Sjögren’s Syndrome

Overview of Sjögren’s Syndrome

• The typical age of onset is between 40 and 50, with a 9 : 1 female preponderance.
• The disease may occur in isolation (primary Sjögren’s syndrome) or in patients with other autoimmune diseases (secondary Sjögren’s syndrome). [1]
• Sjogren’s causes your immune system to go haywire and attack healthy cells instead of invading bacteria or viruses.
• Conditions like this are called autoimmune diseases.
• Your white blood cells, which normally protect you from germs, attack the glands that are in charge of making moisture.
• When that happens, they can’t produce tears and saliva.
• About half of people with Sjogren’s also have another autoimmune disease, like rheumatoid arthritis or lupus.
• Dry eyes and dry mouth are the most common symptoms.
• You can sometimes get problems in other parts of your body, such as swollen glands around your face and neck, dry skin or nasal passages, or painful and stiff joints. [3]

Causes of Sjögren’s Syndrome

• In Sjögren’s syndrome, the body’s immune system produces inflammation that damages the tissues and nerve signals to certain glands in the body.
• The inflammation mainly attacks glands that produce fluids, such as the lacrimal glands behind the eyelids, and the salivary glands in the mouth. This reduces the amount of saliva, tears and other fluids the glands produce.
• We don’t know what causes the immune system to start attacking the body, but it’s thought it could be partly due to the genes we inherit from our parents.
• However, this doesn’t mean that children of people with Sjögren’s syndrome will automatically get the condition.
• It’s also thought that the female hormone oestrogen could be linked to Sjögren’s syndrome, as more women get the condition than men. [4]

Risk Factors of Sjögren’s Syndrome

• Age: Sjogren’s syndrome is usually diagnosed in people older than 40.
• Sex: Women are much more likely to have Sjogren’s syndrome.
• Rheumatic disease: It’s common for people who have Sjogren’s syndrome to also have a rheumatic disease, such as rheumatoid arthritis or lupus. [2]
• HLA-B8/DR3 [1]

Pathogenesis of Sjögren’s Syndrome

• Lymphocytic Infiltration: The hallmark of Sjogren’s syndrome is the infiltration of lymphocytes, primarily CD4+ T cells, into the exocrine glands, particularly the salivary and lacrimal glands. This infiltration leads to inflammation and subsequent destruction of the glands, resulting in decreased tear and saliva production.
• Autoantibodies: Patients with Sjogren’s syndrome often develop autoantibodies, including anti-Ro/SSA and anti-La/SSB antibodies. The exact role of these antibodies in the disease process is not fully understood, but they are associated with disease activity and certain clinical manifestations.
• Genetic Predisposition: There is evidence of a genetic predisposition to Sjogren’s syndrome, with certain HLA alleles being associated with increased risk.
• Environmental Triggers: It is believed that environmental triggers, such as viral infections or hormonal changes, may play a role in initiating the autoimmune response in genetically susceptible individuals.[10]

Pathophysiology

• Lymphocytic Infiltration and Glandular Destruction: The infiltration of lymphocytes, mainly CD4+ T cells, into the exocrine glands (salivary and lacrimal glands) leads to inflammation and progressive destruction of the glandular tissue. This results in decreased production of tears and saliva, causing the characteristic dryness symptoms.
• Autoimmune Response: Sjogren’s syndrome is characterized by an autoimmune response, where the body’s immune system mistakenly attacks its own tissues. This immune response involves the production of autoantibodies, including anti-Ro/SSA and anti-La/SSB antibodies, which are directed against specific cellular components.
• Role of Cytokines: The inflammatory process in Sjogren’s syndrome is mediated by various cytokines, including interferon-gamma, tumor necrosis factor-alpha, and interleukin-1. These cytokines contribute to the tissue damage and dysfunction of the exocrine glands.

Clinical Features

• Sicca Symptoms:

  • Dry eyes (keratoconjunctivitis sicca): This manifests as a gritty or burning sensation in the eyes, foreign body sensation, redness, and blurred vision.
  • Dry mouth (xerostomia): Patients experience difficulty swallowing, altered taste, increased thirst, and dental caries.

• Extraglandular Manifestations:

  • Arthralgias or arthritis: Joint pain or inflammation can affect various joints.
  • Fatigue: A persistent feeling of tiredness or exhaustion.
  • Raynaud’s phenomenon: Cold-induced discoloration of the fingers or toes.
  • Skin manifestations: Dry skin, rashes, or vasculitis.
  • Pulmonary involvement: Interstitial lung disease or pleuritis.
  • Renal involvement: Interstitial nephritis or glomerulonephritis.
  • Neurologic involvement: Peripheral neuropathy or central nervous system manifestations.
  • Lymphoma: There is an increased risk of developing lymphoma in patients with Sjogren’s syndrome.[10]

Sign & Symptoms

Many people who have the condition don’t have any other symptoms.

However, symptoms can vary a lot from person to person.

Some people find they can manage their condition well, while others find it has a big effect on their daily life.

Sjögren’s Syndrome

Eye:

• Eyes may be dry and feel sore, irritable or as if they have grit or sand in them.
• Some people feel discomfort under strong lights, or find their eyes become sticky with mucus.
• Sometimes the eyelids might be sore or swollen, or your vision might become blurred.

Mouth and throat problems:

• Mouth may be dry and you may get mouth ulcers.
• Throat or mouth might also feel sticky.
• It can sometimes be hard to swallow, or you might find that things taste different.
• You may find that you need to drink water while eating.
• Your voice may be hoarse or weak, and some people have a dry cough.
• You could also have cracks in the skin at the corners of your mouth, or your tongue might look red and smooth.
• These include fungal infections such as thrush, an unpleasant taste in your mouth, and more tooth decay.
• Your salivary glands, which are on your face, just in front of your ears, may feel painful and swollen.

Fatigue:

• Fatigue is an overwhelming feeling of tiredness that can happen after doing very little and has no obvious cause.
• You may feel extremely tired, both physically and mentally, and you might find it difficult to concentrate.
• Some people start to feel down or depressed as a result of their condition and the fatigue it causes.

Aches and pains:

• Your joints may be painful and swollen due to inflammation, or you might feel that various parts of your body, such as your muscles, are achy and tender.
• Some people with this condition have headaches and migraines.

Other symptoms:

You may feel as though other parts of the body are drier than normal, such as:

• Bowel – this may cause symptoms similar to irritable bowel syndrome (IBS), such as tummy pain, diarrhoea and constipation.
• Bladder – this may cause irritation, soreness, or the need to pee more than usual.
• Vagina – this can make sex uncomfortable and could cause symptoms of the menopause to feel worse.
• Skin – this can make you feel itchy or unusually sensitive to strong sunlight & also get rashes.
• Air passages – this may make you more sensitive to things in the air, such as smoke or dust, and cause a dry cough. [4]

Associated autoimmune disorders:

• SLE
• Progressive systemic sclerosis
• Primary biliary cirrhosis
• Chronic active hepatitis
• Myasthenia gravis [1]

Clinical Examination

• Eyes:

  • Schirmer’s test: Measures tear production by placing filter paper strips under the lower eyelids.
  • Slit-lamp examination: Evaluates the cornea and conjunctiva for dryness, inflammation, and damage.
  • Rose bengal or lissamine green staining: Stains damaged areas of the cornea and conjunctiva, indicating dryness.

• Mouth:

  • Inspection: Look for dryness of the oral mucosa, cracked lips, and dental caries.
  • Palpation: Assess the salivary glands for enlargement or tenderness.

• Extraglandular Manifestations:

  • Joint examination: Evaluate for joint swelling, tenderness, and range of motion to assess for arthritis.
  • Skin examination: Look for dryness, rashes, or evidence of vasculitis.
  • Neurologic examination: Assess for sensory or motor deficits suggestive of peripheral neuropathy.
  • Lymph node examination: Palpate for enlarged lymph nodes, which may indicate lymphoma.

Diagnosis

There are a number of tests that can help a doctor diagnose this condition.

Tear production:

• The diagnosis can be established by the 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.
• Staining with rose Bengal may show punctate epithelial abnormalities over the area not covered by the open eyelid. [1]

Eye examination: 

• During this test, an eye specialist will put a dye into your eyes and use a microscope with a light to examine them.
• The light makes it possible to see the film of liquid over the surface of each eye.
• If you don’t have enough of this liquid, you may have Sjögren’s syndrome.

Spit test:

• This test involves getting you to spit or dribble into a container over five minutes.
• The amount of saliva that you produce in that time is measured.
• If it’s lower than expected, this could be a sign of this condition.

Ultrasound scans:

• Ultrasound scans can sometimes help with a diagnosis, with high-frequency sound waves used to build up pictures of your salivary glands.
• The glands usually appear grey on ultrasound images, but in people with Sjögren’s syndrome, round black areas can be seen in the scans.

Sialography:

• A sialography is an x-ray of the salivary glands and ducts.
• In this x-ray, contrast dye is injected so that the glands and ducts show up clearly in the images.
• This can be used for diagnosis, but is mainly used to identify blockages of the ducts in people who keep getting salivary gland infections.

MRI scans:

• Occasionally, magnetic resonance imaging (MRI) scans are used if there is uncertainty over the diagnosis, or if it is thought you may have another condition, such as lymphoma.
• MRI scans use radio waves in a strong magnetic field to build up pictures of the inside of the body.

Blood tests:

• People with Sjögren’s syndrome often have high levels of inflammation in their blood, which can measure with blood tests, such as an erythrocyte (er-i-throw-site) sedimentation rate (ESR) test.
• Two autoantibodies, called anti-Ro and anti-La, are important in diagnosis and can found in blood tests.
• About two-thirds of people with primary Sjögren’s syndrome have anti-Ro or anti-La antibodies, or both.
• The presence of one or more of these autoantibodies in a person with dry eyes or a dry mouth strongly suggests they have the condition.

Lip biopsy:

• A lip biopsy is done to see if any clumps of white blood cells have formed inside your salivary glands, and might also be used to check if you’re at risk of lymphoma.
• During the biopsy, a tiny piece of tissue containing several tiny salivary glands is removed from your lower lip under local anaesthetic and examined under a microscope. [4]

Differential Diagnosis

• Other autoimmune diseases:

  • Rheumatoid arthritis
  • Systemic lupus erythematosus
  • Scleroderma
  • Primary biliary cirrhosis

• Infections:

  • Hepatitis C
  • HIV
  • Sarcoidosis

• Medications:

  • Anticholinergic drugs
  • Antidepressants
  • Antihistamines

• Other conditions:

  • Graft-versus-host disease
  • Amyloidosis
  • Head and neck radiation therapy.

Complications

• Ocular Complications:

  • Corneal ulceration and scarring
  • Bacterial keratitis
  • Decreased visual acuity

• Oral Complications:

  • Dental caries
  • Oral candidiasis
  • Difficulty eating and speaking

• Other Complications:

  • Increased risk of lymphoma (especially non-Hodgkin’s lymphoma)
  • Interstitial lung disease
  • Interstitial nephritis
  • Peripheral neuropathy
  • Vasculitis
  • Depression.[10]

Investigations

• Blood tests:

  • Antinuclear antibodies (ANA): Positive in most patients with Sjogren’s syndrome.
  • Anti-Ro/SSA and anti-La/SSB antibodies: Specific for Sjogren’s syndrome and associated with increased disease activity.
  • Rheumatoid factor: May be positive in some patients.
  • Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP): Elevated in active disease.

• Ocular tests:

  • Schirmer’s test: Measures tear production.
  • Slit-lamp examination: Evaluates the cornea and conjunctiva for dryness and damage.
  • Rose bengal or lissamine green staining: Stains damaged areas of the cornea and conjunctiva.

• Salivary gland tests:

  • Salivary flow rate: Measures saliva production.
  • Sialography: X-ray imaging of the salivary glands after injecting contrast dye.
  • Salivary gland biopsy: Microscopic examination of a small sample of salivary gland tissue.

• Other tests:

  • Chest X-ray or CT scan: To assess for lung involvement.
  • Kidney function tests: To evaluate for kidney involvement.
  • Nerve conduction studies: To assess for peripheral neuropathy.[10]

Treatment

• Lachrymal substitutes, such as hypromellose, should be used during the day in combination with more viscous lubricating ointment at night.
• Soft contact lenses can useful for corneal protection in patients with filamentary keratitis, and occlusion of the lachrymal ducts is occasionally needed.
• Artificial saliva and oral gels can tried for xerostomia but are often not effective.
• Stimulation of saliva flow by sugar free chewing gum or lozenges may be helpful.
• Adequate postprandial oral hygiene and prompt treatment of oral candidiasis are essential.
• Vaginal dryness treat with lubricants such as K­Y jelly.
• Extra glandular and musculo skeletal manifestations may respond to steroids, and if so, immuno suppressive drugs can added for their steroid­sparing effect.
• Fatigue is difficult to treat; this is usually due to non­ restorative sleep (often because of xerostomia) and is unresponsive to steroids.
• If lymphadenopathy or salivary gland enlargement develops, biopsy should perform to exclude malignancy. [1]

Prevention

This is because the exact cause of the autoimmune response that leads to Sjogren’s syndrome remains unknown. However, the following points may be relevant:

• Genetic Predisposition: Individuals with a family history of autoimmune diseases may be at a higher risk. While genetics cannot be changed, being aware of the risk can lead to early detection and management.
• Environmental Triggers: Although specific triggers are not fully understood, avoiding potential triggers like certain viral infections or environmental toxins may be beneficial.
• Early Diagnosis and Management: While prevention may not be possible, early diagnosis and management can significantly improve the quality of life and minimize complications associated with Sjogren’s syndrome.[10]

Homeopathic Treatment 

Homeopathy treats the person as a whole. It means that homeopathic treatment focuses on the patient as a person, as well as his pathological condition. The homeopathic medicines selected after a full individualizing examination and case-analysis.

which includes

• The medical history of the patient,
• Physical and mental constitution,
• Family history,
• Presenting symptoms,
• Underlying pathology,
• Possible causative factors etc.

A miasmatic tendency (predisposition/susceptibility) also often taken into account for the treatment of chronic conditions.

What Homoeopathic doctors do?

A homeopathy doctor tries to treat more than just the presenting symptoms. The focus is usually on what caused the disease condition? Why ‘this patient’ is sick ‘this way’?.

The disease diagnosis is important but in homeopathy, the cause of disease not just probed to the level of bacteria and viruses. Other factors like mental, emotional and physical stress that could predispose a person to illness also looked for. No a days, even modern medicine also considers a large number of diseases as psychosomatic. The correct homeopathy remedy tries to correct this disease predisposition.

The focus is not on curing the disease but to cure the person who is sick, to restore the health. If a disease pathology not very advanced, homeopathy remedies do give a hope for cure but even in incurable cases, the quality of life can greatly improved with homeopathic medicines.

Homeopathic Medicines :

The homeopathic remedies (medicines) given below indicate the therapeutic affinity but this is not a complete and definite guide to the homeopathy treatment of this condition. The symptoms listed against each homeopathic remedy may not be directly related to this disease because in homeopathy general symptoms and constitutional indications also taken into account for selecting a remedy.

Medicines:

Nux Moschata – For dry mouth:

• Nux Moschata is a homeopathic medicine prepared from seeds of the Nutmeg plant.
• The natural order of this plant is Myristicaceae.
• Nux Moschata is beneficial for treating dry mouth in cases of Sjogren’s syndrome.
• People who need Nux Moschata have an intensely dry mouth.
• The dryness is so marked that the tongue sticks to the roof of the mouth.
• The dryness of the mouth is most experienced while sleeping.
• A peculiar symptom is a lack of thirst in spite of a dry mouth.
• Besides this, A bad smell from the mouth may be present.
• The lips are also dry.
• The dryness may extend to throat from mouth.

Belladonna – For managing dry eyes:

• Belladonna is a natural treatment for Sjogren’s syndrome prepared from the plant Deadly Nightshade.
• It belongs to the natural order Solanaceae.
• Belladonna is helpful for managing dry eyes in Sjogren’s syndrome.
• Along with dryness, the eyes also appear red.
• Itching in the eyes may be present, and in some cases, a burning, smarting and stinging sensation in eyes is present.
• Sand-like gritty sensation in the eyes along with the above symptoms may also be present.

Rhus Toxicodendron – For joint pains:

• Rhus Tox is an effective homeopathic cure for joint pains in cases of Sjogren’s syndrome.
• People needing rhus tox experience pain, swelling, redness, heat in joints with marked stiffness.
• Cracking on stretching the joints also appears.
• They may feel better by warm application.
• For using rhus tox, a characteristic feature is a joint pain that gets worse during rest whereas better by motion.

Arsenic Album – For fatigue:

• Arsenic Album is an effective medicine especially for Sjogren’s Syndrome where the person experiences extreme fatigue.
• Weariness and weakness prevail all the time, and intense fatigue makes a person want to lie down constantly.
• The slightest exertion can cause fatigue, and there may be anxiety or restlessness.

Bryonia Alba – For dry cough:

• Bryonia is a natural medicine prepared especially from the root of a plant named Bryonia Alba, of the natural order Cucurbitaceae.
• It is well-indicated to treat a dry cough in cases of Sjogren’s syndrome.
• People needing bryonia have a dry, spasmodic cough.
• A tickling sensation in the throat may be present along with a cough.
• The cough may worsen after either eating or drinking.
• Pain in the head and chest appear with a cough.
• Apart from the above symptoms, dryness of the mouth and cracking of the lips may be present.
• Joint pains that are worse from motion, on the other hand better by rest is another prominent attending symptom.

Sulphur – For dry, itching skin:

• Sulphur is a natural remedy specifically for dry, itchy skin in Sjogren’s syndrome.
• The itching may be either localized or widespread all over the body.
• Intense itching is present, also scratching the skin causes a burning sensation.
• The itchy spots may also become painful after scratching.
• In some cases, bleeding appears upon scratching the skin.
• The skin itching is most worse in the evening and night time, especially upon getting warm in the bed.
• Along with the above symptoms, a stitching, prickling sensation on the skin may also appear.

Sepia – For vaginal dryness:

• Sepia is an effective natural medicine especially for vaginal dryness in sjogren’s syndrome.
• Furthermore, Women needing sepia may experience itching in the vagina along with dryness.
• They also complain of painful coition (intercourse) from extreme dryness of the vagina. [5]

Diet & Regimen

• Hydration: Due to the dryness associated with Sjogren’s syndrome, maintaining adequate hydration is crucial.Regular intake of water and other fluids throughout the day can help manage dry mouth and eyes.
• Moistening Foods: Consuming soft, moist foods and avoiding dry, crunchy, or spicy foods can ease discomfort associated with dry mouth.
• Oral Hygiene: Maintaining good oral hygiene is essential to prevent dental complications. This includes regular brushing, flossing, and dental checkups.   
• Artificial Tears and Saliva: Over-the-counter or prescription artificial tears and saliva substitutes can provide relief from dryness symptoms.
• Symptom Management: Medications and other therapies may be recommended by a healthcare professional to manage specific symptoms and complications associated with Sjogren’s syndrome.
• Healthy Lifestyle: Maintaining a healthy lifestyle with regular exercise, adequate sleep, and stress management techniques can help improve overall well-being and potentially mitigate disease activity.

References

1. Davidson’s Principles and Practice of Medicine (22nd edition) Ch. 25
2. https://www.mayoclinic.org/diseases-conditions/sjogrens-syndrome/symptoms-causes/syc-20353216
3. https://www.webmd.com/a-to-z-guides/sjogrens-syndrome#1
4. https://www.versusarthritis.org/about-arthritis/conditions/sjoegrens-syndrome/
5. https://www.drhomeo.com/homeopathic-treatment/homeopathic-remedies-sjogrens-syndrome
6. Aggarwal A, Misra R, Tandon R. Primary Sjogren’s syndrome: rarity in India. J Assoc Physicians India. 2003 Dec;51:1091-4.
7. Aggarwal S, Misra DP, Dwivedi SN, Misra R. Prevalence of secondary Sjogren’s syndrome in Indian patients with rheumatoid arthritis: a single-center study. Rheumatol Int. 2017 Feb;37(2):285-290.
8. Bhatia A, Mishra S, Aggarwal A, Misra R. Clinical Characteristics and Outcome of Primary Sjogren’s Syndrome: A Large Asian Indian Cohort. Open Rheumatol J. 2014;9:36-43.
9. Singh J and Jaiswal AK. AB0318 PREVALENCE OF SECONDARY SJOGREN’S SYNDROME IN PATIENTS WITH RHEUMATOID ARTHRITIS- A SINGLE CENTER STUDY FROM NORTHERN INDIA. Annals of the Rheumatic Diseases 2022;81:1283-1283.
10. "Rheumatology Secrets (5th Edition)" by Sterling G. West (2015) published by Elsevier.