Description

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Context

While global estimates suggest that Sjögren’s affects 1 in 1,000 people, there is precise data for India.

It is roughly 10 times more common in women than in men and usually manifests in the 30s and 40s.

Sjögren’s Disease

Sjögren’s disease, also called Sjögren’s syndrome, is a chronic autoimmune disorder primarily affecting the exocrine glands leading to decreased secretion of saliva and tears.

Named after Swedish ophthalmologist Henrik Sjögren, who first described it in 1933 the disease involves systemic immune-mediated damage beyond the glands with potential impacts on various organs.

Characteristics of Sjögren’s Disease

Aspect	Details
Primary Sjögren’s	Occurs as an isolated condition primarily targeting exocrine glands. Exocrine glands release (secrete) substances through openings (ducts) onto your body surfaces. Exocrine glands secrete sweat, tears, saliva, milk and digestive juices. A gland is a unit of cells that work together to create and secrete these substances.
Secondary Sjögren’s	Associated with other autoimmune disorders like rheumatoid arthritis or systemic lupus erythematosus.
Prevalence	Affects predominantly women with a female-to-male ratio of 9:1. Often manifests between ages 40-60.
Pathophysiology	-Characterized by lymphocytic infiltration of exocrine glands. -Autoantibodies (e.g., anti-Ro/SSA, anti-La/SSB) target glandular epithelial cells.

Autoimmune Mechanism

Genetics: Associated with HLA-DR and HLA-DQ gene polymorphisms. HLA-DR and HLA-DQ are genes that encode MHC class II molecules, which play a key role in the immune system. HLA-DR and HLA-DQ are expressed in the development of CD4 T cells in the thymus. They process and present antigens to CD4 T cells and help mature CD4 T cells respond to antigens during infections.

Polymorphisms in these genes are associated with a number of conditions like diabetes, rheumatoid arthritis etc.

Environmental Triggers: Viral infections (e.g., Epstein-Barr virus) may initiate an aberrant immune response.

Impact on Body Systems

System Affected	Manifestations
Ocular System	Dryness, corneal ulcers, conjunctivitis.
Oral Cavity	Increased dental caries, candidiasis, difficulty in chewing and swallowing.
Musculoskeletal	Arthritis, joint stiffness and muscle pain.
Respiratory	Interstitial lung disease, chronic cough.
Renal	Renal tubular acidosis, interstitial nephritis.
Hematological	Increased risk of lymphoma, anemia and thrombocytopenia.
Neurological	Peripheral neuropathy, cognitive dysfunction ("brain fog").

Treatment

Symptom Management

Artificial tears and saliva substitutes.

Medicines to stimulate glandular secretion.

Immunomodulatory Therapy

Corticosteroids and hydroxychloroquine for systemic symptoms.

Biologics like rituximab target B-cell-mediated pathways.

Sources:

HINDU

PRACTICE QUESTION

Q.With reference to Sjögren’s disease, consider the following statements:

It is an autoimmune disorder that primarily affects the moisture-producing glands of the body.
Common symptoms include dry eyes and dry mouth.
It is caused by bacterial infections that damage salivary and lacrimal glands.

Which of the statements given above is/are correct?

(a) 1 and 2 only

(b) 2 and 3 only

(d) 1, 2 and 3

Answer: (a)

Explanation

Statement 1 is correct

Sjögren’s disease is a chronic autoimmune disorder where the body's immune system attacks its own moisture-producing glands such as the salivary and lacrimal (tear-producing) glands. This leads to decreased gland function.

Statement 2 is correct

The hallmark symptoms of Sjögren’s disease are xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). These symptoms occur due to the reduced secretion of saliva and tears.

Statement 3 is incorrect

Sjögren’s disease is not caused by bacterial infections. It is an autoimmune condition and the exact cause remains unclear though it is associated with genetic and environmental factors. However, bacterial or viral infections might trigger or exacerbate the disease in genetically predisposed individuals.