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Summer FDTN Exam 4 > Rheum 2 > Flashcards

Rheum 2 Flashcards

1
Q

What is SLE

A

AI disorder affecting multiple organs that has relapses and remissions
MC in young women and black women
*Put this on your DDx for anyone w/ multisystem disease and +ANA

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2
Q

What is the pathophys of SLE

A

chronic inflammation to relatively every organ
Autoantibodies to nuclear antigens
Sx 2/2 trapping antigen-antibody complexes in capillaries OR antibody destruction or host cells (platelets)

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3
Q

RF for SLE are

A

15-40 y/o
Women
Black
Genetic predisposition (HLA-DR2/3, +ANA, other rheumatic diseases)

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4
Q

How does SLE present clinically

A 
Relapsing remitting pattern 
**Fatigue! Fever, anorexia, weight loss, malaise, skin lesions  
*Arthralgias, myalgias, pleural effusion
vascular manifestation (Raynaud's, VTE) 
Pericarditis 
Seizure, psychosis 
conjunctivitis, photophobia
anemia, leukopenia, LAD 
Lupus nephritis (hematuria, proteinuria)
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5
Q

What skin lesions are associated with SLE

A 
*Butterfly rash (malar distribution)*
Photosensitive 
Small vessel vasculitis (purpura, petechiae, splinter hemorrhage, etc.)
Discoid plaques 
Periungal erythema 
Nail fold infarcts 
Alopecia
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6
Q

How do you diagnose lupus

A

Presence of Sx (H&P) + Labs

-need 4 or more criteria

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7
Q

You need 4 of the following 11 criteria to diagnose SLE

A

Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
Kidney disease (>.5g proteinuria, casts, or 3+ dipstick proteinuria)
Neurologic disease (Sz or psychosis)
Hematologic disease (hemolytic anemia, leukopenia <4, Lymphopenia <1500, thrombo <100)
Immunologic abnormality (Abs to native DNA/Sm/APA)
Positive ANA

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8
Q

What labs are used in diagnosing SLE

A 
\+ANA
Reflex to antibodies- **Anti-dsDNA**, Anti-Sm*, Anti-ro/la/U1 
Diminished serum compliment 
Anemia, leukopenia, thrombocytopenia
BUN, Cr, UA
ESR, CRP
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9
Q

MC lab abnormalities in SLE are

A

Anemia, Hypocomplementemia, ANA, Anti-native DNA

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10
Q

How do you Tx SLE

A 
Sun protection, exercise 
NSAIDs 
Antimalarials (hydroxychloroquin)
Corticosteroids (topical for skin, PO for flares) 
Methotrexate (arthritis) 
Immunosuppressives 
Resistant: Belimumab
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11
Q

What should you monitor SLE

A

Atherosclerosis, pulmonary HTN, antiphospholipid syndrome, Osteoporosis/osteopenia

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12
Q

What provokes flares in SLE many times

A

Sulfa drugs!

A lot of people with SLE are allergic to them

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13
Q

Most M&M in SLE is 2/2

A

Glomerulonephritis
CNS disease
Antiphospholipid antibodies

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14
Q

Variants of SLE are

A

Acute Cutaneous LE
Subacute Cutaneous LE
Chronic Cutaneous LE: Discoid LE**
Drug induced lupus

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15
Q

Sx of ACLE are

A

Facial eruption
Generalized eruption
TEN

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16
Q

Sx of SCLE are

A

Small erythematous, scaly papules evolving into psoriasis plaques
Over shoulders, forearms, neck, upper torso
Photosensitivity
+ ANA
Arthralgias
Oral ulcers
Drug induced lupus

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17
Q

How does DLE present

A

Erythematous, indurated plaques covered with scale
Inflammation and scarring lesions
Over face, neck, scalp, ears
20% develop SLE

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18
Q

What drugs trigger Drug induced Lupus

A 
Procaine 
Hydralazine 
Minocycline 
Diltiazem 
Penicillamine, Isoniazid, Quinidine, Methyldopa, Chlorpromazine, Practolol
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19
Q

How does Drug induced Lupus present

A 
Fever
malaise
myalgias
arthralgias
ANA and Anti-histone + 
-Rare: cutaneous manifestation, renal or neuro involvement. Anti-DNA/SM are negative
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20
Q

How is pregnancy affected by SLE

A

High rate Spontaneous abortion, pre-term birth, IUGR
Passive autoimmunity )anti-SSA/SSB cross placenta
Neonatal lupus: Congenital heart block, cutaneous involvement that resolves in 6 mo.

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21
Q

What is antiphospholipid antibody syndrome

A

Autoimmune hypercoagulability disorder
Recurrent thromboses (venous or arterial)
Leads to pregnancy complications (miscarriage)

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22
Q

What are the types of APS

A

Primary: absence of other disease
Secondary: 2/2 other AI diseases (SLE)

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23
Q

How does APS usually present

A

Asx until:

Recurrent pregnancy loss, SVT, pulmonary embolism, CVA, Budd-chiari syndrome, Cerebral vein thrombosis, MI

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24
Q

What in “recurrent pregnancy loss” that may present in those with APS

A

Unexplained loss after first trimester
1+ premature births (<34) 2/2 Eclampsia or Pre-E
3+ unexplained fetal death in first trimester

25
Q

How do you diagnose APS

A

1 clinical event + Positive Ab blood test

26
Q

Positive APS antibody blood tests are

A

Anti-cardiolipin antibodies (false + syphilis test)
Antibody to beta-2-glycoprotein
Lupus anticoagulant antibody

27
Q

How do you treat APS

A

Anticoagulation for life (Warfarin) to maintain INR 2-3
If pregnant: SubQ heparin and low dose aspirin
-Other: IV heparin, high dose corticosteroids, IVIG, plasmapheresis

28
Q

What is Raynaud phenomenon

A

Syndrome of sudden onset digital ischemia
Initial phase: Excess vasoconstriction
Recovery phase: Vasodilation

29
Q

What triggers raynaud’s phenomenon

A

Emotional stress, or cold temp!

Relieved by warmth

30
Q

What are the types of Raynaud’s phenomenon

A

Primary (R disease): idiopathic, benign, symmetric, young women
Secondary (R syndrome): scleroderma, ulceration, gangrene, nail fold capillary changes
Primary MC than Secondary (D>S)

31
Q

Causes of Raynaud’s (just the categories bc there are SO many damn causes)

A 
Rheumatic diseases 
Neurovascular compression
Occupational (vibration injury)
Hematologic disorder 
Meda 
Endocrine disorder 
Miscellaneous
32
Q

Clinical presentation of Raynaud’s is

A

Initial: well demarcated digital pallor or cyanosis (numb, stiff)
Recovery: intense hyperemia, rubor, throbbing, paresthesia, pain
Affects fingers**, toes, nose, ears
ASx between attacks!!!!

33
Q

DDx for Raynaud’s is

A

Scleroderma (also Sx in secondary R)
SLE
Buerger disease

34
Q

How do you treat Raynaud’s (non-pharm)

A 
Wear gloves, mittens, or socks 
Keep body warm 
Moisturize skin 
Stop smoking 
Avoid sympathomimetic drugs (decongestants, diet pills, amphetamines)
35
Q

How do you treat Raynaud’s (pharm)

A

1** CCB- Nifedipine, Amlodipine, Felodipine
PDE-5 inhibitor (sildenafil), topical nitroglycerin, Prazosin, Losartan, prostaglandin
Surgery: Sympathectomy when Sx severe and med therapy fails. Good for primary, sot secondary!

36
Q

What is scleroderma (systemic sclerosis

A

Diffuse fibrosis of skin and internal organs 2/2 Unknown etiology
W>M, 20-40 y/o peak

37
Q

What is the pathophys of Scleroderma

A

Deposition of collagen in skin, kidney, heart, lungs, stomach causing fibrosis of skin and organs

38
Q

What are types of scleroderma

A

Diffuse: hands, face, trunk, proximal extremities develop skin changes- Kidney, interstitial lung, and cardiac Dz
Limited*MC: Face and hands, pulmonary HTN late in disease (CREST syndrome)

39
Q

How does Scleroderma present

A

Skin changes
Polyarthralgias
Esophageal dysfunction
Hand and finger swelling, +/- spread to trunk and face
Localized scleroderma (morphea, linear rash)

40
Q

What is CREST syndrome (limited scleroderma)

A 
Calcinosis cutis 
Raynaud phenomenon 
Esophageal dysfunction 
Sclerodactyly
Telangectasia
41
Q

How do you diagnose Scleroderma

A

+ANA
+anti-centromere antibody in CREST
+anti-SCL70 in diffuse (worse outcome)

42
Q

How do you treat scleroderms

A

No cure!
Monitor for HTN (indicates kidney involvement)
Organ specific Tx: PPI (reflux), ACE-I (renal), CCB (raynaud’s), Immunosuppressive (PHTN)

43
Q

What is the prognosis of Scleroderma

A

Poor, 10 yea survival is 40-60

But, better if CREST syndrome (limited)

44
Q

What is polymyositis and dermatomyositis

A

Inflammation of striated muscle, proximal muscle weakness, dermatomyositis
W>M, 40-50 (if >50 think paraneopalstic)
Lowest in Japanese, highest in African american

45
Q

Etiology of P&D is

A

Unknown!

Possible 2/2 muscle inflammation, and associated with occult malignancy (ovary, lung, pancreas)

46
Q

How does P&D present

A

Painless muscle weakness around neck, shoulders, hips
Trouble climbing stairs, or standing from seated
SOB
Cough
Dysphagia (choke while eating, aspiration)
Polyarthralgias
Muscle atrophy

47
Q

What is one way to differentiate P&D form SCD

A

SCD: Choking as in difficulty getting bolus down 2/2 smooth muscle affected
P&D: Striated muscle so difficulty initial swallowing

48
Q

Skin rash with dermatomyositis is

A

Malar
Heliotrope (peri-ocular erythema)
Gottron’s papules
Shawl sign (over sun exposed area)

49
Q

How do you diagnose P&D

A 
Elevated CK and aldolase (muscle enzymes) 
Electromyogram 
Muscle biopsy (definitve!)
50
Q

Diagnostic criteria is

A 
Proximal muscle weakness
Elevated muscle enzymes 
Muscle biopsy evidence 
EMG changes 
Skin rash
51
Q

How do you treat P&D

A

High dose steroids

Immunosuppressives/DMARDS (methotrexate, azathioprine)

52
Q

What is Sjogren Syndrome

A

AI disorder associated with other connective tissue d/o
F>M, 40-60
Also called Sicca or Dry eye syndrome

53
Q

What is the pathophys of Sjogrens

A

Autoantibodies destroy salivary and lacrimal glands= decreased saliva and tear production

54
Q

How does Sjogrens present

A

Xerostomia
Dry eyes
Enlarged parotids
Systemic: dysphagia, pleuritis, small vessel vasculitis, obstructive airway Dz, neuropsych dysfunction, pancreatitis, RTA, chronic interstitial nephritis

55
Q

How do you diagnose Sjogrens

A

+RF
+ANA
*+ anti-LA antibodies (antiSSB)
*+anti-RO antibodies (antiSSA)
*Lip biopsy (definitive, shows gland fibrosis)
+Shirmer test (eval tear secretion- pos if <5mm of filter paper is wet after 5 min)

56
Q

Criteria for Sjogrens is

A

Ocular Sx (at least 1)- dry eyes >3 mo, FB sensation, artificial tears >3xday
Oral Sx (at least 1)-dry mouth >3mo, recurrent swollen salivary glands, need liquid to swallow dry food
Ocular sign (at least 1)- schirmer test, positive vital dye stain
Oral sign (at least 1)- Schirmer test, positive vital dye stain
Positive lip biopsy
Positive anti-SSA/SSB (Ro/La)

57
Q

How do you treat Sjogrens

A

Sx Tx!
Avoid anticholinergics and antihistamines
Keep mucosa moist
Pilocarpine (increase salivary flow- muscarinic agonist, secretagogues)
Cyclosporine (restasis eye drops)

58
Q

What is Rose Bengal stain

A

Vital dye stain for oral region

59
Q

What is van Bijsterveld

A

Vital dye stain for Ocular

Summer FDTN Exam 4 (14 decks)

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