Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Fitz 9th > 68 - Sjogren Syndrome > Flashcards

68 - Sjogren Syndrome Flashcards

1
Q

Chronic systemic autoimmune disorder characterized by lymphocytic infiltration and destruction of exocrine glands and epithelia leading to dry mouth, dry eyes, and B lymphocyte hyperreactivity

A

Sjogren syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Isolated SS disorder

A

Primary Sjogren syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

SS associated with other autoimmune condition

A

Secondary Sjogren syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

SS predominantly affects

A

Women

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

SS patients are most commonly diagnosed in their _____ decades of life

A

Fourth and fifth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Genetic loci significantly associated with pSS

A

HLA DQA1*0501

HLA DQB1*0201

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Important transcription factor involved in upregulating the Type I IFN pathway, which is activated by toll-like receptor signals or viral infection and the production of inflammatory cytokines

A

IRF5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Protein involved in the Type II IFN pathway and activating the adaptive immune system

A

STAT4

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

_____ mutation has been correlated with an increased risk of lymphoma

A

TNFAIP3

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Thought to be the first and main target in pSS

A

Salivary glands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

The role of the salivary gland epithelial cells in pSS has been established. Studies have indicated that epithelial cells play an active role in disease pathogenesis and this is referred to as

A

“Autoimmune epithelitis”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Best-defined autoantibodies in SS

A

Anti-Ro/SSA

Anti-La/SSB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Recognizes 2 RNA-binding proteins (the 52-kDa or the 60-kDa protein)

A

Anti-Ro/SSA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Recognize RNA polymerase III

A

Anti-La/SSB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Found in more than 70% of patients with SS but are not specific for SS and are frequently found in SLE and other autoimmune diseases

A

Anti-Ro/SSA

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

More specific for SS

Present in 50% of patients with pSS or SS/SLE but is rarely seen in other diseases

A

Anti-La/SSB

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Frequently present in patients with both and primary SS

Although they lack specificity, they are markers of a systemic autoimmune response

A

Antinuclear antibodies

Rheumatoid factor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Major stimulus for saliva production

A

Binding of acetylcholine to muscarinic acetylcholine receptors

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

A small group of patients positive for anticentromere autoantibody (ACA) present a clinical picture similar to that of

A

Limited scleroderma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

The ACA-positive group usually has a higher prevalence of

A

Raynaud phenomenon

Thyroid dysfunction

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

The ACA-positive group also shows a higher frequency of

A

Vasculitis
Peripheral neuropathy
Primary biliary cirrhosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Positivity for antimitochondrial antibody (AMA) also has been demonstrated to be related to

A

Primary biliary cirrhosis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Sjogren syndrome is a multifocal autoimmune disease, with systemic involvement in _____ of patients

A

One-third

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

The characteristic feature of SS is exocrine gland dysfunction, leading to classic sicca symptoms of

A

Xerostomia (dry mouth)

Xerophthalmia or keratoconjunctivitis sicca (dry eyes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Medical causes of oral dryness, such as _____, should be considered when evaluating a patient for Sjogren syndrome

A

Dehydration
Diabetes
Viral infections
Drug treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

Flat, nonpalpable, blanching purpura
Characterized by polyclonal hypergammaglobulinemia and rheumatoid factor positivity
Skin biopsies reveal ruptured blood vessels with complement deposition

A

Hypergammaglobulinemic purpura

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

Y/N: Palpable purpura represents an important marker of more severe disease and is associated with an increased risk of lymphoma development and mortality

A

Yes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

Histopathologically, palpable purpura can be divided into 2 groups

A

Neutrophilic inflammatory vascular disease

Mononuclear inflammatory vascular disease

29
Q

Predominantly neutrophilic infiltrate, fibrinoid necrosis, occlusion of the lumen, and extravasation of red blood cells, and is indistinguishable form classical leukocytoclastic vasculitis

A

Neutrophilic inflammatory vascular disease

30
Q

Mononuclear inflammatory infiltrate, with invasion of the blood vessel walls

A

Mononuclear inflammatory vascular disease

31
Q

The clinical presentation of the 2 forms of palpable purpura are indistinguishable, but _____ is associated more strongly with markers of systemic autoimmunity

A

Neutrophilic inflammatory vascular disease

32
Q

Second most frequent form of cutaneous vasculitis in SS

A

Urticarial vasculitis

33
Q

Present as palpable purpuric lesions of the lower extremities, which may ulcerate, finally resolving within 1 to 4 weeks

A

Necrotizing vasculitis

34
Q

Annular erythema associated with pSS is found primarily among

A

Asian pSS patients who have anti-Ro/SSA and anti-La/SSB antibodies

35
Q

Erythematous lesion with a wide elevated border and central clearing
Localized mainly on the faces of Asian patients

A

Annular erythema associated with pSS

36
Q

May be triggers of the lesions of annular erythema associated with pSS

A

Sunlight
Cold exposure
Mental stress
Pregnancy

37
Q

Annular erythema associated with pSS shares a clinical presentation with

A

Subacute cutaneous lupus

38
Q

On histopathology of (annular erythema associated with pSS/SCLE), the dense cell infiltration is localized mainly around sweat glands, and nuclear dust can also be seen when they have vasculitis

A

Annular erythema associated with pSS

39
Q

(Annular erythema associated with pSS/SCLE) are common in Asians, but rare in whites, whereas (annular erythema associated with pSS/SCLE) are more common in whites but rare in Asians

A

Annular erythema associated with pSS

SCLE

40
Q

Presence of erythema nodosum should raise the suspicion for

A

Sarcoidosis

41
Q

Probably the most common abnormality in SS

Can precede sicca symptoms by many years

A

Raynaud phenomenon

42
Q

Raynaud phenomenon in SS is not accompanied by _____, as seen in systemic sclerosis

A

Telangiectasias

43
Q

Although Raynaud phenomenon is usually mild in pSS, it is a marker of a subgroup with increased

A

Extraglandular manifestations

44
Q

The absence of rheumatoid factor and anti-CCP antibodies, and the absence of erosions on radiographs would favor SS over

A

Rheumatoid arthritis

45
Q

Thyroid dysfunction is a very common symptom, occurring in 10% to 70% of SS patients. It often presents as

A

Fatigue

46
Q

Most common manifestation of peripheral nervous system involvement in SS

A

Peripheral axonal polyneuropathies, which are typically sensory

47
Q

Most common form of cranial neuropathy in SS

A

Unilateral trigeminal neuropathy

48
Q

The incidence of lymphoma in SS patients is increased _____-fold

A

15 to 44

49
Q

Y/N: Recent studies have suggested that women with pSS may experience more complications in pregnancy than healthy controls

A

Yes - significant increases in the rates of spontaneous abortions, preterm deliveries, and low-body-weight infants

50
Q

Anti-Ro/SSA and anti-La/SSA can cause _____ in the fetus

A

Congenital heart block

Neonatal lupus

51
Q

Characterized by an annular rash with central regression or mild atrophy in the scalp and around the eyes, as well as hepatic and hematologic abnormalities

A

Neonatal lupus

52
Q

Involves measurement of the total saliva produced from all salivary glands in a time period of 15 min

A

Sialometry

53
Q

The whole unstimulated salivary flow is considered suggestive of SS if it is

A

<1.5 mL in 15 minutes

54
Q

A “stimulated” salivary flow can be measured after administration of

A

Lemon juice or citric acid

55
Q

Functional study to assess saliva production by measuring the secretion of a radioisotope into the oral cavity

A

Salivary gland scintigraphy

56
Q

Can potentially distinguish between decreased production and/or decreased excretion of saliva

A

Salivary gland scintigraphy

57
Q

Imaging method based on retrograde injection of contrast media into the parotid duct

A

Sialography

58
Q

Performed by placing a standardized paper strip in the inferior fornix of each eye and measuring the length of filter paper that becomes wet after 5 minutes

A

Schirmer test

59
Q

The American-European classification system uses a cut-off value for the Schirmer test of

A

5 mm in 5 minutes

60
Q

Dye that preferentially stains the devitalized cornea and conjunctiva

A

Rose Bengal or lissamine green

61
Q

The most reliable objective diagnostic feature of SS is seen on

A

Biopsy of the minor salivary gland

62
Q

There are 2 patterns of pSS that define disease categories with very different clinical risks

A 
Type I (High-risk)
Type II (Low-risk)
63
Q

Patients with _____ may be classified as high-risk disease syndrome (Type I)

A

Low complement C4 levels and/or

Palpable purpura early in their disease course

64
Q

The high-risk disease syndrome group carries a significantly increased risk of _____; it also has an increased _____

A

Lymphoproliferative disease

Mortality rate

65
Q

Two drugs approved for the treatment of dry mouth

A

Pilocarpine 5 mg 4 times/d

Cevimeline 30 mg 3 times/d

66
Q

Pilocarpine and cevimeline are contraindicated in

A

Narrow-angle glaucoma

Uncontrolled asthma

67
Q

Cholinergic side effects

A

Excessive sweating
Urinary frequency
Flushing
Headaches

68
Q

Has been approved by the FDA for the treatment of keratoconjunctivitis sicca

A

Cyclosporine 0.05% ophthalmic solution

69
Q

Most SS-associated lymphomas are

A

Low-grade B cell-lymphomas, localized to the exocrine glands

Fitz 9th (108 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions