren’s syndrome may have another autoimmune disorder called “Devic’s syndrome,” or “Neuromyelitis Optica.” Neuromyelitis Optica (NMO) causes inflammation of the nerves connecting the eye to the brain, or “optic neuritis,” as well as myelitis. The pattern of myelitis which can occur in Neuromyelitis Optica is much different than Multiple Sclerosis. Typically, the myelitis in Neuromyelitis Optica is more severe, causing severe weakness, and may cause future relapses.
Again, the treatment for Neuromyelitis Optica is much different than MS. Patients with Sjögren’s syndrome, who are diagnosed with MS, need to be skeptical about receiving these dual diagnoses.
The most common complications of Sjogren’s syndrome involve your eyes and mouth.
- Dental cavities. Because saliva helps protect the teeth from the bacteria that cause cavities, you’re more prone to developing cavities if your mouth is dry.
- Yeast infections. People with Sjogren’s syndrome are much more likely to develop oral thrush, a yeast infection in the mouth.
- Vision problems. Dry eyes can lead to light sensitivity, blurred vision and corneal ulcers.
Less common complications may affect your:
- Lungs, kidneys or liver. Inflammation may cause pneumonia, bronchitis or other problems in your lungs; may lead to problems with kidney function; and may cause hepatitis or cirrhosis in your liver.
- Lymph nodes. A small percentage of people with Sjogren’s syndrome develop cancer of the lymph nodes (lymphoma).
- You may develop numbness, tingling and burning in your hands and feet (peripheral neuropathy).
What causes it?
The cause of Sjögren’s syndrome is not known, but it is an autoimmune disorder. People with this disease have abnormal proteins in their blood. This suggests that the immune system, which normally functions to protect the body against cancers and infections, is reacting against its own tissue. – See more at: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Sjogrens-Syndrome#sthash.1y9lhFVa.dpuf
How is it Diagnosed?
Diagnosis depends on a combination of symptoms, physical examination, blood tests, and sometimes special studies. Dry eyes and mouth may be early signs of the condition but require further investigation, because these symptoms can be caused by many other conditions or medications. Special tests may assess any decrease in tear or saliva production. An eye examination is helpful in detecting any eye changes seen in Sjögren’s. Blood tests can determine the presence of antibodies (immune system proteins that help destroy foreign invaders) typical of the disease. Typical antibodies include anti-nuclear antibodies (ANA), anti-SSA and SSB antibodies or rheumatoid factor, but these are not always present. Biopsies of saliva glands around the face or under the surface of the inner lip also may be used to make a diagnosis. – See more at: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Sjogrens-Syndrome#sthash.1y9lhFVa.dpuf
How is it treated?
Treatment is designed to lessen the most bothersome symptoms. Dry eyes usually respond to artificial tears applied regularly during the day or to gels applied at night. Other measures, such as plugging or blocking tear ducts, can be used in more severe cases. Eye drops that reduce inflammation in the glands around the eyes, such as cyclosporine (Restasis), may be used to increase tear production. Drinking water, chewing gum, or using saliva substitutes may relieve dry mouth. Some patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine (Salagen) or cevimuline (Evoxac). If patients develop yeast infections, anti-fungal therapies may be used. Humidifiers and nasal saline irrigation may improve nasal dryness. Medications that reduce gastric acid (such as proton-pump inhibitors and H2 blockers) may lessen symptoms of acid reflux. Treatments may help relieve some of the dryness, but usually some dryness persists.
All patients should receive regular dental care to prevent cavities and tooth loss that may occur as a complication of Sjögren’s. Patients with dry eyes should see an ophthalmologist (eye doctor) regularly for signs of damage to the cornea. Patients with excessive redness and pain in the eyes should be evaluated for infections.
Hydroxychloroquine (Plaquenil), an antimalarial drug used in lupus and rheumatoid arthritis, may be helpful in some patients with Sjögren’s syndrome by reducing joint pain and rash experienced by some patients. Patients with rare but serious systemic symptoms, such as fever, rashes, abdominal pain, or lung or kidney problems, may require treatment with corticosteroids such as prednisone (Deltasone and others) and/or immunosuppressive agents like methotrexate (Rheumatrex), azathioprine (Imuran), mycophenolate (Cellcept) or cyclophosphamide (Cytoxan). In addition, researchers are evaluating rituximab (Rituxan) and other biological therapies to treat cases of Sjögren’s that affect the entire body.