INTRODUCTION:

Sjögren's syndrome is an inflammatory disease that can affect many different parts of the body, but most often affects the tear and saliva glands. “Primary” Sjögren's syndrome occurs in people with no other rheumatic disease. “Secondary” Sjögren's occurs in people who have another rheumatologic disease, most often systemic lupus erythematosus and rheumatoid arthritis [1]. Sjogren's causes immune system to go haywire and attack healthy cells instead of invading bacteria or viruses. Conditions like this are called autoimmune diseases. White blood cells, protect from germs, attack the glands that are in charge of making moisture. When that happens, it can't produce tears and saliva.

A dry mouth can feel chalky or full of cotton, and affected individuals may have difficulty speaking, tasting food, or swallowing. Because saliva helps protect the teeth and the tissues of the oral cavity, people with Sjögren syndrome are at increased risk of tooth decay and infections in the mouth. [3]. In Sjögren syndrome, extraglandular involvement may result in painful inflammation of the joints and muscles; dry, itchy skin and skin rashes; chronic cough; a hoarse voice; kidney and liver problems; numbness or tingling in the hands and feet; and, in women, vaginal dryness [5]. A small number of people with Sjögren syndrome develop lymphoma, a blood-related cancer that causes tumor formation in the lymph nodes [4].

INCIDENCE:

Sjögren syndrome is a relatively common disorder; it occurs in 0.1 to 4 percent of the population. It is difficult to determine the exact prevalence because the characteristic features of this disorder, dry eyes and dry mouth, can also be caused by many other conditions. [4]

ETIOLOGY:

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. The decrease in tears and saliva seen in Sjögren's syndrome occurs when the glands that produce these fluids are damaged by inflammation. [1]

Researchers believe that variations in many genes affect the risk of developing Sjögren syndrome, but that development of the condition may be triggered by something in the environment. In particular, viral or bacterial infections, which activate the immune system, may have the potential to encourage the development of Sjögren syndrome in susceptible individuals. [3][4] A defective gene that's linked to Sjogren's, and then get an infection. Immune system swings into action.White blood cells normally lead the attack against the germs. But because of faulty gene, white blood cells target healthy cells in the glands that make saliva and tears. [2]

CLINICAL FEATURES:

The symptoms of Sjogren's vary a lot from person to person.

· Dry mouth- Patient complaints mouth has a chalky feeling. Patient feels hard to swallow. The dryness can also lead to yeast infections in your mouth. Patient don't have enough saliva, which helps protect your teeth from decay, get more cavities than other people, also get inflammation of your gums, called gingivitis.

· Dry eyes- burning sensation, itching, or feel gritty, infections around eyes. It can harm cornea.

· Dryness in throat, nose, lips, or skin. The glands in neck and face might swell up. Women may get dryness in their vagina.

· Some people also get swelling, pain, and stiffness in their joints. This can happen even if patient don't also have rheumatoid arthritis, which has those same symptoms.

· Heartburn, a sensation of burning that moves from stomach to chest.

· It's rarer, but patient could have other problems like inflammation in lungs,liver, and kidneys. Some patients feel tired and have numbness and tingling in some parts of their body. [2] [10]

[14]

Diagnostic Evaluation:

· Physical examination -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.

· An eye examination is helpful in detecting any eye changes seen in Sjögren's.

· Schirmer test of tear production

· Complete blood chemistry with liver enzymes

· Blood tests can determine the presence of antibodies (immune system proteins that help destroy foreign invaders) typical of the disease.

· Urinalysis

· Test for cryoglobulins

· Protein electrophoresis

· Test for hepatitis C and HIV (if at risk)

· Thyroid tests

· Biopsies of saliva glands around the face or under the surface of the inner lip also may be used to make a diagnosis. [7]

· Skin biopsy if a rash is present

· Imaging of the salivary gland: by ultrasound or by MRI

· Chest X-ray

· Typical antibodies include anti-nuclear antibodies (ANA), anti-SSA and SSB antibodies or rheumatoid factor, but these are not always present. [11] Antibodies are proteins that your immune system makes to fight foreign substances like viruses and bacteria. But an antinuclear antibody attacks your own healthy cells instead. It's called "antinuclear" because it targets the nucleus (center) of the cells. [11] [12]

· Blood tests can also reveal if you have high amounts of proteins called immunoglobulins.. A high level could be a sign of Sjogren's. [7] A rheumatoid factor (RF) test measures the amount of rheumatoid factor (RF) in blood. Rheumatoid factors are proteins produced by the immune system.It is most often used to help diagnose rheumatoid arthritis. Rheumatoid arthritisis a type of autoimmune disorder that causes pain, swelling, and stiffness of the joints. Rheumatoid factors may also be a sign of other autoimmune disorders, such as juvenile arthritis, certain infections, and some types of cancer. [13]

TREATMENT MODALITIES:

· 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. [6]. If artificial tears aren't helping, then drugs for dry eyes, including:Cequa, Lacrisert, Restasis. Lacrisert is tiny rod-shaped medicine. Put it into eye with a special applicator, usually once or twice a day. Cequa and Restasis come in the form of drops, twice a day.[2]

· 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.

· Dry mouth: 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). To help your dry mouth, drugs that boost the amount of saliva, including:Cevimeline (Evoxac), supersaturated calciumphosphate rinse (NeutraSal), pilocarpine (Salagen) [2].If Candida is present, it may be treated with sugar-free miconazole or nystatin preparations.

· All patients should receive regular dental care to prevent cavities and tooth loss that may occur as a complication of Sjögren's.

· Joint pain: 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. [10]. A medicine called hydroxychloroquine (Plaquenil) to treat your joint pain. It's a drug that's also used to treat malaria, lupus, and rheumatoid arthritis. [2]. Disease-modifying antirheumatic drugs (DMARDs) similar to those used for RA may improve the symptoms of Sjögren syndrome. These include tumor necrosis factor (TNF) inhibiting drugs such as Enbrel, Humira or Remicaide.

· 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. [1] [10].

· 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.

Some things you can do to ease symptoms include:

· Sip water throughout the day

· Chew sugarless gum

· Avoid medicines that can cause mouth dryness, such as antihistamines and decongestants

· Avoid alcohol

Talk with your dentist about:

· Mouth rinses to replace minerals in your teeth

· Saliva substitutes

· Drugs that help your salivary glands make more saliva

To prevent dental decay caused by mouth dryness:

· Brush and floss your teeth often

· Visit the dentist for regular checkups and cleanings [6]

· If dry skin is a problem, use warm water, not hot, when bathe or shower. Instead of using a towel after showering, let "drip dry". Skin will absorb the moisture from the shower. [2]

· Apply a warm, wet compress to the closed eyes using a washcloth heated in tolerably warm water from the sink or shower. Apply at bedtime and upon awakening for 5 minutes or more often if desired [8]

Complication:

· Patients with dry eyes are at increased risk for infections around the eye and may have damage to the cornea.

· Dry mouth may cause an increase in dental decay, gingivitis (gum inflammation), and oral yeast infections (thrush) that may cause pain and burning. Some patients have episodes of painful swelling in the saliva glands around the face.

· Pain and stiffness in the joints with mild swelling may occur in some patients, even in those without rheumatoid arthritis or lupus.

· Rashes on the arms and legs related to inflammation in small blood vessels (vasculitis) and inflammation in the lungs, liver, and kidney may occur rarely and be difficult to diagnose.

· The parotid gland is at the edge of the jaw and can become swollen and inflamed in some people with Sjögren's Syndrome. [1] [9]

Other Names for This Condition:

· Dacryosialoadenopathia atrophicans

· Gougerot-Houwer-Sjogren syndrome

· Gougerot-Sjogren syndrome

· Keratoconjunctivitis sicca

· Keratoconjunctivitis sicca-xerostomia

· Secreto-inhibitor-xerodermostenosis

· Sicca syndrome

· Sjogren-Gougerot syndrome

· Sjogren's syndrome [3]

PROGNOSIS:

Most people with Sjogren syndrome have limited symptoms such as dry eyes and dry mouth. Their general health and life expectancy are largely unaffected. Increased mortality may be related to conditions associated with Sjogren syndrome, such as systemic lupus erythematous (SLE), rheumatoid arthritis (RA), or primary biliary cirrhosis. People with Sjogren syndrome who don't develop a lymphoproliferative disordercan generally expect to have a normal life span.

REFERENCE

1. Ali Duarte, American College of Rheumatology Committee, available at https://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Sjogrens-Syndrome, Updated March 2019, assessed on 05-06-2020.

2. Brian S. Boxer Wachler, WebMD Medical Reference, available at https://www.webmd.com/a-to-z-guides/sjogrens-syndrome#1, on September 25, 2018, assessed on 05-06-2020.

3. Genetics Home References, https://ghr.nlm.nih.gov/condition/ sjogren-syndrome#resources, assessed on 25-06-2020.

4. Bayetto K, Logan RM. Sjögren's syndrome: a review of aetiology, pathogenesis, diagnosis and management. Aust Dent J. 2010 Jun;55 Suppl 1:39-47. doi: 10.1111/j.1834-7819.2010.01197.

5. Delaleu N, Jonsson MV, Appel S, Jonsson R. New concepts in the pathogenesis of Sjögren's syndrome. Rheum Dis Clin North Am. 2008 Nov;34(4):833-45, vii. doi: 10.1016/j.rdc.2008.08.004. Review.

6. Foulks GN, Forstot SL, Donshik PC, Forstot JZ, Goldstein MH, Lemp MA, Nelson JD, Nichols KK, Pflugfelder SC, Tanzer JM, Asbell P, Hammitt K, Jacobs DS. Clinical guidelines for management of dry eye associated with Sjögren disease. Ocul Surf. 2015 Apr;13(2):118-32. doi: 10.1016/j.jtos.2014.12.001. Epub 2015 Jan 15.

7. Rockville Pike, Bethesda, Medline Plus, U.S. Department of Health and Human Services National Institutes of Health, available at https:// medlineplus.gov/sjogrenssyndrome.html, Page last updated on 15 May 2020 Topic last reviewed: 7 March 2016. https://medlineplus.gov/lab-tests/ana-antinuclear-antibody-test/, Page last updated on 20 February 2020 Page last reviewed: 18 May 2018. https:// medlineplus.gov/lab-tests/rheumatoid-factor-rf-test/, Page last updated on 26 February 2020 Page last reviewed: 28 June 2018

8. J. Daniel Nelson,Specialty Care HealthPartners Medical Group & Clinics, and Professor of Ophthalmology, University of Minnesota, Minneapolis, Minnesota, available at https:// www.sjogrens.org/sites/default/files/inline -files/Dry%20Eye%20Patient%20Education%20Sheet.pdf.

9. Gordon A. Starkebaum, MD, MACR, ABIM Board Certified in Rheumatology, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Medline Plus, available at , https://medlineplus.gov/ency/article/000456.htm, Review Date 4/8/2019

10. Tincani A, Andreoli L, Cavazzana I, Doria A, Favero M, Fenini MG, Franceschini F, Lojacono A, Nascimbeni G, Santoro A, Semeraro F, Toniati P, Shoenfeld Y, Novel aspects of Sjögren's syndrome in 2012, BMC Med. 2013 Apr 4; 11:93., available at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3616867/

11. American College of Rheumatology [Internet]. Atlanta: American College of Rheumatology; c2017. Antinuclear Antibodies (ANA) [updated 2017 Mar; cited 2017 Nov 17]; [about 3 screens]. Available from: https://www.rheumatology.org/I-Am-A/Patient -Caregiver/Diseases-Conditions/Antinuclear-Antibodies-ANA

12. Hinkle J, Cheever K. Brunner & Suddarth's Handbook of Laboratory and Diagnostic Tests. 2nd Ed, Kindle. Philadelphia: Wolters Kluwer Health, Lippincott Williams & Wilkins; c2014. Antinuclear Antibodies (ANAS); 53 p.

13. Arthritis Foundation [Internet]. Atlanta: Arthritis Foundation; Rheumatoid Arthritis Diagnosis [cited 2018 Feb 28]; [about 4 screens]. Available from: https://www.arthritis.org/about-arthritis/types/ rheumatoid-arthritis/diagnosing.php

14. Sjogren’s Syndrome symptoms, available at https:// creakyjoints.org/symptoms/sjogrens-syndrome-symptoms/, assessed on 03/07/2020

Received on 09.07.2020 Modified on 11.08.2020

Int. J. Nur. Edu. and Research. 2020; 8(4):539-542.

DOI: 10.5958/2454-2660.2020.00120.9