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autoimmune 2 Flashcards

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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
myasthenia dx x4
- ice pack (+ = improved sx) - tensilon (+ = no changes in sx) serologic: - MUSK antibodies - AChR -ab electrophysiologic - MG + = ↓ nerve stimulation
26
** meds that may aggravate myasthenia **
``` NMB **ABX - aminoglycosides* - clindamycin - fluoroquinolones - vanc CV - BB - procainamide ``` MISC - Mg (supplementation, etc) - choloroquine/hydroxychloroquine - botulinum
27
gliosis significance
hypertrophy/proliferation of varied neural cells - nonfxnal patch, like scar tissue occurs in ALS - replaces the dead nerves
28
ALS s/s
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
ALS dx
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
mainstay of ALS dx + what confirms
electrodiagnostic study | muscle biopsy confirms
31
ALS tx
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
dermatomyositis is...
one of the idiopathic inflammatory myopathies | skin manifestations prior to development of symmetric & proximal muscle weakness
33
idiopathic inflammatory myopathy patho/facts
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
polymyositis s/s
abrupt presentation GRADUAL weakness- proximal muscle groups (UE, LE, neck, includes esophagus/dysphagia!) - LEG precedes arm weakness
35
polymyositis weakness nota bene
abrupt presentation GRADUAL weakness LEG precedes arm weakness
36
dermatomyositis s/s
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
heliotrope eruption
periorbital edema + purple suffusion over eyelids | DERMATOMYOSITIS
38
Gottron sign
erythema over PIP & MCP joints DERMATOMYOSITIS
39
dermato & poly common s/s
antisynthetase syndrome: inflammatory arthritis Raynaud phenomenon “mechanics hands” (lotion doesn’t work ; not gangrenous) ILD
40
idiopathic inflammatory myopathy dx
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
only specific dx test for idiopathic inflammatory myopathies
biopsy of affected muscle
42
** idiopathic inflammatory myopathy management **
methotrexate: drug of choice!corticosteroids (NOT prednisone) 40 - 60 mg daily, lifelong azathioprine, mycophenolate last resort: immunoglobulin, rituximab
43
SLE s/s
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
** major s/s with SLE **x3
JOINT PAIN! (90%) Raynauds (20%) restrictive lung disease
45
SLE most often used dx tests
ANA (98%) - non-specificanti-dsDNA (70%) - fluctutates w disease activity/glomerulonephritis
46
SLE dx tests (all)
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
** SLE main rx tx ** x2
Hydroxychloroquine 200 to 400 mg/d | Chloroquine
48
SLE all rx tx
``` MAIN Hydroxychloroquine 200 to 400 mg/d Chloroquine ADDITIONAL - corticosteroids (burst therapy) - immunosuppressants: mycophenolate, azathioprine, csa - antineoplastic: cyclophosphamide - B-cell MABS: -umab ```
49
SLE mgmt
- 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
granulomatosis w/ polyangitis mortality
fatal less than 1 year if not treated
51
granulomatosis w/ polyangitis s/s
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
granulomatosis w/ polyangitis: see this and progress to what
glomerulonephritis (75% can progress to CKD in weeks)
53
** granulomatosis w/ polyangitis: test of choice **
ANCA (anti-neutrophil cytoplasmic autoantibodies)- against proteinaise-3 and MPO-ANCA- high sensitivity- positive or negative, doesn't correlate with severity
54
granulomatosis w/ polyangitis: mgmt
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
granulomatosis w polyangitis VS polyarteritis nodosa
gwp: SMALL vessels pn: MEDIUM vessels
56
polyarteritis nodosa
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
polyarteritis nodosa: dx
tissue biopsy (70% sensitivity)
58
polyarteritis nodosa: tx
prednisone - up to 60 mg/dpulse methylprednisolone - 1 gm/d/3daysimmunosuppressants: cyclophosphamide (cytoxan)
59
hereditary angioedema usually triggered by
illness, stress
60
foot drop associate with which autoimmune
polyarteritis nodosa
61
hereditary angioedema: dx
- C1 - C1 esterase inhibitor low or non-fxnal - C2 level (correlates with severity of disease) - normal C4 activity rules out HAE - CT scan
62
hereditary angioedema: mgmt
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
rules out hereditary angioedema
normal C4 activity

monster management (29 decks)

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