Sjögren syndrome

Sjögren's syndrome is a chronic, systemic autoimmune disease characterized by lymphocytic infiltration of the exocrine glands. The most common clinical manifestation is dryness of the eyes (xerophthalmia) and mouth (xerostomia), often accompanied by systemic features. The disease may also affect glands in the stomach, pancreas, and intestines leading to various gastrointestinal symptoms. The disease often coexists with other autoimmune conditions such as rheumatoid arthritis and lupus.

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Secondary Raynaud's in Sjögren's syndrome 2

Classification

Sjögren's syndrome can be categorized into two main forms: primary and secondary. Primary Sjögren's syndrome occurs alone, whereas secondary Sjögren's syndrome occurs in combination with another autoimmune disease, most commonly Rheumatoid Arthritis, Lupus, or Scleroderma.

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Sjogren's syndrome (2)

Pathophysiology

The exact cause of Sjögren's syndrome is unknown, but it's believed to involve a combination of genetic and environmental factors. The disease is characterized by the abnormal production of extra antibodies in the blood that are directed against various tissues of the body. This leads to an inflammatory response where the body's immune cells mistakenly attack its own healthy cells, particularly the glands that produce tears and saliva.

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SSA SSB ANA

Clinical Features

Exocrine Gland Involvement

Dryness of the eyes (keratoconjunctivitis sicca) and mouth (xerostomia) due to lymphocytic infiltration of the lacrimal and salivary glands are the hallmark features of Sjögren's syndrome. These symptoms can lead to complications such as dental caries, oral candidiasis, and corneal ulceration.

Vasculopathy in Sjögren's syndrome

Patients may also experience dryness in other areas including the nose, throat, air passages, skin, and vagina, which can result in a variety of symptoms such as dry cough, recurrent sinusitis, dry skin, and dyspareunia.

Systemic Features

Systemic manifestations are diverse and may affect various organs and systems:

• Musculoskeletal system: Many patients suffer from arthralgia or arthritis, myalgia and fatigue.
• Nervous system: Peripheral neuropathy can occur, causing symptoms such as tingling in the fingers and toes.
• Respiratory system: Interstitial lung disease and recurrent pneumonia can occur due to dryness and inflammation of the airways.
• Skin: Patients may have cutaneous vasculitis presenting as palpable purpura, urticaria or ulcers.

Diagnosis

There is no single test for Sjögren's syndrome. The diagnosis is based on a combination of symptoms, physical examination, and laboratory tests, which may include:

• Serological tests: Autoantibodies such as Anti-Ro/SSA and Anti-La/SSB are present in the majority of patients with Sjögren's syndrome. Other tests may include antinuclear antibody (ANA) and rheumatoid factor (RF).
• Ophthalmologic tests: Tests such as Schirmer's test, tear break-up time (TBUT) and rose bengal staining can be used to assess eye involvement.
• Oral tests: Salivary gland function can be evaluated through salivary flow rates or salivary scintigraphy. Salivary gland biopsy is sometimes performed.
• Imaging: Imaging of the salivary glands, such as ultrasound or sialography, may show characteristic changes.
• Lung function tests: These may be performed in patients with respiratory symptoms to diagnose interstitial lung disease.

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Cytokine profile in Sjogren’s syndrome (SS)

Management

Management of Sjögren's syndrome is largely symptomatic and supportive, as there is currently no cure for the condition. Treatment strategies are primarily aimed at relieving symptoms, preventing complications, and addressing systemic manifestations when they occur.

• Symptomatic relief: Artificial tears and saliva substitutes can be used to alleviate dryness. Regular eye and dental check-ups are important to prevent complications. Nasal and vaginal moisturizers can also be beneficial for dryness in these areas.
• Immunomodulatory therapy: Systemic manifestations and severe glandular disease may require immunosuppressive treatment such as hydroxychloroquine, methotrexate, azathioprine, or corticosteroids. Biological agents like rituximab may be considered in refractory cases.
• Lifestyle modifications: Patients are advised to stay hydrated, use humidifiers, and avoid medications that can worsen dryness such as antihistamines and diuretics.

Epidemiology

Sjögren's syndrome affects people worldwide of all ages, though symptoms commonly appear between the ages of 45 and 55. Women are nine times more likely to be affected than men.

Prognosis

The course of Sjögren's syndrome varies greatly among individuals. While it can be debilitating in some people, others may experience only mild discomfort. The disease itself is not life-threatening, but complications can reduce life expectancy. For instance, patients with Sjögren's syndrome are at a higher risk of developing lymphomas compared to the general population.

Research

Ongoing research is looking into the genetic and environmental factors that predispose individuals to developing Sjögren's syndrome, with the ultimate goal of developing new treatments that can target these underlying mechanisms.

See also

• Autoimmune diseases
• Rheumatoid arthritis
• Systemic lupus erythematosus
• Systemic sclerosis
• International Sjögren's Syndrome Registry

Sjögren syndrome Resources
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Rheumatologic diseases

Arthritis is often used to refer to any disorder that affects the joints. Rheumatic diseases usually affect joints, tendons, ligaments, bones, and muscles. Rheumatologic diseases usually affect joints, tendons, ligaments, bones, and muscles.

Connective Tissue Diseases

Rheumatology and	Connective Tissue Diseases
Ankylosing spondylitis	Arthritis
Arthritis and Rheumatic diseases	Autoimmune diseases
Autoinflammatory diseases	Behçet’s disease
Bursitis	Giant cell arteritis
Gout	Juvenile arthritis
Knee problems	Lupus
Osteoarthritis	Polymyalgia rheumatica
Psoriatic arthritis	Reactive arthritis
Rheumatoid arthritis	Scleroderma
Sjögren’s syndrome	Systemic lupus erythematosus (Lupus)
Tendinitis	Rheumatologic diseases