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Summer FDTN Exam 4 > Rheum 2 > Flashcards

Rheum 2 Flashcards

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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

Study These Flashcards
A

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

25
How do you diagnose APS
1 clinical event + Positive Ab blood test
26
Positive APS antibody blood tests are
Anti-cardiolipin antibodies (false + syphilis test) Antibody to beta-2-glycoprotein Lupus anticoagulant antibody
27
How do you treat APS
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
What is Raynaud phenomenon
Syndrome of sudden onset digital ischemia Initial phase: Excess vasoconstriction Recovery phase: Vasodilation
29
What triggers raynaud's phenomenon
Emotional stress, or cold temp! | Relieved by warmth
30
What are the types of Raynaud's phenomenon
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
Causes of Raynaud's (just the categories bc there are SO many damn causes)
``` Rheumatic diseases Neurovascular compression Occupational (vibration injury) Hematologic disorder Meda Endocrine disorder Miscellaneous ```
32
Clinical presentation of Raynaud's is
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
DDx for Raynaud's is
Scleroderma (also Sx in secondary R) SLE Buerger disease
34
How do you treat Raynaud's (non-pharm)
``` Wear gloves, mittens, or socks Keep body warm Moisturize skin Stop smoking Avoid sympathomimetic drugs (decongestants, diet pills, amphetamines) ```
35
How do you treat Raynaud's (pharm)
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
What is scleroderma (systemic sclerosis
Diffuse fibrosis of skin and internal organs 2/2 Unknown etiology W>M, 20-40 y/o peak
37
What is the pathophys of Scleroderma
Deposition of collagen in skin, kidney, heart, lungs, stomach causing fibrosis of skin and organs
38
What are types of scleroderma
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
How does Scleroderma present
Skin changes Polyarthralgias Esophageal dysfunction Hand and finger swelling, +/- spread to trunk and face Localized scleroderma (morphea, linear rash)
40
What is CREST syndrome (limited scleroderma)
``` Calcinosis cutis Raynaud phenomenon Esophageal dysfunction Sclerodactyly Telangectasia ```
41
How do you diagnose Scleroderma
+ANA +anti-centromere antibody in CREST +anti-SCL70 in diffuse (worse outcome)
42
How do you treat scleroderms
No cure! Monitor for HTN (indicates kidney involvement) Organ specific Tx: PPI (reflux), ACE-I (renal), CCB (raynaud's), Immunosuppressive (PHTN)
43
What is the prognosis of Scleroderma
Poor, 10 yea survival is 40-60 | But, better if CREST syndrome (limited)
44
What is polymyositis and dermatomyositis
Inflammation of striated muscle, proximal muscle weakness, dermatomyositis W>M, 40-50 (if >50 think paraneopalstic) Lowest in Japanese, highest in African american
45
Etiology of P&D is
Unknown! | Possible 2/2 muscle inflammation, and associated with occult malignancy (ovary, lung, pancreas)
46
How does P&D present
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
What is one way to differentiate P&D form SCD
SCD: Choking as in difficulty getting bolus down 2/2 smooth muscle affected P&D: Striated muscle so difficulty initial swallowing
48
Skin rash with dermatomyositis is
Malar Heliotrope (peri-ocular erythema) Gottron's papules Shawl sign (over sun exposed area)
49
How do you diagnose P&D
``` Elevated CK and aldolase (muscle enzymes) Electromyogram Muscle biopsy (definitve!) ```
50
Diagnostic criteria is
``` Proximal muscle weakness Elevated muscle enzymes Muscle biopsy evidence EMG changes Skin rash ```
51
How do you treat P&D
High dose steroids | Immunosuppressives/DMARDS (methotrexate, azathioprine)
52
What is Sjogren Syndrome
AI disorder associated with other connective tissue d/o F>M, 40-60 Also called Sicca or Dry eye syndrome
53
What is the pathophys of Sjogrens
Autoantibodies destroy salivary and lacrimal glands= decreased saliva and tear production
54
How does Sjogrens present
Xerostomia Dry eyes Enlarged parotids Systemic: dysphagia, pleuritis, small vessel vasculitis, obstructive airway Dz, neuropsych dysfunction, pancreatitis, RTA, chronic interstitial nephritis
55
How do you diagnose Sjogrens
+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
Criteria for Sjogrens is
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
How do you treat Sjogrens
Sx Tx! Avoid anticholinergics and antihistamines Keep mucosa moist Pilocarpine (increase salivary flow- muscarinic agonist, secretagogues) Cyclosporine (restasis eye drops)
58
What is Rose Bengal stain
Vital dye stain for oral region
59
What is van Bijsterveld
Vital dye stain for Ocular

Summer FDTN Exam 4 (14 decks)

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