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

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 include persistently low numbers of lymphocytes (leukocytes) in blood and low complement factors C3 and C4 (reference: Jonsson MW, 2012).

Pregnancy at Sjøgren's syndrome

• An increased risk of spontaneous abortion and premature birth at Sjøgren's syndrome has been found. SSA and SSB antibody may 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

referral

Patients resident in Oslo

• Referred Rheumatology Department, Rikshospitalet, Oslo Postbox 4950 Nydalen, 0424 OSLO. The department can receive patients from the Health Region South East and the rest of the country when the capacity allows

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