- 1 Definition
- 2 Disease Cause
- 3 Symptoms and findings of medical examinations
- 4 The doctor's investigation
- 5 Complications
- 6 Classification criteria
- 7 Incorrect diagnosis? (Similar diseases / differential diagnoses)
- 8 Treatment
- 9 Diet and lifestyle
- 10 Pregnancy
- 11 Medical prognosis
- 12 Control and follow-up of rheumatologist
- 13 Referral to specialist
- 14 Journal writing at Sjögren's syndrome
- 15 Medical examinations at Sjögren's syndrome
- 16 Ultrasound of salivary glands at Sjögrens syndrome
- 17 Fatigue Severity Score (FSS)
- 18 Guidelines, Criteria and Misc Links here (EMEUNET)
- 19 Diet in rheumatic diseases
- 20 Guidelines
- 21 Literature
Sjögren's syndrome is a chronic rheumatic disease characterized by
- Daily dryness
- Dry eyes
- Dry mouth and tongue
- Often other dry mucous membranes (skins)
- Fatigue and exhaustion with increased need of sleep
- Rheumatic joint pain
- Severe rash in blood tests (ANA and SSA / Ro antibody)
- Women are affected more than ten times more frequently than men
The disease is rare among children. Dryness is very common at high age, but often caused by other causes than Seagrass Syndrome. The disease reduces the quality of life, but is rarely the cause of reduced life expectancy.
- Most feared complication is lymph node cancer (Lymphoma) that affects 5-10% with Sjøgren's syndrome during life (reference: Jonsson MW, 2012)
Seagrass syndrome is described among all breeds. Gender distribution: 10-20 times more common among women. The prevalence in the population is between 0,05 and 1% when using US / EU classification criteria (see below) (Göransson LG, 2011). Not all feeling of dry mucous membranes is due to Sjøgren's disease. The incidence of Sjøgren's syndrome among patients with dryness symptoms is 5-15%. The disease is most often diagnosed at the age of 40-55 years. Debut before the age of 31 is seen in 15%. Children (3-9 years) can also get the disease in the form of juvenile Sjøgren's syndrome.
Primary Sjögren's syndrome
In the case of primarily Sjögren's syndrome, no other underlying symptomatic explanatory disease exists
Secondary Sjögren's syndrome
Secondary Seagrass Syndrome is characterized by the symptoms occurring after another systemic Autoimmune disease are diagnosed and that there is a probable link between them.
- Combination with SLE, Systemic sclerosis, Rheumatoid arthritis, MCTD, Myositisor Autoimmune liver disease, autoimmune thyroid disease (metabolism) may occur.
- Criteria for classification is described here (in Danish)
Symptoms and findings of medical examinations
Daily dryness and feeling of sand on the eyes. Relief when using “artificial tear fluid” from pharmacy. Other symptoms of dry eyes are itching, burning sensation, varying visual acuity and red eyes.
- The ophthalmologist finds signs that tears and tear film does not cover and protects the eye properly and may find damage to the cornea (color score ≥ 3). Schirmer's test demonstrates reduced lacrimal gland function (less than 5mm of moisture within 5 minutes indicates reduced function. Results between 5mm and 10mm are limit values.
- The tear fluid has often changed the composition of Sjøgren's syndrome, resulting in a reduced ability to "lubricate" and protect the eyes. The cause is often reduced function in Meibomian glands which should add oil and fat to the eye. An ophthalmologist can assess the lubricating function of the tear fluid (Break up time) and the Meibomian glands.
Dryness in the mouth also during the day. One must drink to get swallowed dry food (biscuits, crispbread). Dry mucous membranes in the mouth are detected by inspection and examination.
- Measurement of saliva production ("Sialometry") unstimulated over 15 minutes typically shows low values (<1,5 ml / 15 min). However, the test is not very accurate
- Tissue specimen (biopsy) from small salivary glands from inside the lower lip often demonstrates typical Sjøgren changes in the form of rheumatic inflammation (lymphocyte infiltrations and "focus cuts" ≥ 1)
- The risk when taking biopsy is infection of the wound and sustained reduction of nerve damage in part of the lip. A Finnish study showed that nerve damage occurred in one of 191 biopsy cases (0,5%) (reference: Teppo H, 2007). The information page at a large US center indicates (in 2019) that the risk of numbness lasting for 2-3 months is 1-XNUM% (Johns Hopkins Hospital)
- Reduced sense of smell and taste, bad breath and burning sensation in the mouth are also more common ailments at Sjøgren's syndrome than among healthy ones. The symptoms can affect what you choose to eat (diet and nutritional intake) (reference: Singh PB, 2019)
- Clinical examination of the mouth can show if there are signs of candidiasis (fungal) infection that worsens the symptoms. Symptoms compatible with «burning mouth syndrome»Should also be excluded
Dryness of the vagina
Sjøgren's syndrome often causes glands in the vagina to produce less secretions. This causes dryness problems in many women.
- SSA is short for Sjøgren's Syndrome A and SSB for Sjøgrens Syndrome B.
Many also have elevated immunoglobulin "IgG" and blood lowering reaction (SR) as an expression of increased activity in the immune system. White blood cells, lymphocytes and complement factors C3 and C4 may be lower than normal.
The doctor's investigation
The doctor checks the salivary glands and throat for swelling and noticeable lymph nodes. The oral cavity, joints, lungs and other organs are assessed in more detail for current symptoms.
Blood tests are checked. Rash in antibody SSA (Sjøgrens Syndrome A) is common. Some also have high IgG and / or low numbers of a type of white blood cell (lymphocyte).
- You can prepare yourself for the medical examination with more information here (in Danish).
Serious complications at Sjøgren's syndrome are rare, but some can be attacked.
Most feared is the increased risk of developing lymphoma.
- A common symptom is a persistent and increasing hard swelling of a salivary gland (gland in front of the ears or submandibula below the jaw angle) or strikingly large and growing lymph nodes on the neck. Other locations are less common.
Although the fewest (5-10%) with Sjøgrens' disease get lymphoma, an annual medical check-up is recommended among those who have signs of active disease. A rheumatologist can assess the individual risk factors in more detail (please read more under the section on controls and follow-up below). Research indicates that approximately 17% have lymphoma that occurs before Sjøgren's syndrome is diagnosed. These lymphomas usually do not differ from lymphoma later in the course and are probably part of the disease (reference: Vasaitis L, 2019). The most common type of lymphoma in Sjøgren's syndrome is extra-nodal non-Hodgkin's B-cell lymphoma in mucosa-associated lymphoid tissue (MALT lymphoma) (reference: Nocturne G, 2019)
About one in five (20%) develops lung changes (reference: Palm, O 2013), but the complication is rarely severe and usually does not require treatment.
- CT images are the best method of examining lung symptoms (persistent dry cough, striking heavy breathing). Most typically are cavities (cysts) that represent lymphocytic interstitial pneumonia (LIP).
Urinary tract and kidneys
Frequent urination without the detection of urinary tract infection or antibiotics may be due interstitial cystitis which is more common at Sjøgren's syndrome than in the general population. The diagnosis is made by examining the urinary bladder by urologist (reference: Emmungil H, 2012).
Nephritis (Renal inflammation)
Renal disease at Sjøgren's syndrome is rarely detected. However, mild forms can be difficult to detect. Kidney diseases can be Glomerulonephritis (symptoms: traces of protein and blood in the urine) and tubular interstitial nephritis. In tubular interstitial nephritis, the disease mechanism is complicated. There is reduced acid secretion and increased urinary potassium excretion. In the blood, the acid level (acidosis) increases and potassium becomes low. A so-called anion gap is present, which is a description of the difference between the blood concentration of Na+ions on one side, and the sum of chlorine (Cl-) and bicarbonate (HCO3-) -ions on the other hand. Normally, the gap is 12-16 mmol / l (reference: SNL, XNUM). In the disease process, calcium is mobilized from the skeleton.
- Calcium is then excreted via the kidneys, resulting in the risk of kidney stones (calcium stones). The treatment is then Potassium citrate tablets so that the acidosis is corrected.
No other rheumatic disease has had as many different criteria for diagnosis (and classification) as Sjøgren's disease. In principle, the classification criteria should be used for research, but are also often used in practice to confirm the diagnosis. When new criteria replace the former, some patients with the diagnosis will no longer satisfy the most relevant criteria, which can be frustrating.
- Since 2002 has US / EU criteria been clearly the most widely used.
- In 2016 new ACR / EULAR criteria (follow the link below) were published. In these kidney criteria, previously diagnosed lymphoma does not prevent Sjøgren diagnosis. Probably the academic community will use these in research studies and to some extent also to make the Sjøgren diagnosis in clinical practice going forward.
Both sets of criteria require the presence of either SSA antibody (Ro) in the blood sample or typical changes in the tissue sample (biopsy).
Most people with Sjøgren's syndrome use artificial tear fluid, for example Artelac or similar several times daily. Viscotear gel is often used in the evening or at night. Several types of artificial tear fluid are available on a "blue prescription" (code L40) for reduced tear production and a definite diagnosis. The pharmacy can provide further information. Specialist (ophthalmologist or rheumatologist) prescribes for the first time, GP can renew prescriptions.
- Some people with a lot of ailments benefit from ciclosporin eye drops (Ikervis). The indication should be made by the ophthalmologist
- Mini-Scleral contact lenses can also reduce dryness (reference: Harthan JS, 2018)
- A few benefit from stopping the drainage of tear fluid from the eyes by sealing the tear ducts. There are various methods that are performed by the ophthalmologist
It is important to have good oral and dental hygiene because teeth are easily damaged (caries) when too little spit is produced
- Water is often drunk and in small slugs at a time
- Sugar-free pastilles, sugar-free chewing gum and fluoride tablets will stimulate residual saliva production
- Avoid much sugar
- Use toothpaste that does not foam (Zendium)
- Avoid toothpaste containing sodium lauryl sulfate (soap)
- Mouthwash containing alcohol provides dry mucous membranes and should not be used
- NycoDent Saliva (Sugary tablets) contain malic acid that can stimulate saliva production and xylitol that counteract bacteria and "lubricate" mucous membranes
- XyliMelts lozenge tablets is useful to anyone
- Biotene mouth spray contains glycerin which can add moisture
- Those who use dental prostheses should clean them regularly
- Food should be chewed thoroughly and long before swallowing because dryness in the throat can cause larger bites to get stuck
In case of severe large tooth injuries and certainly reduced saliva production (hyposalivation), the dentist may apply for Coverage of expenses from NAV.
Dryness of the vagina
For symptoms of vaginal dryness, medical examination is recommended. Treatment may consist of substitution with estrogens, either as a vaginal tablet (for example, "Vagifem") or creams (for example, "Ovesterine"). Use of lubricant is also relevant. The GP, gynecologist and pharmacy can give further advice.
Most people with Sjøgren's syndrome are not used to using drugs regularly. It is uncertain whether immunosuppressive drugs help with dryness symptoms, fatigueOr the prognosis of the disease. In some cases, however, such drugs are used, although Sjøgren's syndrome is not among the indications that the manufacturers have approved.
- Plaquenil (hydroxychlorokine, tablets 200mg x 1)
- Effects on joint pain have been reported, but not well documented effect on dryness or fatigue
- Methotrexate (tablets or injections)
- By Arthritis (arthritis-like course)
- Azathioprine (azathioprine tablets)
- On special indications. May increase the risk of lymphoma
- MabThera / Rixathon (rituximab intravenously)
- Uncertain effect on the most common symptoms. Can be tried in severe systemic disease (in several organs) when other treatment has not been sufficient. Effect is expected on symptoms associated cryoglobulinemia which exists in a few.
- Benlysta (belimumab intravenously)
- Uncertain effect. Can be tried as in severe systemic disease (in several organs without effect of rituximab)
- Reference: Ramos-Casals M, 2019 (EULAR recommendations)
DHEA og evening primrose has been used previously, but surveys have not confirmed safe effect, and popularity has slowed. There has been a lot of interest recently LDN (low dose naltrexone), but documentation of effect is lacking. Salagen (pilocarpine) and Evoax (cevilemine) tablets can increase saliva production if a residual function is present, but the drugs can have side effects such as sweating, palpitations, nausea and loose stools. Salagen and Evoax are not approved for normal use in Norway. A doctor can apply to the Norwegian Medicines Agency for approval for use in each individual case.
- Reference: Ramos-Casals M, 2019 (EULAR recommendations)
Diet and lifestyle
Sjögren's syndrome hardly affected by special diet. However, one should be aware that coffee can be uncomfortable on the mucous membranes of the mouth and throat. And alcohol can contribute to increased dryness.
Dryness in the mouth and throat requires a drink for each meal. The food should be chewed carefully and not too spicy. Dry and hard foods should be soaked, for example, in sauces, dressing or in milk. Dry mouth greatly contributes to the risk of tooth decay and gum damage. Sugar and sugary drinks enhance the risk of caries. Juice and some fruits and vegetables may also contain adverse acidity. Thirst aggravates the symptoms. Ample drinks (water) are important for the fluid balance. Someone with Sjøgren's syndrome has intolerance to milk and milk products or Celiac Disease (pain and air complaints from the intestine, loose stools) and should avoid milk and gluten-containing foods respectively (reference: Liden M, 2007).
Many with Sjøgren's syndrome are afflicted with striking fatigue. Large, high-fat meals can aggravate these symptoms. Small and frequent meals provide smoother blood sugar and fewer fatigue attacks. Regular physical activity (walking, cycling, swimming, yoga, tai chi) and 78 hours of sleep each night should be strived.
Normally saliva helps neutralize stomach acid. At Sjøgren's disease, much of the saliva is lacking, and ailments from stomach acid can occur. Acid-inhibiting drugs (proton pump inhibitors) can help. Highly spicy foods can also irritate the stomach and should be avoided.
In rare cases, the pancreas (pancreas) can be attacked and develop impaired function. Blood samples can show high enzymes as signs of inflammation (amylase, lipase). Symptoms can be loose, malodorous stools and weight loss. This should then be examined by a doctor. Intake of pancreatic enzymes and adapted diet (consult with nutritionist) may correct the problem.
- Diet, oral and dental health here (supervisor The Norwegian Directorate of Health)
- In general about diet for rheumatic diseases here: diet
- Please read about Sjögren's syndrome and pregnancy here (in Danish)
- Generally about pregnancy in rheumatic disease here (in Danish)
- More info here (in Danish) (National Competence Center, St Olavs, Trondheim)
Most people with Sjögren's syndrome have a normal life span. Symptoms of dryness and fatigue / exhaustion however, often persists over many years and reduces the quality of life (reference: Enger TB, 2011).
Control and follow-up of rheumatologist
Those most prone to lymphoma (lymphoma) have high levels antibody high disease activity, low white blood cell counts (especially lymphocytes) and low complements (C3, C4) in blood samples and / or ectopic germinal centers in salivary gland biopsy (tissue sample) (reference: Jonsson MW, 2012). Such cases are identified by rheumatologist. Early treatment is important to ensure a good prognosis for lymphoma. Therefore, exposed individuals should be regularly monitored by their GP or rheumatoid arthritis 1-2 times a year. In case of severe lung manifestation, controls are also relevant in the lung doctor.
- Ramos-Casals M, 2019 (EULAR recommendations)
- Holdgate N, 2016
- Rades S, 2019 (dry eyes)
- Grans Compendium in Rheumatology
- Great Norwegian encyclopedia