Lymphocytic hypophysitis

Changed by Bruno Di Muzio, 13 Apr 2016

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Lymphocytic hypophysitis (LH) is an uncommon non-neoplastic inflammatory condition that affects the pituitary gland. It is closely related to other inflammatory conditions in the region, namely orbital pseudotumour and Tolosa-Hunt syndrome.

Epidemiology

Lymphocytic hypophysitis is seen most frequently in women (strong female predilection with a M:FF:M of ~ 9:1), and often in the postpartum period or in the third trimester of pregnancy.

Clinical presentation

Clinical presentation is varied depends ofon the part of the pituitary affected and on the size of the lesion. Lymphocytic hypophysitis can thus be classified classified as:

  • anterior pituitary: lymphocytic adenohypophysitis (LAH)
    • most common
    • mimics a pituitary adenoma
    • endocrine hormone deficits are common
    • mass effects on adjacent structures (e.g. optic chiasm)
  • posterior pituitary: lymphocytic Infundibularinfundibular neurohypophysitis (LINH)
    • rare
    • diabetes insipidus
  • both anterior and posterior pituitary: lymphocytic Infudibularinfudibular panhypophysitis (LIPH)

Associations 

Pathology

It is characterised by infiltration of thepituitary stalk with lymphocytes (as, as the name would suggest).

Radiographic features

CT

Coronal CT and multiplanar reconstructions are able tocan visualise the pituitary region reasonably well. Lymphocytic hypophysitis appears as an enhancing soft tissue mass involving the pituitary and extending into the suprasellar region.

MRI

MRI, as is the case with other pituitary lesions, is the best modality for assessing this condition which appears as a pituitary region mass.

  • T1
    • affected regionarea is isointense with slight signal heterogeneity
    • normal posterior pituitary bright spot may be absent 8
  • T1 C+ (Gd)
    • can variably enhance, usually homogeneously 7
    • dural enhancement may be present 8
    • infundibulum may be thickened 8
  • T2
    • hypointensity in parasellar region can be present and may be useful in differentiating from a pituitary adenoma 4

Treatment and prognosis

Lymphocytic hypophysitis is usually self limiting-limiting and spontaneous recovery can occur. Corticosteroids are sometimes given and deficient hormones can be replaced 8.

Differential diagnosis

The differential diagnosis is essentiallyprimarily that of other pituitary region masses. Considerations include:

  • -<p><strong>Lymphocytic hypophysitis (LH)</strong> is an uncommon non-neoplastic inflammatory condition that affects the pituitary gland. It is closely related to other inflammatory conditions in the region, namely <a href="/articles/orbital-pseudotumour">orbital pseudotumour</a> and <a href="/articles/tolosa-hunt-syndrome-2">Tolosa-Hunt syndrome</a>.</p><h4>Epidemiology</h4><p>Lymphocytic hypophysitis is seen most frequently in women (strong female predilection with a M:F of ~ 9:1), and often in the postpartum period or in the third trimester of pregnancy.</p><h4>Clinical presentation</h4><p>Clinical presentation is varied depends of part of the pituitary affected and on the size of the lesion. Lymphocytic hypophysitis can thus be classified as:</p><ul>
  • +<p><strong>Lymphocytic hypophysitis (LH)</strong> is an uncommon non-neoplastic inflammatory condition that affects the pituitary gland. It is closely related to other inflammatory conditions in the region, namely <a href="/articles/orbital-pseudotumour">orbital pseudotumour</a> and <a href="/articles/tolosa-hunt-syndrome-2">Tolosa-Hunt syndrome</a>.</p><h4>Epidemiology</h4><p>Lymphocytic hypophysitis is seen most frequently in women (strong female predilection with a F:M of ~ 9:1), and often in the postpartum period or the third trimester of pregnancy.</p><h4>Clinical presentation</h4><p>Clinical presentation is varied depends on the part of the pituitary affected and the size of the lesion. Lymphocytic hypophysitis can thus be classified as:</p><ul>
  • -<li>posterior pituitary: lymphocytic Infundibular neurohypophysitis (LINH)<ul>
  • +<li>posterior pituitary: lymphocytic infundibular neurohypophysitis (LINH)<ul>
  • -<li>both anterior and posterior pituitary: lymphocytic Infudibular panhypophysitis (LIPH)</li>
  • +<li>both anterior and posterior pituitary: lymphocytic infudibular panhypophysitis (LIPH)</li>
  • -<li>auto-immune conditions such as:<ul>
  • +<li>auto-immune conditions such as<ul>
  • -<li>Ipilimumab</li>
  • -</ul><h4>Pathology</h4><p>It is characterised by infiltration of the <a href="/articles/pituitary-stalk">pituitary stalk</a> with lymphocytes (as the name would suggest).</p><h4>Radiographic features</h4><h5>CT</h5><p>Coronal CT and multiplanar reconstructions are able to visualise the pituitary region reasonably well. Lymphocytic hypophysitis appears as an enhancing soft tissue mass involving the pituitary and extending into the suprasellar region.</p><h5>MRI</h5><p>MRI, as is the case with other pituitary lesions, is the best modality for assessing this condition which appears as a <a href="/articles/pituitary-region-masses">pituitary region mass</a>.</p><ul>
  • +<li>ipilimumab: monoclonal antibody drug</li>
  • +</ul><h4>Pathology</h4><p>It is characterised by infiltration of the <a href="/articles/pituitary-stalk">pituitary stalk</a> with lymphocytes, as the name would suggest.</p><h4>Radiographic features</h4><h5>CT</h5><p>Coronal CT and multiplanar reconstructions can visualise the pituitary region reasonably well. Lymphocytic hypophysitis appears as an enhancing soft tissue mass involving the pituitary and extending into the suprasellar region.</p><h5>MRI</h5><p>MRI, as is the case with other pituitary lesions, is the best modality for assessing this condition which appears as a <a href="/articles/pituitary-region-masses">pituitary region mass</a>.</p><ul>
  • -<li>affected region is isointense with slight signal heterogeneity</li>
  • +<li>affected area is isointense with slight signal heterogeneity</li>
  • -</ul><h4>Treatment and prognosis</h4><p>Lymphocytic hypophysitis is usually self limiting and spontaneous recovery can occur. Corticosteroids are sometimes given and deficient hormones can be replaced <sup>8</sup>.</p><h4>Differential diagnosis</h4><p>The differential diagnosis is essentially that of other <a href="/articles/pituitary-region-masses">pituitary region masses</a>. Considerations include:</p><ul>
  • -<li><a href="/articles/pituitary-adenoma">pituitary adenoma</a></li>
  • +</ul><h4>Treatment and prognosis</h4><p>Lymphocytic hypophysitis is usually self-limiting and spontaneous recovery can occur. Corticosteroids are sometimes given and deficient hormones can be replaced <sup>8</sup>.</p><h4>Differential diagnosis</h4><p>The differential diagnosis is primarily that of other <a href="/articles/pituitary-region-masses">pituitary region masses</a>. Considerations include:</p><ul>
  • +<li>
  • +<a href="/articles/pituitary-adenoma">pituitary adenoma</a><ul><li>macroadenomas are expected to enlarge the sella turcica </li></ul>
  • +</li>
  • -<li>suprasellar <a href="/articles/meningioma">meningioma</a>
  • +<li>suprasellar <a href="/articles/meningioma">meningioma</a><ul>
  • +<li>dural based aspect</li>
  • +<li>usually follows the cerebral cortex intensity</li>
  • +</ul>
  • -<li><a href="/articles/pituitary-metastasis">pituitary metastasis</a></li>
  • +<li><a href="/articles/pituitary-metastases-1">pituitary metastasis</a></li>
  • -<a href="/articles/granulomatous-hypophysitis">granulomatous hypophysitis</a> (idiopathic of secondary to systemic illness e.g sarcoidosis, syphilis, and tuberculosis)</li>
  • +<a href="/articles/granulomatous-hypophysitis">granulomatous hypophysitis</a> (idiopathic of secondary to systemic illness e.g. sarcoidosis, syphilis, and tuberculosis)</li>

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