Definition: Primary Sjögren’s syndrome (PSS) is characterised by lymphocytic infiltration of salivary and lacrimal glands, leading to glandular fibrosis and exocrine failure.
Sjogren’s syndrome is an autoimmune disease. This means that patients immune system attacks parts of his/her own body by mistake. In Sjogren’s syndrome, it attacks the glands that make tears and saliva.
The disease may occur with other autoimmune diseases that is called secondary Sjögren’s syndrome.
When Sjogren’s syndrome occurs by itself, it’s called primary Sjogren’s syndrome.
If patient already have another autoimmune disease, the condition is called secondary Sjogren’s syndrome.With secondary Sjogren’s, you might have a milder form of the condition. But you’ll still experience symptoms of the coexisting disease. The most common cause of secondary Sjogren’s is rheumatoid arthritis (RA), lupus, or scleroderma.
Associated Autoimmune Disorders:
- Systemic lupus erythematosus.
- Systemic sclerosis.
- Coeliac disease.
- Primary biliary cholangitis.
- Chronic active hepatitis.
- Myasthenia gravis.
Risk Factor: Most people with Sjogren’s syndrome are women. The typical age of onset is between 40 and 50, with a 9 : 1 female-to-male ratio.
Common clinical features:
Less common features:
- Dry eyes.
- Dry Mouth.
- Keratoconjunctivitis sicca.
- Salivary gland enlargement.
- Rashes/skin irritation.
- Non-erosive arthralgia.
- Generalised osteoarthritis.
- Raynaud’s phenomenon.
- Low-grade fever.
- Interstitial lung disease.
- Anaemia, leucopenia.
- Peripheral neuropathy.
- Lymphoreticular lymphoma.
- Interstitial nephritis.
- Renal tubular acidosis.
1. Schirmer tear test : which measures tear flow over 5 minutes using absorbent paper strips placed on the lower eyelid; a normal result is more than 6 mm of wetting.
2. Salivary Gland Biopsy : Demonstration of focal lymphocytic infiltrate.
3. ESR And Gamma-Globulin: Most Patients have elevated ESR level And HyperGammaGlobulinaemia.
4. Detection Of Antibodies : Antibodies that are frequently detected :
- Rheumatoid factor
- Antinuclear antibody
- SS-A (anti-Ro) [Anti-Sjögren’s-syndrome-related antigen A, also called anti–Ro, or the combination anti–SSA/Ro or anti Ro/SSA autoantibodies]
- SS-B (anti-La) [Anti-Sjögren’s-syndrome-related antigen B, also called anti–La]
- Gastric parietal cell
- Yeast infections. People with Sjogren’s syndrome develop oral thrush. (a yeast infection)
- Vision problems. Dry eyes can lead to light sensitivity, blurred vision and corneal damage.
- Dental cavities. Because saliva helps protect the teeth from the bacteria that cause cavities, if mouth is dry than its easy get infected by those Bacteria.
Less common complications might affect:
- Lungs, kidneys or liver: pneumonia, bronchitis in lungs; can damage kidney function; and cause hepatitis or cirrhosis in liver.
- Lymph nodes: A small percentage of people develop lymphoma.
- Nerves: numbness, tingling and burning sensation in hands and feets (peripheral neuropathy).
- Management is symptomatic.
- No treatments that have disease-modifying effects have yet been identified.
- During Day – Lacrimal substitutes, such as hypromellose, should be used.
- During Night – Lacrimal substitutes in combination with more viscous lubricating application used.
- In filamentary keratitis – Soft contact lenses can be useful.
- In Xerostomia – Artificial saliva sprays, saliva-stimulating tablets, and pastilles and oral gels can be tried even chewing gum is most effective.
- In Oral candidiasis – Adequate postprandial oral hygiene and prompt treatment is used.
- Hydroxychloroquine (200 mg twice daily) is often used to address skin and musculoskeletal features and may help fatigue.