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Sjogren's Syndrome, Cause, Types, Pathogenesis, Clinical Signs and Complications.



sjogren's-syndrome-details

Sjogren's syndrome:
Sjogren's syndrome (sho'grenz syndrome) refers to a pathological condition, characterized by:
1. Dry eyes,
2. Dry mouth
3. Lack of moisture at sites where it normally present

Cause:
Dry eyes occur due to autoimmune destruction of Lacrimal glands and dry mouth occurs due to autoimmune destruction of salivary glands.
Autoimmune destruction means immune system of body kills its own cells instead of killing only foreign cells.
Lacrimal glands are responsible for tear secretion and Salivary glands secretes saliva, destruction of these glands result in symptoms like dry eyes and dry mouth.

Etiology:
a) Hyperactivity of B cells leading to auto antibodies formation, these antibodies combine with the receptors present of the salivary and lacrimal glands and result in their destruction
b) Hyper activity of helper T cells and Cytotoxic T cells.
c) Genetic factors also contribute to the Sjogren's syndrome, it is associated with HLA-DR3 and HLA DR4. any abnormality in these genes is a predisposing factors to Sjogren's syndrome.

Pathogenesis:
1. Role Of B cells:
B cells hyeractivity leads to formation of auto antibodies, that is antinuclear antibodies and rheumatoid factor, this leads to formation of immune complexes which will finally result in destruction of lacrimal and salivary glands.

2. Role of T cells.
a) Helper T cells aid local formation of antibodies and activation of cytotoxic T cells,
b) Cytotoxic T cells may cause tissue destruction but the firm evidence is lacking regarding the role of helper T cells and Cytotoxic cells in the pathogenesis of sjogren's syndrome.

Organs Involved:
Lacrimal and salivary glands are most affected, but this syndrome also affects glands of respiratory tract and vagina, CNS, Skin, kidney and muscles.

Types:
Primary Sjogren's syndrome:
It refers to sjogren's syndrome that occurs as an isolated disorder.

Secondary sjogren's syndrome.
It refers to "sjogren's syndrome that occurs in association with other autoimmune diseases such as rheumatoid arthritis.
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Predisposing factors:
1. Age: More common over 40 year of age.
2. Sex: more common in females.

Clinical Manifestations and signs:

1. Primary Manifestations and signs:
a) dry eye
b) Dry mouth
c) Lack of moisture where it is normally present.

2. Secondary Manifestations:
a) dryness of eye leads to corneal inflammation, erosion and ulceration.
b) Dryness of mouth leads to atrophy, inflammatory fissuring and ulceration of oral mucosa.
c) Dryness and  crusting of nose lead to ulceration and perforation of nasal septum.
d) Respiratory tract involvement leads to secondary laryngitis, bronchitis, and pneumonitis.
e) Renal involvement leads to renal tubular acidosis, uricosuria, phosphaturia and generalized aminoaciduria (fanconi's syndrome)

Complications:
All the secondary manifestations are the complications of sjogren's syndrome. moreover neoplastic transformation may also occur.
1. B cell lymphomas develop in one percent of patients.
2. Pseudo-lymphomas develop in 10 percent of patients, these comprise inflammatory hyper-plastic changes within salivary glands.
3. True malignant lymphoid tumors develop in some patients.

1 comment:

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