Contents
- 1 The diagnosis is based on
- 2 Sickness (* is included in classification criteria)
- 3 * Investigational Findings
- 4 Other investigations
- 5 Additional investigation that can be considered
- 6 Lymphoma risk at Sjögren's syndrome
- 7 Pregnancy at Sjøgren's syndrome
- 8 Doubt if the diagnosis is correct?
- 9 Excludes dryness caused by other condition
- 10 Treatment
The diagnosis is based on
- Disease history
- Examinations
- Antibody (SSA) or lip biopsy
- Exclude another disease (Differential diagnoses)
Sickness (* is included in classification criteria)
- Time for:
- Symptom debut
- Diagnosis
- * Subjective symptoms of dryness
- *Eyes
- Daily dry eyes for at least 3 months or
- Repeated feeling of debris in the eyes
- Do you use artificial fog fluid at least 3 daily
- *Mouth
- Daily feeling of dry mouth for at least 3 months
- Must drink often to swallow dry foods
- *Eyes
Other symptoms
- Exhaustion / fatigue (despite a lot of sleep)
- Vaginal dryness
- arthralgia / Arthritis
- Muscle pain (some have low grade Myositis with increased CK in serum)
- Parotitis (had swollen parotis or submandibular glands
- Purpura (usually on the legs and arms)
- Sun rash by SSA antibody in blood
- Lung symptoms (Dry cough, dyspnea at stress)
- Kidney stones (Renal tubular acidosis)
- Earlier Lymphoma (non-Hodgkin's lymphoma may be related to Sjøgren's syndrome)
* Investigational Findings
- * Tear production is reduced
- Schirmers test less than 5mm on at least one of the eyes
- Staining score (corneal examination by ophthalmologist)
- * Saliva (saliva) production is reduced
- Dry mouth and drink for dry food
- * Antibody (SSA = Sjøgren's Syndrome A) is present
- * Focus score on salivary gland biopsy (lip) shows at least 1 foci / 4 square millimeter
- Tongue sticking, sialometry less than 1,5ml / 15min). Vaginal dryness
Other investigations
- Weight (reference for later)
- Palpate:
- Gland glands
- Submandibularis glands
- Lymph nodes on the neck
- Look in the mouth
- Dry oral mucous membranes
- Oral candida
- Bad tooth status
- Heart (rhythm, murmur)
- Lungs (crepitations)
- Blood pressure
- Skin (often dry skin, eczema, purpura)
- Arthritis (Arthritis)
- Neurological (sensibility, neuralgia, movements)
- Schirmer's test (can be performed by a nurse at the rheumatological department)
- Less than 5mm moisture bilaterally (5mm or lower in just one eye is less specific, but sufficient for classification criteria), more than 10mm is normal
- Sialometry / Unstimulated Saliva Measurement (Can be Performed by Nursing at Rheumatology Department)
- Do not drink, eat or chew gum last hour before the examination
- The patient is at ease
- Measured over 15 min
- Less than 1,5ml / 15 is pathologically low
Additional investigation that can be considered
Blood tests
- SSA (Sjøgrens Syndrome A, Ro antibody) in high titer (over 240) at most. SSA also occurs at SLE and Myositis (often lower titer) and in many healthy ones
- SSB (Sjøgrens syndrome B, La antibody) occurs with SSA at less than 50%
- Both SSA and SSB dispose of fetal heart block in fetus in the event of pregnancy
- Sedimentation rate (ESR) often high at high IgG (common)
- CRP (often low)
- TSH, f-T4 (concomitant hypothyroidism),
- Calcium in high values (hyperparathyroid function can cause drowsiness, nausea and pain)
- ALT, creatinine, p-amylase (pancreatitis component), CK (myositite component), electrophoresis, complement factors C3 and C4.
Urine-stix
- To exclude nephritis (more typically by SLE) and glucosuria
Radiological
- CT examination of lungs (matt-glass, fibrosis. cysts: LIP-suspect)
- MRI caput if CNS symptoms (MS-like)
Lymphoma risk at Sjögren's syndrome
Risk factors for non-Hodgkin's lymphoma are, among other things, persistently low number of lymphocytes (leukocytes) in the blood and low complement factors C3 and C4 (reference: Jonsson MW, 2012).
Pregnancy at Sjøgren's syndrome
- An increased risk has been found for miscarriages and premature birth in Sjøgren's syndrome. SSA and SSB antibodies can cause fetal heart block and / or neonatal lupus (rash in the newborn). Please read more about pregnancy at Sjøgrens syndrome here
Doubt if the diagnosis is correct?
- Most (more than 90%) with Sjøgren's syndrome are women
- All (almost) have subjective dryness (sicca phenomena) in the eyes and mouth
- Most have Antibodies SSA / Ro in high titer (severe rash) in the blood
- At high age (more than 80 years) approx. 40% dryness off Another reason (Age-related degeneration of exocrine glands, drugs)
- Differential diagnoses, please see here
Excludes dryness caused by other condition
- IgG4 related disease
- Hepatitis C
- Amyloidosis
- Sarcoidosis
- Earlier radiation therapy to the head or neck
Drugs that can give dryness
- Antidepressants
- Beta-blocker
- Diuretics
- Antihistamines
- Anticolinergic Parkinson's Drugs
Please read about differential diagnoses here
Treatment
- Artificial tear fluid, extra good oral hygiene.
- Rarely needed immunosuppressive drugs
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