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M2 Musculoskeletal > 4/19 SLE, Systemic Sclerosis - Corbett > Flashcards

4/19 SLE, Systemic Sclerosis - Corbett Flashcards

1
Q

SLE

basics

demographics

etiology x3

A

systemic lupus erythematosus

  • autoimmune disease mediated by tissue-binding autoantibodies and immune complexes
    • deposition in tissues: heart, kidney, skin, etc

demographics

90% pts women, principally childbearing age (65% are b/w 16-55)

racial predisposition: AfAm/Caribbean

etiology

  1. genetic factors
    • family hx, monozygotic twin concordance
    • HLA linkage
    • inherited deficiency of early complement (C2, C4, C1q)
      • lack of complement → imparied removal of circulating ICs by MNP system → favors tissue deposition!
  2. immunologic factors
    • ​​failure of self-tolerance in B and CD4+ T cells → production of high-affinity pathoenic autoantibodies
    • TLR engaged by nuclear DNA and RNA → activates B lymphocytes
    • type 1 IFN play a role in lymphocyte activation
  3. environmental factors
    • UV light exposure
    • gender bias due to actions of sex hormones, also X chromosome
      • XXY males: 14x risk of SLE!
    • drugs: hydralazine, procainamide, d-penicillamine can induce SLE-like response in humans
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2
Q

SLE model

A

strong assoc with defects in apoptotic clearance

  • initiating events vary
  • resulting excessive apoptotic debris has imp pathogenic effect
    • antibodies are generated against nuclear antigens

ANA (anti-nuclear antibody): highly SENSITIVE for SLE, not v specific

  • good rule out test
  • not a great rule in test

anti-dsDNA and antiSm: highly SPECIFIC for SLE

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3
Q

autoantibodies

A

ANA (anti-nuclear antibody): highly SENSITIVE for SLE, not v specific

  • good rule out test
  • not a great rule in test

anti-dsDNA and antiSm: highly SPECIFIC for SLE

anti-dsDNA

  • high specificisy
  • titers fluctuate w disease activity
  • high titer IgG anti-dsDNA abs → active glomerulonephritis

antiSm (nuclear non-histone proteins)

  • not sensitive, HIGHLY SPECIFIC
  • positive even when pt enters remission (levels can fall into normal range when quiescent)
  • “speckled” pattern
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4
Q

mechanism of tissue injury

5steps

A

most systemic lesions caused by immune complexes (type III hypersensitivity)

  • DNA-antiDNA complexes detected in glomeruli, sm blood vessels
  • autoabs specific for RBC, WBC, platelets opsonize and promote phagocytosis/lysis
  • antiphospholipid antibody
  1. loss of self tolerance
  2. production of autoab
  3. deposition of IC
  4. IC-assoc infl
  5. tissue fibrosis and damage
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5
Q

SLE presentation

A

young woman with…

  • fever
  • malar “butterfly” rash (photosensitive)
  • arthralgia (symmetric polyarthritis)
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6
Q

typical SLE patient

Ps

A
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7
Q

characteristic rash: acute cutaneous SLE

A

degeneration fo basal layer of epidermis due to abs and IC at dermal-epidermal border

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8
Q

discoid lupus

nephritic disease

A

discoid lupus

  • primary lesion: erythematous papulse or plaque (slight to moderate scale)
    • hypopig in center, hyperpig at borders

nephritic disease

cardiac issues

  • involvement of pericardium (effusions), myocardium, valves (sterile platelet vegetations → thickening of valves)
  • “Libman Sacks endocarditis”: sterile platelet vegetations w high risk of embolic events, assoc with neuropsych SLE
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9
Q

SLE arthritis

A
  • non-deforming (not erosive)
    • issues due to lax tendons (not bone erosion)
  • migratory
  • symmetric, polyarticular problem (knees, carpal jts, finger jts)
  • brief morning stiffness, mild synovitis
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10
Q

drug induced SLE

age of onset

racial pref

gender pref

dx abs

clinical findings

A
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11
Q

mortality

A

early death?

  • active disease: renal, CNS issues
  • infection: immunosuppression
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12
Q

SLE management

A
  • immunosuppressive agents
  • corticosteroids (flares)
  • azothiaprine
  • methotrexate
  • cyclophosphamide
  • biologics: being investigated
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13
Q

systemic sclerosis

(scleroderma)

A

4-5x more common: F>M

age at dx: 50yo

  • chronic infl due to autoimmunity : CD4+ T ells responding to antigens accumulate in skin, release cytokines that activate infl cells and fibroblasts
  • widespread damage to sm blood vessels
    • intimal damage in digital arteries
  • progressive interstitial and pervascular fibrosis in skin, multiple organs
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14
Q

vascular damage

capillaropathy

A
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15
Q

types of scleroderma

A

limited SSc

  • distal to elbows, distal to knees, on face
  • late visceral involvement
  • benign clinical course

diffuse SSc

  • all over
  • early visceral involvement
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16
Q

CREST syndrome

A

form of ltd scleroderma

  • calcinosis
  • Raynaud phenom
  • esophageal dysmotility
  • sclerodactyly (distal to MCP joints)
  • telangiectasia
17
Q

key clinical ft of scleroderma

x4

two addtl systems

A

most common:

  1. Raynaud phenomenon
    • episodic exaggerated vasoconstr of sm arteries, arterioles, AV shunts, or thermoreg vessels of skin of digits (triggered by stress/cold)
    • pallor → cyanosis → hyperemia (w return of bloodflow)
    • be concerned if: starting brand new in middle aged female
  2. skin changes
    • progressive, insidious swelling of distal extremities (ex. fingers)
    • gradual thickening of skin of fingers
    • diffuse sclerotic atrophy of skin
    • pitting, ulcerations at tips of fingers
  3. swollen fingers and arthralgias
    • arthralgias and myalgias are common → infl myopathy due to collagen deposition in muscle
    • non-rosive arthritis affecting small joints
    • ultimate loss of jt mobility is due to skin thickening/fibrosis
  4. GER with or without dysmotility
    • ​​esoph manometry totally flat: low peristalsis and LES resting tone also low → GERD!!!! w dysphagia
      • atrophy of esoph sm muscl

_*GI issues_

general bowel (large and small) dysmotility too - constipation alternative w diarrhea

  • can develop SIBO
  • associated with GAVE (gastric antral vascular ectasia) aka watermelon stomach

_*lung issues_

  • inflammatory alveolitis → interstitial fibrosis
    • ​early diffuse scleroderma in first 4yr illness
    • anti-topoisomerase abs (anti-Scl70) predicts poor outcome
  • pulmo vascular disease → pulmo a HTN
    • longstanding disease in limited SSc
    • prominent P2
18
Q

key clinical ft of scleroderma

GI

pulm

renal

A

_*GI issues_

general bowel (large and small) dysmotility too - constipation alternative w diarrhea

  • can develop SIBO
  • associated with GAVE (gastric antral vascular ectasia) aka watermelon stomach

_*lung issues_

  • inflammatory alveolitis → interstitial fibrosis
    • ​early diffuse scleroderma in first 4yr illness
    • anti-topoisomerase abs (anti-Scl70) predicts poor outcome
  • pulmo vascular disease → pulmo a HTN
    • longstanding disease in limited SSc
    • prominent P2

*renal issues

  • sudden onset malignant HTN (headache, visual disturbances, enceph w seizure, pulmo edema, pericardial effusion)
    • hyper-renin
  • risks: diffuse SSc, esp early; glucocorticoid tx (avoid in diffuse); RNA polymerase III abs
19
Q

scleroderma renal crisis

A
20
Q

scleroderma antibodies

A

negative-ANA → scleroderma v unlikely

  • but not specific!

other antibodies:

  1. anti-centromere ab
    • limited SSc
  2. anti-topoisomerase (antiScl70)
    • highly specific for SSc (assoc w diffuse)
    • assoc w interstitial lung disease and poor prognosis
  3. antiRNApoly3
    • rapid skin involvement
    • scleroderma renal crisis
21
Q

Sjogren’s syndrome

A

autoimmune disease presenting with sicca sx on mucosal surfaces

  • xerophthalmia
  • xerostomia

glands are destroyed → don’t make saliva/tears

determined by specific testing:

  • ocular (rose bengal staining, Schirmer test)
  • oral (salivary flow measurement, parotid scintigraphy)

relatively common

women (14-24x), 40-60

22
Q

Sjogren’s

clinical features

histo hallmark

A

dry mouth due to decr saliva

  • difficulty speaking
  • incr dental decay
  • difficulty swallowing
  • oral candidiasis

dry eyes due to decr lacrimation

  • gritty sensation
  • blurred vision
  • corneal ulcerations over time

hallmark: focal lymphocytic infiltrations of exocrine glands (check via biopsy)

23
Q

concern with Sjogrens

A

frequent lymphadenopathy

complication: lymphoma! (44x incidence)

24
Q

Sjogren’s clinical manifestations

A
25
Q

Sjogren’s antibodies

A
  • ANA antibodies (common)
  • Rf (75%)
  • anti-ribonucleoprotein abs
    • ​anti-Ro/SS-A and_or anti-La/SS-B are most specific

M2 Musculoskeletal (21 decks)

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