29 Sjögren Syndrome

Question 1

Principal glands affected in Sjögren syndrome

Answer 1

Salivary and lacrimal

Question 2

Gender predominance of pSS

Answer 2

Female

Question 3

MC autoimmune disease present with SS

Answer 3

SLE or RA

Question 4

T/F Parotid enlargement in SS is more frequent in adults

Answer 4

F, more frequent in children

Question 5

MC presenting feature of SS among children

Answer 5

Parotitis

Question 6

Characteristics of Sjogren parotitis

Answer 6

Uni- or bilateral, painful or painless, episodic or chronic

Question 7

Juvenile recurrent parotitis compared to pSS

Answer 7

JRP usually presents at 3-6 years, younger than pSS

Question 8

Stains used to demonstrate damaged ocular epithelium

Answer 8

Rose Bengal or lissamine green

Question 9

Test that measures reflex tear production by using a folded strip of sterile filter paper at the margin of the eye, without anesthesia, and with the eye gently closed

Answer 9

Schirmer test

Question 10

(+) Schirmer test

Answer 10

Wetting of ≤5mm in 5 minutes

Question 11

(+) Rose Bengal score

Answer 11

≥4

Question 12

Cells that infiltrate salivary glands in SS

Answer 12

Lymphocytes

Question 13

Criteria for primary SS using the AECG classification criteria

Answer 13

1) 4/6 items + either histopath or serology (+) 2) 3/4 objective items (ocular, histopath, salivary gland involvement, autoAb)

Question 14

Criteria for secondary SS using the AECG classification criteria

Answer 14

Ocular OR oral symptoms + any 2 ocular signs, histopath, or salivary gland involvement

Question 15

3 objective features included in the ACR-SICCA classification criteria (must satisfy 2/3)

Answer 15

1) Anti-Ro and/or La or RF and ANA titer ≥1:320 2) Salivary gland biopsy exhibiting focal lymphocytic sialadenitis with a focus score of ≥1 focus/4mm2 3) Keratoconjunctivitis sicca with ocular staining ≥3; exclude other causes

Question 16

ACR-EULAR classification criteria score for primary sjögren

Answer 16

≥4

Question 17

ACR-EULAR criteria

Answer 17

1) Labial salivary gland with focal lymphocytic sialadenitis and focus score >1 2) Anti-Ro 3) Ocular staining 5 on at least 1 eye 4) Schirmmer ≤5mm/hr on at least 1 eye 5) Unstimulated whole saliva flow rate ≤0.1mL/min

Question 18

Criteria needed for classification of jpSS according to proposed criteria

Answer 18

≥4

Question 19

Extraglandular manifestations of Sjogren

Answer 19

fatigue
arthritis
arthralgia/myalgia
RP

Question 20

T/F Renal involvement is common in Sjogren

Answer 20

F

Question 21

A syndrome of purely sensory neuropathy said to be relatively unique to LS

Answer 21

Purely sensory neuropathy

Question 22

T/F Among mothers of infants with NLE, SS is more common than SLE

Answer 22

F, as common

Question 23

Primary pathological finding in SS

Answer 23

Lymphocytic infiltration of affected tissues

Question 24

Focus scores are determined by

Answer 24

Counting # of lymphocytes ina. 4-mm2 area and classified on a scale from grade 0 (no abn) to 4 (>1 lymphoid nodule present)

Question 25

Focus score indicative of SS

Answer 25

GRade 3 or 4

Question 26

Hallmark autoAbs of SS

Answer 26

Anti Ro and La High titer RF AutoAb directed against organ-specific antigens such as thyroid cells or gastric mucosa

Question 27

Viruses associated with pSS

Answer 27

HTLV-1 Human retrovirus 5 HCV Coxsackie

Question 28

Majority of lymphocytes affecting granular tissue in pSS are

Answer 28

Activated CD4+ T cells and B cells

Question 29

Innate vs adaptive immunity: SS

Answer 29

Both

Question 30

Genes increasingly expressed in the salivary glands of patients with SS

Answer 30

IFN-regulated genes; pDCs are also present in glands

Question 31

Serologic finding in SS suggestive of poor prog

Answer 31

Cryoglobulin

Question 32

Very elevated ESR in SS is likely related to this concomitant finding which is seen in nearly 100% of patients with SS

Answer 32

Hypergammaglobulinemia

Question 33

Anti-ro and la are found in ___% of patients with ss

Answer 33

50-70% only

Question 34

T/F Sialography is included in classification criteria for SS

Answer 34

F

Question 35

In salviary gland scintigraphy for SS, uptake and secretion of isotope is delayed or absent

Answer 35

T

Question 36

Findings from minor salivary gland biopsy that indicate a diagnosis of SS

Answer 36

Focal lymphocytic sialadenitis, with a focus score of 1 or greater per 4mm2

Question 37

T/F a minor salivary gland biopsy can be a very useful and important procedure to determine a definite diagnosis in a child suspected with SS

Answer 37

T

Question 38

T/F SS may not require therapy if without extraglandular disease

Answer 38

T

Question 39

T/F For patients with SS who do not complain of dry eyes, annual ocular screening should still be done

Answer 39

T

Question 40

T/F dry mouth as a manifestation of ss is rare in children

Answer 40

T

Question 41

May be helpful in treating dry mouth

Answer 41

Pilocarpine HCl

Question 42

May be helpful in treating constitutional symptoms, arthralgia, or fatigue associated with SS

Answer 42

HCQ

Question 43

Patients with SS that may require aggressive treatment

Answer 43

Extraglandular manif Severe ocular or mucosal symp Disabling constitutional symp

Question 44

T/F CS stop the progression of SS and improve salivary flow

Answer 44

F

Question 45

Role of CS in SS

Answer 45

Decrease parotid swelling Improve systemic symptoms

Question 46

Biologic agent shown to be effective for SS

Answer 46

Infliximab and abatacept NOT etanercept

Question 47

Patients with SS have significantly increased risk for this malignancy

Answer 47

B cell lymphoma of the MALT cell type, MC affecting the salivary glands

Question 48

T/F SS prognosis is very good

Answer 48

T

Question 49

Strong predictor of mortality in SS

Answer 49

High ESDAI at time of dx

Question 50

CLINICAL features of SS predictive of lymphoma development

Answer 50

Persistently enlarged parotid LAD Splenomeg Palpable purpura

Question 51

Lab features of SS predictive of lymphoma development

Answer 51

Mixed monoclonal cryoglobulinemia Low C4 Monoclonal bands in serum or urine

Question 52

MC manifestation of IgG4-related disease

Answer 52

Orbital 44%

Question 53

T/F IgG4-related disease is usually responsive to treatment

Answer 53

T

Question 54

First-line biologic therapy for IgG4-related disease

Answer 54

Rituximab