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Sjögren Syndrome

Sjoegrens Syndrome

Sjogren's syndrome is a systemic autoimmune disease characterized by lymphocytic infiltrates in exocrine organs. It is named after Swedish ophthalmologist Henrik Sjögren.


Presentation

The early symptoms of Sjogren's syndrome can be easily overlooked. The main clinical presentation in adults is xerophthalmia and xerostomia. In children, bilateral swelling of the parotid glands is a common sign. Xerophthalmia and xerostomia are often described in the following way by affected individuals:

In addition to these sicca symptoms, various extraglandular manifestations may develop, including arthralgia, arthritis, myalgia, Raynaud phenomenon, pulmonary disease, gastrointestinal disease, pericarditis, pulmonary hypertension, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, xeroderma, purpura, and urticaria. Late complications such as blindness, dental destruction, oral candidiasis, and non-Hodgkin lymphoma may occur [6].

Splenomegaly
  • On admission, she presented with anemia, elevated C-reactive protein levels, anasarca, and hepato-splenomegaly.[ncbi.nlm.nih.gov]
  • Clinical predictors of lymphoma development in SS include persistently enlarged parotid glands, with associated splenomegaly, lymphadenopathy, and cutaneous vasculitis (palpable purpura or leg ulcers).[rheumatologyadvisor.com]
  • For instance, reports suggest that patients with Felty's syndrome (splenomegaly and granulocytopenia linked to severe, long-standing seropositive RA) are at a more markedly (5- to 10-fold) increased risk ( 17, 24 ).[doi.org]
Fatigue
  • BMI, body mass index; VAS, Visual Analogue Scale (0–100, with 0 no fatigue/no pain at all); ProF, Profile of Fatigue (0–112, somatic fatigue 0–7, mental fatigue 0–7, with 0 no problem at all); VO 2max, maximal oxygen consumption; RPE, ratings of perceived[doi.org]
  • Fatigue was evaluated in the patients using the Fatigue Severity Scale (FSS) and a fatigue visual analogue scale (VAS); mood was evaluated using the Beck Depression Inventory (BDI).[ncbi.nlm.nih.gov]
Fever
  • The patient was admitted due to dyspnea, fever, polyarthralgia, and generalized edema.[ncbi.nlm.nih.gov]
  • Fever: axillary temperature 37.5 C, not associated with infectious processes. Lymphadenopathy: enlarged nodes ( 1 cm) in the cervical, axillary, or inguinal regions. Cutaneous vasculitis demonstrated by cutaneous purpura and/ or rash.[doi.org]
  • […] something is in the eye Mouth and throat symptoms: Difficulty swallowing or eating dry foods Loss of sense of taste Problems speaking Thick or stringy saliva Mouth sores or pain Teeth decay and gum inflammation Hoarseness Other symptoms may include: Fatigue Fever[nlm.nih.gov]
Anemia
  • On admission, she presented with anemia, elevated C-reactive protein levels, anasarca, and hepato-splenomegaly.[ncbi.nlm.nih.gov]
  • As a result, patients with Sjogren’s syndrome can present with a variety of symptoms These may include: Dry eyes and/or dry mouth Burning and numbness of the extremities Pain with sexual intercourse Anemia or low white blood count Although Sjogren’s syndrome[hopkinssjogrens.org]
  • Patients with the RA and Sjögren’s combination were also more likely to have blood abnormalities, including decreased numbers of platelets (thrombocytopenia) and decreased numbers of red blood cells (anemia).[rheumatoidarthritis.net]
  • Blood tests may reveal elevated erythrocyte sedimentation rate and transaminase levels, anemia, leukopenia, eosinophilia, hypergammaglobulinemia, presence of antinuclear antibodies, anti–alpha-fodrin antibody and rheumatoid factor.[symptoma.com]
Weight Loss
  • A 65-year-old woman with Sjögren's syndrome presented with recurrent abdominal distension, constipation, weight loss, orthostatic dizziness, loss of sweating and incomplete emptying of the bladder.[ncbi.nlm.nih.gov]
  • The following can all be symptoms of lymphoma: night sweats, fever fatigue unexplained weight loss Call your doctor if you have any of these symptoms.[healthline.com]
  • The criteria for qualifying for disability benefits based on Sjogren’s syndrome are: Two or more body systems or organs affected At least one system/function/organ affected to a moderate or severe level At least two of the following symptoms: weight loss[disability-benefits-help.org]
  • Unexplained weight loss. Itchy skin. Reddened patches on the skin. Many of these can be symptoms of other problems, including Sjögren’s syndrome itself. Nevertheless, it is important to see your doctor if you have any of these symptoms.[niams.nih.gov]
Chronic Fatigue Syndrome
  • Komaroff, The Frequency of HLA Class II Antigens in Chronic Fatigue Syndrome, Journal of Chronic Fatigue Syndrome, 11, 4, (33), (2003). Juan-Manuel Anaya, Paula A. Correa, Ruben D.[doi.org]
  • That's because the symptoms can overlap or look like those of other conditions, including chronic fatigue syndrome, fibromyalgia, lupus, rheumatoid arthritis, or even multiple sclerosis.[stlouischildrens.org]
Cough
  • Unexplained cough was defined as chronic cough of unknown etiology despite algorithm-based evaluation and treatment.[ncbi.nlm.nih.gov]
  • Chronic dry cough. Thyroid problems. Joint and muscle pain. Vaginal dryness. Numbness and tingling in the arms and legs. Fatigue or feeling very tired. People with dry mouth can easily get mouth infections.[niams.nih.gov]
  • Lung involvement: persistent cough and/or dyspnea, with chronic diffuse interstitial infiltrates on X-ray, altered pattern on pulmonary function studies, and/or evidence of pulmonary alveolitis/fibrosis in computed tomography scan.[doi.org]
Dry Cough
  • (The most frequent reason, in 80%, was persistent, dry cough periodically waking up patients at night). The chest HRCT scan revealed lung tissue changes in the course of 29% of all examined patients (of 68).[ncbi.nlm.nih.gov]
  • Other symptoms can include joint swelling and pain, vaginal dryness, persistent dry cough and dry skin. It may also spread to other parts of the body such as blood vessels, nerves, lungs, kidneys and digestive system.[coloradoarthritis.com]
  • […] described in the following way by affected individuals: Unable to eat dry foods Tongue sticks on the roof of the mouth Unable to speak for long period Increased cases of dental caries Change in tastes Painful mouth Sandy sensation in the eyes Persistent, dry[symptoma.com]
  • cough Prolonged fatigue Causes Sjogren's syndrome is an autoimmune disorder.[mayoclinic.org]
Hoarseness
  • Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating.[ninds.nih.gov]
  • Eye symptoms: Itching eyes Feeling that something is in the eye Mouth and throat symptoms: Difficulty swallowing or eating dry foods Loss of sense of taste Problems speaking Thick or stringy saliva Mouth sores or pain Teeth decay and gum inflammation Hoarseness[nlm.nih.gov]
  • You may have a dry cough, hoarseness, a decreased sense of smell, and nosebleeds. It can also lead to pneumonia, bronchitis, and ear problems. Dryness of the vagina. This can cause painful intercourse. Fatigue. is a common complaint.[healthcentral.com]
  • Protect Your Voice You can develop hoarseness if their vocal cords become inflamed or become irritated from throat dryness or coughing. To prevent further strain on your vocal cords, try not to clear your throat before speaking.[niams.nih.gov]
Chronic Cough
  • Unexplained cough was defined as chronic cough of unknown etiology despite algorithm-based evaluation and treatment.[ncbi.nlm.nih.gov]
  • Frequency (%) Arthralgia or nonerosive arthritis characterized by tenderness, swelling, or effusion of peripheral joints 37 to 75 Gastrointestinal symptoms (reflux, dyspepsia, diarrhea, constipation) 54 Autoimmune thyroiditis 15 to 33 Pulmonary disease (chronic[aafp.org]
  • cough, numbness in the arms and legs, feeling tired, muscle and joint pains, and thyroid problems. [4] Those affected are at an increased risk (5%) of lymphoma. [2] [7] While the exact cause is unclear, it is believed to involve a combination of genetics[en.wikipedia.org]
Constipation
  • A 65-year-old woman with Sjögren's syndrome presented with recurrent abdominal distension, constipation, weight loss, orthostatic dizziness, loss of sweating and incomplete emptying of the bladder.[ncbi.nlm.nih.gov]
  • Manifestations of Sjögren Syndrome Clinical signs and symptoms Frequency (%) Arthralgia or nonerosive arthritis characterized by tenderness, swelling, or effusion of peripheral joints 37 to 75 Gastrointestinal symptoms (reflux, dyspepsia, diarrhea, constipation[aafp.org]
Abdominal Pain
  • 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[rheumatology.org]
Xerostomia
  • Eye dryness and xerostomia were assessed by the Schirmer's test, the Fox test and the visual analogue scale (VAS).[ncbi.nlm.nih.gov]
Dental Caries
  • The effect of saliva on dental caries. J Am Dent Assoc. 2008; 139 (Suppl):11S–17S. doi: 10.14219/jada.archive.2008.0347. [ PubMed ] [ Cross Ref ] 23. Hurlbutt M, Novy B, Young D. Dental Caries: A pH-mediated disease. CJDH. 2010; 25 (1):9–15. 24.[web.archive.org]
  • The effect of saliva on dental caries. J Am Dent Assoc. 2008;139(Suppl):11S–7S. View Article PubMed Google Scholar Hurlbutt M, Novy B, Young D. Dental Caries: A pH-mediated disease. CJDH. 2010;25(1):9–15.[doi.org]
  • The effect of saliva on dental caries. J Am Dent Assoc. 2008; 139 (Suppl):11S–17S. doi: 10.14219/jada.archive.2008.0347. [ PubMed ] [ CrossRef ] [ Google Scholar ] 23. Hurlbutt M, Novy B, Young D. Dental Caries: A pH-mediated disease.[ncbi.nlm.nih.gov]
Cracked Lips
  • Using oil or petroleum-based lip balm or lipstick to help dry, cracked lips feel better. Using a saliva substitute prescribed by a doctor to make the mouth feel wet. Using medicine to help your mouth make more saliva.[web.archive.org]
  • lips that may peel Dry skin Dry hair Difficulty chewing Difficulty swallowing Esophagitis Reflux Chronic or recurrent bronchitis Pneumonia Pleurisy Interstitial lung disease Malabsorption B12 and other vitamin deficiencies Stomach upset Gastroparesis[migraine.com]
Cheilitis
  • Other frequent complaints are angular cheilitis, simple cheilitis ( Figures 1 and 2 ), exfoliative cheilitis, lip cracking and dryness ( Table 2 ).[ncbi.nlm.nih.gov]
  • Oral candidiasis and angular cheilitis. Dry eyes tend to cause a gritty sensation. There is a predisposition to blepharitis and the eyes may be sticky in the morning.[patient.info]
  • Clotrimazole or nystatin cream can be used if angular cheilitis is present.[rheumatologyadvisor.com]
  • PMID 28957550. v t e Oral and maxillofacial pathology ( K00–K06, K11–K14, 520–525, 527–529 ) Lips Cheilitis Actinic Angular Plasma cell Cleft lip Congenital lip pit Eclabium Herpes labialis Macrocheilia Microcheilia Nasolabial cyst Sun poisoning Trumpeter's[en.wikipedia.org]
Sore Mouth
  • Other symptoms may include: Contact lens discomfort Dry sinuses and frequent sinus infections or nosebleeds Sore mouth, tongue or throat Difficulty chewing or swallowing Dry or peeling lips Blepharitis Dry and sore skin Muscle pain without accompanying[allaboutvision.com]
Arthralgia
  • The most difficult challenge in diagnosis and therapy is patients with symptoms of fibromyalgia (arthralgia, myalgia, fatigue) and oral and ocular dryness in the presence of circulating antinuclear antibodies.[ncbi.nlm.nih.gov]
  • The clinical manifestations evaluated were defined as follows: Articular involvement: arthralgia and/or nonerosive arthritis characterized by tenderness, swelling, or effusion involving 2 or more peripheral joints.[doi.org]
  • Its use in SS patients as in SLE patients has been effective in decreasing arthralgia, myalgia, rash, and lymphadenopathy.[rheumatologyadvisor.com]
  • […] mouth Unable to speak for long period Increased cases of dental caries Change in tastes Painful mouth Sandy sensation in the eyes Persistent, dry cough In addition to these sicca symptoms, various extraglandular manifestations may develop, including arthralgia[symptoma.com]
Dry Skin
  • Use unscented moisturizers to help relieve dry skin. This condition is generally benign. However, people with severe cases are at increased risk for developing non-Hodgkin’s lymphoma . This is a cancer of the white blood cells.[uvahealth.com]
  • A chronic autoimmune disease like Sjögren's syndrome can affect the entire body, causing dry eyes, dry mouth, dry skin, dental decay, joint pain, extreme fatigue, memory loss, pneumonia and abnormal liver function, among other things.[hospitals.jefferson.edu]
  • For dry skin: Use moisturizing lotions for sensitive skin. Avoid drafts from air conditioners, heaters, and radiators, when possible. Use a humidifier in your house and at work.[healthcentral.com]
  • Other symptoms can include joint swelling and pain, vaginal dryness, persistent dry cough and dry skin. It may also spread to other parts of the body such as blood vessels, nerves, lungs, kidneys and digestive system.[coloradoarthritis.com]
  • There can be dry skin and vaginal dryness causing dyspareunia. Other skin features include purpura and annular erythema.[patient.info]
Urticaria
  • […] manifestations may develop, including arthralgia, arthritis, myalgia, Raynaud phenomenon, pulmonary disease, gastrointestinal disease, pericarditis, pulmonary hypertension, lymphadenopathy, neuropathy, vasculitis, renal tubular acidosis, xeroderma, purpura, and urticaria[symptoma.com]
  • DH in pediatric patients presents as urticaria, and more commonly purpura involving the soles, palms, and sides of the fingers.[dx.doi.org]
  • Gastrointestinal disease Leukopenia Anemia Lymphadenopathy Neuropathy Vasculitis Renal tubular acidosis Lymphoma About 50% of patients with Sjögren syndrome have cutaneous findings, such as dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria[emedicine.com]
Dry Eyes
  • ., systemic lupus erythematosus, rheumatoid arthritis), is a critical etiologic factor in the pathogenesis of dry eye syndromes.[ncbi.nlm.nih.gov]
  • The hallmark symptoms of the disorder are dry mouth and dry eyes.[ninds.nih.gov]
  • Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder.[nlm.nih.gov]
  • Topical cyclosporin A is approved by the US Food and Drug Administration (FDA) as a treatment of dry eye.[emedicine.com]
Lacrimation
  • We further hypothesize that androgen treatment to the ocular surface will promote both lacrimal and meibomian gland function and alleviate both "aqueous-deficient" and "evaporative" dry eye.[ncbi.nlm.nih.gov]
  • […] in 35% of normal lacrimal glands.[emedicine.com]
Blepharitis
  • Private practitioners were more likely to use symptom questionnaires and grading scales and to describe anterior blepharitis. Academic settings were more likely to record TBUT and tear meniscus height.[ncbi.nlm.nih.gov]
  • Evaporation of the water from the tear film can increase due to blepharitis (inflammation of the eyelid), decreased production by the oil glands of the eyelids, or thyroid disease.[hopkinsmedicine.org]
  • Your GP can check for some of the more common causes, such as swelling of the eyelids (blepharitis), diabetes or medication.[nhs.uk]
  • There may be blepharitis. The mouth may look dry and a wooden tongue depressor may stick to the tongue. There may be evidence of infection including oral candidiasis and dental caries.[patient.info]
Blurred Vision
  • Others are forced to cope with blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid glands, hoarseness, and difficulty in swallowing and eating.[ninds.nih.gov]
  • Some individuals also experience blurred vision and heightened light sensitivity.[health.facty.com]
  • Complications Dryness of the eyes may cause light sensitivity, blurred vision and in severe cases may lead to corneal ulcers. Similarly severe dryness of the mouth can cause dental cavities and yeast infections in the mouth.[coloradoarthritis.com]
  • This change of the tear film results in irritation and changes on the eye surface that lead to characteristic symptoms, such as a sandy or burning sensation, discomfort, blurred vision, and redness of the eye that progresses as the day goes on.[hopkinsmedicine.org]
  • Dry eyes can lead to light sensitivity, blurred vision and corneal damage. Less common complications might affect: Lungs, kidneys or liver.[mayoclinic.org]
Lacrimal Gland Enlargement
  • Definition (CSP) symptom complex of unknown etiology, usually occurring in middle-aged or older women, marked by the triad of keratoconjunctivitis sicca with or without lacrimal gland enlargement, xerostomia with or without salivary gland enlargement,[fpnotebook.com]
  • gland enlargement, xerostomia with or without salivary gland enlargement, and the presence of a connective tissue disease, usually rheumatoid arthritis but sometimes systemic lupus erythematosus, scleroderma, or polymyositis; an abnormal immune response[icd9data.com]
  • History The first clinical description of Sjögren syndrome was by Mikulicz, who described a 42-year-old with bilateral parotid and lacrimal gland enlargement in 1892.[eyewiki.org]
  • Historical information The first clinical description of Sjögren syndrome was by Mikulicz, who described a 42-year-old with bilateral parotid and lacrimal gland enlargement in 1892.[emedicine.com]
Paresthesia
  • Peripheral neuropathy: paresthesias, numbness, and/or motor defects of the lower/upper extremities confirmed by electromyography. Sural nerve biopsy showing vasculitis involving vasa nervorum when performed.[doi.org]
  • ) 3 (16.6%) – Non-specific ulcerations 2 (9.1%) 4 (22.2%) 1 (6.7%) Small aphtae 3 (13.6%) 2 (11.1%) 1 (6.7%) Sutton's aphtae 1 (4.5%) – – Geographic tongue – 2 (11.1%) 1 (6.7%) Fissured tongue 1 (4.5%) – – Enlargement of salivary glands 2 (9.1%) – – Paresthesias[ncbi.nlm.nih.gov]
Vaginal Dryness
  • In addition, Sjogren's syndrome may cause skin, nose, and vaginal dryness, and may affect other organs of the body including the kidneys, blood vessels, lungs, liver, pancreas, and brain.[ninds.nih.gov]
  • There are vaginal lubricants available on an over-the-counter basis for women who experience vaginal dryness. These lubricants may ease vaginal dryness. Vaginal lubricants may also be considered for sexual intercourse.[disabled-world.com]
  • For vaginal dryness: Use lubricants made specifically to help vaginal dryness. Do not use petroleum jelly. Exercise and therapy Mild exercise such as walking or swimming can help keep joints and muscles flexible.[orthop.washington.edu]
  • Other symptoms can include joint swelling and pain, vaginal dryness, persistent dry cough and dry skin. It may also spread to other parts of the body such as blood vessels, nerves, lungs, kidneys and digestive system.[coloradoarthritis.com]

Workup

No single examination is sufficiently sensitive or specific in the diagnosis of the disorder. Blood tests may reveal elevated erythrocyte sedimentation rate and transaminase levels, anemia, leukopenia, eosinophilia, hypergammaglobulinemia, presence of antinuclear antibodies, anti–alpha-fodrin antibody and rheumatoid factor. Rose Bengal test measures the function of the lacrimal glands and Schirmer's test the production of tears.

Radiological procedures such as sialography and scintigraphy or a salivary gland biopsy may be recommended to aid in the diagnosis of Sjogren’s syndrome [7] [8] [9].

Schirmer's Test Abnormal
  • BOX New classification criteria (modified according to [20]) Current European–American consensus criteria for the classification of primary Sjögren’s syndrome Unstimulated salivary flow rate* 1 abnormal 0.1 mL/minute (1 point) Abnormal Schirmer’s test[ncbi.nlm.nih.gov]
Lymphocytic Infiltrate
  • The clinical presentation of SjS is heterogeneous and can vary from sicca symptoms to systemic disease (characterized by peri-epithelial lymphocytic infiltration of the affected tissue or the deposition of the immune complex) and lymphoma.[ncbi.nlm.nih.gov]
  • Sjogren's syndrome is a chronic inflammatory disorder characterized by salivary insufficiency and lymphocytic infiltrates in exocrine organs.[symptoma.com]
HLA-DR3
  • We found significantly increased frequency of HLA-DR3 (P less than .001), HLA-DW52a (P less than .001), and HLA-DQA4 (P less than .05), in comparison to normal controls.[ncbi.nlm.nih.gov]
  • Among the 1 SS patients, clinical and laboratory features such as hypergammaglobulinemia, symmetric peripheral neuropathy, and hypothyroidism were significantly associated with HLADR3 ( P .01) but not with HLA‐DR2 ( P .10).[doi.org]
  • The association of Sjögren syndrome with human leukocyte antigen (HLA)–B8, HLA-Dw3, HLA-DR3, and the DQA1*0501 allele supports the notion of genetic susceptibility. [16] An active area of research involves genetic polymorphisms of cytokine genes, including[emedicine.medscape.com]
HLA-B8
  • The association of Sjögren syndrome with human leukocyte antigen (HLA)–B8, HLA-Dw3, HLA-DR3, and the DQA1*0501 allele supports the notion of genetic susceptibility. [16] An active area of research involves genetic polymorphisms of cytokine genes, including[emedicine.medscape.com]
HLA-Dw3
  • The association of Sjögren syndrome with human leukocyte antigen (HLA)–B8, HLA-Dw3, HLA-DR3, and the DQA1*0501 allele supports the notion of genetic susceptibility. [16] An active area of research involves genetic polymorphisms of cytokine genes, including[emedicine.medscape.com]

Treatment

Treatment of the disorder is generally symptomatic and supportive. It depends on the manifestations and varies for every patient. Moisturizing treatments, nonsteroidal anti-inflammatory drugs and low-dose glucocorticoids are most commonly used.

Prognosis

The prognosis of primary Sjogren's syndrome is generally good, but for secondary cases, the prognosis is closely related to the associated disease.

Etiology

Sjogren's syndrome exists as a primary or as a secondary disease. The two forms occur in similar frequency. The secondary form of the disease is associated with disorders such as system lupus erythematosus, polyarteritis nodosa, scleroderma, cryoglobulinemia, and systemic sclerosis. Regardless of whether the syndrome is primary or secondary, the parotid glands, the lungs, eyes, the mouth, the nervous systems, and the skin are affected.

The exact etiology of the disease is currently unknown. So far, it has been established that the pathogenesis is multifactorial. Environmental and hormonal factors are thought to trigger inflammation in individuals with a genetic predisposition to the disorder.

Viruses have been poised as being the number one candidates in possible environmental triggers. Some of the viruses that may play a role include HIV, HTLV-1, human hepersvirus 6, Epstein-Barr virus, cytomegalovirus, and hepatitis C virus [3].

Epidemiology

Sjogren's syndrome is ranked as the second most common rheumatologic disorder behind systemic lupus erythematosus. The disorder affects about 0.1% to 4% of the population in the United States. The wide range reflects the lack of a standard diagnosis method [2].

Globally, the disease has shown to remain homogenous among various ethnic groups and environments. The majority of cases occur in individuals above 40 years of age and mainly women are affected [1].

Sex distribution
Age distribution

Pathophysiology

Sjogren's syndrome is a chronic inflammatory disorder characterized by salivary insufficiency and lymphocytic infiltrates in exocrine organs. Even though the mechanism of its pathology has been studied since the discovery, the roles of different populations of immune cells in the pathogenesis remain inconclusive [4] [5].

Prevention

There are no guidelines for prevention of Sjogren's syndrome.

Summary

Sjogren's syndrome is a chronic inflammatory disorder marked by salivary insufficiency and lymphocytic infiltrates in exocrine organs. It is a relatively common disease, although often under-diagnosed.

In 1933, Dr. Henrik Sjögren published an article describing a group of women in a small Swedish town presenting with keratoconjunctivitis, lymphoid infiltrations of the conjunctiva, cornea, lacrimal glands, and parotid glands, a history of arthritis, swelling of the salivary glands, and dryness of the oronasopharynx. Two years later the observation was connected with Mikulicz’s disease and together formed the basis for this syndrome. In 1936, Duke Elder honored Sjögren by naming the disease Sjogren’s syndrome.

The disorder can occur either primary or secondary. Secondary Sjogren's syndrome appears several years after other associated disorders such as scleroderma, rheumatoid arthritis, and primary billiary cirrhosis.

Sjogren's syndrome is common among women above the age of 40 years. The female-to-male ratio is 9:1. There are no geographical differences in the prevalence of Sjogren's syndrome [1].

The disorder is complicated by the fact that there is no sufficiently sensitive or specific test in the diagnosis of Sjogren's syndrome. Moreover, so far there is no known cure for the disease. Symptomatic treatments control the severity of the symptoms associated with the disease.

Patient Information

Once diagnosed with Sjogren's syndrome, the main challenge is not having the disorder, but not knowing what to do next to live positively with it. The good news is that Sjogren’s syndrome does not have to impede your normal lifestyle.

Several remedies are used to treat Sjogren’s syndrome effects. Artificial tears are effective in remedying against dry eye. Tears in normal eyes are drained through tear ducts. Through surgery, these ducts can be sealed with small plugs. Therefore, the little tears formed in the eyes are not drained hence stay for longer. The process is known as punctual occlusion and it is recommended only after the other possible alternatives have failed.

Possible treatments for dry mouth:

  • Consumption of more liquid
  • Suckling of ice cubes that keeps the mouth moist and lubricated
  • Regular rising of the mouth to protect against infections as well as sooth the mouth
  • Maintenance of good oral hygiene to prevent tooth decay and gum diseases
  • Stop smoking for those who smoke as smoking evaporates the mouth as well as causes an irritation
  • Chewing of sugar free gums which stimulates the production of saliva
  • Use of saliva substitutes

Vaginal dryness can be treated with lubricants, hormone replacement therapy, and estrogen creams. Joint and muscle aching can be relieved with non-steroidal anti-inflammation drugs. Systemic corticosteroids or immunosuppressive agents have been used for various extraglandular symptoms, such as vasculitis, lung involvement and kidney involvement.

References

Article

  1. Mavragani CP, Moutsopoulos HM. The geoepidemiology of Sjogren's syndrome. Autoimmunity Reviews 9 2010, A305-A310
  2. Helmick CG, Felson DT, Lawrence RC, et al. Estimates of the prevalence of arthritis and other rheumatic conditions in the United States. Part I. Arthritis Rheum. Jan 2008;58(1):15-25.
  3. Jonsson R, Vogelsang P, Volchenkov R, Espinosa A. The complexity of Sjogren's syndrome: Novel aspects on pathogenesis. Immunol. Lett. 141 2011, 1; 1-9; 9
  4. Voulgarelis M, Tzioufas AG. Pathogenetic mechanisms in the initiation and perpetuation of Sjogren's syndrome. Nature Reviews Rheumatology 6 2010, 529-537.
  5. Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nature Reviews Rheumatology 9 2013, 544-556.
  6. Tzioufas AG, Voulgarelis M. Update on Sjögren's syndrome autoimmune epithelitis: from classification to increased neoplasias. Best Pract Res Clin Rheumatol. Dec 2007;21(6):989-1010.
  7. Montaño-Loza AJ, Crispín-Acuña JC, Remes-Troche JM, Uribe M. Abnormal hepatic biochemistries and clinical liver disease in patients with primary Sjögren's syndrome. Ann Hepatol. Jul-Sep 2007;6(3):150-5.
  8. Daniels TE. Labial salivary gland biopsy in Sjögren's syndrome. Assessment as a diagnostic criterion in 362 suspected cases. Arthritis Rheum. Feb 1984;27(2):147-56.
  9. García-Carrasco M, Ramos-Casals M, Rosas J, et al. Primary Sjögren syndrome: clinical and immunologic disease patterns in a cohort of 400 patients. Medicine (Baltimore). Jul 2002;81(4):270-80.

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Last updated: 2019-07-11 21:33