Pathophysiology, Diagnosis, and Management of Sjogren's Syndrome

While our immune system is designed to defend the body against infections and diseases, by recognizing them (bacteria, viruses, and pathogens) as foreign, there are cases where the immune system could start to target tissue or another part of the body as foreign. This is the case of autoimmune diseases. Autoimmune diseases are conditions where the immune system mistakenly attacks its own cell, tissues, or organs, treating them as foreign to the body. This attack could lead to inflammation and damage of the tissue, cell, or organ, as well as cause other health problems. While it is not common, there are over 80 different autoimmune diseases which range from Lupus, rheumatoid arthritis, multiple sclerosis, and Sjogren's syndrome. In today's post, I will be writing about Sjogren's syndrome.

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Sjogren's syndrome is most times mistaken for dry mouth and dry eye because it is part of its symptoms, but it goes beyond that. Sjogren's syndrome is an autoimmune disorder that affects the glands that produce tears and saliva, as well as inflammatory arthritis. The dry eye is known as Keratoconjunctivitis sicca. Keratoconjunctivitis sicca is a condition where the gland that produces tears in the eyes is of poor quality. This causes dryness, grittiness, burning of the eyes, and itching. The eye will become sensitive to light, and blurred vision.

The dry mouth condition is known as Xerostomia. It is a condition where there is a decrease in the production of saliva in the mouth. Saliva is important in maintaining the health of the south, keeping it moist, and comfortable. It also neutralizes acids in the mouth.

Sjogren's syndrome also has arthritis which falls under Rheumatologic diseases. Rheumatologic diseases affect the joints bones, and muscles of the body and are often characterized by stiffness, pain, and depending on the disease it can be characterized by inflammation. Non-inflammatory Rheumatologic diseases are Osteoarthritis, Fibromyalgia, Osteoporosis, neuropathy (Charcot joint), and Alkaptonuria. Inflammatory Rheumatologic diseases are Rheumatoid arthritis, Lupus, Sjogren, Septic Arthritis, Seronegative Spondyloarthritis, Pseudogout, and Gout.

In autoimmune diseases, there is organ-specific disorder and systemic disorder. Organ-specific disorder as the name implies mistakes and attacks specific organs and tissues in the body. Examples of such diseases are Histomoto, Addison's disease, Pernicious anemia, Type 1 diabetes (that attacks the pancreas), Graves' disease, and Rheumatoid arthritis. While Organ-specific disorder affects a specific organs, systemic autoimmune diseases is when the immune system attacks multiple organs and tissues in the body. Example of such diseases are Systemic lupus erythematosus (SLE), Scleroderma, Sjogren's syndrome, and Polymyositis. When you talk about Autoimmune system, you mention autoantibodies. Autoantibodies are protein immune system, produced by the B lymphocyte cell white blood cells. B cells defend the body against foreign bodies but in autoimmune diseases, the B cell starts to attack its own cells, tissues, and organs.

With the above, we can say that Sjogren's syndrome is a systemic autoimmune diseases which could cause inflammatory arthritis. It is non-erosive, not destroying the joint where the arthritis affects. Sjogren's syndrome is a lymphocyte autoimmune disorder that affects the glands that produce saliva, the lacrimal gland that produces tears, and the joints in the body. this causes dry mouth, eyes, and arthritis which could cause fatigue, joint pain, and difficulty in swallowing. The dry mouth with Sjogren's syndrome can lead to dental caries (tooth decay), infections and other salivary gland problems. It is important to know that Sjogren's syndrome is a type four hypersensitivity disease, and so it usually has to do with the T-lymphocytes as well.

Epidemiologically, Sjogren syndrome is a very rare disease affecting between 0.5% to 1% of the population, usually affecting more females to men within the age of 40 years to 60 years old. Majority of patients who suffer from Sjogren's syndrome have rheumatoid arthritis and connective tissue diseases such as Lupus. It can also be associated with EBV (Epstein-Barr virus), CMV (cytomegalovirus), and HCV (hepatitis C virus). It is also associated with fibromyalgia which is common in women. It is also associated with HLA-DQ (human leukocyte antigen DQ), and HLA-DR (human leukocyte antigen DR).

In the past, Sjogren's syndrome was classified into primary and secondary sjogren's syndrome. Primary Sjogren's syndrome doesn't have an underlying rheumatic disorder while Secondary Sjogren's syndrome is associated with one or more underlying rhematic disease. Currently Sjogren's syndrome is identified with the patients facing neurological issues including depression (CNS) and Neuropathy (PNS), respiratory diseases such as restrictive lung disease, interstitial lung disease. Patients with Sjogren syndrome would experience Xerosis cutis (dry mouth), Dysphagia and gastritis in the gastrointestinal tract. Patients could also suffer from anemia, Leukopenia, Dyspareumia, hypogammoglobulinemia, pericarditis, thyroid, and lymphoma.

Diagnosis of Sjogren's Syndrome will have to be with examining the symptoms as well as the underlying diseases if any. With the Dry eye, (Schirmer test, Ocular Surface Staining, Tear Breakup time can be done), Dry mouth (Saxon test, and whole sialometry), Assessment of autoantibodies (ANA greater thanor equals to 1, RF positive, Anti-SS-A, and Anti-SS-B) shows positive to Sjogren syndrome. Salivary gland Imaging, MRI and Ultrasound will be helpful in glandular parenchymal abnormalities, Lip biopsy for the salivary gland to identify focal lymphocytic sialadenitis.

Management of Sjogren's syndrome would include patients quit smoking, use of artificial tears for lubrication, Cyclic AMP can be used, Parasympathomimetic for increasing parasympathetic functions, artificial saliva, goo hygiene, use sugar-free and flavoured drugs, Use antibiotic, Hydroxylchloroquine, Methotrexate, steroids. In cases of metabolic acidosis, use of HCO3-, and incases of lymphoma, R-CHOP can be used. Patients should go for examination regularly. There is no cure for Sjogren's syndrome, but it can be managed properly.

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