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

ItSjogren syndrome is the 2~~^nd ~~commonest~~~~second most common 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.

Clinical presentation

Clinically, it manifests as:

keratoconjunctivitis sicca (xerophthalmia)
dryness of the mucous membrane of the mouth (xerostomia)
bilateral parotid enlargement

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~~nonspecific interstitial pneumonitis (NSIP)~~-considered~~: considered the ~~commonest~~most common 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 1⁶)
other connective tissue diseases
EBV infection
Hepatitis C infection
primary biliary cirrhosis ~~- possible~~ (possible ¹²)
HTLV infection

Markers

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

Radiographic features

MRI

Salivary glands

Parotid gland involvement may give asalt and pepper appearance or a honeycomb ~~appearence~~appearance. 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 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.

History and etymology

This condition is named after Swedish ophthalmologist Henrik Sjögren (1899–1986) although it was first described by WB Hadden and JW Hutchinson in 1871 ¹³.

~~-<p><strong>Sjögren syndrome</strong> is an autoimmune condition of exocrine glands that produce tears and saliva.</p><p>Clinically, it manifests as:</p><ul>~~
+<p><strong>Sjögren syndrome</strong> is an autoimmune condition of exocrine glands that produce tears and saliva.</p><h4>Epidemiology</h4><p>Sjogren syndrome is the second most common 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>Clinical presentation</h4><p>Clinically, it manifests as:</p><ul>
~~-<a href="/articles/keratoconjunctivitis-sicca">keratoconjunctivitis sicca</a> (<a href="/articles/xerophthalmia">xerophthalmia</a>)</li>~~
+<a href="/articles/keratoconjunctivitis-sicca">keratoconjunctivitis sicca</a> (<a href="/articles/xerophthalmia">xerophthalmia</a>)</li>
~~-<li>bilateral parotid enlargement and </li>~~
~~-<li>an autoimmune disorder</li>~~
-</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>
+<li>bilateral <a href="/articles/parotid-enlargement">parotid enlargement</a>
+</li>
+</ul><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">nonspecific interstitial pneumonitis (NSIP)</a>: considered the most common pattern <sup>9-10</sup>
~~-<a href="/articles/mikulicz-syndrome">Mikulicz syndrome </a>(sometimes known as Sjogren type 1 <sup>6</sup>)</li>~~
+<a href="/articles/mikulicz-syndrome">Mikulicz syndrome </a>(sometimes known as Sjogren type 1 <sup>6</sup>)</li>
~~-<a href="/articles/primary-biliary-cirrhosis">primary biliary cirrhosis</a> - possible <sup>12</sup>~~
~~-</li>~~
+<a href="/articles/primary-biliary-cirrhosis">primary biliary cirrhosis</a> (possible <sup>12</sup>)</li>
~~-<li>sjögren A antibody</li>~~
~~-<li>sjögren B antibody</li>~~
-</ul><h4>Radiographic features</h4><h5>MRI </h5><h6>Salivary glands</h6><p>Parotid gland involvement may give a <a href="/articles/salt-and-pepper-sign">salt and pepper</a> appearance or a honeycomb appearence. A change in size of the lacrimal glands associated with accelerated fat deposition may also be seen <sup>3</sup>.</p><h5>Ultrasound</h5><h6>Salivary glands</h6><ul>
+<li>sjögren A antibody</li>
+<li>sjögren B antibody</li>
+</ul><h4>Radiographic features</h4><h5>MRI </h5><h6>Salivary glands</h6><p>Parotid gland involvement may give a <a href="/articles/salt-and-pepper-sign">salt and pepper</a> appearance or a honeycomb appearance. A change in size of the lacrimal glands associated with accelerated fat deposition may also be seen <sup>3</sup>.</p><h5>Ultrasound</h5><h6>Salivary glands</h6><ul>
~~-</ul><h4>Complications</h4><p>One of the complications of this syndrome is the development of <a href="/articles/malignant-lymphoma">malignant lymphoma</a>.</p>~~
+</ul><h4>Complications</h4><p>One of the complications of this syndrome is the development of <a href="/articles/malignant-lymphoma">malignant lymphoma</a>.</p><h4>History and etymology</h4><p>This condition is named after Swedish ophthalmologist <strong>Henrik Sjögren</strong> (1899–1986) although it was first described by <strong>WB Hadden</strong> and <strong>JW Hutchinson</strong> in 1871 <sup>13</sup>. </p>

References changed:

13. Ghafoor M. Sjögren's Before Sjögren: Did Henrik Sjögren (1899-1986) Really Discover Sjögren's Disease? J Maxillofac Oral Surg. 2012;11(3):373-4. <a href="https://doi.org/10.1007/s12663-011-0303-0">doi:10.1007/s12663-011-0303-0</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/23997498">Pubmed</a>

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