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Integrated systems > Autoimmunity, Myopathies, SLE > Flashcards

Autoimmunity, Myopathies, SLE Flashcards

1
Q

Name some organ specific types of autoimmune disease

A
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2
Q

The most at risk population for SLE is _

A

The most at risk population for SLE is women in reproductive years

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3
Q

Fill out this autoimmune ROS:
* Skin:
* Joints:
* Eyes:
* ENT:
* Serositis:
* Urinary:
* Neuro:
* Heme:

A

Fill out this autoimmune ROS:
* Skin: rashes
* Joints: arthralgia, joint swelling
* Eyes: dry eye, irritation
* ENT: dry mouth, oral ulcers
* Serositis: pleuritic chest pain
* Urinary: hematuria, frothy urine
* Neuro: headache, seizures, neuropathic sx
* Heme: clots, anemia

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4
Q

Signs of hemolytic anemia include:
1. _ Haptoglobin
2. _ Lactate dehydrogenase
3. _ RBCs

A

Signs of hemolytic anemia include:
1. Low Haptoglobin
2. Elevated Lactate dehydrogenase
3. Spherocytes RBCs

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5
Q

Autoantibodies _ and _ are most specific for SLE

A

Autoantibodies anti-dsDNA and anti-Smith are most specific for SLE

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6
Q

What are three major reasons of low complement?

A
  1. Chronic liver disease
  2. Use of complement (autoimmune)
  3. Deficiency
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7
Q

Autoimmune diseases/ Type III hypersensitivity can activate the _ complement pathway

A

Autoimmune diseases/ Type III hypersensitivity can activate the classical complement pathway
* Activated by antigen-antibody complex

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8
Q

Recall that the lectin pathway is activated by _

A

Recall that the lectin pathway is activated by microbial carbohydrates (ex: MBL)

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9
Q

Recall that the alternative pathway is activated by _

A

Recall that the alternative pathway is activated by activating surfaces

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10
Q

Therapy for acute flare of lupus is _ + _

A

Therapy for acute flare of lupus is corticosteroids + hydroxychloroquine
* We avoid long term use of corticosteroids

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11
Q

Name the various inflammatory myopathies

A
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12
Q

Dermatomyositis and polymyositis both occur in [populations] and have an acute to subacute presentation of weeks to months

A

Dermatomyositis and polymyositis both occur in children and adults and have an acute to subacute presentation of weeks to months

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13
Q

The muscle distribution affected in dermatomyositis and polymyositis is _

A

The muscle distribution affected in dermatomyositis and polymyositis is symmetrical, proximal involvement
* Classically affects shoulder and hip girdle and preserves distal muscles
* May cause dysphagia, weakness of respiratory muscles, heart involvement

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14
Q

Inclusion body myositis classically affects [population] and has _ onset

A

Inclusion body myositis classically affects older adults and has chronic, insidious onset (months to years)

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15
Q

The muscle distribution affected in inclusion body myositis is _

A

The muscle distribution affected in inclusion body myositis is asymmetric, proximal & distal muscle groups
* Does NOT spare distal muscle groups!

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16
Q

The confirmatory diagnosis for inflammatory myopathies is _

A

The confirmatory diagnosis for inflammatory myopathies is muscle biopsy

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17
Q

Inflammatory myopathies may present with elevated muscle enzymes, _ and _

A

Inflammatory myopathies may present with elevated muscle enzymes, CK and Aldolase
* Sometimes LFTs are elevated, esp AST

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18
Q

Common autoantibodies assocaited with dermatomyositis include:

A

Common autoantibodies assocaited with dermatomyositis include: anti-Jo-1, anti-Mi-2, anti-MDA5

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19
Q

Polymyositis involves infiltrative _ cells in the [muscle layer]

A

Polymyositis involves infiltrative CD8+ cells in the endomysium
* May see diffuse regions of necrotic and regenerating myofibers

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20
Q

Dermatomyositis involves infiltrative _ cells in the [muscle layer]

A

Dermatomyositis involves infiltrative CD4+ cells in the perimysium
* Inflammation and vasculitis leads to diminished blood flow to the peripheral muscle bundles –> perifascicular atrophy

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21
Q
A

Polymyositis

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22
Q
A

Dermatomyositis

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23
Q
A

Inclusion body myositis- CD8+ cells invading myofiber; presence of sarcoplasmic rimmed vacuoles; may see amyloid deposits

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24
Q

Patients with polymositis and dermatomyositis are at increased risk of [lung manifestation]

A

Patients with polymositis and dermatomyositis are at increased risk of interstitial lung disease

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25
Q

Name some extra-muscular manifestations in inflammatory myopathies

A
  • Constitutional symptoms (fatigue, fevers, weight loss)
  • Arthritis
  • Gastroesophageal dysmotility
  • Pulmonary hypertension
  • Myocarditis
  • Vasculitis (more prominent in pediatric population)
  • Raynaud’s Phenomenon
  • Increased risk for occult malignancy
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26
Q

First line pharmocologic treatment for inflammatory myositis is _ ; steroid sparing agents like _ can also be used

A

First line pharmocologic treatment for inflammatory myositis is corticosteroids ; steroid sparing agents like methotrexate can also be used

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27
Q

Name the cutaneous manifestations of dermatomyositis

A
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28
Q

Joint stiffness that is worse in the morning is more suggestive of _

A

Joint stiffness that is worse in the morning is more suggestive of inflammatory arthritis
* Inflammatory symptoms will get better with movement but mechanical ones get worse

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29
Q

Rheumatoid arthritis is a multi-system, [symmetry] inflammatory polyarthritis that predominantly affects the hands and feet early

A

Rheumatoid arthritis is a multi-system, symmetric inflammatory polyarthritis that predominantly affects the hands and feet early

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30
Q

The peak onset of RA is [age]

A

The peak onset of RA is 45-65

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31
Q

(RA/ osteoarthritis) is more likely to involve the DIP

A

Osteoarthritis is more likely to involve the DIP
* Both involve the PIP

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32
Q

(RA/ osteoarthritis) is more likely to involve the MCP

A

RA is more likely to involve the MCP
* Both involve the PIP

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33
Q

The classic autoantibodies involved in RA are _ and _ ; _ is more specific

A

The classic autoantibodies involved in RA are rheumatoid factor and anti-CCP ; anti-CCP is more specific
* A high anti-CCP titer is associated with more damage

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34
Q

Patients with [HLA] are more susceptible to RA

A

Patients with HLA-DRB1 are more susceptible to RA

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35
Q
A

RA nodules

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36
Q

[Eye manifestations] can occur in RA

A

Keratoconjunctivitis sicca, peripheral ulcerative keratitis, scleritis can occur in RA

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37
Q

[Renal disease] is a rare manifestation in RA patients

A

AA amyloidosis is a rare manifestation in RA patients

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38
Q

First line treatment for new onset active RA is _

A

First line treatment for new onset active RA is methotrexate
* Hydroxycloroquine, TNF inhibitors, IL-6 inhibitors, anti-CD20 Abs, JAK inhibitors also exist

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39
Q

Pulmonary manifestations of RA include _

A

Pulmonary manifestations of RA include ILD, pleuritis, pulmonary nodules

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40
Q

CV manifestations of RA include:

A

CV manifestations of RA include: pericarditis, coronary artery disease

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41
Q

Hematologic manifestations of RA include _

A

Hematologic manifestations of RA include anemia, thrombocytosis, leukemia/lymphoma

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42
Q

Sjogren’s disease is an autoimmune disease characterized by lymphocytic infiltration of _

A

Sjogren’s disease is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands
* Associated with systemic disease and risk of lymphoma

43
Q

_ is a test in which calibrated filter paper strip is placed in the lower eyelid of each eye, and the patient keeps their eyes closed for five minutes. The paper’s length that becomes wet is then measured

A

Schirmer’s test is a test in which calibrated filter paper strip is placed in the lower eyelid of each eye, and the patient keeps their eyes closed for five minutes. The paper’s length that becomes wet is then measured

44
Q

The gold standard test for Sjogren’s is _

A

The gold standard test for Sjogren’s is salivary lip gland biopsy
* To identify lymphocytic sialadenitis

45
Q

Sjogren’s disease is associated with a [type] RTA

A

Sjogren’s disease is associated with a distal Type 1 RTA
* Impaired distal tubule/ collecting duct acidification
* Alpha-intercalated cells are unable to secrete H+
* No new bicarb is generated leading to metabolic acidosis
* Bicarb will be low, K will be low

46
Q

RA is caused by _ antibodies against the Fc portion of _

A

RA is caused by IgM antibodies (RF) against the Fc portion of IgG

47
Q

Risk factors for RA include smoking, HLA _ or _

A

Risk factors for RA include smoking, HLA DR1 or DR4

48
Q

Felty syndrome is a rare extra-articular manifestation of seropositive rheumatoid arthritis characterized by RA, _ and _

A

Felty syndrome is a rare extra-articular manifestation of seropositive rheumatoid arthritis characterized by RA, neutropenia and splenomegaly

49
Q

_ is an extra-articular manifestation of rheumatoid arthritis characterized by pneumoconiosis

A

Caplan syndrome is an extra-articular manifestation of rheumatoid arthritis characterized by pneumoconiosis

50
Q

_ is a joint deformity seen in RA in which there is PIP flexion and DIP hyperextension

A

Boutonniere deformity is a joint deformity seen in RA in which there is PIP flexion and DIP hyperextension

51
Q

_ is a joint deformity seen in RA in which there is DIP flexion and PIP hyperextension

A

Swan neck deformity is a joint deformity seen in RA in which there is DIP flexion and PIP hyperextension

52
Q

SLE is associated with HLA _ and _

A

SLE is associated with HLA DR2 and DR3

53
Q

Msk effects of lupus include _

A

Msk effects of lupus include symmetrical polyarthritis with inflammation of the MCP + PIP without deformity

54
Q

[Endocarditis] is a manifestation of lupus

A

Libman-Sacks endocarditis is a manifestation of lupus

55
Q

Sjogren syndrome is associated with [gallbladder pathology]

A

Sjogren syndrome is associated with primary biliary cirrhosis

56
Q

Sjogren syndrome is most commonly associated with [autoantibodies (2)]

A

Sjogren syndrome is most commonly associated with anti-Ro/SSA + anti-La/SSB
* Non-specific serology include rheumatoid factor, ANA, elevated CRP/ESR

57
Q

Sjogren syndrome patients have a risk of [heme malignancy]

A

Sjogren syndrome patients have a risk of B cell non-hodgkin lymphoma like MALT lymphoma

58
Q

[Inflammatory myopathy] involves cell-mediated cellular damage while _ involves antibody mediated cellular damage

A

Polymyositis involves cell-mediated cellular damage while dermatomyositis involves antibody mediated cellular damage

59
Q

_ is associated with heliotrope edema

A

Dermatomyositis is associated with heliotrope edema
* Additionally, gottron papules, shawl and V signs

60
Q

Patients with polymyositis or dermatomyositis should be tested for underlying _

A

Patients with polymyositis or dermatomyositis should be tested for underlying malignancy

61
Q

Scleroderma is caused by an autoimmune non-inflammatory vasculopathy that leads to _ activation and _ deposition

A

Scleroderma is caused by an autoimmune non-inflammatory vasculopathy that leads to fibroblast activation and collagen deposition

62
Q

Scleroderma is most common in [population]

A

Scleroderma is most common in women, 30-50 years old

63
Q

Thickened skin, pruritus, loss of wrinkles, and telangiectasias should raise suspiscion for _

A

Thickened skin, pruritus, loss of wrinkles, and telangiectasias should raise suspiscion for scleroderma

64
Q

Symptoms of limited scleroderma can be remembered with mneumonic _

A

Symptoms of limited scleroderma can be remembered with mneumonic CREST
* Calcinosis cutis
* Raynaud phenomenon
* Esophageal dysmotility
* Sclerodactyly
* Telangiectasia

65
Q

_ form of scleroderma is a more benign course with less skin involvement and a slower onset

A

Limited scleroderma (CREST syndrome) form of scleroderma is a more benign course with less skin involvement and a slower onset

66
Q

Diffuse scleroderma involves _

A

Diffuse scleroderma involves widespread skin thickening multiorgan involvement + life-threatening complications

67
Q

Mixed connective tissue disease has overlapping characteristics of 3 syndromes:

A

Mixed connective tissue disease has overlapping characteristics of 3 syndromes: systemic sclerosis, SLE, polymyositis

68
Q

[Autoantibody] is more specific to limited scleroderma

A

Anti-centromere is more specific to limited scleroderma
* Anti-Scl-70 occurs in both

69
Q

[Autoantibody] is more specific to diffuse scleroderma

A

Anti-RNA polymerase III is more specific to diffuse scleroderma
* Anti-Scl-70 occurs in both

70
Q

Mixed connective tissue disease is associated with [autoantibody]

A

Mixed connective tissue disease is associated with anti-U1 RNP antibody

71
Q

Sarcoidosis involves the formation of _ secondary to antigen trigger and immune response

A

Sarcoidosis involves the formation of noncaseating granuloma formation secondary to antigen trigger and immune response

72
Q

Formation of granulomas involves macrophages activating _ which then release _

A

Formation of granulomas involves macrophages activating Th1 which then release IFN-gamma
* Stimulates the formation of epitheloid cells

73
Q

Hypervitaminosis D and hypercalcemia can occur in sarcoidosis due to increased activity of _

A

Hypervitaminosis D and hypercalcemia can occur in sarcoidosis due to increased activity of 1 alpha-hydroxylase
* Macrophage activity stimulates this enzyme –> increased calcitrol

74
Q

Lupus pernio is a skin rash specific to _

A

Lupus pernio is a skin rash specific to sarcoidosis

75
Q

Lofgren syndrome (sarcoidosis manifestation) involves fever + (3 symptoms)

A

Lofgren syndrome (sarcoidosis manifestation) involves fever +
1. Bilateral hilar lymphadenopathy
2. Erythema nodosum
3. Migratory polyarthritis

76
Q

Sarcoidosis most commonly presents with _ on chest x-ray

A

Sarcoidosis most commonly presents with bilateral hilar lymphadenopathy on chest x-ray
* PFTs would show restrictive lung disease

77
Q

Increased ACE levels in sarcoidosis result from increased formation of _

A

Increased ACE levels in sarcoidosis result from increased formation of epithelioid cells

78
Q

Isolated sarcoidosis does not require treatment; however, symptoms may be treated with _

A

Isolated sarcoidosis does not require treatment; however, symptoms may be treated with glucocorticoids

79
Q

RA patients are at an increased risk of [CV manifestations]

A

RA patients are at an increased risk of pericarditis and MI

80
Q

A patient with a history of dry eyes, dry mouth, dental carries, oral candidiasis, and parotid swelling is concerning for _

A

A patient with a history of dry eyes, dry mouth, dental carries, oral candidiasis, and parotid swelling is concerning for Sjogren’s disease

81
Q

A pregnant woman with a +SSA antibody is at risk of having a baby with _

A

A pregnant woman with a +SSA antibody is at risk of having a baby with neonatal lupus
* Manifestations: congenital heart block, elevated liver enzymes, cytopenias, rash
* However, a mother with Sjogren’s who is SSA+ and SSB+ still has a very low risk of neonatal lupus

82
Q

Neonatal lupus is associated with _ rash, cytopenias, liver disease, and congenital heart block

A

Neonatal lupus is associated with “racoon eye” rash, cytopenias, liver disease, and congenital heart block

83
Q

If a patient with Sjogren’s develops B symptoms or unilateral parotid gland swelling we should be concerned for _

A

If a patient with Sjogren’s develops B symptoms or unilateral parotid gland swelling we should be concerned for MALT lymphoma
* The most common lymphomas in these patients are marginal zone lymphomas like MALT

84
Q

_ is a vasospastic reaction to cold temperatures or emotional stress that leads to sharply demarcated color changes in the skin

A

Raynaud phenomenon is a vasospastic reaction to cold temperatures or emotional stress that leads to sharply demarcated color changes in the skin
* Associations with multiple autoimmune conditions but most notably systemic slcerosis

85
Q

The (3) phases of raynaud phenomenon are:

A

The (3) phases of raynaud phenomenon are:
1. Ischemia
2. Cyanosis
3. Rubor

86
Q

Pharmacologic management of raynaud phenomenon includes:

A

Pharmacologic management of raynaud phenomenon includes:
* Calcium channel blockers
* Topical nitroglycerin (vasodilator)
* PDE inhibitors

87
Q

_ Systemic sclerosis includes early, rapid visceral involvement, involvement of the trunk, ILD, renal crisis, and GI effects

A

Diffuse systemic sclerosis includes early, rapid visceral involvement, involvement of the trunk, ILD, renal crisis, and GI effects

88
Q

Decreased renal blood flow from scleroderma results in overactivation of RAAS and a high _ state

A

Decreased renal blood flow from scleroderma results in overactivation of RAAS and a high renin state
* Scleroderma renal crisis = identical to malignant hypertension

89
Q

Treatment for scleroderma renal crisis is _

A

Treatment for scleroderma renal crisis is ACE-inhibitors
* Scleroderma patients should check blood pressure at least once a week bc of risk

90
Q

Limited cutaneous systemic sclerosis is associated with late development of [lung pathology]

A

Limited cutaneous systemic sclerosis is associated with late development of pulmonary arterial hypertension
* PAH much more common in limited

91
Q

ILD is associated with the presense of [antibody] in scleroderma patients

A

ILD is associated with the presense of Scl-70 (topoisomerase 1) in scleroderma patients
* ILD is much more common in diffuse

92
Q

_ syndrome is a pathognomonic presentation of sarcoidosis that involves anterior uveitis, parotid gland enlargement, facial palsy, and fever

A

Heerfordt’s syndrome is a pathognomonic presentation of sarcoidosis that involves anterior uveitis, parotid gland enlargement, facial palsy, and fever

93
Q

Autoantibodies specific to SLE

A

Anti-dsDNA, anti-Smith

94
Q

Autoantibody specific to drug-induced lupus

A

Anti-histone

95
Q

Autoantibody specific to mixed connective tissue disease

A

Anti-U1 RNP

96
Q

The most specific autoantibody to RA is _

A

The most specific autoantibody to RA is anti-cyclic citrullinated peptide (anti-CCP)

97
Q

Rheumatoid factor is a _ antibody

A

Rheumatoid factor is a IgM antibody against IgG Fc region

98
Q

Autoantibodies specific to Sjogrens

A

Anti-Ro (SSA) + Anti-La (SSB)

99
Q

Diffuse scleroderma is associated with [autoantibody]

A

Diffuse scleroderma is associated with anti-Scl-70 + anti-RNA pol III

100
Q

Anti-Scl-70 is a _ antibody

A

Anti-Scl-70 is an anti-DNA topoisomerase I

101
Q

Limited scleroderma, CREST syndrome is associated with [autoantibody]

A

Limited scleroderma, CREST syndrome is associated with anti-centromere

102
Q

Polymyositis and dermatomyositis are associated with [autoantibodies]

A

Polymyositis and dermatomyositis are associated with anti-synthetase (anti-Jo-1), anti-SRP, anti-helicase (anti-Mi-2)

103
Q

Anti-SSA and anti-SSB are [type] antibodies

A

Anti-SSA and anti-SSB are anti-ribonucleoprotein antibodies

104
Q
A

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