1 Lacrimal Gland Enlargement Enlarged lacrimal gland Two

1 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Hint: NOT ‘benign’ and ‘malignant’— think more broadly than that!

2 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory

3 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Two broad categories in the differential NOT ‘benign’ and ‘malignant’— again, think more broadly than that! Inflammatory

4 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid

5 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Two broad categories in the differential Lymphoid

6 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Two broad categories in the differential Benign (Finally!) Malignant Lymphoid

7 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign ? Malignant ? Most common within each category?

8 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Most common within each category?

9 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma What is the classic pathologic description of adenoid cystic carcinoma? It looks like ‘Swiss cheese’ owing to its cystic nature How is the diagnosis confirmed? Via incisional biopsy There are five subtypes based on histology. All are bad, but which conveys the worst prognosis? Basaloid

10 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma What is the classic pathologic description of adenoid cystic carcinoma? It looks like ‘Swiss cheese’ owing to its cystic nature How is the diagnosis confirmed? Via incisional biopsy There are five subtypes based on histology. All are bad, but which conveys the worst prognosis? Basaloid

11 Lacrimal Gland Enlargement Adenoid cystic carcinoma: Swiss cheese appearance

12 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma What is the classic pathologic description of adenoid cystic carcinoma? It looks like ‘Swiss cheese’ owing to its cystic nature How is the diagnosis confirmed? Via incisional biopsy There are five subtypes based on histology. All are bad, but which conveys the worst prognosis? Basaloid

13 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma What is the classic pathologic description of adenoid cystic carcinoma? It looks like ‘Swiss cheese’ owing to its cystic nature How is the diagnosis confirmed? Via incisional biopsy There are five subtypes based on histology. All are bad, but which conveys the worst prognosis? Basaloid

14 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma What is the classic pathologic description of adenoid cystic carcinoma? It looks like ‘Swiss cheese’ owing to its cystic nature How is the diagnosis confirmed? Via incisional biopsy There are five subtypes based on histology. All are bad, but which conveys the worst prognosis? Basaloid

15 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma What is the classic pathologic description of adenoid cystic carcinoma? It looks like ‘Swiss cheese’ owing to its cystic nature How is the diagnosis confirmed? Via incisional biopsy B asaloid ad There are five subtypes based on histology. All are bad, but which conveys the worst prognosis? Basaloid

16 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma If pleomorphic adenoma is suspected, should incisional biopsy be performed? Malignant No! The tumor should be removed en bloque, without violating its pseudocapsule Adenoid cystic carcinoma Benign lymphoproliferative Why must a pleomorphic diseaseadenoma be managed in this way? Incomplete excision increases the risk of recurrence, which can be malignant (a process known as malignant transformation)

17 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma If pleomorphic adenoma is suspected, should incisional biopsy be performed? Malignant No! The tumor should be removed en bloque, without violating its pseudocapsule Adenoid cystic carcinoma Benign lymphoproliferative Why must a pleomorphic diseaseadenoma be managed in this way? Incomplete excision increases the risk of recurrence, which can be malignant (a process known as malignant transformation)

18 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma If pleomorphic adenoma is suspected, should incisional biopsy be performed? Malignant No! The tumor should be removed en bloque, without violating its pseudocapsule Adenoid cystic carcinoma Benign lymphoproliferative Why must a pleomorphic diseaseadenoma be managed in this way? Incomplete excision increases the risk of recurrence, which can be malignant (a process known as malignant transformation)

19 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma If pleomorphic adenoma is suspected, should incisional biopsy be performed? Malignant No! The tumor should be removed en bloque, without violating its pseudocapsule Adenoid cystic carcinoma Benign lymphoproliferative Why must a pleomorphic diseaseadenoma be managed in this way? Incomplete excision increases the risk of recurrence, which can be malignant (a process known as malignanttwo transformation) words

20 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma If pleomorphic adenoma is suspected, should incisional biopsy be performed? Malignant No! The tumor should be removed en bloque, without violating its pseudocapsule Adenoid cystic carcinoma Benign lymphoproliferative Why must a pleomorphic diseaseadenoma be managed in this way? Incomplete excision increases the risk of recurrence, which can be malignant (a process known as malignant transformation)

21 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Lymphoid Epithelial Two broad categories in the differential Benign Malignant Pleomorphic adenoma NEVER biopsy to make dx Adenoid cystic carcinoma Biopsy to make dx Hold on! How can you know beforehand which you’re dealing with, and therefore whether or not to biopsy? Fungal Thyroid eye dz These entities are usually distinguishable on the basis of Radiographic Features and Clinical Presentation:

22 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Lymphoid Epithelial Two broad categories in the differential Benign Malignant Pleomorphic adenoma NEVER biopsy to make dx Adenoid cystic carcinoma Biopsy to make dx Hold on! How can you know beforehand which you’re dealing with, and therefore whether or not to biopsy? These entities are usually distinguishable on the basis of Radiographic Features and Clinical Presentation:

23 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Lymphoid Epithelial Two broad categories in the differential Benign Malignant Pleomorphic adenoma NEVER biopsy to make dx Hold on! How can you know beforehand which you’re dealing with, and therefore whether or not to biopsy? These entities are usually distinguishable on the basis of Radiographic Features and Clinical Presentation: Adenoid cystic carcinoma Biopsy to make dx Pleomorphic adenoma: ‘Well circumscribed’ Adenoid cystic carcinoma: ‘Bony involvement’

24 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Lymphoid Epithelial Two broad categories in the differential Benign Malignant Pleomorphic adenoma NEVER biopsy to make dx Hold on! How can you know beforehand which you’re dealing with, and therefore whether or not to biopsy? These entities are usually distinguishable on the basis of Radiographic Features and Clinical Presentation: Adenoid cystic carcinoma Biopsy to make dx Pleomorphic adenoma: ‘Well circumscribed’ Adenoid cystic carcinoma: ‘Bony involvement’

25 Lacrimal Gland Enlargement Adenoid cystic: Bony involvement (invading the orbital wall, arrow) Pleomorphic adenoma: Well-circumscribed Adenoid cystic carcinoma vs pleomorphic adenoma: Imaging

26 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Lymphoid Epithelial Two broad categories in the differential Benign Malignant Pleomorphic adenoma NEVER biopsy to make dx Hold on! How can you know beforehand which you’re dealing with, and therefore whether or not to biopsy? These entities are usually distinguishable on the basis of Radiographic Features and Clinical Presentation: Adenoid cystic carcinoma Biopsy to make dx Pleomorphic adenoma: ‘Well circumscribed’ Adenoid cystic carcinoma: ‘Bony involvement’ Pleomorphic adenoma: (rate of growth) ‘Slow growing’ and ‘Painless’ (pain? ) Adenoid cystic carcinoma: (rate of growth’ growth) ‘Rapid and (pain? ) ‘Painful’

27 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Lymphoid Epithelial Two broad categories in the differential Benign Malignant Pleomorphic adenoma NEVER biopsy to make dx Hold on! How can you know beforehand which you’re dealing with, and therefore whether or not to biopsy? These entities are usually distinguishable on the basis of Radiographic Features and Clinical Presentation: Adenoid cystic carcinoma Biopsy to make dx Pleomorphic adenoma: ‘Well circumscribed’ Adenoid cystic carcinoma: ‘Bony involvement’ Pleomorphic adenoma: ‘Slow growing’ and ‘Painless’ Adenoid cystic carcinoma: ‘Rapid growth’ and ‘Painful’

28 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Next…

29 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Malignant

30 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Malignant ? Most common within each category? ?

31 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Malignant Benign lymphoproliferative disease Most common within each category? Lymphoma

32 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Malignant Benign lymphoproliferative disease Lymphoma Next…

33 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious

34 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious ? Malignant Benign lymphoproliferative disease Noninfectious Lymphoma ? ? Types of infections, not specific bugs

35 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Viral Malignant Benign lymphoproliferative disease Lymphoma Bacterial Fungal Noninfectious

36 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Noninfectious Viral Malignant Which virus is Bacterial the most common cause of acute dacryoadenitis? Lymphoma Benign Fungal lymphoproliferative EBV disease

37 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Noninfectious Viral Malignant Which virus is Bacterial the most common cause of acute dacryoadenitis? Lymphoma Benign Fungal lymphoproliferative Epstein-Barr virus disease

38 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Viral Malignant Benign lymphoproliferative disease Noninfectious Lymphoma Which Bacterial bug is the most common cause Fungal of bacterial dacryoadenitis? Bartonella

39 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Viral Malignant Benign lymphoproliferative disease Noninfectious Lymphoma Which Bacterial bug is the most common cause Fungal of bacterial dacryoadenitis? Bartonella

40 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral ? Bacterial ? Fungal ? Three common etiologies

41 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz

42 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz Regarding lacrimal gland enlargement, the very general rule of thumb is this: words --If the enlargement occurs in an inflammatory context, think orbitaltwo pseudotumor first. --If the enlargement occurs in a noninflammatory context, think lymphoproliferative disease first.

43 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz Regarding lacrimal gland enlargement, the very general rule of thumb is this: --If the enlargement occurs in an inflammatory context, think orbital pseudotumor first. --If the enlargement occurs in a noninflammatory context, think lymphoproliferative disease first.

44 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz Regarding lacrimal gland enlargement, the very general rule of thumb is this: --If the enlargement occurs in an inflammatory context, think orbital pseudotumor first. two other words disease first. --If the enlargement occurs in a noninflammatory context, think lymphoproliferative

45 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Two broad categories in the differential Benign Pleomorphic adenoma Malignant Lymphoid Two broad categories in the differential Benign Malignant Adenoid cystic Benign carcinoma lymphoproliferative disease Lymphoma Infectious Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz Regarding lacrimal gland enlargement, the very general rule of thumb is this: --If the enlargement occurs in an inflammatory context, think orbital pseudotumor first. --If the enlargement occurs in a noninflammatory context, think lymphoproliferative disease first.

46 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz If a pt presents with bilateral and symmetrical lacrimal gland enlargement, which condition is the most likely cause?

47 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Pleomorphic adenoma Malignant Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz If a pt presents with bilateral and symmetrical lacrimal gland enlargement, which condition is the most likely cause? Sarcoid

48 Lacrimal Gland Enlargement Bilateral lacrimal gland enlargement in sarcoid

49 Lacrimal Gland Enlargement Enlarged lacrimal gland Two broad categories in the differential Neoplastic Inflammatory Two broad categories in the differential Epithelial Lymphoid Two broad categories in the differential Benign Malignant Pleomorphic adenoma Adenoid cystic carcinoma Benign Infectious Malignant Benign lymphoproliferative disease Lymphoma Noninfectious Viral Pseudotumor Bacterial Sarcoid Fungal Thyroid eye dz If a pt presents with bilateral and symmetrical lacrimal gland enlargement, which condition is the most likely cause? Sarcoid (Sarcoid-induced lac gland enlargement gets its own set of slides…)

50 Q Sarcoid patients % Half have ophthalmic involvement % Half have no ophthalmic involvement

51 A Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement

52 Q Sarcoid patients Half have ophthalmic involvement % Half have lacrimal gland involvement % Half have no lacrimal gland involvement Half have no ophthalmic involvement

53 A Sarcoid patients Half have ophthalmic involvement Half have lacrimal gland involvement Half have no ophthalmic involvement

54 Q Sarcoid patients Half have ophthalmic involvement Half have lacrimal gland involvement Half have no ophthalmic involvement Half have no lacrimal gland involvement eponym Enlargement of the lacrimal and salivary glands + keratoconjunctivitis sicca = Mikulicz syndrome

55 A Sarcoid patients Half have ophthalmic involvement Half have lacrimal gland involvement Half have no ophthalmic involvement Half have no lacrimal gland involvement Enlargement of the lacrimal and salivary glands + keratoconjunctivitis sicca = Mikulicz syndrome

56 Q Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement For what population is the incidence of ophthalmic involvement far higher--around 90%? Very young (<4 years old) children, in whom it is known as early-onset sarcoid. Half have lacrimal Half have no lacrimal Early-onset sarcoid presents classically with a triad of signs/symptoms—what are they? gland involvement --Uveitis --Arthritis --Rash. Early-onset sarcoid is difficult to distinguish from what other immunologic dz of childhood? JRA--juvenile rheumatoid arthritis (aka juvenile idiopathic arthritis, or JIA)

57 A Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement For what population is the incidence of ophthalmic involvement far higher--around 90%? Very young (<4 years old) children, in whom it is known as early-onset sarcoid. Half have lacrimal Half have no lacrimal Early-onset sarcoid presents classically with a triad of signs/symptoms—what are they? gland involvement --Uveitis --Arthritis --Rash. Early-onset sarcoid is difficult to distinguish from what other immunologic dz of childhood? JRA--juvenile rheumatoid arthritis (aka juvenile idiopathic arthritis, or JIA)

58 Q Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement For what population is the incidence of ophthalmic involvement far higher--around 90%? Very young (<4 years old) children, in whom it is known as early-onset sarcoid. Half have lacrimal Half have no lacrimal Early-onset sarcoid presents classically with a triad of signs/symptoms—what are they? gland involvement --Uveitis --Arthritis --Rash. Early-onset sarcoid is difficult to distinguish from what other immunologic dz of childhood? JRA--juvenile rheumatoid arthritis (aka juvenile idiopathic arthritis, or JIA)

59 A Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement For what population is the incidence of ophthalmic involvement far higher--around 90%? Very young (<4 years old) children, in whom it is known as early-onset sarcoid. Half have lacrimal Half have no lacrimal Early-onset sarcoid presents classically with a triad of signs/symptoms—what are they? gland involvement --Uveitis --Arthritis --Rash. Early-onset sarcoid is difficult to distinguish from what other immunologic dz of childhood? JRA--juvenile rheumatoid arthritis (aka juvenile idiopathic arthritis, or JIA)

60 Q Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement For what population is the incidence of ophthalmic involvement far higher--around 90%? Very young (<4 years old) children, in whom it is known as early-onset sarcoid. Half have lacrimal Half have no lacrimal Early-onset sarcoid presents classically with a triad of signs/symptoms—what are they? gland involvement --Uveitis --Arthritis --Rash. Early-onset sarcoid is difficult to distinguish from what other immunologic dz of childhood? JRA--juvenile rheumatoid arthritis (aka juvenile idiopathic arthritis, or JIA)

61 A Sarcoid patients Half have ophthalmic involvement Half have no ophthalmic involvement For what population is the incidence of ophthalmic involvement far higher--around 90%? Very young (<4 years old) children, in whom it is known as early-onset sarcoid. Half have lacrimal Half have no lacrimal Early-onset sarcoid presents classically with a triad of signs/symptoms—what are they? gland involvement --Uveitis --Arthritis --Rash. Early-onset sarcoid is difficult to distinguish from what other immunologic dz of childhood? JRA--juvenile rheumatoid arthritis (aka juvenile idiopathic arthritis, JIA)