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:
- 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 cough
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].
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].
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].
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
- Mavragani CP, Moutsopoulos HM. The geoepidemiology of Sjogren's syndrome. Autoimmunity Reviews 9 2010, A305-A310
- 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.
- 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
- Voulgarelis M, Tzioufas AG. Pathogenetic mechanisms in the initiation and perpetuation of Sjogren's syndrome. Nature Reviews Rheumatology 6 2010, 529-537.
- Nocturne G, Mariette X. Advances in understanding the pathogenesis of primary Sjögren's syndrome. Nature Reviews Rheumatology 9 2013, 544-556.
- 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.
- 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.
- 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.
- 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.