Sjögren's disease

Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
W8MD medical weight loss NYC and sleep center NYC

Sjögren's syndrome
Synonyms	Sicca syndrome
Pronounce	N/A
Specialty	N/A
Symptoms	Dry mouth, dry eyes, fatigue, joint pain
Complications	Lymphoma, dental caries, oral thrush
Onset	Middle age
Duration	Long term
Types	N/A
Causes	Autoimmune disease
Risks	Genetics, environmental factors
Diagnosis	Blood test, Schirmer's test, salivary gland biopsy
Differential diagnosis	Rheumatoid arthritis, systemic lupus erythematosus, fibromyalgia
Prevention	N/A
Treatment	Artificial tears, saliva substitutes, immunosuppressive drugs
Medication	Hydroxychloroquine, corticosteroids, pilocarpine
Prognosis	N/A
Frequency	0.1% to 4% of the population
Deaths	Rare

Autoimmune disease affecting exocrine glands

Sjögren's disease, also known as Sjögren's syndrome, is a chronic autoimmune disease characterized by the body's immune system attacking its own exocrine glands, particularly the salivary glands and lacrimal glands. This results in the hallmark symptoms of dry mouth and dry eyes, known as xerostomia and keratoconjunctivitis sicca, respectively.

Signs and symptoms

The primary symptoms of Sjögren's disease include:

• Dry mouth (xerostomia): Patients often experience difficulty swallowing, speaking, and an increased risk of dental cavities due to reduced saliva production.
• Dry eyes (keratoconjunctivitis sicca): This can lead to irritation, a gritty sensation, and potential damage to the cornea.
• Fatigue: Many patients report chronic fatigue, which can significantly impact quality of life.
• Joint pain and stiffness: Arthralgia is common, and some patients may develop arthritis.

In addition to these primary symptoms, Sjögren's disease can affect other parts of the body, leading to complications such as:

• Skin dryness
• Vaginal dryness
• Chronic cough
• Swelling of the salivary glands
• Neuropathy

Causes

The exact cause of Sjögren's disease is unknown, but it is believed to involve a combination of genetic, environmental, and hormonal factors. The disease is more common in women, suggesting a possible hormonal link. Certain viral infections may trigger the onset of the disease in genetically predisposed individuals.

Pathophysiology

Antinuclear antibody patterns on immunofluorescence, often seen in autoimmune diseases like Sjögren's

Sjögren's disease is characterized by the infiltration of lymphocytes, primarily T cells, into the exocrine glands. This immune response leads to the destruction of glandular tissue and subsequent reduction in secretions. Autoantibodies, such as anti-Ro/SSA and anti-La/SSB, are commonly present in patients and are used as markers for diagnosis.

Diagnosis

The diagnosis of Sjögren's disease is based on a combination of clinical symptoms, serological tests, and sometimes a salivary gland biopsy. Key diagnostic criteria include:

• Schirmer's test: Measures tear production.
• Salivary flow rate: Assesses saliva production.
• Blood tests: Detect the presence of specific autoantibodies.
• Lip biopsy: Examines minor salivary glands for lymphocytic infiltration.

Treatment

Diagram showing the effects of Sjögren's syndrome on the body

There is no cure for Sjögren's disease, but treatment focuses on relieving symptoms and preventing complications. Common treatments include:

• Artificial tears and saliva substitutes to alleviate dryness.
• Immunosuppressive medications such as hydroxychloroquine and methotrexate to reduce immune system activity.
• Pilocarpine and cevimeline to stimulate saliva production.
• Regular dental care to prevent cavities and oral infections.

Prognosis

The prognosis for patients with Sjögren's disease varies. While many individuals experience only mild symptoms, others may develop severe complications, including lymphoma. Regular monitoring and management of symptoms are crucial to maintaining quality of life.

Notable cases

Carrie Ann Inaba, a celebrity diagnosed with Sjögren's syndrome

Several public figures have been diagnosed with Sjögren's disease, raising awareness about the condition. Notably, Carrie Ann Inaba, a television personality and dancer, has publicly discussed her experiences with the disease.

See also

• Autoimmune disease
• Rheumatoid arthritis
• Systemic lupus erythematosus
• Keratoconjunctivitis sicca