Sheehan's syndrome differential diagnosis: Difference between revisions

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Sheehan's syndrome should be differentiated from other diseases causing hypopituitarism.<ref name="pmid9541295">{{cite journal |vauthors=Sato N, Sze G, Endo K |title=Hypophysitis: endocrinologic and dynamic MR findings |journal=AJNR Am J Neuroradiol |volume=19 |issue=3 |pages=439–44 |year=1998 |pmid=9541295 |doi= |url=}}</ref><ref name="pmid7758238">{{cite journal |vauthors=Powrie JK, Powell M, Ayers AB, Lowy C, Sönksen PH |title=Lymphocytic adenohypophysitis: magnetic resonance imaging features of two new cases and a review of the literature |journal=Clin. Endocrinol. (Oxf) |volume=42 |issue=3 |pages=315–22 |year=1995 |pmid=7758238 |doi= |url=}}</ref><ref name="pmid26262437">{{cite journal |vauthors=Honegger J, Schlaffer S, Menzel C, Droste M, Werner S, Elbelt U, Strasburger C, Störmann S, Küppers A, Streetz-van der Werf C, Deutschbein T, Stieg M, Rotermund R, Milian M, Petersenn S |title=Diagnosis of Primary Hypophysitis in Germany |journal=J. Clin. Endocrinol. Metab. |volume=100 |issue=10 |pages=3841–9 |year=2015 |pmid=26262437 |doi=10.1210/jc.2015-2152 |url=}}</ref><ref name="pmid7629223">{{cite journal |vauthors=Thodou E, Asa SL, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S |title=Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings |journal=J. Clin. Endocrinol. Metab. |volume=80 |issue=8 |pages=2302–11 |year=1995 |pmid=7629223 |doi=10.1210/jcem.80.8.7629223 |url=}}</ref><ref name="pmid8345854">{{cite journal |vauthors=Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamabe H |title=Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus |journal=N. Engl. J. Med. |volume=329 |issue=10 |pages=683–9 |year=1993 |pmid=8345854 |doi=10.1056/NEJM199309023291002 |url=}}</ref><ref name="pmid21668725">{{cite journal |vauthors=Hsieh CY, Liu BY, Yang YN, Yin WH, Young MS |title=Massive pericardial effusion with diastolic right ventricular compression secondary to hypothyroidism in a 73-year-old woman |journal=Emerg Med Australas |volume=23 |issue=3 |pages=372–5 |year=2011 |pmid=21668725 |doi=10.1111/j.1742-6723.2011.01425.x |url=}}</ref>
Sheehan's syndrome should be differentiated from other diseases causing hypopituitarism.<ref name="pmid9541295">{{cite journal |vauthors=Sato N, Sze G, Endo K |title=Hypophysitis: endocrinologic and dynamic MR findings |journal=AJNR Am J Neuroradiol |volume=19 |issue=3 |pages=439–44 |year=1998 |pmid=9541295 |doi= |url=}}</ref><ref name="pmid7758238">{{cite journal |vauthors=Powrie JK, Powell M, Ayers AB, Lowy C, Sönksen PH |title=Lymphocytic adenohypophysitis: magnetic resonance imaging features of two new cases and a review of the literature |journal=Clin. Endocrinol. (Oxf) |volume=42 |issue=3 |pages=315–22 |year=1995 |pmid=7758238 |doi= |url=}}</ref><ref name="pmid26262437">{{cite journal |vauthors=Honegger J, Schlaffer S, Menzel C, Droste M, Werner S, Elbelt U, Strasburger C, Störmann S, Küppers A, Streetz-van der Werf C, Deutschbein T, Stieg M, Rotermund R, Milian M, Petersenn S |title=Diagnosis of Primary Hypophysitis in Germany |journal=J. Clin. Endocrinol. Metab. |volume=100 |issue=10 |pages=3841–9 |year=2015 |pmid=26262437 |doi=10.1210/jc.2015-2152 |url=}}</ref><ref name="pmid7629223">{{cite journal |vauthors=Thodou E, Asa SL, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S |title=Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings |journal=J. Clin. Endocrinol. Metab. |volume=80 |issue=8 |pages=2302–11 |year=1995 |pmid=7629223 |doi=10.1210/jcem.80.8.7629223 |url=}}</ref><ref name="pmid8345854">{{cite journal |vauthors=Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamabe H |title=Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus |journal=N. Engl. J. Med. |volume=329 |issue=10 |pages=683–9 |year=1993 |pmid=8345854 |doi=10.1056/NEJM199309023291002 |url=}}</ref><ref name="pmid21668725">{{cite journal |vauthors=Hsieh CY, Liu BY, Yang YN, Yin WH, Young MS |title=Massive pericardial effusion with diastolic right ventricular compression secondary to hypothyroidism in a 73-year-old woman |journal=Emerg Med Australas |volume=23 |issue=3 |pages=372–5 |year=2011 |pmid=21668725 |doi=10.1111/j.1742-6723.2011.01425.x |url=}}</ref>
{| class="wikitable"
{| class="wikitable"
! rowspan="3" |Diseases
! rowspan="3" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Diseases}}
! rowspan="3" |Onset
! rowspan="3" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Onset}}
! colspan="5" |Manifestations
! colspan="5" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Manifestations}}
! colspan="4" |Diagnosis
! colspan="4" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Diagnosis}}
|-
|-
! colspan="4" |History and Symptoms
! colspan="4" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|History and Symptoms}}
! rowspan="2" |Physical examination
! rowspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Physical examination}}
! rowspan="2" |Laboratory findings
! rowspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Laboratory findings}}
! rowspan="2" |Gold standard
! rowspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Gold standard}}
! rowspan="2" |Imaging
! rowspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF| Imaging}}
! rowspan="2" |Other investigation findings
! rowspan="2" style="background: #4479BA; width: 200px;" | {{fontcolor|#FFF|Other investigation findings}}
|-
|-
!Trumatic delivery
!Trumatic delivery
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* Decreased axillary and pubic hair growth
* Decreased axillary and pubic hair growth
|Pancytopenia
|
* Pancytopenia


Eosinophilia
* Eosinophilia


Hyponatremia
* Hyponatremia


Low fasting plasma glucose
* Low fasting plasma glucose


Decreased levels of anterior pituitary hormones in blood.
* Decreased levels of anterior pituitary hormones in blood.


|Dx is clinical  with key feature of traumatic delivery.
|
-Most senitive test is low baseline prolactin levels w/o response to TRH.
* Dx is clinical   
|Sequential changes of pituitary enlargement followed by shrinkage and necrosis leading to decreased sellar volume or empty sella.
 
* Most senitive test is low baseline prolactin levels w/o response to TRH.
|CT/MRI shows sequential changes of pituitary enlargement followed by shrinkage and necrosis leading to decreased sellar volume or empty sella.
|
|
|-
|-
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* [[CT]] scan without [[Contrast medium|contrast]] is the initial test of choice. [[Pituitary hemorrhage|Pituitary hemorrhag]]<nowiki/>e on [[CT]] presents as a hyper-dense lesion.
* [[CT]] scan without [[Contrast medium|contrast]] is the initial test of choice. [[Pituitary hemorrhage|Pituitary hemorrhag]]<nowiki/>e on [[CT]] presents as a hyper-dense lesion.


* [[MRI]] is done in cases of inconclusive [[CT]]. An [[MRI]] is more sensitive in identifying [[intrasellar]]<nowiki/>mass and soft tissue changes
* [[MRI]] is done in cases of inconclusive [[CT]].  
|
|
[[Blood tests]] may be done to check:
[[Blood tests]] may be done to check:
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* Autoimmune thyroiditis
* Autoimmune thyroiditis
|<nowiki>-Decreased pituitary hormones(Gonadotropins most common)</nowiki>
|
* Decreased pituitary hormones(Gonadotropins most common)


-Hyperprolactinemia
* Hyperprolactinemia(40%)


-GH excess
* GH excess
|Pituitary biopsy
|Pituitary biopsy
|[[CT]] & [[MRI]] typically reveal --features of a [[Pituitary gland|pituitary]] [[mass]]
|
-diffuse and homogeneous contrast enhancement
* [[CT]] & [[MRI]] typically reveal --features of a [[Pituitary gland|pituitary]] [[mass]]
 
* Diffuse and homogeneous contrast enhancement
|The most accurate test is a [[Pituitary gland|pituitary]][[biopsy]] which will show [[lymphocytic]][[Infiltration (medical)|infiltration]].
|The most accurate test is a [[Pituitary gland|pituitary]][[biopsy]] which will show [[lymphocytic]][[Infiltration (medical)|infiltration]].
|-
|-
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|[[Digital subtraction angiography]]
|[[Digital subtraction angiography]]
|
|
* The [[modality]] of choice for [[diagnosis]] of [[subarachnoid hemorrhage]] is noncontrast head [[Computed tomography|computed tomography (CT)]], with or without [[lumbar puncture]].<sup>[[Subarachnoid hemorrhage CT#cite note-pmid7897421-1|[1]]]</sup>
* Noncontrast head [[Computed tomography|computed tomography (CT)]], with or without [[lumbar puncture]].<sup>[[Subarachnoid hemorrhage CT#cite note-pmid7897421-1|[1]]]</sup>
* [[Computed tomography|CT]] shows hyperattenuating material filling the [[subarachnoid space]].
* [[Computed tomography|CT]] shows hyperattenuating material filling the [[subarachnoid space]].
|
|
[[Lumbar puncture|Lumbar puncture (LP)]] is necessary when there is a strong suspicion of [[subarachnoid hemorrhage]]. LP will show:
[[Lumbar puncture|Lumbar puncture (LP)]]  shows:
* Elevated opening [[pressure]]
* Elevated opening [[pressure]]
* Elevated [[Red blood cell|red blood cell (RBC)]]
* Elevated [[Red blood cell|red blood cell (RBC)]]
Line 166: Line 172:
* Loss of body hair
* Loss of body hair
|Decreased levels of anterior pituitary hormones in blood.
|Decreased levels of anterior pituitary hormones in blood.
|
|MRI
|
|
|
|
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* Delayed relaxation phase of deep tendon reflexes
* Delayed relaxation phase of deep tendon reflexes
|low T3,T4
|
* Low T3,T4


High TSH
* High TSH


Rest of pituitary hormone levels WNL
* Rest of pituitary hormone levels WNL
|TSH levels
|TSH levels
|Done to rule out ant pituitary cause
|Done to rule out ant pituitary cause
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* Decreased libido
* Decreased libido
|
|
|Low estrogen, testosterone
|
* Low estrogen, testosterone


High FSH/LH
* High FSH/LH
|
|
|
|
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* Genetic tests including a karyotype to check chromosomal structure
* Genetic tests including a karyotype to check chromosomal structure
* Prolactin level (milk hormone)
* Prolactin level (milk hormone)
* Sperm count
* Thyroid tests
|-
|-
|Hypoprolactinemia
|Hypoprolactinemia
Line 235: Line 241:
|
|
|Done to rule out any pituitary cause
|Done to rule out any pituitary cause
|<nowiki>-Prolactin assay in 3rd trimester</nowiki>
|
* Prolactin assay in 3rd trimester


-LH, FSH
* LH, FSH


-Thyrotropin and free thyroxine
* Thyrotropin and free thyroxine
|-
|-
|Panhypopituitarism
|Panhypopituitarism
Line 246: Line 253:
|<nowiki>+</nowiki>
|<nowiki>+</nowiki>
|Oligo/amenorrhea
|Oligo/amenorrhea
|Polyuria
|
* Polyuria


Polydipsia
* Polydipsia
|<nowiki>-Growth failure</nowiki>
* Features of hypothyroidism and hypoadrenalism
|
* Growth failure


-B/L hemianopsia
* B/L hemianopsia


-Papilledema
* Papilledema
|all pituitary hormones decreased
|All pituitary hormones decreased
|MRI
|MRI
|
|
Line 264: Line 274:
| -
| -
|<nowiki>-</nowiki>
|<nowiki>-</nowiki>
|Hypoglycemia
|
* Hypoglycemia


Hypotension
* Hypotension
|<nowiki>-Dehydration</nowiki>
|
* Dehydration


-Hyperpigmentation
* Hyperpigmentation


-loss of pubic and axillary hair
* loss of pubic and axillary hair


|<nowiki>-Hyponatremia with/without hyperkalemia</nowiki>
|
* Hyponatremia with/without hyperkalemia


-Plasma renin activity to aldosterone ratio
* Plasma renin activity to aldosterone ratio
|CT abdomen
|CT abdomen
|CT abdomen
|CT abdomen
|<nowiki>-Serum cortisol testing</nowiki>
|
* Serum cortisol testing


-Serum ACTH testing
* Serum ACTH testing


-Antiadrenal Ab testing
* Anti-adrenal Ab testing
|-
|-
|Menopause
|Menopause

Revision as of 20:21, 22 August 2017

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Iqra Qamar M.D.[2]

Overview

Sheehan syndrome must be differentiated from lymphocytic hypophysitis, pituitary apoplexy, hypothyroidism, Addison's disease, panhypopititarism, empty sella syndrome, hypogonadotropic hypogonadism, Simmond's disease, hypoprolactinemia, menopause,female athlete triadand SAH.[1][2][3]

Differentiating Sheehan's Syndrome from other Diseases

Sheehan's syndrome should be differentiated from other diseases causing hypopituitarism.[4][5][6][7][8][9]

Diseases Onset Manifestations Diagnosis
History and Symptoms Physical examination Laboratory findings Gold standard Imaging Other investigation findings
Trumatic delivery Lactation failure Menstrual irregularities Other features
Sheehan's syndrome Acute ++ ++ Oligo/amenorrhea
  • Adrenal insufficiency symptoms
  • Hypothyroidism features
  • Breast tissue atrophy
  • Decreased axillary and pubic hair growth
  • Pancytopenia
  • Eosinophilia
  • Hyponatremia
  • Low fasting plasma glucose
  • Decreased levels of anterior pituitary hormones in blood.
  • Dx is clinical
  • Most senitive test is low baseline prolactin levels w/o response to TRH.
 CT/MRI shows sequential changes of pituitary enlargement followed by shrinkage and necrosis leading to decreased sellar volume or empty sella.
Pituitary apoplexy Acute +/- ++ Oligo/amenorrhea Severe headache
  • Visual acuity defects
  • CN palsies (nerves III, IV, V , and VI)
 Decreased levels of anterior pituitary hormones in blood. MRI
  • MRI is done in cases of inconclusive CT.

Blood tests may be done to check:

Lymphocytic hypophysitis Acute +/- + Oligo/amenorrhea
  • Associated with autoimmune conditions
  • Retro-orbital or Bitemporal pain
  • DI
  • Autoimmune thyroiditis
  • Decreased pituitary hormones(Gonadotropins most common)
  • Hyperprolactinemia(40%)
  • GH excess
 Pituitary biopsy
  • Diffuse and homogeneous contrast enhancement
 The most accurate test is a pituitarybiopsy which will show lymphocyticinfiltration.
Subarachnoid hemorrhage Acute - - - Signs of meningeal irritation Xanthochromia Digital subtraction angiography

Lumbar puncture (LP)  shows:

Empty sella syndrome Chronic - + Oligo/amenorrhea
  • Erectile dysfunction
  • Headache
  • Low libido
  • Signs of raised intracranial pressure may be present
  • Nipple discharge
 Decreased levels of pituitary hormones in blood. MRI Empty sella containing CSF
Simmond's disease/Pituitary chachexia Chronic +/- + Oligo/amenorrhea
  • Cachexia
  • Premature aging
  • Progressive emaciation
  • Loss of body hair
 Decreased levels of anterior pituitary hormones in blood. MRI
Primary hypothyroidism Chronic +/- - Oligomenorrhea/menorrhagia
  • Cold intolerance
  • Constipation
  • Dry skin
  • Bradycardia
  • Hair loss
  • Myxedema
  • Delayed relaxation phase of deep tendon reflexes
  • Low T3,T4
  • High TSH
  • Rest of pituitary hormone levels WNL
 TSH levels Done to rule out ant pituitary cause
Primary hypogonadotropic hypogonadism Chronic - - Oligo/amenorrhea
  • Hot flushes
  • Energy and mood changes
  • Decreased libido
  • Low estrogen, testosterone
  • High FSH/LH
  • Done to rule out any pituitary cause
  • Blood tests for anemia and iron
  • Genetic tests including a karyotype to check chromosomal structure
  • Prolactin level (milk hormone)
Hypoprolactinemia Chronic - + - Infertility

Subfertiliy

Puerperal agalactogenesis No workup is necessary Done to rule out any pituitary cause
  • Prolactin assay in 3rd trimester
  • LH, FSH
  • Thyrotropin and free thyroxine
Panhypopituitarism Chronic - + Oligo/amenorrhea
  • Polyuria
  • Polydipsia
  • Features of hypothyroidism and hypoadrenalism
  • Growth failure
  • B/L hemianopsia
  • Papilledema
 All pituitary hormones decreased MRI Left hand and wrist radiograph for bone age
Primary adrenal insufficiency/Addison's disease Chronic - - -
  • Hypoglycemia
  • Hypotension
  • Dehydration
  • Hyperpigmentation
  • loss of pubic and axillary hair
  • Hyponatremia with/without hyperkalemia
  • Plasma renin activity to aldosterone ratio
 CT abdomen CT abdomen
  • Serum cortisol testing
  • Serum ACTH testing
  • Anti-adrenal Ab testing
Menopause Chronic - +/- Oligo/amenorrhea
Female athlete triad Chronic - - Oligo/amenorrhea
  1. ↑ Rolih CA, Ober KP (1993). "Pituitary apoplexy". Endocrinol. Metab. Clin. North Am. 22 (2): 291–302. PMID 8325288.
  2. ↑ Vidal E, Cevallos R, Vidal J, Ravon R, Moreau JJ, Rogues AM, Loustaud V, Liozon F (1992). "Twelve cases of pituitary apoplexy". Arch. Intern. Med. 152 (9): 1893–9. PMID 1520058.
  3. ↑ Lazaro CM, Guo WY, Sami M, Hindmarsh T, Ericson K, Hulting AL, Wersäll J (1994). "Haemorrhagic pituitary tumours". Neuroradiology. 36 (2): 111–4. PMID 8183446.
  4. ↑ Sato N, Sze G, Endo K (1998). "Hypophysitis: endocrinologic and dynamic MR findings". AJNR Am J Neuroradiol. 19 (3): 439–44. PMID 9541295.
  5. ↑ Powrie JK, Powell M, Ayers AB, Lowy C, Sönksen PH (1995). "Lymphocytic adenohypophysitis: magnetic resonance imaging features of two new cases and a review of the literature". Clin. Endocrinol. (Oxf). 42 (3): 315–22. PMID 7758238.
  6. ↑ Honegger J, Schlaffer S, Menzel C, Droste M, Werner S, Elbelt U, Strasburger C, Störmann S, Küppers A, Streetz-van der Werf C, Deutschbein T, Stieg M, Rotermund R, Milian M, Petersenn S (2015). "Diagnosis of Primary Hypophysitis in Germany". J. Clin. Endocrinol. Metab. 100 (10): 3841–9. doi:10.1210/jc.2015-2152. PMID 26262437.
  7. ↑ Thodou E, Asa SL, Kontogeorgos G, Kovacs K, Horvath E, Ezzat S (1995). "Clinical case seminar: lymphocytic hypophysitis: clinicopathological findings". J. Clin. Endocrinol. Metab. 80 (8): 2302–11. doi:10.1210/jcem.80.8.7629223. PMID 7629223.
  8. ↑ Imura H, Nakao K, Shimatsu A, Ogawa Y, Sando T, Fujisawa I, Yamabe H (1993). "Lymphocytic infundibuloneurohypophysitis as a cause of central diabetes insipidus". N. Engl. J. Med. 329 (10): 683–9. doi:10.1056/NEJM199309023291002. PMID 8345854.
  9. ↑ Hsieh CY, Liu BY, Yang YN, Yin WH, Young MS (2011). "Massive pericardial effusion with diastolic right ventricular compression secondary to hypothyroidism in a 73-year-old woman". Emerg Med Australas. 23 (3): 372–5. doi:10.1111/j.1742-6723.2011.01425.x. PMID 21668725.

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