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monster management > autoimmune 2 > Flashcards

autoimmune 2 Flashcards

1
Q

multiple sclerosis is…

A

immune system attacks myelin of nerves → communication problems

NO CURE** paresthesia / motor weakness **

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2
Q

myasthenia gravis is…

A

chronic neuromuscular disease w varying degrees of weakness of skeletal muscles

THINK: T-cell immune response @ proteins in neuromuscular junction

** profound fatigue WITH skeletal muscle weakness **

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3
Q

ALS is…

A

amyotrophic lateral sclerosis

progressive incurable neurodegenerative disorder (“neuro failure” - motor neuron degeneration/death) / 3-5 year survival

ETC

  • cortical motor cell loss + retrograde axonal loss- gliosis replaces lost neurons
  • spinal cord atrophy + loss of large myelinated fibers in motor nerves
  • intracellular inclusions in degenerating neurons & glia
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4
Q

idiopathic inflammatory myopathies are…

A

INFLAMMATION OF SKELETAL MUSCLES

polymyositis: “chronic inflammatory myopathy”
- SKELETAL muscle weakness

dermatomyositis: “connective tissue disease w inflammation”
- SKIN manifestation before muscle involvement
SEE: dermato w/ poly more often

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5
Q

systemic lupus erythematosis is…

A

immune system mistakenly attacks healthy tissue, often: skin, joints, kidneys, brain

** JOINT PAINT **

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6
Q

granulomatosis w/ polyangitis is…

A

aka Wegener’s
chronic inflammation of vessels = INHIBITS BLOOD FLOW = hypoperfusion

** sinusitis most common s/s **

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7
Q

polyarteritis nodosa is…

A

inflammatory vessel disease: necrotizing arteritis of MEDIUM VESSELS involving…

skin, peripheral nerves, mesenteric vessels, heart, brain

** PAIN prominent early s/s **

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8
Q

hereditary angioedema is…

A

genetic disease = edema of face, airways, internal organs

C1 INHIBITOR low or non-fxn

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9
Q

what is C1 + why is it important + which disease

A

C1 = bradykinins + inflammatory molecules
need C1-inhib or else excess levels
hereditary angioedema

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10
Q

arteritis

A

inflammation of arteries, usually d/t infection or autoimmune

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11
Q

MS progression

A

most: slow, progressive, secondary disease with steady neuro deterioration

85% = relapse/remit phases

5% = “benign MS” - largely asymp 10 to 15 years out

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12
Q

MS: more relapse/remission cycles = …

A

increased likelihood of advanced disease progression

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13
Q

MS s/s

A 
paresthesia (37-45%) ← non-specificmotor weakness (20-27%)
gait/balance disturbance (13-35%)
vision loss/optic neuritis (15-17%)
diplopia &/or vertigo (10-13%)
hyperreflexia, extensor plantar response (babinski)
LE ataxia
impaired rapid alternating movements
loss of vibration/proprioception
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14
Q

MS dx

A

dissemination in TIME
- 2+ attacks
OR
- gadolinium enhancing lesions (MRI) any time OR
- new MRI T2 enhancing lesion + prev doc lesion

dissemination in SPACE
- objective clinical evidence 2+ lesions
OR
- 1+ T2 lesion in 2/4 typical locations (periventricular, juxtacortical, infratentorial, SPINAL CORD)

primary progressive
1+ T2 lesions in at least 1 typical area
OR
2+ T2 lesions in cord

optional: positive CSF (oligo clonal bands and/or increased IgG)

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15
Q

** MS tx **

A 
goal: decrease # cycles
primary progressive: none (supportive care)
relapse/remit: any of...
- immunosuppressants
- interferon beta

secondary pregressive: Mitoxantrone

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16
Q

** primary progressive MS tx **

A

none (supportive care)

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17
Q

nota bene interferon beta tx for MS

A

DOES NOT CROSS BBB

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18
Q

** secondary pregressive MS tx **

A

Mitoxantrone

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19
Q

myasthenia gravis s/s

A

PROFOUND fatigue WITH weakness (fluctuating skeletal muscle)

presenting: - ocular (50%)
- diplopia, ptosis- bulbar (15%) - dysarthria, dysphagia, mastication difficulties (speech: phonation, enunciation; esophageal persistalsis impaired, stricture)- Limb weakness (

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20
Q

what is bulbar

A

cranial nerves; head/face/neck in addition to respiratory

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21
Q

what is myasthenia crisis

A

acute respiratory failure usually assoc w/ severe bulbar symptoms

warning sx:↑ bulbar/generalized weakness & muscle fatigue (difficult to tell) ↑ dysphagia, difficulty masticating

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22
Q

warning symptoms of myasthenia crisis

A

↑ bulbar/generalized weakness & muscle fatigue (difficult to tell) ↑ dysphagia, difficulty masticating

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23
Q

** myasthenia crisis tx **

A 
ICU: manage resp failure
note: vent weaning difficult - weigh risk/benefit
rapid tx: plasmapheresis OR IvG
immunosuppressants:
- glucocorticoids like methylpred (short term, follow with...)
- mycophenolate
- azathioprine
- cyclosporin
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24
Q

myasthenia crisis immune suppressant meds

A
  • glucocorticoids like methylpred (short term, follow with…)
  • mycophenolate- azathioprine- cyclosporin
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25
Q

myasthenia dx x4

A
  • ice pack (+ = improved sx)
  • tensilon (+ = no changes in sx)
    serologic:
  • MUSK antibodies
  • AChR
    -ab
    electrophysiologic
  • MG + = ↓ nerve stimulation
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26
Q

** meds that may aggravate myasthenia **

A 
NMB 
**ABX
- aminoglycosides*
- clindamycin
- fluoroquinolones
- vanc
CV
- BB
- procainamide

MISC

  • Mg (supplementation, etc)
  • choloroquine/hydroxychloroquine
  • botulinum
27
Q

gliosis significance

A

hypertrophy/proliferation of varied neural cells - nonfxnal patch, like scar tissue occurs in ALS - replaces the dead nerves

28
Q

ALS s/s

A

limb: spasticity, hyperreflexia, stiffness + movement incoordination, weakness/gait disorder, muscle atrophy/fasciculation
bulbar: jaw stiffness, dysphonia, dysphagia, laryngospasm, sialorrhea (drooling)
axial: neck weakness, bent spine, absent abdominal reflexes, ↑ lumbar lordosis
respiratory: tachypnea, weak voice/speech, weak cough, accessory muscle use ; → resp failure

29
Q

ALS dx

A

only need 1 of the following / suspect = DNA test or electrodiagnostic study

  • electrodiagnostic study
  • genetic testing
  • muscle biopsy: CONFIRMS
  • MRI
  • neuromuscular US
  • labs: creatinine kinase (non-specific neuromuscular dysfxn), hypercalcemia (hyperparathyroidism), HIV/heavy metals (r/o ALS)
30
Q

mainstay of ALS dx + what confirms

A

electrodiagnostic study

muscle biopsy confirms

31
Q

ALS tx

A

tx is symptomatic!
- multidisciplinary care to maintain best QoL- general care: sialorrhea, thick mucus, resp failure, trach/bronch toileting- dysphagia- weakness, spasms, pain, loss of fxn- palliative vs hospice

32
Q

dermatomyositis is…

A

one of the idiopathic inflammatory myopathies

skin manifestations prior to development of symmetric & proximal muscle weakness

33
Q

idiopathic inflammatory myopathy patho/facts

A

gradual progression of weakness (takes weeks to months)
30 - 50, female, AA, usually assoc with other connective disease (scleroderma, SLE, Sjogren)
UNUSUAL TO SEE ALONE

34
Q

polymyositis s/s

A

abrupt presentation
GRADUAL weakness- proximal muscle groups (UE, LE, neck, includes esophagus/dysphagia!)
- LEG precedes arm weakness

35
Q

polymyositis weakness nota bene

A

abrupt presentation
GRADUAL weakness
LEG precedes arm weakness

36
Q

dermatomyositis s/s

A

dusky red rash; malar (mimics SLE)
surrounding facial erythema beyond malar (+ neck, shoulders, upper chest/back “shawl sign”)
heliotrope eruption: periorbital edema + purple suffusion over eyelids
periungal erythema + dilation of nail bed capillaries
erythema over PIP & MCP joints (Gottron sign)
scalp involvement (mimics psoriasis)

37
Q

heliotrope eruption

A

periorbital edema + purple suffusion over eyelids

DERMATOMYOSITIS

38
Q

Gottron sign

A

erythema over PIP & MCP joints DERMATOMYOSITIS

39
Q

dermato & poly common s/s

A

antisynthetase syndrome: inflammatory arthritis
Raynaud phenomenon
“mechanics hands” (lotion doesn’t work ; not gangrenous)
ILD

40
Q

idiopathic inflammatory myopathy dx

A

ONLY SPECIFIC DX TEST: biopsy of affected muscle
MRI - detects early/patchy muscle involvement + guides biopsy
usually normal: CPC, troponin, ESR, CRP)
non-specific: ANA
rheumatoid factor: pos in 50%proteins

wtf like I’m going to remember these- Myositis specific autoantibodies

  • Anti-Jo
  • 1(anti-synthetase)
  • Anti-SRP (signal recognition particle)
  • Anti-Mi-2 (nuclear helicase)
41
Q

only specific dx test for idiopathic inflammatory myopathies

A

biopsy of affected muscle

42
Q

** idiopathic inflammatory myopathy management **

A

methotrexate: drug of choice!corticosteroids (NOT prednisone) 40 - 60 mg daily, lifelong
azathioprine, mycophenolate
last resort: immunoglobulin, rituximab

43
Q

SLE s/s

A

JOINT PAIN (90%)
Raynauds (20%)
arthritis, fever, malaise, anorexia/weight loss

ocular: conjunctivitis, cotton wool spots on retina, photosens

malar rash, discoid rash, oral ulcers, serositis

renal: proteinuria gt 0.5 g/d OR dipstick >3+ ; casts
neuro: seizures, psychosis

heme - hemolytic anemia, leukopenia (lt 4K /mcL), lymphopenia (lt 1500/mcL), thrombocytopenia (lt 100K/mcL)

immunologic disease: positive ANA
pulmonary (pleurisy, Effusions, Bronchopneumonia, Pneumonitis, restrictive lung disease)

44
Q

** major s/s with SLE **x3

A

JOINT PAIN! (90%)
Raynauds (20%)
restrictive lung disease

45
Q

SLE most often used dx tests

A

ANA (98%) - non-specificanti-dsDNA (70%) - fluctutates w disease activity/glomerulonephritis

46
Q

SLE dx tests (all)

A

ANA (98%) anti-dsDNA (70%)Antihistone (70%) - more r/t drug inducedAntierythrocyte (60%) - erythrocyte basement membranes; some hemolysisAntiphospholipid (50%)Lupus anticoagulant/LA (7%)CBC: anemia, leukopenia, thrombocytopeniaESR,CRP (nonspecific), UA (proteinuria, casts, hematuria), CrGFR (lupus nephritis), C3/C4 (decreased levels = active lupus)

47
Q

** SLE main rx tx ** x2

A

Hydroxychloroquine 200 to 400 mg/d

Chloroquine

48
Q

SLE all rx tx

A 
MAIN
Hydroxychloroquine 200 to 400 mg/d
Chloroquine
ADDITIONAL
- corticosteroids (burst therapy)
- immunosuppressants: mycophenolate, azathioprine, csa
- antineoplastic: cyclophosphamide
- B-cell 
MABS: -umab
49
Q

SLE mgmt

A
  • Hydroxychloroquine 200 to 400 mg/d
  • Chloroquine
    supportive care
  • lifelong anticoag if antiphospholipid syndrome
  • acetaminophen (fever)
  • COX2 inhib (chronic muscle pain - AVOID OPIOIDS)
  • allogenic mesenchyma SC
    T- vaccinations- limit tobacco- cancer screenings
50
Q

granulomatosis w/ polyangitis mortality

A

fatal less than 1 year if not treated

51
Q

granulomatosis w/ polyangitis s/s

A

sinusitis (most common)
- saddle deformity- necrotizing granulomatous lesions in U(90%)/L(60%) resp tract - vasculitis of SMALL - spontaneous VTE- new HTN- otitis media- polyarteritis nodosa- glomerulonephritis (75%; can progress to CKD in weeks)- tissue bx- chest CT

52
Q

granulomatosis w/ polyangitis: see this and progress to what

A

glomerulonephritis (75% can progress to CKD in weeks)

53
Q

** granulomatosis w/ polyangitis: test of choice **

A

ANCA (anti-neutrophil cytoplasmic autoantibodies)- against proteinaise-3 and MPO-ANCA- high sensitivity- positive or negative, doesn’t correlate with severity

54
Q

granulomatosis w/ polyangitis: mgmt

A

prednisone 1 mg/kg/d - for flares! long term possible but remember: SE!!!
Cyclophosphamide 2 mg/kg/d- good longterm, can pair w pred for acute- malignancy riskAzathioprine up to 2 mg/kg/dMethotrexate 20-25 mg/wkRituximab

55
Q

granulomatosis w polyangitis VS polyarteritis nodosa

A

gwp: SMALL vessels
pn: MEDIUM vessels

56
Q

polyarteritis nodosa

A

INSIDIOUS ONSET
PAIN PROMINENT early
- pain: bigger vessels, arthralgia, myalgia, neuropathy- fever, malaise, wt loss, develop for wks- mononeuritis multiplex– foot drop ← abnormal, not disuse syndrome; progressive gait issues Skin: Livedo reticularis, subcutaneous nodules, LE ulcerationsAbdominal pain: N&V, acalculous cholecystitis , appendicitis, perforation, micro-aneurysm ruptureSubclinical cardiac

57
Q

polyarteritis nodosa: dx

A

tissue biopsy (70% sensitivity)

58
Q

polyarteritis nodosa: tx

A

prednisone - up to 60 mg/dpulse methylprednisolone - 1 gm/d/3daysimmunosuppressants: cyclophosphamide (cytoxan)

59
Q

hereditary angioedema usually triggered by

A

illness, stress

60
Q

foot drop associate with which autoimmune

A

polyarteritis nodosa

61
Q

hereditary angioedema: dx

A
  • C1
  • C1 esterase inhibitor low or non-fxnal
  • C2 level (correlates with severity of disease)
  • normal C4 activity rules out HAE
  • CT scan
62
Q

hereditary angioedema: mgmt

A

antihistamines DO NOT WORK- pain meds & fluids
- C1 esterase inhibitor concentrate (derived from human plasma)- FFP - contains C1 inhibitor (not as good as concentrated C1 esterase inhibitor)- Ecallantide - ⊣ plasma kallikrein (consult!!!)- icatibant - bradykinin B2 receptor inhibitor (Europe)- androgens - danazol: ↑ production C1 inhibitor

63
Q

rules out hereditary angioedema

A

normal C4 activity

monster management (29 decks)

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