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Inflammatory Connective Tissue Disease > 29 Sjögren Syndrome > Flashcards

29 Sjögren Syndrome Flashcards

1
Q

Principal glands affected in Sjögren syndrome

A

Salivary and lacrimal

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2
Q

Gender predominance of pSS

A

Female

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3
Q

MC autoimmune disease present with SS

A

SLE or RA

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4
Q

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

A

F, more frequent in children

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5
Q

MC presenting feature of SS among children

A

Parotitis

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6
Q

Characteristics of Sjogren parotitis

A

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

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7
Q

Juvenile recurrent parotitis compared to pSS

A

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

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8
Q

Stains used to demonstrate damaged ocular epithelium

A

Rose Bengal or lissamine green

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9
Q

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

A

Schirmer test

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10
Q

(+) Schirmer test

A

Wetting of ≤5mm in 5 minutes

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11
Q

(+) Rose Bengal score

A

≥4

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12
Q

Cells that infiltrate salivary glands in SS

A

Lymphocytes

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13
Q

Criteria for primary SS using the AECG classification criteria

A

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

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14
Q

Criteria for secondary SS using the AECG classification criteria

A

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

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15
Q

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

A

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

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16
Q

ACR-EULAR classification criteria score for primary sjögren

A

≥4

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17
Q

ACR-EULAR criteria

A

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

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18
Q

Criteria needed for classification of jpSS according to proposed criteria

A

≥4

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19
Q

Extraglandular manifestations of Sjogren

A

fatigue
arthritis
arthralgia/myalgia
RP

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20
Q

T/F Renal involvement is common in Sjogren

A

F

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21
Q

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

A

Purely sensory neuropathy

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22
Q

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

A

F, as common

23
Q

Primary pathological finding in SS

A

Lymphocytic infiltration of affected tissues

24
Q

Focus scores are determined by

A

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

25
Q

Focus score indicative of SS

A

GRade 3 or 4

26
Q

Hallmark autoAbs of SS

A

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

27
Q

Viruses associated with pSS

A

HTLV-1
Human retrovirus 5
HCV
Coxsackie

28
Q

Majority of lymphocytes affecting granular tissue in pSS are

A

Activated CD4+ T cells and B cells

29
Q

Innate vs adaptive immunity: SS

A

Both

30
Q

Genes increasingly expressed in the salivary glands of patients with SS

A

IFN-regulated genes; pDCs are also present in glands

31
Q

Serologic finding in SS suggestive of poor prog

A

Cryoglobulin

32
Q

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

A

Hypergammaglobulinemia

33
Q

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

A

50-70% only

34
Q

T/F Sialography is included in classification criteria for SS

A

F

35
Q

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

A

T

36
Q

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

A

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

37
Q

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

A

T

38
Q

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

A

T

39
Q

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

A

T

40
Q

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

A

T

41
Q

May be helpful in treating dry mouth

A

Pilocarpine HCl

42
Q

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

A

HCQ

43
Q

Patients with SS that may require aggressive treatment

A

Extraglandular manif
Severe ocular or mucosal symp
Disabling constitutional symp

44
Q

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

A

F

45
Q

Role of CS in SS

A

Decrease parotid swelling
Improve systemic symptoms

46
Q

Biologic agent shown to be effective for SS

A

Infliximab and abatacept
NOT etanercept

47
Q

Patients with SS have significantly increased risk for this malignancy

A

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

48
Q

T/F SS prognosis is very good

A

T

49
Q

Strong predictor of mortality in SS

A

High ESDAI at time of dx

50
Q

CLINICAL features of SS predictive of lymphoma development

A

Persistently enlarged parotid
LAD
Splenomeg
Palpable purpura

51
Q

Lab features of SS predictive of lymphoma development

A

Mixed monoclonal cryoglobulinemia
Low C4
Monoclonal bands in serum or urine

52
Q

MC manifestation of IgG4-related disease

A

Orbital 44%

53
Q

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

A

T

54
Q

First-line biologic therapy for IgG4-related disease

A

Rituximab

Inflammatory Connective Tissue Disease (8 decks)

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