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Clinical Medicine > Lupus and Rheumatic Disorders > Flashcards

Lupus and Rheumatic Disorders Flashcards

1
Q

What is systemic lupus erythematosus (SLE)?

A

chronic multisystemic inflammatory autoimmune disease

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2
Q

Cause of SLE?

A

unknown

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3
Q

Process of SLE

A

abnormal immune response -> pathogenic autoantibodies -> deposit in tissue

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4
Q

What autoantibodies are produced in SLE?

A

antinuclear; antiphospholipid; anti-double-stranded DNA

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5
Q

x% of SLE patients have a family history

A

10%

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6
Q

Genes affected in SLE have impact on?

A

innate and/or adaptive immune responses; either increased activation or impaired regulation

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7
Q

Environmental factors of SLE

A

UV light; drugs (procainamide, hydralazine, quinidine, isoniazid)

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8
Q

Immunologic triggers of SLE

A

epstein barr, CMV, dietary factors (amino acid L-canavanine)

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9
Q

Hormonal triggers of SLE

A

strong link to females

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10
Q

Does SLE impact men or women more?

A

women

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11
Q

Key age of SLE

A

15-44

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12
Q

constitutional symptoms of SLE

A

fatigue, malaise, weight loss, fever, lymphadenopathy

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13
Q

skin/hair symptoms of SLE

A

malar rash, discoid rash, photosensitive rash, Raynaud’s phenomenon, alopecia

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14
Q

MSK symptoms of SLE

A

polyarthritis, polyarthralgias, myositis

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15
Q

kidney symptoms of SLE

A

HTN, proteinuria, hematuria, renal failure

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16
Q

CNS symptoms of SLE

A

seizures, psychosis, stroke, depression, HA

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17
Q

Cardiopulmonary symptoms of SLE

A

pericarditis, myocarditis, conduction abnormalities, pleuritis, parenchymal lung disease, pulmonary thrombosis, accelerated atherosclerosis with vascular endothelial dysfunction

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18
Q

GI symptoms of SLE

A

hepatosplenomegaly, pancreatitis, bowel vasculitis

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19
Q

Hematologic system symptoms of SLE

A

hemolytic anemia, thrombocytopenia

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20
Q

ocular symptoms of SLE

A

retinal vasculitis, scleritis

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21
Q

Is SLE erosive or non-erosive?

A

non-erosive

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22
Q

number of joints SLE impacts?

A

2 or more peripheral joints

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23
Q

Is SLE symmetrical or asymmetrical joint pain?

A

Symmetrical

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24
Q

Common SLE impacted joints

A

wrist, MCP, PIP

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25
Q

What’s a key MSK finding that may make you think SLE?

A

pain/stiffness out of proportion to PE

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26
Q

When do kidney manifestations commonly occur with SLE?

A

1st two years of onset

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27
Q

SLE can lead to what in the kidneys?

A

renal insufficiency and failure

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28
Q

How to determine if SLE has impacted the kidneys?

A

renal biopsy

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29
Q

What are the two major causes of SLE death?

A

kidney failure, accelerated atherosclerosis with vascular endothelial dysfunction

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30
Q

How many neuropyschiatric syndromes are associated with SLE?

A

19

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31
Q

What’s the most common neuropsychiatric symptom of SLE?

A

cognitive impairment

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32
Q

Which two neuropsychiatric symptoms are part of ACR Lupus diagnostic criteria?

A

seizure, psychosis

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33
Q

11 features of ACR SLE criteria

A

malar rash, discoid rash, photosensitivity, oral ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, antinuclear antibody

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34
Q

HOw many ACR SLE features do you need to be diagnosed?

A

4/11

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35
Q

Describe renal disorder ACR SLE classifications

A

proteinuria (>3+) or cellular casts (red cell; hemoglobin; granular; tubular or mixed)

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36
Q

Describe neurologic disorder ACR SLE classifications

A

seizures or psychosis

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37
Q

Describe hematologic disorder ACR SLE classifications

A

hemolytic anemia with reticulocytosis; leukopenia; lymphopenia; thrombocytopenia

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38
Q

Describe immunologic disorder ACR SLE classifications

A

ANA to double-stranded DNA; positive antibody to Smith nuclear antigen; positive antiphospholipid antibody

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39
Q

Labs for SLE work up

A

CBC with diff, CMP, ESR, CRP, PT, PTT, ANA, urinalysis

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40
Q

What will you see in the CBC with diff of a SLE patient?

A

anemia, leukopenia, lymphopenia, thrombocytopenia

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41
Q

95% specificity for SLE; fluctuates with dz; assc with glomerulonephritis: test

A

Anti-dsDNA

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42
Q

99% specific for SLE: Test

A

Anti-Smith

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43
Q

Mixed connective tissue disease: test

A

Anti-U1RNP

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44
Q

Sjӧgren’s syndrome, photosensitivity: test

A

Anti-Ro/SSA

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45
Q

Sjӧgren’s syndrome: test

A

Anti-La/SSB

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46
Q

Drug induced lupus test

A

Antihistone

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47
Q

Arterial and venous thrombosis; pregnancy morbidity: test

A

Antiphospholipid

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48
Q

Two tests with highest prevalence in SLE

A

antihistone (70%); anti-dsDNA (60%)

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49
Q

Imaging for SLE workup

A

plain films of joints; CXR or Chest CT; ECG/Echocardiogram, renal biopsy

50
Q

What are you looking for in CXR of SLE?

A

pleural effusion, pulmonary infiltrates, interstitial lung disease

51
Q

What are you looking for in ECG/Echocardiogram of SLE?

A

pericarditis, pericardial effusion

52
Q

What are you looking for with renal biopsy of SLE?

A

definitive diagnosis for lupus nephritis

53
Q

What’s the leading cause of m/m in SLE?

A

atherosclerosis

54
Q

What should SLE patients stop?

A

smoking

55
Q

Conservative lifestyle modifcations for SLE?

A

avoid sunlight/high SPF sunscreen, avoid tobacco, avoid fatigue, adequate sleep

56
Q

4 main SLE medication categories?

A

antimalarials, NSAIDs, corticosteroids, DMARDs

57
Q

SLE 5 year survival rate?

A

90%

58
Q

SLE 15 year survival rate?

A

85%

59
Q

What’s Sjӧgren’s Syndrome?

A

Autoimmune disorder affecting exocrine glands; lymphocytic and plasma cell infiltration (salivary and lacrimal glands)

60
Q

Sjӧgren’s Syndrome is associated with?

A

human leukocyte antigen (genetics)

61
Q

Secondary Sjӧgren’s Syndrome is associated with?

A

other autoimmune connective tissue diseases

62
Q

Etiology of Sjӧgren’s Syndrome

A

unknown

63
Q

Environmental triggers of Sjӧgren’s Syndrome

A

viruses (maybe)–EBV, retroviruses, coxsackieviruses, CMV, Hep C; hormones

64
Q

Who is impacted more by Sjӧgren’s Syndrome men or women?

A

women

65
Q

When does Sjӧgren’s Syndrome peak?

A

4th and 5th decade

66
Q

CM of Sjӧgren’s Syndrome

A

insidious onset, xerophthalmia (dry eyes), xerostomia (dry mouth), bilateral parotid gland swelling

67
Q

Xerophthalmia in Sjӧgren’s Syndrome can lead to?

A

Keratoconjunctivitis sicca, corneal ulcers and scarring, bacterial keratitis, eyelid infections, visual impairment

68
Q

Xerostomia in Sjӧgren’s Syndrome can lead to?

A

difficulty swallowing, altered sense of taste, dental caries/infections, fungal infections (candidiasis), pain with eating salty/spicy foods, difficulty talking/develop hoarseness, parotitis

69
Q

How is sicca syndrome associated with Sjӧgren’s Syndrome?

A

it’s independent of Sjӧgren’s Syndrome

70
Q

What is sicca syndrome?

A

dryness of exocrine glands from a variety of causes

71
Q

Constitutional symptoms of Sjӧgren’s Syndrome

A

fatigue; low-grade fever

72
Q

Skin symptoms of Sjӧgren’s Syndrome

A

xerosis, palpable purpura, Raynaud’s phenomenon, photosensitivity

73
Q

pulmonary symptoms of Sjӧgren’s Syndrome

A

dry cough, chronic bronchitis, bronchiectasis, COPD

74
Q

MSK symptoms of Sjӧgren’s Syndrome

A

Polyarthritis; polymyositis

75
Q

Renal symptoms of Sjӧgren’s Syndrome

A

Tubular interstitial nephritis; glomerulonephritis; renal calculi

76
Q

Neurologic symptoms of Sjӧgren’s Syndrome

A

Peripheral neuropathy; optic neuropathy; myelopathy

77
Q

Neoplastic symptoms of Sjӧgren’s Syndrome

A

Lymphadenopathy; lymphoma; Non-Hodgkin’s lymphoma

78
Q

Sjӧgren’s Syndrome work up

A

ocular and oral exams, Schirmer test, Rose Bengal staining, labs

79
Q

Labs for Sjӧgren’s Syndrome

A

CBC with diff, CMP, RF, ANA, anti-ss-A (rho), anti-ss-B (La), elevated ESR; salivary gland biopsy maybe

80
Q

What will the lab work look like for Sjӧgren’s Syndrome?

A

+RF; + ANA, + anti-ss-A (rho), +anti-SS-B (La), elevated ESR

81
Q

6 diagnostic criteria for Sjӧgren’s Syndrome

A

ocular symptoms; oral symptoms; ocular signs; oral signs; positive minor salivary gland biopsy findings; positive anti-SSA or anti-SSB antibody results

82
Q

What are the specific ocular symptom criteria for Sjӧgren’s Syndrome

A

dry eyes > 3 months; FBS; use of AT >3x a day

83
Q

What are specific oral symptom criteria for Sjӧgren’s Syndrome?

A

dry mouth >3 months, recurrent swollen salivary glands, use of liquids to aid swallowing

84
Q

What are specific ocular sign criteria for Sjӧgren’s Syndrome

A

positive test for schirmer or rose bengal stain

85
Q

What are specific oral sign criteria for Sjӧgren’s Syndrome

A

abnormal salivary scintigraphy, parotid sialography, abnormal salivary flow

86
Q

How many criteria do you need for Sjӧgren’s Syndrome diagnosis?

A

4+

87
Q

Treatment for Sjӧgren’s Syndrome (non-rx)

A

fluid replacement, skin lotion, humidifiers, proper oral hygiene, annual eye/dental exams, treat secondary symptoms

88
Q

Rx treatment for dry eyes (Sjӧgren’s Syndrome)

A

artificial tears, topical steroids, cyclosporine ophthalmic (restasis), lacrimal puncta plugging, glasses with moisture shields

89
Q

Dry mouth treatment (Sjӧgren’s Syndrome)

A

encourage increased fluid intake, humidifiers, artificial saliva (salivart, saliment, mouthkote)

90
Q

What is polymyalgia rheumatica?

A

Inflammatory condition affecting shoulders and pelvic girdle; non-erosive synovitis and tenosynovitis

91
Q

Etiology of polymyalgia rheumatica?

A

unknown; associated with HLA-DR4 (activates immune system leading to inflammation)

92
Q

Does polymyalgia rheumatica impact men or women more frequently?

A

women

93
Q

CM of polymyalgia rheumatica

A

sudden onset of muscle pain and stiffness; neck, shoulders, arms; pelvic girdle and thigh muscles; possible muscle weakness; possible fatigue/malaise/loss of appetite

94
Q

What condition is polymyalgia rheumatica associated with?

A

giant cell arteritis

95
Q

giant cell arteritis targets?

A

medium to large extracranial blood vessels

96
Q

Commonly affected arteries by giant cell arteritis

A

superficial temporal, ophthalmic, occipital, vertebral, posterior ciliary, proximal vertebral

97
Q

RF for giant cell arteritis

A

women, age

98
Q

S/S of giant cell arteritis

A

HA, TTP over temporal artery, visual changes, possible fatigue/malaise/fever

99
Q

laboratory testing for polymyalgia rheumatica

A

ESR, CRP, CBC with diff, RF or anti-CCP, creatine phosphokinase, GCA-temporal artery biopsy

100
Q

What’s the gold standard for GCA?

A

temporal artery biopsy

101
Q

What do you expect with the ESR in polymyalgia rheumatica?

A

> 40

102
Q

polymyalgia rheumatica diagnostic criteria

A

morning stiffness >45 minute (2 pts); hip pain/limited ROM (1 point); absence of RF and/or anti-CCP (2 points); absence of peripheral joint pain (1 point)

103
Q

Treatment of polymyalgia rheumatica

A

prednisone 12.5-25 mg/day (long term add PPI to protect GI); Ca/Vit D

104
Q

Treatment of giant cell arteritis

A

Prednisone 40-60 mg x 4 weeks; then decrease 10 mg every 2 weeks until reach 20mg, then reduce by 2.5 mg every 2-4 weeks to 10 mg; then decrease 1 mg every 1-2 months as long as no relapse

105
Q

What is polyarteritis nodosa?

A

systemic vasculitic syndrome; Necrotizing inflammation of arterial media and inflammatory cell infiltration

106
Q

What vessels does polyarteritis nodosa impact?

A

small and medium

107
Q

Where in the vessel does polyarteritis nodosa impact?

A

bifurcations

108
Q

What happens when vessels weaken in polyarteritis nodosa?

A

aneurysms, thrombi development, leads to hemorrhage and organ ischemia or infarction

109
Q

Etiology of polyarteritis nodosa?

A

unknown, maybe Hep B/C, CECR1 gene mutation (encodes for adenosine deaminase 2 -> affects immune system)

110
Q

Does polyarteritis nodosa impact men or women more frequently?

A

men

111
Q

Polyarteritis nodosa is associated with?

A

hairy cell leukemia

112
Q

Constitutional symptoms of polyarteritis nodosa?

A

fever, malaise, fatigue, anorexia, myalgia

113
Q

NS symptoms of polyarteritis nodosa?

A

transient cerebral ischemia and monocular blindness, peripheral neuropathy

114
Q

cutaneous symptoms of polyarteritis nodosa?

A

rash, purpura, gangrene, nodules, livedo reticularis, raynaud’s

115
Q

What’s livedo reticularis?

A

common skin finding consisting of a mottled reticulated vascular pattern that appears as a lace-like purplish discoloration of the skin.

116
Q

What condition is livedo reticularis associated with?

A

polyarteritis nodosa

117
Q

GI symptoms of polyarteritis nodosa?

A

postprandial abdominal pain, N/V, organ infarction

118
Q

renal symptoms of polyarteritis nodosa?

A

renal failure

119
Q

labs for polyarteritis nodosa

A

CBC with diff, CRP, ESR, liver function, HBV/HCV serology, RF (negative), ANA (negative), angiography (aneurysmal dilation–renal, mesenteric, or hepatic arteries), biopsy, abdominal CT

120
Q

Diagnostic criteria of polyarteritis nodosa (3/10)

A

Weight loss > 4 kg, Livedo reticularis, Testicular pain/tenderness, Myalgias, weakness or leg tenderness, Neuropathy, Diastolic bold pressure > 90 mmHg, Elevated BUN > 40 mg/dl or creatinine >1.5 mg/dl, Positive Hep B virus, Arteriography with aneurysms, Arterial biopsy with necrotizing inflammatory infiltrate

121
Q

Treatment of polyarteritis nodosa?

A

prednisone 1-2 mg/kg/day (start higher and taper), maybe methotrexate with steroids, anti-viral for hep b, treat HTN

122
Q

Prognosis of polyarteritis nodosa?

A

if untreated 5 year survival rate <20%; if treated increases to 50%

Clinical Medicine (13 decks)

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