DEFINITION:

Sjögren's syndrome is an autoimmune disorder that causes your glands to produce less moisture than
they should, resulting in chronic dryness throughout the body, especially in the eyes and mouth ¹. It is a
systemic chronic inflammatory disorder characterized by lymphocytic infiltrates in exocrine organs, and
it affects mostly women, with the median age of onset around 50 to 60 years ².

CAUSES

Sjögren's syndrome is an autoimmune condition, meaning that instead of protecting the body from
infection or illness, the immune system mistakenly attacks healthy parts of the body ¹. The exact cause
of Sjögren's syndrome is unknown, but it is thought to be linked to genetics, hormones and other
autoimmune conditions

PATHOPHYSIOLOGY:

The pathophysiology of Sjögren's syndrome involves a complex interplay of genetic, environmental, and
hormonal factors, leading to:

1. Autoantibody production: The immune system produces autoantibodies against glandular tissues,
particularly the salivary and lacrimal glands.

2. Lymphocytic infiltration: White blood cells (lymphocytes) accumulate in the glands, leading to
inflammation and tissue damage.

3. Glandular dysfunction: Inflammation and damage disrupt glandular function, reducing tear and saliva
production.

4. Epithelial cell activation: Epithelial cells in the glands become activated, contributing to the
inflammatory response.

5. Cytokine imbalance: Pro-inflammatory cytokines (e.g., IL-1, TNF-alpha) dominate, perpetuating

inflammation.

6. Hormonal influences: Estrogen and androgen hormones may play a role in the development and
progression of Sjögren's syndrome.

7. Genetic predisposition: Certain genetic markers (e.g., HLA-B8, HLA-DR3) increase the risk of
developing Sjögren's syndrome.

This pathophysiological process leads to the characteristic symptoms of Sjögren's syndrome, including
dry eyes, dry mouth, and systemic manifestations such as joint pain and fatigue.
DIAGNOSTIC INVESTIGATION:

The diagnostic investigation of Sjögren's syndrome includes ¹ ² ³ ⁴:

1. Blood tests to detect specific antibodies

2. Sialometry to measure saliva production

3. Lip biopsy to examine salivary glands

4. Eye exam, including Schirmer's test and epithelial staining test

5. Physical exam and medical history assessment

6. Blood and urine tests to rule out other conditions

7. Salivary gland function scans and ultrasonography of major salivary glands

8. Nerve conduction and electromyography for neurologic complications

9. Cutaneous nerve/skin biopsy and lumbar puncture for neurologic complications

10. MRI of the brain and spinal cord for neurologic complications

SIGNS AND SYMPTOMS:

The signs and symptoms of Sjögren's syndrome include ¹ ² ³:

- Dry eyes, mouth, skin, nose, upper respiratory tract and vagina

- Difficulty swallowing, tasting or speaking

- Tooth decay and recurring oral thrush

- Persistent dry cough

- Fatigue

- Joint pain

- Swollen salivary glands

- Rashes, especially after being in the sun

- Vaginal dryness

- Difficulty concentrating and memory issues

- Difficulty sleeping

- Shortness of breath

- Muscle weakness

- Numbness and tingling, especially in the arms and legs

COMPLICATIONS:

The complications of Sjögren's syndrome include ¹ ² ³ ⁴:

- Dental cavities: Saliva helps protect teeth from bacteria that cause cavities, so dry mouth makes you
more prone to developing cavities.

- Yeast infections: People with Sjögren's syndrome are more likely to develop oral thrush, a yeast
infection in the mouth.

- Vision problems: Dry eyes can lead to light sensitivity, blurred vision and corneal damage.

- Lung problems: Sjögren's syndrome can cause lung infections, widening of the airways in the lungs, and
scarring of the lungs.

- Pregnancy complications: Pregnant women with Sjögren's syndrome may have a small risk of
complications.

- Cancer: People with Sjögren's syndrome have an increased risk of getting non-Hodgkin lymphoma.

- Raynaud's phenomenon: This condition causes restricted blood flow to the hands and feet.

- Underactive thyroid gland: This condition can cause tiredness and weight gain.

- Irritable bowel syndrome: This condition can cause tummy pain, diarrhea or constipation.

- Peripheral neuropathy: This condition causes loss of feeling in the hands and feet.

- Kidney problems: Sjögren's syndrome can cause kidney inflammation or kidney stones.

- Inflammation of the blood vessels: This condition can cause a rash.

RISK FACTORS:

The risk factors for Sjögren's syndrome include ¹ ² ³ ⁴ ⁵:

- Age: Sjögren's syndrome is usually diagnosed in people older than 40.

- Sex: Women are much more likely to have Sjögren's syndrome.

- Rheumatic disease: It's common for people who have Sjögren's syndrome to also have a rheumatic
disease.

- Family history of autoimmune disease: A positive family history of autoimmune disease is associated
with Sjögren's syndrome.

- Infection: Persistent infection could trigger autoimmune disorders.

MEDICAL MANAGEMENT:

Pharmacologic Intervention includes,Corticosteroids and immunosuppressants such as

cyclophosphamide are used in severe cases.Antifungal agents therapeutically or prophylactically for
superimposed fungal infections of mouth or vagina.Cevimeline 30 mg t.i.d., a cholinergic agonist for
treatment of dry moutDepending on your symptoms, your doctor might suggest medications
that:Decrease eye inflammation. Prescription eyedrops such as cyclosporine (Restasis) or lifitegrast
(Xiidra) may be recommended by your eye doctor if you have moderate to severe dry eyes.Increase
production of saliva. Drugs such as pilocarpine (Salagen) and cevimeline (Evoxac) can increase the
production of saliva, and sometimes tears. Side effects can include sweating, abdominal pain, flushing
and increased urination.Address specific complications. If you develop arthritis symptoms, you might
benefit from nonsteroidal anti-inflammatory drugs (NSAIDs) or other arthritis medications. Yeast
infections in the mouth should be treated with antifungal medications.Treat systemwide symptoms.
Hydroxychloroquine (Plaquenil), a drug designed to treat malaria, is often helpful in treating Sjogren's
syndrome. Drugs that suppress the immune system, such as methotrexate (Trexall), also might be
prescribed.Dry eyes usually respond to artificial tears applied regularly during the day or to gels applied
at night. We usually have patients use a preservative-free drop during the day and a preservative-free
gel or ointment at night.Punctal occlusion with plugs or cauterization is frequently needed in more
severe cases. Cauterization should only be performed after a trial of plugs to ensure the patient is not
left with excessive tearing.Eye drops that reduce inflammation in the glands around the eyes, such as
cyclosporine (Restasis) are used to increase tear production.Many patients are helped by serum tears
which are derived from their own blood. Most eye centers now have the capability of making these for
the patient. Drinking water, chewing gum, or using saliva substitutes may relieve dry mouth.Some
patients benefit from using prescription medications that stimulate saliva flow, such as pilocarpine
(Salagen) or cevimuline (Evoxac).If patients develop yeast infections, anti-fungal therapies may be used.
Humidifiers and nasal saline irrigation may improve nasal dryness. Medications that reduce gastric acid
(such as proton-pump inhibitors and H2 blockers) may lessen symptoms of acid reflux. Treatments may
help relieve some of the dryness, but usually some dryness persists.The first approach to extra-glandular
(systemic) major organ-system disease is oral/parenteral corticosteroids.

NURSING MANAGEMENT USING THE NURSING PROCESS:

Here is the nursing management of Sjögren's syndrome using the nursing process:

*Assessment:*

- Physical examination: assess for dry eyes, mouth, and skin; joint pain and swelling; and lymph node
enlargement

- Review medical history: autoimmune disorders, rheumatic diseases, and medications

- Lab tests: autoantibody screening, complete blood count, and inflammatory markers

*Diagnosis:*
- Identify specific nursing diagnoses:

- Impaired oral mucous membrane

- Dry eye

- Pain

- Fatigue

- Deficient knowledge

*Planning:*

- Develop individualized care plan with patient goals and outcomes

- Collaborate with healthcare team: rheumatologist, ophthalmologist, dentist

*Interventions:*

- Oral care: frequent water intake, saliva substitutes, and oral hygiene

- Eye care: artificial tears, humid environment, and eye protection

- Pain management: analgesics, heat/cold therapy, and relaxation techniques

- Fatigue management: energy conservation, rest, and stress reduction

- Education: disease process, self-care, and medication management

*Evaluation:*

- Monitor patient progress and adjust care plan as needed

- Assess symptom management and quality of life

- Evaluate patient understanding and adherence to self-care regimen

This nursing process framework guides nursing management of Sjögren's syndrome, addressing physical
and emotional symptoms, promoting patient education and self-care, and enhancing quality of life.
NURSING DIAGNOSIS:

Here is the nursing management of Sjögren's syndrome using nursing diagnoses:

_Impaired Oral Mucous Membrane_

- Assessment: dry mouth, difficulty swallowing, oral lesions

- Goals: maintain oral hydration, prevent oral infections

- Interventions: frequent water intake, saliva substitutes, oral hygiene, mouthwash

_Dry Eye_

- Assessment: dry eyes, blurred vision, eye discomfort

- Goals: maintain eye moisture, prevent eye damage

- Interventions: artificial tears, humid environment, eye protection, avoid rubbing eyes

_Pain_

- Assessment: joint pain, muscle pain, fatigue

- Goals: manage pain, improve mobility

- Interventions: analgesics, heat/cold therapy, relaxation techniques, gentle exercise

_Fatigue_

- Assessment: persistent fatigue, lack of energy

- Goals: manage fatigue, improve energy levels

- Interventions: energy conservation, rest, stress reduction, prioritize activities

_Deficient Knowledge_
- Assessment: lack of understanding of disease process, self-care

- Goals: improve knowledge, promote self-care

- Interventions: education on disease process, self-care techniques, medication management

These nursing diagnoses guide nursing management of Sjögren's syndrome, addressing specific patient
needs and promoting individualized care.

OUTCOME:

Mild Sjogren disease has a good prognosis but those with moderate to severe disease have a very poor
quality of life. The dry mouth and eyes often cause irritable symptoms which are not well tolerated. In
addition, many of these patients develop moderate to severe arthritis and ambulation can be difficult.
With advancing age, as the function of the exocrine glands subsides, the symptoms also tend to worsen.
In the long run, there is a risk that patients with Sjogren syndrome will develop a lymphoproliferative
disorder which can lower the life expectancy. Women with Sjogren syndrome are also at a high risk for
developing complications during pregnancy. Premature deliveries and spontaneous abortions are not
uncommon. Finally, patients with Sjogren syndrome who have antiphospholipid antibodies are at risk for
developing vascular thrombotic disorders and fetal demise.