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Med 1 > Connective Tissue/Jt Disease > Flashcards

Connective Tissue/Jt Disease Flashcards

1
Q

Types of SLE (4)

A

spontaneous
Discoid lupus - skin lesionsw/out systemic
drug induced
ANA negative lupus(arthritis, raynuds, Ro antiSS +, risk neonatal lupus in infants)

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2
Q

SLE epidemiology

A

Women of childbrearing age
African Americans

appears late childhoors

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3
Q

Clinical Features(11)

need 4/11

A

Serositis-pleursy, pericarditis
Oral Ulcers -nasopharyngeal/painless
Arthritis
Photosensitivity

Blood disorders -leukopenia, thrombopenia
Renal involvement- proteinuria(nephrotic), casts
ANA
Immunity phenom- anti smith, anti dsDNA, anti phos
Neurologic - seizure/psychosis

Malar rash
Doscoid Rash

fatigue, malaise, fever weight loss

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4
Q

Libman sacks endocarditis

A

SLE associated complication

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5
Q

Antihistone ABs present in

A

Drug induced SLE

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6
Q

ANA elv in?(7)

A

SLE, RA, Scleroderma, Sjogrens, mixed connective, polyomyositis, drug induced lupes

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7
Q

Anti Ro(SS-A) and La(SS-B)

Found in(5)

A

Sjogrens
Sub cutaneous SLE
Neonatal lupes (w/ congenital heartblock)

Complement deficiency (C2+C4)
ANA negative lupus
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8
Q

Reumatoid factor (2)

A

70% of RA

healthy 3%

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9
Q

C- anca

A

Wegeners

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10
Q

P-anca

A

polyarthritis nodosa

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11
Q

ESR elv(4)

A

infection
malignancy
rheumatologic
miscellaneous (pregnancy, necrosis)

r/o inflam and monitor course of inflamm

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12
Q

C reactive protein (6)

A 
inflame states/infection
MI
vaculitis
trauma
malignancy
pancreatitis

used for infection, more sense and specific than ESR.

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13
Q

HLA DR2 and DR3(3)

A

SLE both

DR3 - sjogrens
DR4 - RA

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14
Q

HLA B27 (3)

A

ankylosinf spondylitis
Reiters
psoriatic arthrits

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15
Q

Tx for SLE(4)

A

steriods for flares
NSAIDs for less severe

long term - hydroxychloroquine for constitutional symptoms, eye exam for retinal toxicity

cyclophosphamide - glomerulonephritis

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16
Q

Scleroderma

A

high quantity of collagen deposited -> complications and fibrosis

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17
Q

What is NOT part of drug SLE

A

Renal and CNS involvement

also usually not butterfly, alopecia and ulcers

Anti hitone antibodies

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18
Q

Drugs -> lupus (6)

A 
Hydralazine*
Procainamide *
Isoniazaid*
Chlopromazine*
mthyldopa
quinidine

See anti histone antibodies

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19
Q

Anticentomere

A

CREST 80%

sclreoderma 30%

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20
Q

antiscleroderma 70

A

Scleroderma 20%

Crest 10%

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21
Q

most common finding in SLE

A 
Lupus glomerulonephritis - 5 types
minimal lesion,
 mesangial lupus, 
focal proliferation,
 diffuse proliferative (40% and bad), 
membranous
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22
Q

Scleroderma types (2)

A

women 35-50

Diffuse - ANA and antitopoisomerase(anti-Scl 70), widespread skin, rapid onset, visceral involvement early, POOR prognosis–peropheral edema, polyarthritis, fatigue, weak, Renal failure?, interstial lung disease

Limited - anticentromere w/ CREST, limited to disatl extremities and face, delayedonst, pulm HTN and ischemic vascular disease, better prognisis

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23
Q

CREST syndrome

A

Limited scleroderma anticentormere positive

Calcinosis of digits
Raynauds
Esophageal dysmotility
Scerodactyl of finfgers
Telangiectasis - digits and nails
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24
Q

Ddx of Reynauds(7)

A 
primary
Scleroderma
SLE
Mixed connective disease
vasculitis - bergers
Medication - beta blockers, nicotine
thromoboangitis obliterans
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25
Features of sclerodema general (6)
Raynauds Cutaneous fibrosis - tight skin of face, extremities (sclerodactyl) GI- both diffuse, limited - dysphagia, reflux Pulm involvment- cause death Cardiac - pericardial effusion, -> CHF, arrythmia Renal Crisis - rare today
26
antiphospholipid antibody Syndrome Find?
Hypercoagulable state - idiopathic, SLE to other collagen disease recurrent venous thrombosis- PE risk Arterial thrombosis, fetal loss recurrent, thrombocytopenia, Prolonged PTT of PT not correctable w/ plasma addition Tx - Warfarin
27
Antitopoisomerase
Specific for Diffuse Scleroderma antiscleroderma 70
28
Tx of scleroderm
no cure Tx symptoms - NSAIDs (mkstl pain), H2/PPIs Tx pulm and renal complications
29
Sjogrens Syndrome 2 types
Autoimmune in women Multiorgan disease -> skin, lungs, thyroid, vessels, liver Primary - dry mouth/eyes w/ lymphocytic infiltration of minor salivary glands Secondary - Dry eyes/mouth + RA/scleroderma/SLE.polysyositis
30
Sjogrens has increase risk of death 2/2
nonhodgkins lymphoma
31
Dx of Sjogrens
Dry eyes (burning, red, blurred) out, arthralgia, extra glandular arthritis, nephritis, vasculitis ANA, Anti Ro and La Normocytic, normochromic anemia Schirmer test - tilter paper -> lacrimal output
32
Pregnant people w/ antibodies to ro (SS-A) have increased risk of
child w/ neonatal SLE
33
Tx sjogrens
Pilocarpine /ceimeline Artifical tears NSAIDs, steroids
34
Mixed connective tissue disease
overlap syndrome Features of SLE, RA, scleroderma, polymyosistis Fins: pulm invovelment, esophageal dysmotility, polyarthritis, sclerodactyl cutaneous manifestations, myopathy, Raynauds ANTi U1-RNP High ANA + RF?
35
Arthritis is LESS likely RA if (3)
joints not symmetric DIP is involved( can be every other jt) Constitutional symptoms absent (especially in morning)
36
most common jts for RA
**Hands - PIP and MCP and wrist See ulnar deviation
37
Boulonniere deformaties Swan neck contractures
- PIP flexed and DIP extended | - MCP flexed and PIP extended and DIP dlexed
38
Extra-articular manifestations of RA 2 main, a lot of others
malaise, weight loss, fever * rheumatoid nodules (elbow, sacrum, occiput) * Pleural effusion- has low glucose Cardiac - nodules, pericarditis (40%), effusion eyes- scleritis, scleromalacia, Nervous - infarction of nerve trunk, systemic vasculutus- can't move arm Feltys syndrome Blood - anemia, thrombocytosis Cascultis
39
feltys triad
RA, neutropenia, splenomegaly anemia, thrombocytopenia, LAD Late in disease process
40
Osteophytes found in
osteoarthritis changes in jts w/ RA usually more extensive w synovial involvement
41
Dx of RA 2 factors
high titres fo RF -> more severe disease but also nonspecific Anticitullinated peptide/protein antibodies (ACPA) more sensitive Elv ESR, C reactive protein Anemia sympotms >6wks Radiograph: periarticular erosion, osteopenia, narrow jt space seen late
42
Tx of RA
NSAIDs - pain control Corticosteroids low dose if not manages DMARDs early in Dx, have slow onset of action: Methotrexate*(SFx GI, oral ulcers, alopecia, hepatocellular injury), Leflunamide Hydroxychloraquine - (eye exam for retinal risk) Sulfasalzine Antitumor necrosis factor - etanercept, infliximab
43
Variants of RA (2)
Feltys syndrome - anemia, neutropenia, splenomegaly, RA Juvenile RA - befroe age 18, estra articular manifestations predom (STILLS disease_ or arthritis predom
44
Increase uric acid production in (3) Decrease excretion
hypoxanthine-guanine phosphoribosyltranserase deficiency - Lesch Nyhan syndrome Phophoribysl pyrophosate synthetase overactivity increases cel turnover Renal disease NSAIDs, Diuretics Acidosis
45
Asymptomatic hyperuricema Tx
should NOT be - 95% % remain asymptomatic
46
Acute gouty attack precipitates (5)
``` dehydration decrease in temp stress - emotional.physical alcoholic use starvation ```
47
Synovial fluid anaysis in presumed gout attack
gram stain and culture to r.o septic arthritis
48
Dx of gout Role of serum Uric Acid
Needle shaped and negative biofringence rate crystals Uric acid levels NOT helpful in Dx and can be normal Radiographs -> punched out erosions
49
Complications of gout (2)
nephrolithiasis - < 1%/yr Degenrative arthritis in <15% pts
50
Meds that increase uric acid levels other causes?
thizide and loop diuretics obesity Alcohol Dietary purine - red meat/seafood
51
Acute gout Tx? (3)
Bed rest NSAIDS - indomethacin - not ASA( aggravates) or tylenol (no anti inflame) Colchicine - alt for NSAIDs - N/V, abdominal cramps, diarrhea, NOT for use w/ renal insufficiency and cytosine Corticosteriods (oral prednisone 7-10 days), maybe intraarticular injection
52
Prophylactic therapy for gout
wait till 2-3 acute gouty attacks ~/yr allopurinol - xanthine oxidase inhibitor, decreases production, NEVER acutely. SFx rash/SJ syndrome probenacid/ sulfinpyrazone - increase renal excretion (need working kidneys)
53
Conditions -> increase Ca crystal deposition
hemochromatosis, hyperparathyroidism, hypothyroidsim, Bartter's syndrome Age and w/OA of joints
54
Presentation of pseudo gout Dx?
Commonlu affects knees and wrists usually monoarticular, but can be poly weakly positively birefringent rod/rhomboid crystals Radiograph - chondrocalcinosis
55
Tx of pseudogout
Tx underlying disorder, NSAIDs, cochicine, intra-articular steriodinjections Total joint replacement if appropriate
56
Polymyosistis diagnostic criteria(4)
2/4 probable w/ increasing likelihood Symmetric proximal muscle weakness elvation in serum creatine phosphokinase EMG finding of myopathy Biopsy evident myositis Rash = dermatomyositis Usually females
57
Inclusion body myositis -
oddball | M>F, no autoantibodies, distal muscles, and low CK
58
Causes of dermatomyositis and polymyositis
genetically susceptible + env trigger -> immune activation Dermato - hummoral immune mech Poly - cell mediated
59
dermatomyositis features increased risk of?
symmetrical proximal muscle weakness over weeks-months (dysphagia 30%) Rash - heliotrope (eyes, bridge of nose, cheeks) Gottrons papules - papular erythematous scaly lesions over knuckles V sign - rah on face, neck anterior chest Shawl sign - rash on shoulders, upper back, elbows, knees periungunal erythema Risk of - vasculitis - GI, kidneys, lungs, eyes - CA, dermatomyocitis remits once CA removed
60
Elv CK,** LDH, Aldolase, AST and ALT elv anti Jo 1 Antibodies(antisynthetase) or anti Mi2 antibodies
Dermatomyocitis polymyocitis
61
Tx of dermatomyositis and polymyositis
Corticosteriods immunosuppression if not responsive - methotrexate, cyclophosphamide, chlorambucil PT
62
Polymyalgia Rheumatica epidemiology association w/ what disease?
elderly - rare M autoimmune cause, self limited 1-2 yrs 10%-> temporal arteritis
63
Features of Polymyalgia Rheumatica
Hip and shoulder pain - bilateral; pain w/ movement and normal strength Morning stiffness common Malaise, fever, depression Jt swelling , 20% knees wriss, hands
64
Dx of polymyalgia rheumatica Tx?
clinical diagnosis ESR elv >50-100 corticosteroids w/ response 1-7 days - stop w/in 2 yrs for most,can be longer
65
Fibromyalgia Epi and Key Dx points
Adult women w/ chronic non progressive waxing and waning pain KEY - maple trigger points, tender to palpaion, symmetrical (18)' Unknown etiology
66
Symptoms of fibromyalgia
Stiffness**, body aches changes w/ weather, sleep, stress, cold, worse in morning Sleep dysruptions/unrefreshing Anxiety/depression
67
Dx of fibromyalgia
Pain (including axial) for min 3 months 11/18 tender pt sites r/o: myofascial syndrome, rheumatoid, polymyalgia rheumatica, ankylosing spondylitis, spondyloarthropathy, chronic fatigue syndrome, lymes, hypothyroid, polymyosistis
68
Tx of fibromyalgia
Reassure - > active and productive Rx don't work - SSRIs, TCAs? cognitive behavioral therapy
69
Seronegative spondyloarthropathies Types(4) and associations
Associated w/ HLA B27 M>W Ankylosing spondyltitis reactive arthritis/reiters Psoriatic arthrits Arthropathy of IBD
70
Common features of seronegative spondyloarthropathies (7)
negative RF HLA B27 assocaition Oligoarthritis -asymmetrical enthesitis - inflam insertion of tendon onto bone Inflam arthritis (axial/sacroiliac) extra-articular features (eye -anterior uveitis, cardiac, renal) Familial deposition(IBD, ankylosing spondylitis, psoriasis)
71
characteristic of ankylosing spondylitis
low back pain worse in the morning and better w. use and warm showers
72
Complications of ankylosing spondylitis(5)
``` restrictive lung disease cauda equina syndrome spine Fx w/ cord injury Osteoporosis Spondylodiscitis ```
73
Polyarticular Jt pain (8)
``` RA SLE viral arthritis Reiters Syndrome Rheumatic Fever Lymes Gonococcal Drug induced ```
74
Monoarticular Jt pain (6)
``` osteoarthritis Gout pseudogout Truama septic arthritis Hemarthosis ```
75
Tx of seronegative spondyloarthropathies (ankylosing spondylitis, psoriatic, IBD, Reactive)
NSAIDs Anti TNF PT surgery?
76
Reactive arthritis Characteristics and causes
clinicial diagnosis - suspected w/ moving arthritis inflam oligoarthitis of usually lower its - preceeded by infection 1-4wks prior Reiters syndrome is an example Causes salmonella, shigella, camplobacter, chlamydia, yesernia
77
GI infection of UTI infection followed by asymmetric arthritis 1-4wks later that moves
Reactive arthritis 2/2-> salmonella, shigella, camplobacter, chlamydia, yesernia Fatigue, malaise, weight loss, fever
78
Undifferentiated spondyloarthropathy
Features of reactive arthritis w/out evidence of UTI or GI infection
79
Reiter syndrome
Can't see(anterior uveitis) Can't pee(urethritis) Can't climb tree(arthritis) may not always occur together, Post infection
80
Dx of active arthritis /reiters
synovial fluid does not grow anything, Antibody response r/o infeciton and crystals
81
Tx of reactive arthritis
NSAIDs, maybe sulfasalazine and azathioprine Abs generally NOT used
82
Psoriatic arthritis
10% of psoriasis Pts gradual onset after skin condition Aymmetric and polyarticular Upper extremities and small its more common NSAIDs
83
Temporal arteritis/giant cell arteritis affects what? Whos affected
Vasculitis of unknown cause >50 yrs W>M temporal arteries frequently but also aorta or carotids -> increase risk of aneurism and dissection
84
Temporal arteritis featured(5) Associated w/
``` constitutional - malaise, fatogue, fever HA - severe Visual impairment * only 25-50% Jaw apin w/ chewing Tenderness of temporal artery, no pulse ``` 40% have polymyalgia rheumatica
85
Vasculitis types
Large vessel - takayasu;s arteritis, temporal arteritis Medium - PAN, kawasaki, Wegeners, Churg straus, mucrscopic polyangitis Small - henrocj stolen purpura, hypersensitivity vasculitis, Behcet's Syndrome
86
Dx of temporal arthritis (5)
``` ESR elv biopsy key (neg does not r/o) ``` Age >50 New HA tender/palpable artery jaw claudication
87
Tx of temporal artheritis
High dose steroids early* - do NOT wait for biopsy for fear of blindness Tx 4wks minimum but maybe up to 2-3 yrs Follow ESR
88
Takyasu's Arteritis demographic Affects what artery Dx?
young asian women vasculitis of aortic arch and branches -> stenosis Dx w/ areriogram
89
Young women w/ decreased peripheral pulses, discrepent PB of arm vs leg and arterial bruits
Takyasu arteritis
90
Features of Takyasu arteritis
Constitutional - fever, night swears, malaise, arthalgias Pain/tender vessels *Absent pulses in carotid, radial ulnar artris, aortic regurg Ischmia signs -> limb ischemia, aoritc regurg, stroke, secondary HTN (renal stenosis)
91
Tx of takasu
Steriods Treat HTN 2/2 renal stenis Surgery to recannulate stenosis
92
Churg Strauss Associated w/ ?
vasculitis of many organ systems fever, fatigue, weight loss Prominent respiratory symptoms: asthma/dyspnea Skin lesions - sub Q nodules and palpable purpura P-ANCA positive poor prognosis - Steroids Tx
93
Wegener's Granulomatosis Affects? Features
Vasculitis of kidneys and lower respiratory tract Up respiratory symptoms, purulent/bloody d/c Oral ulcers Pulm symptoms - cough hemoptysis, dyspnea Renal involvement - glomerulonephritis (death) Eye - conjunctivitis/scleritis Arthralgia/myalgia Tracheal stenosis
94
Dx of wegenrs
CXR abnormal - nodules or infiltrates Elv ESR, Anemia, hematuria C-ANCA biopsy confirms
95
Prognosis and Tx of wegeners
POOR, die w/in yr Tx - cyclophosphamide and corticosteroids -> remission Renal transplant?
96
Polyarteritis nodosa Associated w/ (3)
vasculitis of nervous system and GI tract Hep B, HIV, drug Rxns PMN invasion of all layers and fibrinoid necrosis _ intimal proliferation -> luminal decrease
97
Symptoms of Polyarteritis nodusa
NO pulm involvement Fever/weakenss, weight loss, myalgia, arthralgia ab pain w/ bowel angina HTN, mononeuritic multiples, livedo reticularis Maybe P-Anca +
98
Polyarteritis nodusa Dx
P anca + can be present ESR elveatied Fecal occult blood Tx w/ steriods, cyclophosphamide POOR prognosis
99
Burgers Disease - Thromboangiitis obliterans
Young men who smoke Acute inflame -> ischemia nd gangrene Clausdicaion, cold cyanotic, painful extremities, ulceration STOP smoking
100
Hypersensitivity Vasculitis
Small vessel vasculitis in rsponse to: Drug, (penicllin, sulf), infection or other Skin involved -> palpable purport, macule, vesicles; painful lesions constitutional sympoms Dx w/ biopsy Prognosis good w/ withdrawal of agent

Med 1 (11 decks)

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