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Clin med sem 2 > Rheum- Adults > Flashcards

Rheum- Adults Flashcards

1
Q

Gout- pathophysiology

A

Hyperuricemia

  • serum urate >6.8
  • rapid flucation in serum levels

Monosodium urate (MSU) level has to be high enough - crystals preciptiatate -> inflammatory response

Resolution of acute inflammation - mediated by immune mechanisms

Chronic inflammatory -> osteoclasts, blasts, chondrocytes - conttribut to tophi formation, erosion of bone, and joint injury - deforming bones

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2
Q

Gout- cause

A

Uric Acid Balance

Underexcreters - Majority

  • Renal insufficiency
  • Meds - low dose ASA, thiazides, loop diuretcis, nicotinic acid, cyclosporine, levodopa, ethambutol, pyrazinamide
  • Acidosis - DM ketoacidosis, ketogenic diet, lactic acidosis
  • Volume depletion/dehydration
  • lead exposure

Overproducers - minor

  • inherited defect of metabolism - lesch Nyhan syndrome, Kelley-Seegmiller
  • Myeloproliferative and lymphoproliferative disorders, polycythemia, carcinoma - TLS
  • Chronic hemolytic anemias
  • transient hyperuricemia associated w/ ATP consumption
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3
Q

Gout- epidemiology

A 
M>W
M 40-60, W >60
Pacific islanders
Alcohol - more drinks
Food w/ hyperuricema - read meat, seafood fructose
Obesity
Chronic dis - HTN, hyperlipidemia, DM
Meds aff urate balance - thiazide & loop diuretics, low dose ASA, cyclosporine, niacin
Protective - estrogen
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4
Q

Gout- S/S & PE

A

S/S:

  • Big toe hurts
  • sudden onset - hurts at night
  • painful, tender, swellen joint - red, arm
  • maybe fever
  • Max severity - 12-24 hrs
  • hx of similar attacks

PE:
Swollen, very tender red and warm overlying skin
Desquamation - skin peeling off
Tophi - irregular, asymmetrci macroscopic deposits of urate
- PATHOGNOMIC FOR GOUT
- Sites - external ear, hands, olecranon, feet, knee, Achilles tendon, forearm
- Usually painless
- after years
- maintain state of inflammation -> promtoes joint and tissue destruction around them
MTP OF GREAT TOE - podagra
- usualy in one joint !

Renal

  • Uric acid nephrolithiasis - kidney stones
  • chronic urate nephropathy - MSU crystals are deposted in the renal medulla and pyramids
  • Uric acid nephropathy - acute renal failure large amounts of uric acid crystals precipitate in the collecting ducrs and ureters
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5
Q

Gout- diagnosis

A

Stages:

  1. Acute gouty arthritis
  2. Intercritical gout - interval -> agttack resolved
  3. Chronic articular and tophaceous gout - may not totally resolve

Synovial fluid aspirate - monosodium urate crystals
- Negatively birefringent - needly-like, when viewed with polarized light microscopy

Ultrasound - hyperechoic linear density over:

  • joint - double contour sign
  • tophi - hypoechoic cloudy area

Xray - rat bite lesions - later in disease
- if next to tophus = GOUT

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6
Q

Gout- labs & imaging

A

Serum uric acid- inc or nl or low
Peripher WBC- inc
ESR/CRP- inc
- CRP- can inc at first

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7
Q

Gout- treatment

A

Asymptomatic hyperuricemia - don’t treat!
Life style mod - lose weight, reduce alcohol consuption, reduce purine-rich food consumption, DRINK FLUIDS >2L
Avoid hyperuricemic meds

Acute:
Goal - relieve pain ASAP
Start treatment as soon as possible - 12-24hrs
Urate- lowering meds - dont start during acute attack!! - continue if already on it - allopurinol

1st - NSAIDS

  • Naproxen
  • Indomethacin
  • D/C - after 1-2 days after complete clinical resolution
  • Contraindications - CKD w/CrCl <60

Cochicine - if NSAID intolerance

  • D/C after 2-3 days
  • SE - DIARRHEA and ABDOMINAL cramping
  • reversible peripheral neuropathy
  • Contra - severe hepatic or renal impairment w/ cochicine in 2w, mod-strong inhibitor of P-gp and/or CYP3A4 inhibitor

Corticosteroids - cant take NSAIDs or Cochicine

  • intra-articular - only 1 or 2 joints
  • PO, IV, IM
  • Caution - CHF, glucose intolerance, poor HTN control
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8
Q

Gout- prognosis

A 
Get a 24 hr urine acid test:
<800mg Undersecreter - Uricosuric agent or XOI
>800 mg Overproducer - XOI 
Xanthine Oxidase Inhibotrs (XOI) - reduce production of uric acid - disolve tophi
1. Allopurinol - 100mg/QD
- titrate every 2-5 weeks
- NEED A DOSE of >300 mg/day
- Prophylactic - cochicine 
- SE - rash, cutaneous reactions
2. Febuxostat - 40mg/day 
- titrate up to 80mg at 2 w
- Prophylactic - colchiicine 
- less hypersen reactions
- abnormal LFTS and slightly higher rate of CV events

Uricosuric Meds - Probenecid

  • block tubular reabsorption of urate - inc rate uric acid is renally excreted
  • CrCl >60
  • urinate 2L/day - avoid uric acid in urinary tracte
  • DONT GIVE - G6PD pts

Uricase - Pegloticase

  • breaks urate down - in other animals
  • REFACOTRY TO ALL OTHER THERAPIES
  • IV every 2weeks
  • premeds - infus reactions and
  • Dont give - G6PD
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9
Q

Gout- acute attack and prevention

A

Acute attack - resolve w/out treatment
- time - will become more frequent and last longer
The younger - more pregoressive course
Rever to Rheum
With urate-lowering meds - much less chronic/ tophi/deformity

Prevention - done during intercritical period

  • Serum urate level <6mg/dL - done slowly to prevent attack
  • start low dose when starting therapy - reduce risk of acute flare
  • indications: frequent gout attacks, tophi, clinicor or radiographic signs, renal insuff, ruccurent nephrolithiasis, Premen women, <25 male
  • USE LOW DOSE COLCHICINE when initiating XOI etc
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10
Q

Pseudogout- pathophysiology

A

Idiopathic
Early onset- familial
Excess pyrophosphate production in cartilage —> Ca pyrophosphate supersaturation —> CPR crystals
CPP crystals I- involved w joint inflammation —> degrades cartilage

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11
Q

Pseudogout- cause

A

Arthropathy caused by precipitation of Ca pyrophosphate dehydrate crystals —> acute, recurrent or chronic arthritis

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12
Q

Pseudogout- epidemiology

A 
Older adults- M & W 
- inc w age, rare <55 
Joint trauma 
Familial chondorcalciosis  
Hemochromatosis
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13
Q

Pseudogout- S/S & PE

A

Asymptomatic/incidental - CPP deposition in joint on xray, no symtpoms

Acute arthritis/pseudogout:

  • self-limited, sudden attacks of pain, redness, warmth diability, and swelling, monoarticor or oligoarticular
  • Knee - most common - then wrists, shoulders, elbows, ankles
  • Provoked by surgery, trauma or major illness
  • Assoicated w/ fever/chills

Chronic CPP crystal inflammatory arthrisi - Pseudo-RA

  • Inflammatory arthrisi - CPP crystals can found in joint
  • multi joins in symmetric patterns - similar to RA
  • usually affects joints unaffected by OA - MCPs, wrists, elbows, glenohumeral joints
  • asynchornous waxing and waning of inflamed joints

OA w/ CPPD - psudo-OA

  • progessive joint degernation
  • knees - most comon - wrists, MCP, hips, shoulders, elbows, spine
  • Clinical exam like OA - tenderness, boney enlarment, dec ROM

Pseudo-neuropathic joint ds

  • severe joint degernation form CPP crystal deposition
  • Resemble neuropathic arthropaythy - no neruoloigc imaprirment
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14
Q

Pseudogout- diagnosis

A

Joint aspirate- weakly positively birefingent rhomboid crystals- by polarized light microscopy

X-ray- linear and punctate densities w/in hyaline or articulate cartilage
- often see degenerative changes- subchonral cyss, osteophyte formation, fragmentation of bone and cartilage

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15
Q

Pseudogout- labs & imaging

A

Correct any underlying assoicated metabolic disorders

Acute pseduogout

  • Aspiration -> intraarticular glucocorticoid injection - triamcinolone
  • NSAIDs- indomethacin, naproxen, salicylates
  • Colchine
  • systemic cortiosteroids - PO, iV or IM

OA w/ CPPD - treat like OA

Pseudo-neuropathic joint disease - treat like charcot arthropathy

Prophylaxis
- >3 attacks per year - Cochinine -> doesnt work -> NSAIDS
Pseduo-RA
- NSAIDS - 1st - naproxen, indomethacinf
- Alternatives to NSAIDs - cochicine, hydoxycholorquine, low-dose glucocorticoids

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16
Q

Pseudogout- treatment

A

Attacks will resolve w/out treatment
Can cause chronic symptoms
Refer to rheum

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17
Q

Fibromyalgia- pathophysiology

A 
Unknown cause 
Disorder of altered pain processing and regulation 
- central sensitization 
- allodynia & hyperalgesia 
- functional MRI and PET studies 
Genetic 
Psychosocial 
Neuro endocrine 
Autonomic nervous system 
Stressful events- sexual abuse, war 
Viruses/infections- EBV
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18
Q

Fibromyalgia- cause

A

Chronic clinical syndrome of generalized musculoskeletal pain
- real dx vs psych vs socially constructed

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19
Q

Fibromyalgia- epidemiology

A

W

20-50

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20
Q

Fibromyalgia- S/S & PE

A 
Chronic pain/stiffness - wide spread  - 4 quads of body  - neck, shoulders, low back, hips  
Sleep distrubance - non restorative
Fatigue
Muscle weakness
Paresthesias
Cognitive disturbance
HA
Depression & anxiety
IBS
Dry mouth
Pelvic pain
Bladder symptoms
Tinnitus
Multiple chemical hypersensitivities
TMJ   
Onset: acute injury, infection, childbirth, persistent stress, exposure to toxins  
PE - normal except for pain at tender points - apply 4kg/cm2 to points
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21
Q

Fibromyalgia- labs & imaging

A

Little benefit- no lab abnormalities

CBC, ESR, CRP, TSH

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22
Q

Fibromyalgia- treatment

A

Initial:

  • education
  • good sleep hygiene - dec spleep -> worse pain
  • exercise - low impact aerobic activity
  • CBT

Meds:

  • TCAs - amitriptyline
  • Cycobenzaprine
  • SNRIs - Cymbalta (duloxetine)
  • SSRIs - Prozac
  • Anticonvulsants - Lyrica (pregablin), gabapentin

Refer: Do well w/ close FU

  • Psyc
  • PT
  • Rheum
  • Pain Managment
  • Massage, acupuncture

NOT HELPFUL:

  • Opiods
  • Corticosteroids
  • NSAIDs
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23
Q

Fibromyalgia- prognosis

A

Chronic- not progressive
Pts do well w close PCP FU
Most can work
Worse outcomes- F, low socioeconomic status, unemployed, depression, and obesity

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24
Q

Polymyalgia rheumatica (PMR)- pathophysiology

A

Unknown- associated w polymorphisms of HLA
- DR alleles
Affected joints- lymphocytes and monocytes —> inflammation

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25
Q

Polymyalgia rheumatica (PMR)- cause

A

Inflammatory condition associated w pain and stiffness of HIPS and SHOULDERS

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26
Q

Polymyalgia rheumatica (PMR)- epidemiology

A 
>50 inc w age 
W>M 
Scandinavian & Northern Europeans 
ID?
PMR > GCA
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27
Q

Polymyalgia rheumatica (PMR)- S/S & PE

A

Pain and stiffness - SHOULDERS AND PELVIC

  • shoulders first
  • Morning Stiffnes & Stiffness after activity - gel phenomneon
  • movements inc pain
  • impaired ROM
  • normal muscle strength

Low-grade Fever
Malaise
weight loss

Bursal inflammation/synovitis - wrists, knees, sternoclavicular joints
- sweeling, pitting edema

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28
Q

Polymyalgia rheumatica (PMR)- diagnosis

A

Clinical

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29
Q

Polymyalgia rheumatica (PMR)- labs & imaging

A 
ESR/CRP- inc 
Anemia 
WBC- nl 
Reactive thrombocytosis  
LFTs- inc ALP
Albumin- low
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30
Q

Polymyalgia rheumatica (PMR)- treatment

A

Glucocorticoid therapy- prednisone- 15mg PO QD
- nl start- range 10-20 mg
- no improvement after 7 days —> in 30 mg
Rapid improvement generally seen
After stable and controlled —> taper
- flares common- inc dose and then taper slower

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31
Q

Polymyalgia rheumatica (PMR)- prognosis

A

Good w steroids
Does not cause chronic damage
Morbidity- related to long term steroid use
Refer rheum

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32
Q

Giant cell arteries (GCA)- pathophysiology

A

Unknown- associated w polymorphism of HLA- DR alleles
Infiltration of inflammatory cells into vessels—> vasculitis
- thoracic aorta, large cervical artery, branches of external carotid arteries
Active inflammation —> thrombosis
Fragmentation of elastic almina

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33
Q

Giant cell arteries (GCA)- epidemiology

A

Smoking
DM
plus, all of the above

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34
Q

Giant cell arteries (GCA)- S/S & PE

A

CLASSIC: HA, Scalp Tenderness, Jaw claudication, visual changes
- visual loss - amaruosis fugax, diplpia
- anterior ischemic optic neuropathy - occlusive arteritiis of posterior ciliary artery
HALF HAVE PMR
Fever, fagigue, weight loss, malaise
Atypical - respiratory, neurolligc, otolaryngeal

PE:
Overall look ill
Temporal artery - thickened, tender, prominent, or normal
Funduscopic - pallor, edema of optic disc, scattered cotton-wool patches, small hemorrhages - or nl
CV - asymmetry of pulses in arm, aortic regug, bruits near clavicle

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35
Q

Giant cell arteries (GCA)- diagnosis

A

Temporal artery biopsy- gold
-unilateral biopsy neg —> contralateral biopsy
Classification- does not replace biopsy for def diagnosis

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36
Q

Giant cell arteries (GCA)- labs & imaging

A 
ESR/CRP- inc 
Anemia 
WBC- nl 
Reactive thrombocytosis 
LFTs- inc ALP 
Albumin0 low
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37
Q

Giant cell arteries (GCA)- treatment

A

DON’T WAIT FOR BIOPSY TO START TREATMENT - prevent blindness

Glucocorticoids - Prednisone - 40-60 mg PO QD
- no response - inc dose
- Once conttrolled - start tapering - no faster then 10% every 1-2 w
Will need this for months
Flares common - inc Predni by 10mg

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38
Q

Giant cell arteries (GCA)- prognosis

A 
If not treated- poor prognosis and blindness 
Chronic course and relapses 
Inc CV events 
Long term steroid risk 
Refer rheum
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39
Q

Takayasu arthritis- pathophysiology

A

Chronic vasculitis- affecting aorta and main branches

  • spectrum of PMR/GCA?
  • inflammation of blood vessels
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40
Q

Takayasu arthritis- epidemiology

A

W
Asian
10-40 y

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41
Q

Takayasu arthritis- S/S & PE

A

Constitutional symptoms - early
Vascular insufficiency - later
- claudication, cool extremities, subclavian steal syndrome, syncope, BP differential, arthralgia, skin lesions, pul, abdominal pain, diarrhea, GI hemorrhae, angina pectoris

PE: 
BP differential - 10mmHg
Pulses am/legs - diminished, asymmetrical
Burits
Synovitis - large joints
Renovascular HTN
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42
Q

Takayasu arthritis- diagnosis

A

Clinical

Imaging- arterial luminal narrowing or occlusion w wall thickening

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43
Q

Takayasu arthritis- labs & imaging

A

Anemia
ESR/CRP- inc
Albumin- low

44
Q

Takayasu arthritis- treatment

A

Glucocorticoids- prednisone 45-60mg PO QAM
- taper w controlled labs good
- may need chronic use
- 2nd line- immunosuppressed
Surgery- PCTA, bypass grafting, aortic repair

45
Q

Takayasu arthritis- prognosis

A

Chronic
Relapse- remitting
Self limited
Vascular- progressive

46
Q

Polyarteritis nodosa (PAN)- pathophysiology

A

Inflamed wall of vessel thickens and lumen narrows —> less blood flow (thrombosis) —> ischemia or infarction of tissue

Systemic necrotizing vasculitis

  • medium sized or small arteries
  • VEINS not involved

Can affect any organ
- skin, muscle, peripheral nerves, kidneys, GI tract, heart- most common
LUNGS SPARED

47
Q

Polyarteritis nodosa (PAN)- cause

A

Idiopathic

Associated- Hep B, Hep C, hairy cell leukemia

48
Q

Polyarteritis nodosa (PAN)- epidemiology

A 
M
40-60 
No racial group 
1/100,000
Hep B
49
Q

Polyarteritis nodosa (PAN)- S/S & PE

A

Systemic - fatiuge, arthralgia, fever, weight loss, weakness, pain in extremities
Skin:
- LOWER EXTREMITIY ULCERATION - classic
- subcut nodules, Livedo reticularis, Bullous or vesicular eruption, palpable purpura, Infarction or dgital gangrene
Renal:
- MOST COMMON AFFECTED ORGAN
- HTN and RENAL INSUFF
- severe - infarct
- perirenal hematoma - rupture of renal arterial aneurysms
GI
- ABDOMINAL PAIN - after meals
- N/V/D, Infarc - lead to acalculous cholecysts
Cardiac - MI, CHF w/ uncontrolled HTN or ICM
Lungs - NOT INVOLVED
MSK - pain in extremities, arthralgia, myalgia, muscles weakness
Eye - retinal hemorrhage, visual impairment, optic ischemia
Nervous System:
- MONONEURITIS multiplex - foot drop
- sensory -> then motor
- CNS involvment - encephalopathy, seizure, stroke
Other - ischemic orchitis

50
Q

Polyarteritis nodosa (PAN)- diagnosis

A

Biopsy of involved organ- necrotizing inflammation of medium sized arteries

Angiogram

  • small aneurysm- rosary sign
  • irregular constrictions of large vessels and occlusion to smaller vessels

CT and MR angio- show extent of organ involvement

Clinical- need 3/10

51
Q

Polyarteritis nodosa (PAN)- labs & imaging

A 
ESR/CRP- inc
LFTs/Hep panel- abnormal 
Cr- inc 
Anemia- GI involvement/chronic disease 
CK- inc
52
Q

Polyarteritis nodosa (PAN)- treatment

A

Viral hep? —> antiviral- limit steroids
Mild disease:
- corticosteroids alone- prednisone- 1 mg/kg QD
- taper after 4 weeks- if improved
- resistance?—> methotrexate, azathioprine

Mod/severe- renal, HTN, ischemia, infarct

  • high dose corticosteroid + immunosuppressant
  • prednisone and cyclophosphamide- IV or PO
  • live/organ threatening- IV Methylprednisone
  • Max 12 m on cyclophsom
53
Q

Polyarteritis nodosa (PAN)- prognosis

A

No treatment- V bad
W treatment- 80%
Worse- CKD w CR >1.6, proteinuria >1g/dl, GI ischemia, CNS dis, CV invol

54
Q

Reactive arthritis (ReA)- pathophysiology

A

Asymmetric polyarthritis that develops after GI or GU infection- large lower extremity joint

55
Q

Reactive arthritis (ReA)- cause

A

GI infections- shigella, salmonella, Yersinia, campylobacter, E. coli, C diff
- inflammation in gut dec wall integrity and activates pro inflammatory cytokines —> microlesions —> cytokines migrate to joint

STI- CT, ureaplama urealyticum

HLA-B27- predisposed

56
Q

Reactive arthritis (ReA)- epidemiology

A

Rare
M>F
YA
HLA-B27 gene

57
Q

Reactive arthritis (ReA)- S/S & PE

A

Diarrhea or urethritis - 1-4 weeks earlier
Asymmetric Arthritis
- large weight bearing joints - knee, ankle
- Spine, SI joint
Enthesitis - swelling at heel
Dactylitis - sausage joints

General - fever, fatigue, weight loss
Eye - conjunctivits, anterior uveitis
Mucocutaneous - balanitis, stomatitis, keratoderma, blennorrhagicum
Nails - minic psoriasis 
- CANT SEE, CANT PEE, CANT climb a tree
58
Q

Reactive arthritis (ReA)- diagnosis

A

Clinical- no single test
MSK findings
Preceding infect
No other thing

59
Q

Reactive arthritis (ReA)- labs & imaging

A

CRP/ESR- inc
Stool culture or urethral swab- pos
Synovial fluid analysis- WBC inc- neutrophils

60
Q

Reactive arthritis (ReA)- treatment

A 
Treat underlying infection 
Arthritis: 
NSAIDS- mainstay/initial 
- Naproxen, diclofenac, indomenthacin- 2w 
No response 
- intraarticular glucocorticoids 
- PO glucocorticoids 
- DMARD- sulfasalazine or methotrexate
61
Q

Reactive arthritis (ReA)- prognosis

A

Self limiting
Lasts 3-5m- remit 6-12
Require PT
Refer to rheum

62
Q

Sjogren syndrome- pathophysiology

A

Systemic autoimmune disease- affects LACRIMAL and SALIVARY glands —> mouth and eye dryness aka xerostomia and keratoconjunctivitis sicca

63
Q

Sjogren syndrome- cause

A

Primary or sec to another autoimmune dx

- by itself or with another autoimmune

64
Q

Sjogren syndrome- epidemiology

A 
F >M 
Middle aged 
All races- Caucasian 
HLA-DQ
HLA- DB
65
Q

Sjogren syndrome- S/S or PE

A

Ocular: Keratoconjuntivitis Sicca
- diminished aqueous tear production
- dry, gritty, sandy, foreign body sensation, burning, itching, intolerance to contacts, photphbia, eye fatigue
- Conjunctival or cornal damge - Rose bengal, lissamine green or fluorescein stainging
- Schirmer test - dec tear production
- Mucus filaments, dilation of bulbar conjunctival vessels, lacrimal gland enlargement
Mouth - Xerostomia
- cotton mouth, dysphagia, trouble w/ prolonged speaking, loss of taste
- Dental caries, gingival recession, oral candidiasis common, laryngotracheal reflus, chronic esophagitis
- salivary gland enlarmentt - partoid
- Saxon test - chew on dry sponge and weigh to see how much produced
- Sialometry - spit out saliva
Constiutional - Fatigue, low-grade fever
Skin - xerosis, purpura, raynaud phenomenon, cutaneous vasculitis, angular chelitis, annular erythema
MSK - arthraliga, symmetric nondeforming, intermittent - mild myopathy w/ weakness
Lungs - dry cough, excessive throat clearning, rhinitis, sinusistis, hoarseness, pneumonitis, pneumonia
CV - rare - pericarditis, heart block
GI - atrophic chronic gastritis, celiac dis, biliary cirrhosis, autoimmune hep
Renal - chronic interstitial nerphritis, glomerular disease
UG - vaginal dryness, dyspareunia, pruritis, dysuria, urinary frequency/urgency, nocturia in absence of UTI
Neurologic/Psych - peripheral neuropathy, ataxia, trigeminal neuropathy, mono, neuropathics, CNS , affective disorders - depression

66
Q

Sjogren syndrome- diagnosis

A

Salivary gland biopsy- gold

  • real life- only do this if diag unclear
  • > 4 lobules removed from inner lip
  • positive= focus score >1 per 4mm2
  • focus= >50 lymphocytes
  1. Objective test of dry eye or xerostomia or imaginst consistent w glandular abnormalities
  2. Anti-Ro/SSa and/or anti-La/SSb antibodies, positive lip biop, or established rheum dis
67
Q

Sjogren syndrome- labs & imaging

A 
ANA- pos 
Anti-Ro/SSA and anti-La/SS-B- pos antibodies 
RF- pos 
Centromere antibodies, anti-CCp, antimitrochondrial 
Anemia- mild
Cytopenia- mild 
Hypergammaglobulnemia- mild
ESR- inc 
CRP- nl/inc
LFTs- abnormal 
Renal impairment- inc cr and dec bicarb
68
Q

Sjogren syndrome- treatment

A

Oral:

  • Symptomatic
  • Artificial Saliva
  • Mouth lubrication - sugar free hard candies
  • Oral care

Ocular:

  • Environment management
  • artificial tears - every 2-4 hrs, at night ointments
  • still no relief -> ocular cyclosporine
  • topical steroids - if all else fails
  • punctal occlusion plugs

Immunosuppressive therapy

  • Hydroxychoroquine
  • Methotrexate

Refer to rheum

69
Q

Sjogren syndrome- prognosis

A

Depends on extent
Severe exocrine involvemnt - higher lymphoma risk - DLBCL
Risk - autoimmune
Extraglandular dx - inc M&M
Refer if - systemic signs/symptoms, oculur dryness doesn’t respond to artifical tears

70
Q

Rheumatoid arthritis (RA)- pathophysiology

A

HLA- DR MHC genes
-shared epitope
Synovial tissues main target —>
Tcells, Bcells, and macrophages, synovial cells go to synovial tissues —>
synovial tissue proliferates —>
excess synovial fluid and pannus forms —>
pannus invades nearby bone and cartilage, destroying them and stretching the joint capsule —>
destruction and deformity

71
Q

Rheumatoid arthritis (RA)- epidemiology

A 
F>M
F- 4th-5th decade 
M- 6th-8th decade 
Genetic 
Smoking
72
Q

Rheumatoid arthritis (RA)- S/S & PE

A

Indisious onset
Morning stiffness >30 min
Symmetric swelling of joints
Tender, pianful joints
Joints affected: PIP, MCp, wrists, ankles, knees, MTPs
Synovial cysts, tendon rupture, entrapment syndrome
Hands
- Ulnar deviation of MCP joints
- Swan neck - hyperextension of PIP, flexion of DIP
- Boutonniere - Flexion of PIP, extension of DIP
- Z deformity - hyperextended interphalangeal joint
General - fatigue, weight loss, low-grade fever
Skin
- Rheumatoid nodlues - subcutaneous nodules, extensor surfaces over joints, firm/nontender
- vasculitis
Ocular - keratoconjunctivitis sicca, scleritis/episcleritis, scleromalacia
Pulm - pleural effusions, rheumatoid nodues, interstitial lung disease
CV - pericardial effusions, pericarditis

Felty Syndrome Triad - splenomegaly, neutropenia, RA

73
Q

Rheumatoid arthritis (RA)- diagnosis

A 
Inflammatory arthritis involving >3 joints 
Pos RF and/or anti-CCP- can be neg 
Elevated ESR and/or CRP
Duration >6w 
Excluded everything else
74
Q

Rheumatoid arthritis (RA)- lab & imaging

A

Anti-CCP antibodies - most specific, more aggressive
RF - more severe disease
- 15% are seronegative - neg anti-CCP and RF

ESR/CRP - inc
Anemia - mild
Thormbocytosis - mild
WBC - normla/mild leukocytosis
Synovial fluid - inflammatory effusion, leukocytes 1500-2500, PMNs predominate

Xray - most specific

  • early - nl
  • Initial - soft tissue swelling, osteopenia - wrists or feet
  • later - joint space narrowing and erosions

MRI - more sensitive then xray
- see erosions earlier

U/S - more senstivie then xray

75
Q

Rheumatoid arthritis (RA)- treatment

A

Control pain and inflammation
preserve function, prevent deformity
Early diagnosis and intiation of DMARDs!!!
Target of treatment - remission or low disease activity
Refer to Rheum

Pretreatment Screening:

  • Hep B&C
  • Baseline CBC, Cr, LFTs, ESR, CRP
  • Ophthalmic screening
  • Check for latent TB
  • Baseline radiographs

NSAIDs - help w/ symptoms
- Not monotherapy
- All about equal
Corticosteroids - Prednisone 5-20mg QD
- very helpful for symptom relief and slowing rate of joint damage
- Not recommended for monotherapy or long-term use
- Bridge - as start DMARD

DMARD

  1. Methotrexate - INITIAL
    - starting - 7.5mg PO weekly
    - improve at 2-6w
    - Contraindicated - prego, liver disease, heavy alcohol, severe renal impairment
    - SE: GI, stomatitis
    - Close monitor - CBC, LFTs
    - ALL TAKE FOLIC ACID or leucovorin calcium
  2. TNF inhibitors - Etanercept
    - SQ or IV
    - Expensive!!
    - well tolerated
    - higher risk of serious bacterial infections - must screen for latent TB
    - worsen CHF
    - work well w/ severe disease

F/U

  • assess symptoms and functional status
  • monitor lab work - disease activty and toxicities
  • radiographs every 2 yr
76
Q

Rheumatoid arthritis (RA)- prognosis

A
  • Pre MTX - very poor
  • good treatment - better
  • disease flares
  • higher mortality w/ CV disease
  • Poor - RF or anti-CCP pos, extrarticular dis, functional limitation, eroisions on xray
77
Q

Systemic sclerosis (Scleroderma)- pathophysiology

A

Chronic systemic autoimmune with fibrosis of skin and other organs
2 froms: Limited, Diffuse

Unknown exact
Widespread proliferative/obliterative vasculopathy of small & medium arteries and capillary rarefaction - Hallmark!!

  1. Vascular injury + activation
  2. Hypertorphy of intima
  3. Excessive deposition of fibrin can occlude lume -> loss vascular supply -> tissue hypoxia

Fibroblasts overporduce extracellular matrix -> inc collagen deposition in the skin
Collagen cross-linking -> skin tightening

78
Q

Systemic sclerosis (Scleroderma)- cause

A 
1. Limited- CREST 
Calcinosis Cutis
Raynaud
Esophageal dysmotility 
Sclerodactyly- local thickening /tightness 
Telangiectasia
  1. Diffuse
79
Q

Systemic sclerosis (Scleroderma)- epidemiology

A 
F>M
30-50yo
AA- early onset, diffuse 
NA in OK 
1st degree relatives
80
Q

Systemic sclerosis (Scleroderma)- S/S & PE

A

Raynaud - first sign
Constitutional symtpoms - diffuse disease
Skin:
- initial - edema + pruritis
- later - thick hide-like skin
- hyperpigmentation, digital ulcers, pitting of fingertips, loss of appendicular hair, telangiectasias calcinosis
GI:
- Dysphagia, reflux symptoms, hypomotility
- Vascular ectasia - stomach antrum - watermelon stomach
Lung:
- pulmonary fibrosis
- pulmonary vascular dis -> pHTN
CV:
- pericarditis
- heart block
- myocardial fibrosis
- heart failure
- arrhythmia
Sicca syndrome - sjoren syndrome
Reanal:
- SCLERODERMA RENAL CRISIS - sudden onset of mealignant HTN can lead to renal failure and death
Thromboembolic disease - inc risk
Musculoskeletal - joint pain, contractures, immobility, tendon friction rubs
Neuro - neuropathies, myopathy/myosisits
GU - eractile dysfunction, femal sexual dysfunction

81
Q

Systemic sclerosis (Scleroderma)- diagnosis

A

> 9 = sclera derma

82
Q

Systemic sclerosis (Scleroderma)- labs & imaging

A

ANA - pos
Anti-SCL-70 - pos
Anti-centromere antibodies - limited skin involvement, pHTN, & digital ischemia
Anti-RNA polymerase III antibody - diffuse disease - inc SRC
Anemia - mild
Peripheral smear - microangiopathic hemolytic anemia - SRC
Proteinuria w/ renal involvment

CT - lung changes
PFTs - baseline, repeat Q4-12m
Echo - pHTN
EGD - Barrett’s

83
Q

Systemic sclerosis (Scleroderma)- treatment

A

No proven disease-modifying drug - TREAT TARGETED ORGANS
Refer to Rheum and any other specialist!

Diffuse skin/ systemic involvement - immune process target
- glucocorticoids, methotrexate, mycophenolate mofetil, cycophophamide, IVIG

Raynaud phenomenon - CCB, Sildenafil, exposure precautions

GI

  • prokinetic agents - metoclopramide, erythromycin
  • Malabsorption - treat small bowel bacterial overgrowth with abx
  • Reflux - lifestyle mod, PPIs,H2blockers

Lung

  • pHTN - dont smoke, diuretic, digoxin, sildenafil or prostaglandins
  • ILD - immunosuppreants - cyclophosphamide, MMF

Renal
- SRC - ACEI to return to baseline, dialysis, transplant

C V - treat per usual

Erectile dysfunction - on-demand pills dont work, Viagra, Cialis

84
Q

Systemic sclerosis (Scleroderma)- prognosis

A

Depends on organ involvment
Lung dx - may cause of mortality
Worse prog - diffuse, M, pulmonary invovlement, SRC, CV

limited - 90%
Diffuse - 70-80%

May be linked w/ breast and lung cancer

85
Q

Systemic lupus erythematosus- pathophysiology

A

Chronic inflammatory disease w antibodies and vary presentations that can affect every organ —> fatigue, joint pain, rash

Exact etiology unclear
Lupus antibodies target intracellular particles that have nucleic acid and nucleic acid binding proteins

Antibodies bind to targets —> activate inflammatory pathway —> immune complexes and complement deposited in organs- esp skin, kidneys, heart valves

86
Q

Systemic lupus erythematosus- epidemiology

A

Childbearing females- use of estrogen
OCP, early monarch, estrogen, post menopausal

Black, Hispanic, Asian > Caucasian

Urban

MHC and genes code for complement pathway

Smoking

87
Q

Systemic lupus erythematosus- S/S & PE

A

Constitutional symptoms - fever, fatigue, malaise, weight loss
Skin
1. Acute cutaneous lupus erythematosus -
- Localized: malar ““butterfly”” rash, spares nasolabial folds, triggered by sun exposure, scaly, indurated
- Generalized: maculopapular lesions in a photosensitive distribution - any part of body
2. Subacute cutaneous lupus erythematosus
- MOST photosensitive of all rashes
- scaly, erythematous papules
3. Chronic cutaneous lupus erythematosus
- discoid lupus - raised erythematous plaques w/ scale, raised erythmeatous ring around lesion
- lead to scaring -> skin atrophy
- lupus panniculitis
- Alopecia, plaques or psoriasiform lesions, nail fold infarcts, periungual erythema, livedo reticularis, mucosal ulcerations - not painful, on hard palate
MSK - arthralgia and nonerosive arthritis - early - PIP, MCP, writs, knees
Renal - commonly involved - poor prognostic indicator - lupus glomerulonephritis
Vascular
- raynaud phenomenonz
- Vasculitis - all sizes, esp small
- Thromboembolis - antiphospholipid antibodies
CV - pericarditis, inc risk CAD, arrhthmias, MR from atypical verrucous endocarditis
Lung - pleuritis, pleural effusion, ILD, pneumonitis, pHTN, alveolar hemorrhage
GI - dysphagia, PUD, intestinal pseudo-obstruction, protein-losing enteropathy, mesenteric vasculitis, acute pancreatitis,peritonitis, perfoation
Eye - Keratoconjunctivitis sicca, retinal vasculopathy
Neuro - cognitive dysfucntion, psychosis, mood disorder, seizures, cerebrovascular disease, neuropathy, HA
Lymphadenopathy - cervical, axillary, inguinal nodes
Hematologic - anemia of chronic disease, leukopenia and midl thrombocytopenia

88
Q

Systemic lupus erythematosus- diagnosis

A

Even if it doesn’t meet criteria- still dx w 2-3 features plus 1 other feature e

89
Q

Systemic lupus erythematosus- labs & imaging

A

ANA - pos
Anti-dsDNA - specific, but not sensitive - levels correspond to disease
Anti-SM - specific, but not sensitive
Antiphospholipid antibodies - false pos for syphilis
Anti-Ro and anti-LA - maybe!

Anemia
Leukopenia
Thrombocytopenia
Elevated Cr
False-positive suphilis
Proteinuria - abnormal urine sediment
Hematuria
Hypocomplementemia
Direct coombs - pos
90
Q

Systemic lupus erythematosus- treatment

A

Regular exercise
Smoking cessation
Sun protection - DAILY
Healthy diet
Prevent glucocortioid induced osteoposis
Stay up to date on vacc
Treated w/ antimalarials
Cutaneous:
- initial - topical glucocorticoids - start low
- can do - intralesional injections of triamcinolone - refractory
- may need systemic - hydroxycholorquin
Musculoskeletal - NSAIDs or Tylenol - may add hydroxychloroquine
Serositis - NSAIDs or/and low-mod glucocorticoids
- hydroxychlorquine or PO colchicine - persistent/recurrent
- mod/high steroids - taper -> switch to MTX, azathioprine, or MMF - severe or refractory
Renal - renal biopsy -> get exact path
- ACEI or ARB - HTN/proteinuria
- immunosuppressent?
Neuropsychiatric -depends
- Seizures - anticonvulsants
- CVA - b/c of antiphopholipid? - long term anticoag
- Glucorticoids - controversial
Hematologic
- leukopenic w/ recurrent infection - prednisone (inc WBC)
- Thrombocytopenia <20k or < 50k + bleeding and severe autoimmue anemai
- high dose glucocorticoids - taper
- IVIG, immunosuppressants, Splenectomy - last

91
Q

Systemic lupus erythematosus- prognosis

A

Biomodal mortality
Early- infections, renal, CNS
Later- atherosclerosis form chronic inflammation- MI
Higher malignancy risk
A vascular necrosis of bone- long term effects of steroids

92
Q

Drug induced lupus

A

Procainamide, hydralazine, isoniazid, chloropromazine, methydopa, minocycline, quindine

F=M
No nephritis and CNS involvement
No anti-dsDNA antibodies or hypocomplementemia
Labs and S/S normalize once drug is discontinued

93
Q

Polymyositis- pathophysiology

A

Autoimmune myopathy characterized by proximal muscle weakness

Myosistis-specific antibodies and myosistits associated antibodies
Inflammatory cell infiltrates muscle tissue
Non immune also plays a role?
Path poorly understood

94
Q

Polymyositis- epidemiology

A 
Rare
F>M
Black > Caucasian 
45-60 yo 
HLA polymorphism
95
Q

Polymyositis- S/S & PE

A

Proximal muscle weakness/muscle fatigue
- gradual and progressive
- symmetric
- leg weakness- first
- arm and neck weakness
- trouble rising out of chair, climbing stairs, washing hair
- dysphagia, difficulty breathing, fetal incontinence
Muscle pain/tenderness
Intestinal lung disease, myocarditis, overlap syndrome- connective tissue disease

96
Q

Polymyositis- diagnosis

A 
No validated way 
Can do w/out biopsy if:
- characteristic clinical symptoms 
- abnormal muscle enzyme 
- no better explanation 
Get an EMG or MRI
97
Q

Polymyositis- labs & imaging

A 
CK - inc 
Troponin - see if CV involved
LDH, aldolase, AST, ALT - inc
ESR/CRP - not helpful
ANA - pos
Anti-Jo-1 antibody 
Anti-SRP - progressive, severe
Anti-Mi-2 & anti-155/140- dermatomyositis

Muscle Biopsy - Gold
- muscle that is weak, but not atrophied
- Quds or delts
- Inflammatory infiltrate - invade fascicle
EMG & MRI - identify which muslce to biopsy
- bx oposite side - wont get needle artifact
- differentiate myosisit rom a neruopathic cause
- EMG - early recrutiment
- MRI - muscle inflammation, edema, fibrosis, calcification

98
Q

Polymyositis- treatment

A

Steroid- Prednisone
- 4-6 w for disease control
- Taper
Osteoporosis prophylaxis

Immunosuppressant - combined

  • initial or when steroid fail
  • Azathioprine
  • Methotrexate

Exercise!!

PT

99
Q

Polymyositis- prognosis

A 
Good w/ meds
Delayed treatment  - worse prognosis
Don’t regain completely
Anti-Jo-1 - don’t completely respond to treatment
Inc risk of malignancy
Steroid - SE from long term use
100
Q

Psoriatic arthritis- pathophysiology

A

Chronic inflammatory arthritis associated w psoriasis
Unknown
HLA alleles
Develops after or same time as onset of psoriasis

101
Q

Psoriatic arthritis- S/S & PE

A

Pain and stiffness - affected joints
- morning stiffness >30min - worse w/ no movement

Symmetric polyarthritis - common
Asymmetirc oligoarthritis - less common

Distal arthritis - DIP

Arthritis mutilans - deforming destructive

Spondyloarthritis - sarcoiliitis & spondylitis - C spine involvement

Enthesitis - inflammation at site of insertion of tendons
- soft tissue swelling, TTP
Tenosynovitis - flexor tendons of hands, extensor carpi ulnaris
Dactylitis - sausage digit - diffuse swelling of entire finger or toe

Skin lessions - onset
- erythematous plaques with scaling

Nails - pitting, ridging, onycholysis, nail bed hyperkeratosis, splinter hemorrahage

Pitting edema in hands/feet

Eyes - ocular inflammation - uveitis, conjunctivitis

102
Q

Psoriatic arthritis- diagnosis

A 
Nonspecific 
Both psoriasis and inflammatory arthritis 
Diagnose in a sense of psoriasis 
- distal joint involvement 
- asymmetric distribution 
- nail lesion
- dactylitis 
- FH of psoriasis 
- presence of HLA-C
103
Q

Psoriatic arthritis- labs & imaging

A

ESR/CRP- inc
Synovial fluid- inflammatory
RF- neg

Imaging

  • joint space narrowing
  • erosions involving DIP, PIP
  • pencil-in-cup deformity
104
Q

Psoriatic arthritis- treatment

A

Refer to Derm and Rheum
Early treatment
PT/OT/exercise
Weight reduction

Mild peripheral or axial - NSAIDs - naproxen, celebrex

Severe or unresponsive to NSAIDs

  • DMARDs - Methotrexate, sulfasalazine, azathioprine, cyclospoirne, hydroxychloroquine
  • Biologic TNF inhibitors - etanercept, infliximab, adalimumab, golimumab, cerolizumab pegol
  • Immunosupprssion - reactivation of latent TB

DONT USE PO STEROIDS!!- cause flare

105
Q

Psoriatic arthritis- prognosis

A

Comorbidities

  • inc risk of CV
  • DM, atherosclerosis, metabolic syndrome
  • inc rates of depression and anxiety

Clin med sem 2 (30 decks)

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