Rheumatology Flashcards

1
Q

RA

  • define
  • involves?
  • intra or extra articular manifestations?
  • Age of onset? MC in who
  • etiology
  • CM: MC?
  • which joints MC involved and spares?
  • list some characteristic hand deformities
  • constitutional s/s
  • PE?
A
  • chronic systemic inflamm autoimmune dz involving synovium of joints
  • inflammed synovium can cause damage to cartilage + bone
  • EXTRA articular manifestations

onset= 20-40—W»M

Etiology unknown—-infection, genetic predispo

CM

  • systemic/constitutional s/s: fever, fatigue, wt. loss, anorexia
  • joint pain + stiffness: morning stiffness >1 hour and improves later in the day, decr ROM
  • MC affects small joints: wrist, hands (MCP, PIP), MTP, ankle
  • SPARES DIP
  • characterisitc hand deformities
    1. Boutonniere deformities of PIP
    2. Swan-neck contractures of fingers
    3. ulnar deviation of metacarpophalangeal joints

PE: SYMMETRIC***** inflamed joints

  • warm, erythematous, boggy/soft,
  • rheumatoid nodules over bony prominences
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2
Q

RA

  • diagnosis (labs, radiographs)
  • xray findings for general, severe, early and lte RA
  • 5 diagnostic criteria
A

DIAGNOSIS:

  1. LABS
    * elevated RF
    * (+) Anticitrullinated peptide/protein antibodies
    * elevated ESR
    * elevated CRP
  2. RADIOGRAPHS
    *xray: symmetric joint narrowing, osteopenia, bone & joint erosions
    SEVERE RA: joint subluxation
    EARLY RA: xray normal
    LATE RA: narrowing of joint space (bc of thinning of articlar cartilage)

FIVE DIAGNOSIS CRITERIA:

  1. inflammatory arthritis of 3 or more joints
  2. s/s > 6 weeks
  3. elevated ESR and CRP
  4. (+) serum RF and ACPA
  5. xray changes consistent with RA—erosions + periarticular decalcification
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3
Q

Is RF helpful to rend in RA pts?

A

NO

because it rarely changes with dz activity

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4
Q

best initial lab test for RA?

most specific lab test for RA?

A

Rheumatoid factor— initial

anti-citrullinated peptide antibodies–most specific

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5
Q

Tx for RA

A

goal is to minimize pain + swelling, prvent progression, help PT remain as functionl as possible

  1. exercise to maintain ROM and muscle strength
  2. DMARD (methotrexate or Leflunomide) + NSAID for immediate sympt control
    * **DMARDs started early!!
  3. Corticos second line for ss control— this does not slow the dz process tho
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6
Q

why are DMARDs rx for RA

  • onset of action?
  • ex
A
  • ***Reduce morbidity and mortality by:
  • *slow dz progression
  • preserve joint function
  • limit complications

***needs to be initiated early—at time of diagnosis
onset= 6+ weeks

EX:

  • methotrexate, leflunomide, hydroxycholoquine, sulfasalazine= SYNTHETIC
  • adalimumab, infliximab=TNF inhibitors aka BIOLOGIC dmards
  • Abatacept, anakinra, rituximab–other biologics
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7
Q

best initial DMARD for RA?

  • alternative to this drug?
  • adjunct?
  • alternative first line?
  • which is used in less severe cases
A

Methotrexate=best initial

Leflunomide=alternative to methotrexate or adjunct

hydroxychloroquine and sulfasalazine= alternative for first line if cannot give methotrexate–BUT less effective + not usually used

Hydroxychloroquine used in less severe cases

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8
Q

which DMARD is safe in pregnancy

A

hydroxychloroquine

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9
Q

SE of methotrexate

A
  1. GI upset
  2. oral ulcers
  3. mild alopecia
  4. bone marrow suppresion
    * **HAVE TO GIVE WITH FOLIC ACID
  5. hepatocellualr injury
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10
Q

second line if all DMARDs dont work for RA?

A

anti-tumor necross factor (TNF) inhibiting drugs

  • etanercept
  • infliximab
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11
Q

Reactive Arthritis

  • what is it
  • also called?
  • when does it show and what causes it
  • MC occurs in?
  • CM
  • which joints MC affected
A
  • inflammatory arthritis in resp to an infection or inflammation in another part of the body
  • also called Reiter’s Syndrome
  • MC seen 1-4 weeks after infections–>Chlamydia trachomatis or GI (salmonella, shigella, campy, yersinia)

MC occurs in HLA-B27 +

CM: *recent GI or genitourinary infection

  1. asymmetric arthritis–> new joints may be involved over days–painful with effusions and lack of mobility
    * joint pain can persist or recur over long-term pd
    * lower extrem joints MC affected–knees
    ***
  2. systemic: fatigue, malaise, weight loss and fever
  3. TRIAD: arthritis + ocular s/s (conjunctivitis, uveitis) + genital (urethritis, cervicitis)
  4. Keratoderma blennorrhagicum—hyperkeratinized lesions on palms and soles
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12
Q

Reactive arthritis

  • diagnosis–lab findings, etc
  • tx–1st?
A

DIAGNOSIS

  • arthrocentesis (synovial fluid analysis)— r/o crystals or septic arthritis
  • incrs WBC (<50,000), negative cultures
  • Incrs ESR
  • incr IgG

TX:

  1. first line: NSAIDs
  2. if no response: sulfasalazine or Methotrexate second line +/- intraarticular glucocorticoid injections
    * NO ABX*—- they do not fix the reactive arthritis—- only used to treat the underlying cause (GI or GU infection)
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13
Q

Polyarteritis Nodosa (PAN)

  • what is it
  • MC affects what part?
  • spares what part?
  • MC in who
  • Assoc with?
  • CM
  • Diagnosis–labs, imaging, definitve?
  • TX
A
  • *systemic vasculitis MC medium sized vessels: renal, CNS, GI
  • *SPARES pulmonary vessels

MC in men 40-60 YO
INCR assoc with chronic Hep B and C***** HIV, and drug rxns

CM

  1. Renal HTN: renal artery stenosis, renal ischemia–NOT assoc with glomerulonephritis
  2. GI: abd pain worse when eating (intestinal angina), N/V
  3. Constitutional: fever, arthalgia, myalgias
  4. CNS: neuropathy, stroke, Monoeuritis multiplex (type of peripheral neropathy), foot drop**, ulnar nerve neuropathy
  5. Dermatologic: ulcers, livdeo reticularis (skin appears mottled/purplish), rynaouds

DIAGNOSIS:

  1. LABS
    * incr ESR
    * proteinuria
    * ANCA (-)
  2. ANGIO:
    * renal or mesenteric: microanurysms “beading”/strung together —> “rosary sign”
  3. BIOPSY=definitive
    * necrotizing medium vessel vasculities and NO GRANULOMAS

TX:

  1. Glucocorticoids +/- Cyclophosphamide if severe or refractory
  2. Hep B+: tx for BHV and possible tx with plasmaphoresis
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14
Q

what distinguishes PAN from other vasculities

A

PAN spares the pulmonary vessels

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15
Q

Polymyalgia Rheumatica

  • define
  • MC in who
  • etiology
  • duration of dz
  • CM— worse when? most promient s/s?
  • PE
A
  • **idiopathic inflammation of: joints, bursae and tendons
  • **closely assoc with giant cell arteritis

**MC in elderly (rare before 50) with avg onset=70YO
W»M

ETIOLOGY: unknown–auto immune

DURATION: self limiting–lasting 1-2 years

CM–usually begins ABRUPTLY

  • pain + stiffness in proximal joints and muscles: shoulders, neck, hips and pelvic girdle >2 weeks
  • BILAT pain
  • stiffness in shoulder and hip regions after period of inactivity—-MC s/s AND profound morning stiffness and sometimes daylong stiffness
  • Pain on movement
  • can have diff combing hair or getting up from chair
  • constitutional: fever, malaise, wt loss, depression

PE:

  1. normal muscle strength ****
  2. decr active and passive ROM
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16
Q

Polymyalgia Rheumatica

  • diagnosis–labs
  • tx
A

LABS

  • incr ESR and CRP
  • normal muscle enzymes (distinguishes PAN from polymyositis)

TX

  1. low-dose corticos=initial toc
  2. methotrexate is no resp to corticos OR if corticos are contraindicated
    - ->Response usually occurs in 1-7 days
    - ->After 4-6 weeks, begin to taper slowly
    - ->Most patients can stop corticosteroids within 2 years; a few patients have symptoms up to 10 years
    - ->Steroids are not curative, but are effective in suppressing inflammation until the disease resolves itself.
17
Q

what distinguishes polymyalgia rheumatica from polymyositis

what is simialr about the two dz?

A

LABS–>muscle enzymes

  1. normal muscle enzymes= polymyalgia rheumatica
  2. incr muscle enzymes (CK and aldolase)= Polymyositis

PE:

  1. normal muscle strength in polymyalgia rheumatica
  2. decr muscle strength in polymyositis
  3. +weakness in polymyositis
  4. NO weakness in polymyalgia rheumatica

ONSET OF SYMPTOMS:

  1. polymyalgia rheumatica—abruptly
  2. polymyositis–more sub-acute–weeks-months
  3. 30-50YO for polymyositis
  4. > 50 YO polymyalgia rheumatica

TREATMENT

  1. polymyalgia rheumatica gets low dose corticos
  2. polymyositis gets high dose corticos

SIMILARITY:

  1. hip and shoulder pain
  2. diff rising from chair
  3. diff combing hair
18
Q

Polymyositis

  • what is it
  • who is aff more
  • MC affects what parts
  • if skin is invovled–what is it called
  • CM-MC affected ?
  • PE
  • diagnosis– best initial? most specific? definitive?
A
  • *idopathic autoimune dz leading to MUSCLE INFLAMMATION
  • *MC limbs, neck and pharynx–can aff heart, lungs, GI tract

W»M
30-50 YO

Skin involvement=Dermatomyositis

CM

  • progressive symmetric proximal muscle weakness (hips and shoulders)–dev over weeks or months
  • diff combing hair, going up stairs or geting up from chairs
  • earliest and most severely affected muscle groups=neck flexors, shoulder girdle & pelvic girdle
  • +/- myalgia, dysphagia

PE

  • decr muscle strength—esp proximal muscles and often symmetrical
  • muscle atrophy
DIAGNOSIS 
LABS: 
1. sig incr muscle enzymes (CK and aldolase)--- best initial test 
2. AUTOANTIBODIES: 
*anti-Jo-1 (myositis-specific antibody)
*anti-signal recognition protein (most specific for this dz) 
*(+) ANA 
3. Incr ESR and CRP 
4. Incr RF

BIOPSY=definitive diagnosis–>endomysial inflammation
ELECTROMYOGRAPHY is abnm in 90% of patients

TX

  1. high dose corticosteroids first line
  2. Methotrexate, Azathioprine, IVIG, Mycophenolate—refrac to steroids or if contra
19
Q

Dermatomyositis

  • define
  • PE
  • diagnosis– most specific, best initial test, definitive
A
  • idiopathic inflamm leading to dermatologic s/s + muscle inflammation
  • polymyositis + derm s/s**

CM= same as polymyositis

PE=same as polymyositis AND:

  • heliotrope rash–> edema & blue or purple discoloration of the upper eyelids
  • Gottron’s papules–>raised violaceous scaly patches (dorsum of the PIP and DIP MC)
  • shawl sign: erythema shoulders, upper chest and back
  • V sign: erythema of neck and upper chest

DIAGNOSIS:
1. INITAL TEST= incr muslce enzymes (CK and aldolase)
2. autoantibodies: anti-Jo 1 and Anti-Mi-2**** MOST SPECIFIC
3. incr ESR, CRP, RF,
4 BIOPSY=definitive
5. EMG abnormal

TX
1. high dose glucocorticoids first line
2 Hydroxychloroquine useful for skin lesions
3. Immunosuppressive agents for patients who don’t respond to steroids or if corticosteroids are contraindicated (methotrexate, azathioprine, IVIG, mycophenolate)

20
Q

Fibromyalgia

  • MC in?
  • what is it
  • CM–aggrivatd by? better by?
  • Diagnosis
A

MC in adult women—-80-90% of cases–>20-55YO
**waxing/waning in severity

*abnormal pain perception of unknown etiology

CM

  • MSK pain–>constant and aching,
  • —>pain aggrivated by weather changes, strs, sleep deprivation and cold temps, worse in am
  • —>rest, warmth and exercise improve pain
  • extreme fatigue
  • stiffness
  • sleep + cognitive disturbances (“fibro fog)
  • HA
  • neuro s/s: numbness
  • anxiety
  • depression

DIAGNOSIS

  • primarily clinical
  • CRITERIA: tenderness in at least 11/18 trigger points + chronic/widespread pain >3 MO

TX

  1. conservative tx: initial tx–>stay active + low intensity exercise
  2. 1st line medical tx–>amitriptyline (TCA)
    * SNNRI: Duloxetine or Milnacipram OR cyclobenzaprine alternative
  3. Local anesthetic at trigger points
  4. Pregabalin FDA approved esp for sleep s/s
21
Q

Sjogren Syndrome

  • define
  • primary vs secondary
  • assoc with?
  • MC in who?
  • cm
  • tx
  • complications of dz
A
  • autoimmune dz affecting exocrine glands
  • multi-organ dz
  • Lymphocytes infiltrate and destroy exocrine glands (lacrimal and salivary glands)
  • primary=occurs alone
  • secondary: assoc with other autoimmune dz (Hashimoto, SLE, RA)
  • MC W>M 40-60YO
  • Assoc with HLDA-DR52 gene

CM
1. dry mucous membranes: xerostomia, dry eyes (keratoconjunctivitis sicca), vaginal dryness, bilateral parotid gland enlargement, dental caries (comp from xerostomia)

TX
1. Increase mucosal secretions–>artificial tears to prevent corneal ulcer, increase fluid intake, sugar free gum, artificial saliva and fluoride treatments
2. Cholinergic drugs: Pilocarpine or Cevimeline=incrs salivation + lacrimation
SE: diaphoresis, flushing, sweating, bradycardia, dirrhea, N/V

COMPS

  1. incr risk of non-hodgkin lymphoma, pneumonitis, interstitial nephritis
  2. incr risk of congenital HB if mom has high antibody titers while preg
22
Q

how to diagnose Sjogren Syndrome

  • best initial
  • definitive?
A

SCREENING LABS:
Best initial test: (+)Anti-nuclear antibodies (ANA)—specifically AntiSS-A (Ro) & antiSS-B (La)
OTHER LABS: +RF, anemia, leukopenia

(+) Schirmer test: decry tear production—– wetting of <5cm filter paper placed in lower lid for 5 mins

DEFINITIVE= lip or parotid gland biopsy–>gland fibrosis & lymphocytic infiltration

23
Q

Systemic sclerosis aka

  • define
  • Mc in who
  • describe the two types
  • diagnosis
  • tx
A

scleroderma

  • *systemic autoimmue CT dz where collagen deposition leads to fibrosis of the skin + internal organs (Lung, kidney, heart, GI)
  • high quantity of collagen deposition that leads to problems*

MC women 35-40YO

TWO TYPES:
1. Limited (CREST) syndrome: 80% of cases
*tight, shiny, thick skin of the face, neck and skin distal to the elbows and knees–SPARES trunk
Calcinosis cutis–>Ca deposites in skin
Raynaud’s
Esophageal motility disorder
Sclerodactyly (claw hand–localized thickness +tightness of skin on fingers/toes
Telangiectasis

  1. Diffuse: 20% cases
    * tight, shiny, thickened skin involving trunk & proximal extremities
    * assoc with greater internal organ involvement–>resitrictive lung dz, myocardial fibrosis

DIAGNOSIS

  • anti-centromere antibodies->specific for CREST
  • anti-SCL-70 antibodies–assoc with diffuse dz and multi organ involvement—-poorer prognosis
  • (+) ANA– not specific but positive in 90% of patients

TX

  • organ specific
    1. GERD: PPIs
    2. Hypertensive renal dz: ACEI
    3. Raynauds: vasodilators–CCBs
    4. severe: DMARDs: methotrexate, Mycophenolate, Cyclophosphamide for refrac or severe
    5. Pulmonary fibrosis: Cyclophosphamide
    6. Pulm HTN: Bosentan, Sildenafil
24
Q

Systemic Lupus Erythematous (SLE)

  • define
  • RF + onset
  • types
A
  • idiopathic chronic systemic, multi-organ, auto immune dz of CT
  • genetic, environmental and hormonal factors invovled
  • primarily type III hypersensitivity

RF

  • young females–> onset 20-40
  • AA
  • hispanic
  • native american
  • genetic
  • environmental
  • sun exposure
  • infections
  • estrogen—OCPs

FOUR TYPES

  1. spontaneous SLE
  2. discoid lupus (skin lesions without systemic dz)
  3. drug-induced lupus
  4. ANA negative lupus
25
Q

SLE

-CM

A

TRIAD: joint pain + fever + malar rash

Constitutional s/s: fever, chills, fatigue, night sweats

Discoid lupus: annular, erythematous pathces on the face and scalp that heal with scarring

Cutaneous s/s: malar rash, photosensitivty, oral or nasopharyngeal ulcers, alopecia, raynauds

MSK: joint pain–>may be first symp of dz, arthritis (inflammatory and symmetric), arthalgias, myalgias

OTHER: HA, serositis, glomerulonephritis, retinitis, oral ulcers, alopecia

26
Q

Diagnostic findings for SLE

A

LABS
(+) ANA–>screening TOC
*anti-double stranded DNA and anti-smith antibodies—>either one is diagnostic of SLE (specific and pathognomonic)
*antiphospholipid antibodies=incr risk of aterial or venous thrombosis
*Pancytopenia: anemia of chronic dz, leukopenia, lymphopenia and thrombocytopenia
*Decreased complement C3, C4

27
Q

Labs for a SLE flare up

A

increase in dsDNA antibodies and complement C3 and C4 decrease

28
Q

what is used for monitoring SLE

A

dsDNA antibodies

29
Q

Tx for SLE

  • mild and define mild
  • mod and define
  • severe and define
A

*tx depends on level of organ involvement
FOR ALL PT: sunscreen + avoidance of prolonged sun exposure

MILD (skin, joint, mucosal s/s)

  • hydroxychloroquine w/ or w/o NSAIDs and/or short term low-dose glucocorticoids
  • sometimes NSAIDs can be used alone for very mild dz

MODERATE: significant but non-organ threatening

  • hydroxychloroquine or chloroquine+ short term glucocorticoid
  • CAN ADD: Belimumab—monoclonal antibody that inhibs B-lymphs—reserved for active cutaneous or MSK dz unresponsive to glucos or other immunosuppressants

SEVERE: life or organ threatening
*high dose glucos or intermittent IV “pulses” of Methylprednisolone with other immunosuppressive agents (cyclophosphamide, Mycophenolate, Rituxmab)

30
Q

Antiphospholipid Syndrome

  • define
  • triggers
  • diagnosis
  • tx–asympto and sympto
A

Idiopathic disorder characterized by venous or arterial thromboses due to antibodies against negatively charged phospholipids
*can occur as primary OR with other Dz— such as SLE

TRIGGERS: smoking, prolonged immob, estrogen, CA, hyperlipidemia, HTN

CM
*increased risk of arterial and venous thromboses (atherosclerosis, recurrent DVT or PE, recurrent miscarriages)

DIAGNOSIS

  • anticardiolipin antibodies
  • Lupus-anticoagulant=incr PTT

TX

  • asympto: no tx
  • recurrent thrombosis may require lifelong Warfarin or other type of anticoag
  • low molecular weight Heparin used in pregnancy
31
Q

Jeuvenille (idiopathic) Rheumatoid Arthritis

  • define
  • types and CM
A

*autoimmune mono or polyarthritis in <16 y/ for >6 weeks

THREE TYPES 
1. Systemic (Still's Dz): 20% of all cases 
*daily high fever 
*daily arthritis pain 
*salmon-colored pink migratory rash 
*lymphadenopathy 
NO UVEITIS 
  1. Pauci (oligo) articular= 50% of cases
    * less than 5 joints involved, typically large joints (knees, ankles)
    * YES uveitis
  2. Polyarticular
    * 5+ small joints
    * usually symmetric
    * Uveitis
    * similar to adult RA— + morning stiffness, can be RF +
    * worse prognosis
32
Q

Jeuvenille (idiopathic) Rheumatoid Arthritis
-dx
-tx–1st, 2nd, severe
tx for (+) ANA?

A
DX=clinical 
*incr ESR and CRP 
\+ANA if oligoarticular 
15%= RF(+) 
Still's DZ often assoc with negative RF and ANA

TX

  1. NSAIDs=1st line
  2. Steroids=2nd or NSAIDs not eff
    * *PT

Severe or as second line: Anakinra (interleukin-1 rec inhib), methotrexate, lefluonmide

IF (+)ANA= routine eye exams every 3 MO bc of uveitis