Sjögren syndrome

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Sjögren syndrome is an autoimmune condition of exocrine glands that produce tears and saliva.

Clinically, it manifests as:

keratoconjunctivitis sicca (xerophthalmia)
dryness of the mucous membrane of the mouth (xerostomia)
bilateral parotid enlargement and
an autoimmune disorder

Epidemiology

It is the 2^nd commonest autoimmune disorder after rheumatoid arthritis. There is a recognised female predilection with F:M ratio of ≈ 9:1. Patients typically present around the 4^thto 5^th decades.

Pathology

It is a chronic autoimmune disorder involving mainly the salivary and lacrimal glands and is associated with hyperactivity of the B-lymphocytes and with autoantibody and immune complex production.

Associations

Approximately ~~~ 40~~ ~40% of cases occur in isolation. Known associations include

thoracic changes associated with Sjögren syndrome
- non specific interstitial pneumonitis (NSIP) ~~- considered~~-considered the commonest pattern ^9-10
- usual interstitial pneumonitis ¹¹
- lymphoid interstitial pneumonia (LIP)
- focal lymphoid hyperplasia of the lung
- small airways disease ^7-8
Mikulicz syndrome (sometimes known as~~Sjogren~~ Sjogren type 1 ⁶)
other connective tissue diseases
EBV infection
Hepatitis C infection
HTLV infection

Markers

anti SSa(Ro) antibody
anti SSb(La) antibody
sjögren A antibody
sjögren B antibody

Radiographic features

MRI

Salivary glands

Parotid gland involvement may give a salt and pepper appearance or a honeycomb appearence. A change in size of the lacrimal glands associated with accelerated fat deposition may also be seen ³.

Ultrasound

Salivary glands

early stage- - the gland can be normal or become enlarged and hyperechoic ⁵
late stages ~~- may~~-may characteristically show a multicystic or reticular pattern within an atrophic gland ⁵

Complications

One of the complications of this syndrome is the development of malignant lymphoma.

-</ul><h4>Epidemiology</h4><p>It is the 2<sup>nd</sup> commonest autoimmune disorder after <a href="/articles/rheumatoid-arthritis">rheumatoid arthritis</a>. There is a recognised female predilection with F:M ratio of ≈ 9:1. Patients typically present around the 4<sup>th </sup>to 5<sup>th</sup> decades.</p><h4>Pathology</h4><p>It is a chronic autoimmune disorder involving mainly the salivary and lacrimal glands and is associated with hyperactivity of the B-lymphocytes and with autoantibody and immune complex production. </p><h5>Associations</h5><p>Approximately ~ 40 % of cases occur in isolation. Known associations include</p><ul>
+</ul><h4>Epidemiology</h4><p>It is the 2<sup>nd</sup> commonest autoimmune disorder after <a href="/articles/rheumatoid-arthritis">rheumatoid arthritis</a>. There is a recognised female predilection with F:M ratio of ≈ 9:1. Patients typically present around the 4<sup>th </sup>to 5<sup>th</sup> decades.</p><h4>Pathology</h4><p>It is a chronic autoimmune disorder involving mainly the salivary and lacrimal glands and is associated with hyperactivity of the B-lymphocytes and with autoantibody and immune complex production. </p><h5>Associations</h5><p>Approximately ~40% of cases occur in isolation. Known associations include</p><ul>
~~-<a href="/articles/non-specific-interstitial-pneumonia-1">non specific interstitial pneumonitis (NSIP)</a> - considered the commonest pattern <sup>9-10</sup>~~
+<a href="/articles/non-specific-interstitial-pneumonia-1">non specific interstitial pneumonitis (NSIP)</a>-considered the commonest pattern <sup>9-10</sup>
~~-<a href="/articles/mikulicz-syndrome">Mikulicz syndrome </a>(sometimes known as <strong>Sjogren type 1</strong> <sup>6</sup>)</li>~~
+<a href="/articles/mikulicz-syndrome">Mikulicz syndrome </a>(sometimes known as Sjogren type 1 <sup>6</sup>)</li>
~~-<li>~~
~~-<strong>early stage</strong> - the gland can be normal or become enlarged and hyperechoic <sup>5</sup> </li>~~
~~-<li>~~
~~-<strong>late stages</strong> - may characteristically show a multicystic or reticular pattern within an atrophic gland <sup>5</sup>~~
+<li>early stage<strong>-</strong>the gland can be normal or become enlarged and hyperechoic <sup>5</sup> </li>
+<li>late stages-may characteristically show a multicystic or reticular pattern within an atrophic gland <sup>5</sup>

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