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Table 1 Previous reports of sellar involvement in GPA

From: Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review

1st Author & year

PMID

No. of cases

Age at GPA diagnosis

Age at time of sellar involvement

Sex

Pituitary Imaging (MRI unless stated)

Endocrinological abnormalities

Non-CNS features

ANCA

Haynes 1978 [29]

692550

1

26*

25

M

NA

DI Hyperprolactinaemia Normal TSH

Normal LH & FSH

Lung, renal

N/A

Hurst 1983 [30]

6625709

1

47*

47

F

CT normal

DI Normal prolactin Normal TSH

Post-menopausal LH/FSH

Polyarthritis, ENT, ocular, mucocutaneous, pulmonary

N/A

Lohr 1988 [31]

3172100

1

19*

19

F

Intrasellar mass

N/A

ENT, pulmonary

N/A

Rosete 1991 [32]

1865428

1

51*

51

F

CT: pituitary enlargement

DI

N/A

N/A

Czarnecki 1995 [33]

7611087

1

31

34

F

Sellar mass with suprasellar extension. Enhancement of the stalk and hypothalamus. Loss of PS.

DI Hyperprolactinaemia

ENT, arthralgia

N/A

Roberts 1995 [34]

7758239

2

71*

71

F

Intrasellar mass with suprasellar extension

DI (post-surgery)

↓ TSH

↓ LH & FSH

↓ cortisol

Normal prolactin

None

cANCA +ve

28*

28

F

Intrasellar mass with low-density centre

DI (post-surgery) Normal prolactin Normal TSH

Normal LH & FSH

Normal cortisol

Ocular, arthralgia, cutaneous, renal

cANCA +ve

Bertken 1997 [35]

9265867

1

36*

36

F

Macrocystic pituitary mass with suprasellar extension. Hydrocephalus

DI (post surgery) ↓ TSH response ↓ LH & FSH responses

ENT, pulmonary, ocular

ANCA -ve

Hajj-Ali 1999 [36]

10461488

1

N/A

21

F

Normal

DI

N/A

N/A

Katzman 1999 [21]

10219422

2

41*

41

F

Pituitary enlargement. Loss of PS

DI Hyperprolactinaemia Normal TSH Normal LH & FSH

Constitutional, arthralgia, ENT, ocular, cutaneous

cANCA +ve

18*

18

F

Pituitary enlargement with contrast-enhancement.

Loss of PS

DI

Hyperprolactinaemia

Normal TSH

Normal LH & FSH

ENT, pulmonary

cANCA +ve

Miesen 1999 [37]

10069203

1

46*

45

M

Stalk thickening, contrast-enhancement.

Loss of PS

DI

Hyperprolactinaemia

Hypogonadism

Normal TSH

Normal LH & FSH

Normal cortisol & ACTH

ENT, renal, pulmonary

ANCA +ve

Goyal 2000 [38]

11003280

1

N/A (many years before pituitary involvement)

48

F

Sellar mass with suprasellar extension. Contrast-enhancement

DI

↓ TSH

Renal, pulmonary

cANCA +ve

Tappouni 2000 [39]

11096156

1

58*

57

F

Pituitary mass

DI

Constitutional, ENT, cutaneous, renal, pulmonary

PR3 +ve

Woywodt 2000 [40]

11028850

1

30*

30

M

N/A (diagnosed at autopsy)

N/A

N/A

N/A

Garovic 2001 [41]

11136194

1

47*

47

F

Cystic enlargement of the pituitary.

Non-enhancing with gadolinium

DI

↓ prolactin

↓ TSH

↓ gonadotropins

Constitutional, cutaneous, pulmonary

cANCA +ve

Tao 2003 [42]

14642162

1

N/A

19

F

Pituitary and stalk enlargement with heterogenous enhancement

DI

↓ TSH

Hypogonadism

N/A

N/A

Muir 2004 [43]

15150009

1

13*

13

M

Diffuse pituitary enlargement.

Foci of ↑ T1 signal. More extensive ↑ T2 signal. Central contrast-enhancement.

Loss of PS.

DI

ENT, pulmonary

ANCA +ve

Vittaz 2004 [44]

15687906

2

45

47

M

Pituitary mass, with contrast-enhancement.

Pituitary stalk thickened & infiltrated.

Loss of PS

Hyperprolactinaemia

↓ testosterone

↓ LH, normal FSH

↓ cortisol & ACTH

Normal TSH

Constitutional, polyarthritis, peripheral neuropathy, pulmonary

PR3 +ve

46

50

F

Pituitary enlargement

DI

Hyperprolactinaemia

Hypogonadism

Polyarthritis, ocular, mucocutaneous, ENT

ANCA +ve

Duzgun 2005 [45]

15864593

1

47*

47

F

Loss of PS

DI

Anterior pituitary hormones normal

Polyarthritis, pulmonary, ENT, renal

PR3 +ve

Seror 2006 [16]

16523054

3

45

50

F

Nodular pituitary enlargement. Homogenous contrast-enhancement. Loss of PS

DI

Hyperprolactinaemia

Thyrotropic deficiency

Hypogonadism

Corticotropic deficiency

ENT, ocular, mucocutaneous

PR3 +ve

26

41

F

Nodular pituitary enlargement. Contrast- enhancement

DI

Normal anterior pituitary hormones

ENT, arthralgia, ocular, renal

PR3 +ve

55

57

M

Pituitary enlargement & central necrosis. Heterogenous enhancement.

DI

Hyperprolactinaemia

Panhypopituitarism

Peripheral neuropathy, pulmonary, retinal vasculitis, digital, cerebral and renal infarcts

PR3 +ve

Spisek 2006 [46]

16322901

1

30*

29

M

Sellar cystic lesion

DI

↓ TSH

↓ LH & FSH

↓ ACTH

↓ IGF-1

ENT

PR3 +ve

McIntyre 2007 [47]

17318440

1

22*

22

F

Heterogeneous enhancing pituitary mass

DI

Hypogonadism

Cutaneous, ocular, renal

PR3 +ve

Thiryayi

2007 [48]

17188492

1

21*

21

F

Sellar mass with central hypo-intensity

DI (post-surgery)

Hypogonadism (post-surgery)

Constitutional, arthralgia

cANCA +ve

Yong 2008 [49]

17492510

1

33*

33

M

Pituitary stalk thickening. Contrast-enhancing nodule at the superior aspect

of the stalk.

Loss of PS.

DI

Hypogonadism

↓ ACTH

Normal prolactin

Normal TSH

Normal IGF-1

ENT

PR3 +ve

Cunnington 2009 [50]

20107566

3

19

24

M

Pituitary enlargement

DI

Anterior pituitary hormones normal

Constitutional, ENT, pulmonary, ocular, cutaneous

PR3 +ve

33

34

F

Diffusely enlarged gland containing a poorly enhancing lesion with supra-sellar extension. Loss of PS.

DI

Normal prolactin

Normal TSH

Constitutional, ENT

cANCA+ve

26

35

M

Diffusely enlarged pituitary and thickened stalk.

DI

↓ TSH

Constitutional, ENT, pulmonary

cANCA +ve

Xue 2009 [51]

19172275

1

63*

63

F

Normal

DI

TSH normal

LH & FSH normal

Constitutional, pulmonary, peripheral neuropathy

PR3 +ve

Barlas 2011 [52]

21116602

1

35

37

F

Anterior enlargement.

Central area with low signal on T1 and high signal on T2. Post contrast enhancement of pituitary and stalk. Loss of PS.

DI

Hyperprolactinaemia

ENT, pulmonary, polyarthritis

cANCA +ve

Santoro 2011 [53]

22147097

1

53*

53

F

Hypointensity of adenohypophysis on T1.

Hyperintense sectors on R side on T2.

Peripheral contrast-enhancement.

Stalk-thickening.

Loss of PS.

DI

↓ TSH

Hypogonadism

Normal prolactin

Polyarthritis, cutaneous, pulmonary, renal

cANCA +ve

Tenorio- Jimenez 2011 [54]

22673710

1

23

38

F

MRI: marked infundibular thickening, sellar mass with hypointensity on T1. Loss of PS

Hyperprolactinaemia

↓ TSH

Hypogonadism

Post pituitary hormones unaffected

ENT, pulmonary, renal

cANCA +ve (−ve by the time of sellar manifestations)

Hughes 2013 [55]

23186961

1

N/A

30

F

Sellar mass

Panhypopituitarism

Ocular

N/A

Pereira 2013 [56]

22898089

1

48*

48

F

Appearances of pituitary microadenoma, but histopathlogy revealed

necrotizing

granulomatous

inflammation

Hyperprolactinaemia

↓ TSH

Post pituitary hormones unaffected

ENT

N/A

Kapoor 2014 [20]

25077899

8

N/A

67

F

Peripherally enhancing cystic sellar mass compressing the stalk

Hyperprolactinaemia

Hypogonadism

Normal TSH

ENT, renal

7/8 cases PR3 +ve

N/A

48

F

Multiple non-enhancing cystic areas in the pituitary, convexity of superior margin of pituitary

DI

↓ prolactin

↓ TSH

Hypogonadism

Corticotropic deficiency

ENT, pulmonary, cutaneous

N/A

28

F

Sellar mass with large zone of central non-enhancement and peripheral enhancement. Stalk displacement

DI

Hypogonadism

Normal prolactin

Normal TSH

Normal IGF-1

Normal cortisol

ENT, pulmonary, renal

N/A

55

M

Sellar mass with suprasellar extension

DI

Hypogonadism

Normal prolactin

Normal TSH

Normal cortisol

Normal IGF-1

ENT, pulmonary, renal, cutaneous, joints

N/A

35

M

Necrotic sellar mass with peripheral enhancement & suprasellar extension. Thickened contrast-enhancing stalk

DI

↓ TSH

Hypogonadism

Normal prolactin

Normal cortisol

Normal IGF-1

ENT

N/A

54

M

Enlarged pituitary measuring 12 mm, with heterogeneous enhancement. Slight diffuse thickening of the stalk

DI

↓ TSH

Hypogonadism

Normal prolactin

Normal IGF-1

ENT, pulmonary, renal, cardiac

N/A

68

M

Homogeneously enhancing sellar mass, extending into the cavernous sinus bilaterally. Stalk preserved.

↓ prolactin

↓ TSH

Hypogonadism

↓ IGF-1

ENT, joints

N/A

28

F

Sellar mass extending into the suprasellar cistern, with low T2 signal in the periphery and a bright centre. Peripheral enhancement with central cystic change. Thickening of pituitary stalk

DI

Normal prolactin

Normal TSH

Normal cortisol

Normal IGF-1

ENT

De Parisot 2015 [19]

25906106

9

46*

46

F

Enlarged posterior pituitary. Infiltration of posterior pituitary. Loss of PS

DI

ENT, ocular

 

60

70

M

Normal

↓ TSH

Hypogonadism

↓ IGF-1

Normal prolactin

ENT, peripheral neuropathy

 

23

24

F

Enlarged pituitary. Irregularity of infundibulum. Heterogeneous enhancement of anterior pituitary. Loss of PS

DI

↓ TSH

Hypogonadism

Normal prolactin

Normal IGF-1

ENT

 

24*

24

M

Enlarged infundibulum

DI

Hyperprolactinaemia

↓ TSH

Hypogonadism

Normal IGF-1

Renal, ocular, joints, gastro-intestinal

 

66

77

M

Enlarged pituitary. Stalk infiltration.

Loss of PS.

↓ TSH

Hypogonadism

↓ IGF-1

Normal prolactin

None

 

67

68

F

Normal

DI

Hyperprolactinaemia

Normal TSH

Normal LH & FSH

Normal IGF-1

ENT

 

28

42

F

Heterogeneous enhancement of pituitary

DI

Hyperprolactinaemia

Hypogonadism

↓ ACTH

ENT, lung

 

55

57

M

Sellar mass, heterogeneous.

Enhancement. Enlargement and infiltration of stalk. Loss of PS

DI

Hyperprolactinaemia

↓ TSH

Hypogonadism

↓ ACTH

Pulmonary

 

46

50

F

Enlargement and infiltration of pituitary. Heterogeneous enhancement, contact with optic chiasm

DI

Hyperprolactinaemia

Hypogonadism

Normal TSH

Normal IGF-1

ENT, ocular

 

Eli 2016 [22]

27521731

1

32*

29

F

Homogenously enhancing sellar mass. Thickened stalk.

Hyperprolactinaemia

ENT, pulmonary

MPO +ve

Esposito 2017 [57]

28540625

3

37*

37

F

Sellar mass extending into the suprasellar cistern with peripheral enhancement and central cystic lesion. Stalk deviation.

DI

Hypogonadism

GH deficiency

Constitutional, ENT

PR3 +ve

36*

36

F

Cystic pituitary mass

DI

Anterior pituitary function normal

Constitutional, myalgia, ENT, pulmonary

PR3 +ve

32*

32

F

Sellar mass with homogeneous. Thickening of the pituitary stalk

DI

Anterior pituitary function normal

ENT

PR3 +ve

  1. Abbreviations: ACTH adrenocorticotropic hormone, cANCA cytoplasmic pattern ANCA staining, DI diabetes insipidus, GH growth hormone, IGF-1 insulin-like growth factor 1, LH luteinising hormone, FSH follicular stimulating hormone, MPO myeloperoxidase, N/A data not available, PMID PubMed ID, PR3 proteinase-3, PS posterior signal.
  2. *indicates cases where pituitary involvement was diagnosed prior to or at the time of GPA diagnosis
  3. For the purposes of the Table, a TSH within the reference range but inappropriately low for the T4 has been included in the category “↓ TSH”