Sjogren's Syndrome

Original Editor - Cassie Shay from Bellarmine University's Pathophysiology of Complex Patient Problems project.

Top Contributors - Cassie Shay, Kim Jackson, Lucinda hampton, Admin, Elaine Lonnemann, 127.0.0.1, Wendy Walker, WikiSysop and Sehriban Ozmen  

Introduction

Signs and symptoms: SS

Sjögren's syndrome (SS) is a chronic systemic autoimmune condition that physiotherapists commonly encounter in clinical practice. The hallmark symptoms are dry eyes and dry mouth (sicca symptoms) caused by immune-mediated damage to the lacrimal and salivary glands.

Key Clinical Features

• Primary symptoms: Persistent dry eyes and mouth due to glandular dysfunction
• Systemic nature: Up to 50% of patients develop extraglandular complications affecting joints, skin, lungs, kidneys, and nervous system
• Comorbidity patterns: Frequently occurs alongside rheumatoid arthritis and systemic lupus erythematosus.

Epidemiology

Population Impact

• Affects 0.5-1.0% of the population (400,000-3.1 million US adults)
• Nearly half as common as rheumatoid arthritis

Patient Demographics

• Peak onset: Ages 45-55
• Gender: 9:1 female predominance
• Distribution: Global occurrence without racial or geographic bias
• Comorbidities: 50% also have rheumatoid arthritis or other connective tissue diseases

Underdiagnosis is common due to lack of standardized screening and underreferral from primary care.

Etiology

It is not known what causes SS. Genes may play a role, combined with an outside trigger eg bacteria or virus.It’s also more likely to affect people who have conditions such as rheumatoid arthritis, scleroderma and lupus.

Clinical Presentation

This 90 second video takes a look at this syndrome. 

The more common symptoms of Sjogren’s syndrome include:

• dry eyes (irritation, feeling gritty or itchy, burning)
• dry mouth (or difficulty chewing or swallowing).

Symptoms: Sjogren’s

Sjogren’s syndrome is a systemic condition, which means it can affect the entire body, other symptoms can include:

• swelling and tenderness of the glands around your face, neck, armpits and groin
• tiredness (fatigue)
• dry skin or rashes
• joint pain and general achiness
• dryness of the nose, ear and throat
• vaginal dryness
• bowel irritation.

Complications can include:

• increased risk of dental decay
• increased risk of developing thrush infections in the mouth
• vision problems
• inflammation of internal organs (such as the kidneys, lungs or liver)
• problems with the circulatory and nervous system.

Associated Co-morbidities

Cytokine profile found in SS

Secondary Sjogren's is a form of the syndrome which develops after the presentation of a primary disease, usually another autoimmune connective tissue disease. Common primary diseases associated with Sjogren's Syndrome include: Polymyositis; Rheumatoid Arthritis; Scleroderma; Systemic Lupus Erythematosus; Lymphoma.

Image R: The cytokine profile found in Sjogren’s syndrome (SS) is imbalanced with the overexpression of numerous pro-inflammatory cytokines (on the left) vs low or undetectable levels of anti-inflammatory cytokines (on the right in image).

Key Treatment Approaches

Dry Eye Management

• Primary: Preservative-free artificial tears (various formulations)
• Advanced: Anti-inflammatory drops, immunosuppressive drops (cyclosporine/tacrolimus)
• Severe cases: Punctal occlusion with plugs or cauterization

Dry Mouth Management

• Basic: Water intake, sugar-free gum, saliva substitutes
• Medications: Pilocarpine or cevimeline to stimulate saliva production
• Complications: Antifungal therapy for secondary yeast infections

Systemic Medications (for extraglandular symptoms)

• Anti-inflammatory: Glucocorticoids, hydroxychloroquine
• Immunosuppressants: Methotrexate, azathioprine, mycophenolate mofetil, leflunomide
• Advanced: Rituximab or cyclophosphamide (rarely used)

Clinical Relevance for Physiotherapy

• Systemic treatment is typically reserved for moderate to severe extraglandular manifestations
• Patients with isolated dry eye/mouth symptoms usually manage with topical/local treatments
• Multiple medications may affect patient tolerance for exercise and rehabilitation activities
• No established superiority between different systemic treatment approaches

Understanding these treatments helps you recognize medication-related effects on patient function and exercise capacity.

Diagnostic Tests

Diagnosis of this disease is based on medical history, a physical exam, and results from some clinical and laboratory tests. Due to the nature of the disease in that the symptoms are similar to other diseases and they appear gradually over time, it may take years for Sjogren's to be properly diagnosed. Nonetheless, diagnostic testing may include the following:

• Schirmer’s test – special blotting paper held to the eye is used to assess the degree of tear production
• eye examination – including the use of special dyes
• biopsy – a small piece of salivary gland tissue is removed (usually from the lip) and examined under a microscope
• blood tests – may be used to check the levels of particular immune system cells in the blood, and to check for any problems with your kidneys or liver.

Physical Therapy Management

Individuals with Sjogren's syndrome have reduced physical capacity and tend to fatigue very easily. While research on the effects of exercise on individuals diagnosed with Sjogren's is limited, the available studies suggest these patients benefit from moderate to high-intensity levels of exercise. This type of activity has a positive influence on aerobic capacity, fatigue, physical function, and mood.

Further research is required to understand the effects exercise may have on individuals with varying severities of this disease, and to determine the long-term effects exercise may have on their symptoms. However, some of the musculoskeletal presentations of SS are muscle and joint pain, along with arthritis. Thererfore, a patient may be treated by a physical therapist to relieve these symptoms.

Lifestyle Changes

There are also several things a patient can do at home in order to manage his or her symptoms. These may include:

• Over the counter eye drops
• Chewing gum or sucking on hard candy to increase oral secretions
• Drinking water to relieve a dry mouth
• Using lip balm to moisten the lips
• Practice thorough oral hygiene to avoid oral infection
• Heavy moisturizing creams or ointment for dry skin
• Humidifier to hydrate the skin and moisten the air for breathing