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Clin Med III: MSK > Rheumatological Conditions > Flashcards

Rheumatological Conditions Flashcards

1
Q

what is the first line of autoantibody defense.

A

B cells develop in the Bone Marrow and any antibody created which would attack a body protein found in the bone marrow is destroyed.

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2
Q

Without ____ of the B Cells, the body will not proliferate the autoantibody; this is the second line of defense against autoantibody production.

A

T Cell activation

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3
Q

causes of autoimmunity

A
  1. genetics
  2. stress
  3. infection
  4. medication SE
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4
Q

Rheumatology, probably more so than other fields of medicine, relies on recognition of _____ gleaned from a good H&P, labs, and imaging as many presentations will generate a large differential and labs and imaging are often non-specific

A

PATTERNS

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5
Q

difference between highly sensitive test vs highly specific test?

A
  • Highly Sensitive Test- Very few false neg; can cause false positives making healthy ppl being improperly dx; ability to R/O dx
  • Highly Specific Test- Very few positive cases will be something other than what the tester is looking for; there are positives which are not picked up - false negatives may occur. People with disease may be missed; ability to rule IN dx
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6
Q

the main lab to order if Suspect an autoimmune disease / Connective Tissue Disease?

A

ANA: Antinuclear Antibody

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6
Q

what is an ANA: Antinuclear Antibody?

A
  • Autoantibodies - antibodies that attack self
  • Will attack the nucleus of healthy cells
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7
Q

ANA is positive if titer is what ratio?

A

≥1:160

  • Amount of saline dilution for antibodies to become undetectable
  • ~5% of healthy ppl
  • Higher titer = Greater likelihood; ≠ severity
  • High Sensitivity; Low Specificity (more test needed)
  • Once ANA is (+) - do not retest
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8
Q

possible causes that increase ANA?

3

A
  1. infection
  2. CA
  3. pregnancy
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9
Q

disease with (+)ANA based on sensitivity

A
  1. Drug-Induced Lupus (Hydralazine, Procainamide, Isoniazid) - 100%
  2. SLE - >95%
  3. Scleroderma - 85%
  4. Sjӧrgen Syndrome - 48%
  5. Dermatomyositis / Polymyositis - 61%
  6. RA - 40%
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10
Q

patterns seen with SLE

5

A
  • Anti-dsDNA - Rim
  • Anti-SSA (Anti-Ro) - Speckled
  • Anti-SSB (Anti-La) - Speckled
  • Anti-Smith - SnRNP
  • Anti-U1-RNP - U1-SnRNP
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11
Q

pattern for Drug Induced Lupus

A

Anti-Histone, Homogenous

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12
Q

Pattern for Progressive Systemic Sclerosis

A
  • Anti-RNA-Polymerase - Nucleolar
  • Anti-Scl-70 - DNA Top I
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13
Q

patterns for Dermatomyositis

A
  • Anti-Mi-2, Nucleolar
  • Anti-Jo-1, Nucleolar
  • Anti-SRP, Cytoplasmic
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14
Q

patterns for Polymyositis

A
  • Anti-Jo-1, Nucleolar
  • Anti-SRP, Cytoplasmic
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15
Q

pattern for crest syndrome

A

Anti-Centromere, Cytoplasmic

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16
Q

pattern for Sjӧgren

A
  • Anti-SSA (Anti-Ro), Speckled
  • Anti-SSB (Anti-La), Speckled
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17
Q
  • Often a reflex lab with (+) ANA
  • Leads the clinician to a more specific disease in the presence of possible Connective Tissue Disease
A

ENA: Extractable Nuclear Antigens

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18
Q

Mixed Connective Tissue Disease is a term describing ?

A

overlap of clinical features of SLE, Systemic Scleroderma, and polymyositis.

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19
Q

First detected in Rheumatoid Arthritis patients
Proteins in blood produced by immune system
RF presence indicates inflammatory process

which lab?

A

RF: Rheumatoid Factor

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20
Q

RF: Rheumatoid Factor is found in what conditions?

A
  1. Rheumatoid Arthritis
  2. Sjogren’s Syndrome
  3. Juvenile Arthritis
  4. Scleroderma
  5. Mononucleosis
  6. TB
  7. Leukemia
  8. Multiple Myeloma
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21
Q

2 most sensitive conditions to rheumatoid factor?

A
  1. Sjogren’s (75-95%)
  2. RA (70-90%)

not specific to RA

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22
Q

what other infectious conditions may present with high rheumatoid factor?

A

syphilis, HCV, TB

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23
Q
  • A newer test more specific for Rheumatoid Arthritis than RF
  • Is found in other diseases than RA, but at significantly lower levels.
A

Anti-CCP: Anti cyclic citrullinated peptide

  • Sensitivity for RA - 66.0%
  • Specificity for RA - 90.4%
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24
Q

Best at identifying Acute Inflammation
Produced by liver (not reliable test w/ liver failure)

A

CRP: C-reactive Protein

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25
Q
  • Best at identifying Chronic Inflammation - Infection, Malignancy, Vasculitis, most Anemias, MI, Inflammatory Arthritis
  • Normal test r/o temporal arteritis and polymyalgia rheumatica
  • ↑ w/ age, pregnancy (2nd/3rd TM), medications (OCP)
A

ESR: Erythrocyte Sedimentation Rate

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26
Q

Two types of test of ANCA

A
  • FA, immunofluorescence, (more sensitive)
  • ELISA (more specific)
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27
Q

p-ANCA (Perinuclear pattern) is MC in what conditions

A

SLE, Sjogren’s, RA, PM, DM

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28
Q

c-ANCA (Cytoplasmic pattern) is MC in what conditions

A

(+) Wegener Granulomatosis & Churg-Strauss Syndrome

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29
Q

A chronic, systemic, multi organ autoimmune disease of connective tissues secondary to antibody formation and immune complex deposition.

A

Systemic Lupus Erythematosus (SLE)

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30
Q

SLE MC affects who?

A
  • female
  • 16-55 y/o
  • Native American > African American > Asian (Chinese, Filipino) > Hispanic and Japanese > Caucasian
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31
Q

Precipitating factors of SLE include:

A
  • Sun exposure
  • Stress
  • Infections
  • Pregnancy
  • Surgery
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32
Q

Low grade fever
Joint Pain
Rash - Covers the cheeks & nasal bridge; spares the nasolabial folds

A

SLE

Malar (butterfly) rash

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33
Q

criteria to dx SLE

A

4 out of 11 common s/s:

  1. Malar Rash
  2. Discoid Rash
  3. Photosensitivity
  4. Oral Ulcers
  5. Arthritis
  6. Kidney dz
  7. Neuro dz (In absence of lyte abnormalities or med SE)
  8. Serositis - pericarditis, pleuritis
  9. Hematologic dz - hemolytic anemia w/ reticulocytosis, lymphopenia, thrombocytopenia
  10. Immunologic Abnormalities
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34
Q

immunologic dx criteria for SLE

A
  1. abnml ANA
  2. Anti-dsDNA in abnml titer OR
  3. Anti-Smith OR
  4. (+) Antiphospholipid Ab:
    - abnml level IgG or IgM anticardiolipin ab OR
    - (+) lupus anticoag using standard method OR
    - false (+) for syphilis known to be positive for at least 6 mo and confirmed by Treponema pallidum immobilization or fluorescent treponemal ab absorption test
35
Q

findings that indicate kidney disease

A
  • > 500mg protein in 24 hrs.
  • ≥3+ protein UA dipstick
  • Casts: Red, Hgb, granular, tubular, mixed
36
Q

what ANA ratio is needed to use the ACR/EULAR 2019 criteria?
points needed?

A

≥ 1:80
10 points needed for inclusion.

37
Q

derm manifestations of SLE

6

A
  1. Malar Rash
  2. Discoid Rash
  3. Photosensitivity
  4. Oral / Nasopharyngeal Ulcers
  5. Raynaud’s
  6. Alopecia
38
Q

MSK manifestations of SLE

A
  1. Arthritis - ≥2 joints, Migratory / Symmetric, Often peripheral, esp. hands
  2. Myalgias
39
Q

CV and pulm manifestations of SLE

A
  1. Pericarditis or Pleuritis
  2. Myocarditis
  3. Valvular dz
  4. Vasculitis
  5. Interstitial Lung Disease
  6. Pulmonary HTN
40
Q

renal manifestations of SLE

A

Lupus nephritis

  • BX proven Lupus Nephritis guarantees SLE
  • Hematuria
  • Proteinuria
  • Elv Creatinine
  • May be asx - UA / BMP if considering SLE Dx
41
Q

neuro manifestations of SLE

A
  • Cognitive dysfunction
  • Stroke
  • Neuropathy
  • Transverse myelitis
  • HA
  • Delirium
  • Depression
42
Q

GI manifestations of SLE

A

Not on the list of 11

  • Dysphagia, abdominal pain, nausea
  • Pancreatitis, gastritis, mesenteric vasculitis
43
Q

systemic manifestations of SLE

A

Not on the list of 11

  • Fatigue, fever, malaise, weight changes
  • weakness
44
Q

what is the most common manifestatio of SLE

A

MSK - 90%

45
Q

what is drug-induced lupus?

A
  • Strong Genetic predisposition
  • resolves with DC of offending agent
  • Not associated w/ CNS/Kidney/Alopecia
  • ANA+ - 100% sensitivity
  • (+) Anti-Histone Ab - 95%
46
Q

common medications that causes SLE

A
  • (High Risk >5%) Procainamide, hydralazine, Penicillamine
  • (Moderate Risk 1-5%) Quinidine
  • Many other Low risk medications…
47
Q

monitoring for SLE

A
  • q 6 mo if completely stable; 3-4 mo
  • Complete H/PE
  • CBC: thrombocytopenia (acute flare); Anemia
  • CMP
  • UA w/ micro
  • +/- ESR, CRP, Complement levels, Anti-dsDNA - fluctuate with flares
48
Q

nonpharm mgmt for SLE

A
  • refer
  • psychological
  • sun protection & SPF >55; avoid meds w/ photosensitivity
  • diet: balanced, vit D supp, daily vitamins if GI concerns
  • exercise
  • smoking cessation
  • immunization: flu, pneuomococcal, HPV, HBV
  • avoid pregnancy during flares - “dormant” x 6 mo before trying
49
Q

what medications to avoid in SLE

A
  1. Sulfa Antibiotics (Bactrim) - rash in >30%,
  2. Minocycline
50
Q

mgmt for mild SLE

A

Skin joint and mucosal

  • Hydroxychloroquine, (+/-) NSAID
  • < 7.5 mg prednisone daily if needed
51
Q

mgmt for moderate SLE

A

Significant but non-organ threatening disease

  • Hydroxychloroquine + NSAID
  • 5-15 mg prednisone daily, tapered
  • Immunosuppressive (MTX or Azathioprine)
52
Q

mgmt for severe SLE

A

Renal/CNS involvement

  • Hydroxychloroquine + NSAID
  • High dose IV prednisone
  • Immunosuppressive agent (Belimumab, Rituximab, Cyclosporine)
53
Q

possible tx options for joint sx in SLE

A
  1. Hydroxychloroquine (Plaquenil)
  2. Naproxen
  3. Prednisone
54
Q

which joint symptom meication is known to cause the “Bull’s Eye Retinopathy”
what is this called?

A
  • Hydroxychloroquine
  • Chloroquine Retinopathy
55
Q

epidemiology of scleroderma

A
  • 1-2/100,000 in USA
  • Females
  • MC 30-50 years of age
56
Q

skin manifestations of scleroderma

A
  1. puffiness
  2. sclerodactyly
  3. rest of body tightens
  4. pigmentation changes and itching
57
Q

vascular manifestations of scleroderma

A
  1. raynauds - 95%
  2. fingertip ulcerations
  3. cutaneous telangiectasia
  4. CAD
58
Q

GI manifestations of scleroderma

A
  1. GERD, hoarsenss, dysphagia
  2. dyspepsia, early satiety, bloating
  3. constipation, fecal incontinence
  4. malnutrition and chronic iron def anemia
59
Q

rsp manifestations of scleroderma

A
  1. dyspnea - progressive
  2. chest pain - pulm HTN
  3. cough, dry - restrictive lung disease
60
Q

MSK manifestations of scleroderma

A
  1. arthralgia, myalgia
  2. Loss of ROM, muscle weakness
  3. carpal tunnel
61
Q

cardiac manifestations of scleroderma

A
  1. CHF
  2. arrhythmias, palpitations
  3. systemic sclerosis - independent risk for MI
62
Q

renal manifestations of scleroderma

A
  1. HTN
  2. AKI
  3. CKD
63
Q

GU manifestation sof scleroderma

A
  1. ED
  2. dyspareunia
  3. vaginal narrowing, dryness
64
Q

ENT manifestations of scleroderma

A
  1. sicca syndrome w/ poor dentition
  2. tongue CA
  3. blindess - retinal artery occlusion

sicca: lymphocytic infiltration of salivary and lacrimal glands

65
Q

neuro manifestations of scleroderma

A
  1. TN
  2. peripheral neuro
  3. HA and CVA
66
Q

There are at minimum 4 subsets of Scleroderma:

A
  1. Limited Cutaneous Systemic Sclerosis - s/s distal to the elbows and the face and neck. Many manifest CREST.
  2. Diffuse Cutaneous Systemic Sclerosis - proximal and even truncal manifestations.
  3. Systemic Sclerosis Sine Scleroderma - other s/s present w/o skin manifestations - RARE
  4. Systemic Sclerosis w/ Overlap Syndrome - Any of the above w/ overlap features of another systemic rheum dz (ex: SLE, Sjogren’s, RA, etc…)
67
Q

what is CREST syndrome?

A
  1. calcinosis
  2. raynauds
  3. esopahgeal dysfunction
  4. sclerodactyly
  5. telangiectasias
68
Q

difference between CREST vs systemic

A

CREST - mainly head and hands
systemic - includes trunk and proximal extremities

69
Q

MC GI manifestation of scleroderma

A

esophageal (90%)

Other GI sx:

  • GERD
  • Choking
  • Cough after swallowing
  • Bloating
  • Early Satiety
  • Dysphagia
70
Q

w/u for scleroderma

A
  • CBC - anemia, dec MCV (iron def and/or blood loss)
  • BMP - dec kidney function
  • ANA (+)
  • Anti-DNA top I (Scl-70) - diffuse cutaneous SSc - risk of lung dz
  • Anti-Centromere - assoc w/ CREST
  • Anti-RNA polymerase III - Diffuse dz - risk of renal failure and CA
71
Q

All patients should have baseline imaging/testing to determine if ____ ____ _____ is present
what are these testings?

scleroderma

A

Interstitial Lung Disease

  • PFTs
  • Echo
  • CT of Chest
72
Q

tx for scleroderma

A
  1. No Definitive Cure
  2. Usually Organ System based
  3. Raynaud’s - CCB, GERD - PPI
  4. Arthritis: Similar to lupus
    - NSAID (If kidneys stable)
    - Inflammatory (RA), add in stepwise approach - Low dose steroids, Hydroxychloroquine, MTX, Biologics
  5. PT and wt loss
  6. Diffuse skin sclerosis or organ involvement - MTX: 15-25 mg weekly
  7. REFER to Rheumatology!!!
73
Q

chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland function with resultant dryness of the eyes and mouth

A

Sjӧgren’s Syndrome

74
Q

epidemiology of Sjӧgren’s Syndrome

A
  • one of the more common autoimmune disorders
  • Female
  • 50-60 y/o
75
Q

pathophys of Sjӧgren’s Syndrome

A

Ab attack:

  • exocrine glands - xerostoma, dry eyes, parotid gland enlargement
  • endocrine glands - thyroid dysfunction
  • extraglandular - arthritis, GI, ILD, neuropathies
76
Q

if secondary Sjӧgren’s Syndrome, it is often associated with what other conditions? (2)

A
  1. RA
  2. SLE
77
Q
  • Dry eyes - burning, itching, difficulty with contacts, ropy secretions, FB sensation, photophobia
  • Dry Tongue - Halitosis, insatiable thirst, cavities - often at the gum line, dysphagia without liquid,
  • Dry skin, vagina, nose, throat, larynx

dx?
w/u?

A

Sjӧgren’s Syndrome

  • Basic labs - pancytopenia, hypokalemia, low bicarb, inc AST/ALT possible
  • Elevated ESR (CRP is not)
  • ANA (-)
  • Anti-SSA (Anti-Ro) / Anti-SSB (Anti-La) (+) in primary disease
  • (+) HLA-DR52 gene
  • Labial salivary gland bx may confirm
  • eye sx - Schirmer Test & surface staining
78
Q

what other conditions may mimic sjogren’s syndrome?

A

HIV, HCV, and Vit A def

79
Q

tx for Sjӧgren’s Syndrome

A
  1. Eye drops
  2. Avoid smoking
  3. Omega-3 Fatty Acids
  4. Counsel women that SS is associated with congenital heart block if high titers of Ro/La
  5. Lotion for skin
  6. Dental exams q 6 mo w/ fluoride tx
  7. Pilocarpine: Cholinergic
    - Xerostomia: 5 mg PO QID
80
Q
  • SE of pilocarpine?
  • what other med can help with SE?
A

diaphoresis, flushing, sweating, bradycardia, diarrhea, N/V, incontinence, blurred vision. (Cyclosporine may help with blurred vision)

81
Q

Epidemiology of Polymyositis / Dermatomyositis

A
  • DM MC > PM
  • females
  • DM in childhood or adulthood vs PM in adulthood
82
Q

s/s of DM and PM

A
  1. Insidious onset
  2. symmetric, progressive, painless proximal weakness, eye and facial muscles spared, +/- dysphagia; extramuscular manifestations (ILD, polyarthritis, skin changes in DM, not PM)
  3. Pain possible
  4. Muscle Atrophy: difficulty rising from a chair, climbing steps, ADLs, etc
  5. Overlap with other autoimmune disease possible
83
Q

DM and PM have an Increased risk of ? 3-5 years after onset

A
  • cancer
  • DM > PM

Ovarian, breast, colon, melanoma, Non-hodgkin’s

84
Q

Skin manifestations common with DM

A
  1. Gottron’s Papules - raised, violaceous scaly eruptions on knuckles
  2. Heliotrope Eruption (Blu-purple)
  3. Mechanic Hands - hyperkeratotic with dirty appearance
  4. Shawl Sign
85
Q

w/u for Polymyositis / Dermatomyositis

A
  1. Basic labs
  2. elevated ESR (CRP is not)
  3. Muscle Enzymes - Aldolase, CK (>10x ULN in active disease, inc CK-MB w/o Troponins)
  4. ANA (+) MC
  5. Anti-Mi-2: DM
  6. Anti-Jo-1: DM/PM - Interstitial Lung Fibrosis, Mechanic Hands
  7. Anti-SRP: DM/PM - More exclusive to PM, MSK weakness and cardiac s/s
  8. Imaging
    - MRI: Muscle edema or atrophy, fibrosis
    - EMG: Myopathic Potentials
  9. Muscle Bx - not necessary
86
Q

tx for Polymyositis / Dermatomyositis

A
  1. CA screening + CT chest, abd, pelvis, TVUS, Mammogram - q 2 years
  2. Swallow evaluation if dysphagia
  3. Prednisone 1 mg / kg [max = 100 mg/QD]
    - Taper after 4-6 weeks
    - +/- DMARD (MTX / Sulfasalazine)
    - topicals for skin
    - PT for muscle weakness

Clin Med III: MSK (14 decks)

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