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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
Name some extra-muscular manifestations in inflammatory myopathies
* 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**
26
First line pharmocologic treatment for inflammatory myositis is _ ; steroid sparing agents like _ can also be used
First line pharmocologic treatment for inflammatory myositis is **corticosteroids** ; steroid sparing agents like **methotrexate** can also be used
27
Name the cutaneous manifestations of dermatomyositis
28
Joint stiffness that is worse in the morning is more suggestive of _
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*
29
Rheumatoid arthritis is a multi-system, [symmetry] inflammatory polyarthritis that predominantly affects the hands and feet early
Rheumatoid arthritis is a multi-system, **symmetric** inflammatory polyarthritis that predominantly affects the hands and feet early
30
The peak onset of RA is [age]
The peak onset of RA is **45-65**
31
(RA/ osteoarthritis) is more likely to involve the DIP
**Osteoarthritis** is more likely to involve the DIP * *Both involve the PIP*
32
(RA/ osteoarthritis) is more likely to involve the MCP
**RA** is more likely to involve the MCP * *Both involve the PIP*
33
The classic autoantibodies involved in RA are _ and _ ; _ is more specific
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*
34
Patients with [HLA] are more susceptible to RA
Patients with **HLA-DRB1** are more susceptible to RA
35
**RA nodules**
36
[Eye manifestations] can occur in RA
**Keratoconjunctivitis sicca, peripheral ulcerative keratitis, scleritis** can occur in RA
37
[Renal disease] is a rare manifestation in RA patients
**AA amyloidosis** is a rare manifestation in RA patients
38
First line treatment for new onset active RA is _
First line treatment for new onset active RA is **methotrexate** * *Hydroxycloroquine, TNF inhibitors, IL-6 inhibitors, anti-CD20 Abs, JAK inhibitors also exist*
39
Pulmonary manifestations of RA include _
Pulmonary manifestations of RA include **ILD, pleuritis, pulmonary nodules**
40
CV manifestations of RA include:
CV manifestations of RA include: **pericarditis, coronary artery disease**
41
Hematologic manifestations of RA include _
Hematologic manifestations of RA include **anemia, thrombocytosis, leukemia/lymphoma**
42
Sjogren's disease is an autoimmune disease characterized by lymphocytic infiltration of _
Sjogren's disease is an autoimmune disease characterized by lymphocytic infiltration of **exocrine glands** * *Associated with systemic disease and risk of lymphoma*
43
_ 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
**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
The gold standard test for Sjogren's is _
The gold standard test for Sjogren's is **salivary lip gland biopsy** * *To identify lymphocytic sialadenitis*
45
Sjogren's disease is associated with a [type] RTA
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
RA is caused by _ antibodies against the Fc portion of _
RA is caused by **IgM** antibodies (RF) against the Fc portion of **IgG**
47
Risk factors for RA include smoking, HLA _ or _
Risk factors for RA include smoking, HLA **DR1** or **DR4**
48
Felty syndrome is a rare extra-articular manifestation of seropositive rheumatoid arthritis characterized by RA, _ and _
Felty syndrome is a rare extra-articular manifestation of seropositive rheumatoid arthritis characterized by RA, **neutropenia** and **splenomegaly**
49
_ is an extra-articular manifestation of rheumatoid arthritis characterized by pneumoconiosis
**Caplan syndrome** is an extra-articular manifestation of rheumatoid arthritis characterized by pneumoconiosis
50
_ is a joint deformity seen in RA in which there is PIP flexion and DIP hyperextension
**Boutonniere deformity** is a joint deformity seen in RA in which there is PIP flexion and DIP hyperextension
51
_ is a joint deformity seen in RA in which there is DIP flexion and PIP hyperextension
**Swan neck deformity** is a joint deformity seen in RA in which there is DIP flexion and PIP hyperextension
52
SLE is associated with HLA _ and _
SLE is associated with HLA **DR2** and **DR3**
53
Msk effects of lupus include _
Msk effects of lupus include **symmetrical polyarthritis with inflammation of the MCP + PIP without deformity**
54
[Endocarditis] is a manifestation of lupus
**Libman-Sacks endocarditis** is a manifestation of lupus
55
Sjogren syndrome is associated with [gallbladder pathology]
Sjogren syndrome is associated with **primary biliary cirrhosis**
56
Sjogren syndrome is most commonly associated with [autoantibodies (2)]
Sjogren syndrome is most commonly associated with **anti-Ro/SSA + anti-La/SSB** * *Non-specific serology include rheumatoid factor, ANA, elevated CRP/ESR*
57
Sjogren syndrome patients have a risk of [heme malignancy]
Sjogren syndrome patients have a risk of **B cell non-hodgkin lymphoma like MALT lymphoma**
58
[Inflammatory myopathy] involves cell-mediated cellular damage while _ involves antibody mediated cellular damage
**Polymyositis** involves cell-mediated cellular damage while **dermatomyositis** involves antibody mediated cellular damage
59
_ is associated with heliotrope edema
**Dermatomyositis** is associated with heliotrope edema * *Additionally, gottron papules, shawl and V signs*
60
Patients with polymyositis or dermatomyositis should be tested for underlying _
Patients with polymyositis or dermatomyositis should be tested for underlying **malignancy**
61
Scleroderma is caused by an autoimmune non-inflammatory vasculopathy that leads to _ activation and _ deposition
Scleroderma is caused by an autoimmune non-inflammatory vasculopathy that leads to **fibroblast** activation and **collagen** deposition
62
Scleroderma is most common in [population]
Scleroderma is most common in **women, 30-50 years old**
63
Thickened skin, pruritus, loss of wrinkles, and telangiectasias should raise suspiscion for _
Thickened skin, pruritus, loss of wrinkles, and telangiectasias should raise suspiscion for **scleroderma**
64
Symptoms of limited scleroderma can be remembered with mneumonic _
Symptoms of limited scleroderma can be remembered with mneumonic **CREST** * Calcinosis cutis * Raynaud phenomenon * Esophageal dysmotility * Sclerodactyly * Telangiectasia
65
_ form of scleroderma is a more benign course with less skin involvement and a slower onset
**Limited scleroderma (CREST syndrome)** form of scleroderma is a more benign course with less skin involvement and a slower onset
66
Diffuse scleroderma involves _
Diffuse scleroderma involves **widespread skin thickening multiorgan involvement + life-threatening complications**
67
Mixed connective tissue disease has overlapping characteristics of 3 syndromes:
Mixed connective tissue disease has overlapping characteristics of 3 syndromes: **systemic sclerosis, SLE, polymyositis**
68
[Autoantibody] is more specific to limited scleroderma
**Anti-centromere** is more specific to limited scleroderma * *Anti-Scl-70 occurs in both*
69
[Autoantibody] is more specific to diffuse scleroderma
**Anti-RNA polymerase III** is more specific to diffuse scleroderma * *Anti-Scl-70 occurs in both*
70
Mixed connective tissue disease is associated with [autoantibody]
Mixed connective tissue disease is associated with **anti-U1 RNP antibody**
71
Sarcoidosis involves the formation of _ secondary to antigen trigger and immune response
Sarcoidosis involves the formation of **noncaseating granuloma formation** secondary to antigen trigger and immune response
72
Formation of granulomas involves macrophages activating _ which then release _
Formation of granulomas involves macrophages activating **Th1** which then release **IFN-gamma** * Stimulates the formation of epitheloid cells
73
Hypervitaminosis D and hypercalcemia can occur in sarcoidosis due to increased activity of _
Hypervitaminosis D and hypercalcemia can occur in sarcoidosis due to increased activity of **1 alpha-hydroxylase** * *Macrophage activity stimulates this enzyme --> increased calcitrol*
74
Lupus pernio is a skin rash specific to _
Lupus pernio is a skin rash specific to **sarcoidosis**
75
Lofgren syndrome (sarcoidosis manifestation) involves fever + (3 symptoms)
Lofgren syndrome (sarcoidosis manifestation) involves fever + 1. **Bilateral hilar lymphadenopathy** 2. **Erythema nodosum** 3. **Migratory polyarthritis**
76
Sarcoidosis most commonly presents with _ on chest x-ray
Sarcoidosis most commonly presents with **bilateral hilar lymphadenopathy** on chest x-ray * *PFTs would show restrictive lung disease*
77
Increased ACE levels in sarcoidosis result from increased formation of _
Increased ACE levels in sarcoidosis result from increased formation of **epithelioid cells**
78
Isolated sarcoidosis does not require treatment; however, symptoms may be treated with _
Isolated sarcoidosis does not require treatment; however, symptoms may be treated with **glucocorticoids**
79
RA patients are at an increased risk of [CV manifestations]
RA patients are at an increased risk of **pericarditis and MI**
80
A patient with a history of dry eyes, dry mouth, dental carries, oral candidiasis, and parotid swelling is concerning for _
A patient with a history of dry eyes, dry mouth, dental carries, oral candidiasis, and parotid swelling is concerning for **Sjogren's disease**
81
A pregnant woman with a +SSA antibody is at risk of having a baby with _
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
Neonatal lupus is associated with _ rash, cytopenias, liver disease, and congenital heart block
Neonatal lupus is associated with **"racoon eye"** rash, cytopenias, liver disease, and congenital heart block
83
If a patient with Sjogren's develops B symptoms or unilateral parotid gland swelling we should be concerned for _
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
_ is a vasospastic reaction to cold temperatures or emotional stress that leads to sharply demarcated color changes in the skin
**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
The (3) phases of raynaud phenomenon are:
The (3) phases of raynaud phenomenon are: 1. **Ischemia** 2. **Cyanosis** 3. **Rubor**
86
Pharmacologic management of raynaud phenomenon includes:
Pharmacologic management of raynaud phenomenon includes: * Calcium channel blockers * Topical nitroglycerin (vasodilator) * PDE inhibitors
87
_ Systemic sclerosis includes early, rapid visceral involvement, involvement of the trunk, ILD, renal crisis, and GI effects
**Diffuse** systemic sclerosis includes early, rapid visceral involvement, involvement of the trunk, ILD, renal crisis, and GI effects
88
Decreased renal blood flow from scleroderma results in overactivation of RAAS and a high _ state
Decreased renal blood flow from scleroderma results in overactivation of RAAS and a **high renin state** * Scleroderma renal crisis = identical to malignant hypertension
89
Treatment for scleroderma renal crisis is _
Treatment for scleroderma renal crisis is **ACE-inhibitors** * *Scleroderma patients should check blood pressure at least once a week bc of risk*
90
Limited cutaneous systemic sclerosis is associated with late development of [lung pathology]
Limited cutaneous systemic sclerosis is associated with late development of **pulmonary arterial hypertension** * *PAH much more common in limited*
91
ILD is associated with the presense of [antibody] in scleroderma patients
ILD is associated with the presense of **Scl-70 (topoisomerase 1)** in scleroderma patients * *ILD is much more common in diffuse*
92
_ syndrome is a pathognomonic presentation of sarcoidosis that involves anterior uveitis, parotid gland enlargement, facial palsy, and fever
**Heerfordt's syndrome** is a pathognomonic presentation of sarcoidosis that involves anterior uveitis, parotid gland enlargement, facial palsy, and fever
93
Autoantibodies specific to SLE
**Anti-dsDNA, anti-Smith**
94
Autoantibody specific to drug-induced lupus
**Anti-histone**
95
Autoantibody specific to mixed connective tissue disease
**Anti-U1 RNP**
96
The most specific autoantibody to RA is _
The most specific autoantibody to RA is **anti-cyclic citrullinated peptide (anti-CCP)**
97
Rheumatoid factor is a _ antibody
Rheumatoid factor is a **IgM antibody against IgG Fc region**
98
Autoantibodies specific to Sjogrens
**Anti-Ro (SSA) + Anti-La (SSB)**
99
Diffuse scleroderma is associated with [autoantibody]
Diffuse scleroderma is associated with **anti-Scl-70 + anti-RNA pol III**
100
Anti-Scl-70 is a _ antibody
Anti-Scl-70 is an **anti-DNA topoisomerase I**
101
Limited scleroderma, CREST syndrome is associated with [autoantibody]
Limited scleroderma, CREST syndrome is associated with **anti-centromere**
102
Polymyositis and dermatomyositis are associated with [autoantibodies]
Polymyositis and dermatomyositis are associated with **anti-synthetase (anti-Jo-1), anti-SRP, anti-helicase (anti-Mi-2)**
103
Anti-SSA and anti-SSB are [type] antibodies
Anti-SSA and anti-SSB are **anti-ribonucleoprotein** antibodies
104

Integrated systems (8 decks)

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