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Clin Med semester 2 > Rheumatology Part 2- Paulson (exam 2) > Flashcards

Rheumatology Part 2- Paulson (exam 2) Flashcards

1
Q

Fibromyalgia: Describe.

A

A chronic clinical syndrome of generalized musculoskeletal pain.
-Controversial condition
“real disease” vs. psychological disease vs. medicalization of socially constructed entity?

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2
Q

Fibromyalgia: Associated symptoms?

A
  • Fatigue
  • Disordered sleep
  • Multiple somatic symptoms
  • Cognitive problems
  • Psychiatric symptoms.
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3
Q

Fibromyalgia: Epidemiology?

A
  • Very common
  • Especially among those visiting rheumatology and pain management providers.
  • Much more common in women
  • Especially 20-50
  • Estimates of percentage affected range from 2-10%
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4
Q

Fibromyalgia: Etiology?

A
  • Unknown
  • Genetic basis
  • Psychosocial factors
  • Neuroendocrine dysfunction
  • ANS dysfunction
  • STRESSFUL EVENTS
  • Viruses/Infections
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5
Q

Fibromyalgia: Pathophysiology? dont memorize

A
  • Unknown cause
  • Disorder of altered pain processing & regulation: –“Central sensitization”
  • -Allodynia & hyperalgesia
  • Functional MRI and PET studies
  • Genetic basis
  • Psychosocial factors
  • Neuroendocrine dysfunction
  • Autonomic nervous system dysfunction
  • Stressful events
  • Viruses/infections
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6
Q

Fibromyalgia: Describe allodynia.

A

Allodynia refers to central pain sensitization (increased response of neurons) following normally non-painful, often repetitive, stimulation. Allodynia can lead to the triggering of a pain response from stimuli which do not normally provoke pain.

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7
Q

Fibromyalgia: Describe hyperalgesia.

A

Hyperalgesia is an enhanced pain response. It can result from either injury to part of the body or from use of opioid painkillers. When a person becomes more sensitive to pain as a result of taking opioid medication, it’s called opioid-induced hyperalgesia (OIH).

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8
Q

Fibromyalgia: What imaging studies have supported the little we understand about fibromyalgia?

A
  • Functional MRI and PET studies.
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9
Q

Fibromyalgia: Presentation?

A
  • Chronic pain/stiffness (generalized): *Involves all 4 quadrants of the body
  • *Pain often described as worst around neck, shoulders, low back, and hips
  • Common associated complaints
  • Onset often after acute injury, infection, childbirth, persistent stress, exposure to toxins.
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10
Q

Fibromyalgia: Common associated complaints?

A
  • Sleep disturbance
  • Fatigue
  • Muscle weakness
  • Paresthesias
  • Cognitive disturbance
  • HA
  • Depression
  • Anxiety
  • IBS
  • Dry mouth
  • Pelvic pain
  • Bladder symptoms
  • Tinnitus
  • Multiple chemical hypersensitivities
  • TMJ issues
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11
Q

Fibromyalgia: What is a unique physical exam associated with fibromyalgia?

A
  • Exam of tender points.
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12
Q

Fibromyalgia: Describe the exam of tender points.

A
  • Exam is Normal apart from pain at tender points.

- Apply 4 kg/cm^2 to the points (apply enough pressure to whiten the nail bed of the fingertip of the examiner)

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13
Q

Fibromyalgia: What is the ACR( american college of rheum) Classification Criteria for tender points?

A
  • 9 pairs (18 total) of tender points.
  • 1190 Dx criteria: 11/18 tender points and symptoms of widespread pain (above and below the waist, and both sides of the body)
  • Note control locations.
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14
Q

Fibromyalgia: When performing an exam of tender points, where are the control locations?

A
  • Thumb
  • Mid-forearm
  • Forehead
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15
Q

Fibromyalgia: Where are the anterior tender points for fibromyalgia?

A
  • Under sternomastoid m.
  • Near 2nd costochondral junction
  • 2 cm distal to lateral epicondyle
  • Prominence of the greater trochanter.
  • Medial fat pad of the knee.
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16
Q

Fibromyalgia: What are the posterior tender points for fibromyalgia?

A
  • Insertion of the suboccipital m.
  • Mid-upper trapezius m.
  • Origin of the supraspinatus m.
  • Upper outer quadrant of the buttock.
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17
Q

Fibromyalgia: Labs/Imaging?

A
  • Generally of little benefit to making the dx.
  • Fibromyalgia itself doesn’t cause any abnormalities.
  • CBC, ESR, CRP, TSH would be reasonable initial lab tests
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18
Q

Fibromyalgia: DDx

A 
RA
SLE
Polymyositis
PMR
OSA
Hypothyroidism
Many, many  more
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19
Q

Fibromyalgia Initial approach/initial tx:

A 
Initial approach:
Pt education
Good sleep hygiene (poor sleep often causes worsening pain)
Exercise (low-impact aerobic activity)
CBT??
Meds (next slide)
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20
Q

Fibromyalgia Tx: What medications are not helpful for these patients?

A

**Not helpful:
Opioids
Corticosteroids
NSAIDs

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21
Q

Fibromyalgia Tx: What medications are associated with helping these patients?

A
  • TCAs
  • Cyclobenzaprine (Flexeril)
  • SNRIs
  • SSRIs
  • Anticonvulsants
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22
Q

Fibromyalgia Tx: Examples of TCAs?

A
  • **Amitriptyline (Elavil)
  • nortriptyline (Pamelor)
  • desipramine (Norpramin)
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23
Q

Fibromyalgia Tx: Examples of SNRIs?

A
  • **duloxetine (Cymbalta)

- minacipran (Savella)

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24
Q

Fibromyalgia Tx: Examples of SSRIs?

A
  • fluoxetine (Prozac)
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25
Q

Fibromyalgia Tx: Examples of anticonvulsants?

A
  • **pregabalin (Lyrica)

- gabapentin (Neurontin)

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26
Q

Fibromyalgia Tx: What are appropriate referrals for these patients?

A

-Studies show patients seem to do well with close PCP follow up
-If needed, can refer to:
Psychiatry
Physical Therapy
Rheumatology
Pain Management
Alternative: massage, acupuncture, etc

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27
Q

Fibromyalgia: Prognosis?

A
  • Chronic, but not considered progressive.
  • Minimal or no improvement in symptoms despite variety of treatment modalities.
  • Pts do well with close PCP follow up
  • Most patients are able to work
  • Female gender, low socioeconomic status, unemployment, depression, and obesity associated with worse outcomes
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28
Q

PMR: What does PMR stand for?

A
  • Polymyalgia rheumatica
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29
Q

GCA: What does GCA stand for?

A

giant cell arteritis

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30
Q

Describe PMR

A

Inflammatory condition associated with pain and stiffness of the hips and shoulders

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31
Q

Describe GCA

A

aka Temporal Arteritis: headache, jaw claudication, and visual symptoms associated with elevated ESR that can cause blindness

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32
Q

Frequently GCA and PMR ____

A

coexist

-Thought to represent a spectrum of the same disease

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33
Q

Epidemiology/Risk factors for GCA and PMR:

A
  • Almost always patients >50 years
  • Increases with increasing age
  • Women&raquo_space;men
  • Highest in Scandinavian populations & northern Europeans
  • Less common in Japanese, Black, and northern Indians
  • *PMR much more common than GCA
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34
Q

_____ increases GCA risk

A

smoking

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35
Q

______ decreases risk of GCA

A

Diabetes

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36
Q

Pathophysiology of GCA/PMR:

  • Both PMR and GCA are assoc. with _____
  • affected joints have..?
A
  • Unknown cause
  • Both PMR and GCA are associated with polymorphisms of HLA-DR alleles.
  • Affected joints have lymphocytes and monocytes causing inflammation in PMR
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37
Q

In GCA, there is infiltration of inflammatory cells into the vessels causing a ________

A

-**vasculitis

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38
Q

MC sites of GCA?

A

MC in the thoracic aorta, large cervical arteries, and branches of the external carotid arteries

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39
Q

In GCA, areas of active inflammation can ______

A

thrombose

-as well as fragmentation of the elastic lamina

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40
Q

Clinical Manifestations- PMR

A
  • **Pain and stiffness in the shoulder and pelvic areas
  • -Usually shoulders come first
  • -Morning stiffness & stiffness after activity. “gel phenomenon”
  • -Movement worsens pain
  • -Impaired range of motion
  • -Should have normal muscle strength
  • Nonspecific systemic symptoms: low-grade fever, malaise, weight loss
  • -Can be the first symptoms
  • Bursal inflammation/synovitis
  • -Wrists, knees, sternoclavicular joints can have swelling and/or pitting edema
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41
Q

Classic Sx of GCA:

A

**headache, scalp tenderness, jaw claudication, visual changes (esp. amaurosis fugax or diplopia)

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42
Q

amaurosis fugax=

A

transient vision loss (NEVER normal!!)

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43
Q

Vision loss in GCA usually stems from?

A
  • anterior ischemic optic neuropathy
  • -Occlusive arteritis of the posterior ciliary artery (branch of ophthalmic artery)
  • Chief blood supply to the optic nerve
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44
Q

About half of GCA Pts have ____

A

PMR

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45
Q

Constitutional Sx of GCA

A

fever, fatigue, weight loss, malaise

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46
Q

Atypical Sx of GCA

A

respiratory symptoms (dry cough), neurologic symptoms, otolaryngeal symptoms

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47
Q

Physical Exam-GCA

A
  • Might look ill overall
  • Temporal artery can be thickened, tender, prominent, or normal appearing

-Funduscopic exam:
Can see pallor and edema of the optic disc, with scattered cotton-wool patches and small hemorrhages, or can have a normal funduscopic exam

-Cardiovascular exam:
Asymmetry of pulses in arms, aortic regurgitation murmur, bruits near clavicle if GCA has affected the aorta or major branches

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48
Q

Labs: GCA

A 
-**Elevated ESR/CRP
Anemia
-WBC usually normal
-May have reactive thrombocytosis
-May have abnormal LFTs (esp ↑ALP)
-Albumin can be low
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49
Q

Define elevated Sed rate

A

normal sed rate is less than 20.

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50
Q

DDx PMR

A

Rheumatoid arthritis
Fibromyalgia
Polymyositis
Infections ie: endocarditis, osteomyelitis
Drug-induced myopathy/myalgia (esp. statins)
-RS3PE syndrome (Remitting Seronegative Symmetrical Synovitis with pitting Edema)
-hypothyroidism

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51
Q

DDx- GCA

A 
Takayasu Arteritis
Migraine headache
Meningitis
Retinal Vein or Artery occlusion
Amyloidosis
Small and medium vessel vasculidities
Wegener’s granulomatosis, polyarteritis nodosa, microscopic polyangitis
Malignancy
Sinus infection
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52
Q

Diagnosis for PMR

A
  • Clinical Diagnosis

- Multiple scoring/classification systems exist

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53
Q

GCA dx (what is the gold standard test**?)

A
  • **Temporal artery biopsy is gold standard
  • If GCA suspected, but a unilateral biopsy is negative –> contralateral biopsy
  • Classification criteria does not replace biopsy for definitive diagnosis
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54
Q

Tx PMR

A

-Glucocorticoid therapy:
**Prednisone 15 mg PO daily
(Normal starting doses might range from 10-20 mg)

-If no improvement after 7 days, could ↑ to 30 mg

-If still no improvement, consider a different Dx.
(**Rapid improvement generally seen– alerts you that you have the correct dx)

  • After stable and Sx controlled, start tapering dose
  • *Flares are common–> increase dose and taper slower
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55
Q

Tx GCA

A

-Don’t wait for biopsy results to start treatment!!
-**Goal is to prevent permanent blindness
-Glucocorticoids: **Prednisone 40-60 mg PO daily
–If no response, increase dose
Once controlled (usually 2-4 weeks), start tapering
-No faster than reducing by 10% every 1-2 weeks
-Will need to be on prednisone for months
-Think about osteoporosis prevention!
-Flares common- increase prednisone by 10 mg. Inflammatory markers are helpful (CRP is the first to increase)

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56
Q

Prognosis: PMR

A
  • Good, with steroid therapy
  • Many patients have a self-limiting course
  • Does not cause chronic damage
  • Most morbidity is related to long-term steroid use
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57
Q

Prognosis: GCA

A
  • **If not treated, can have a poor prognosis and lead to permanent blindness
  • Fair number have a chronic course and relapses
  • Association with increased cardiovascular events (MI, CVA, & PVD)
  • Long-term steroid risk has consequences

-Referral for either: rheumatology

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58
Q

Takayasu Arteritis (TA): describe this condition

A

=**Chronic vasculitis mostly affecting the aorta and main branches
–Speculated to be on the spectrum of PMR/GCA with similar pathophysiology

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59
Q

Takayasu Arteritis (TA): demographic

A

Most common in women, Asians

–Onset usually between 10 and 40

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60
Q

Takayasu Arteritis (TA): early sx and later sx

A
  • Constitutional symptoms common early in disease
  • Later, symptoms of vascular insufficiency: Claudication, cool extremities, subclavian steal syndrome can lead to syncope, BP differential, arthralgias, skin lesions, pulmonary manifestations, abdominal pain/diarrhea/GI hemorrhage, angina pectoris
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61
Q

TA PE findings

A

-BP differential (usually at least 10 mmHg), diminished, asymmetrical arterial pulses in arms/legs, bruits, may have synovitis in large joints, renovascular hypertension

62
Q

TA: labs

A

mild anemia, elevated ESR/CRP, low albumin

63
Q

TA: dx

A

Suspected with clinical features, then imaging showing arterial luminal narrowing or occlusion with wall thickening

64
Q

TA: tx

A

Glucocorticoids: **Prednisone 45-60 mg PO Qam initially

  • Taper when symptoms controlled/labs improved.
  • Many need chronic steroids.
  • Other immunosuppresants are second line.
  • Surgical intervention with PCTA (percutaneous angioplasty), bypass grafting, or aortic repair may be needed.
65
Q

TA: prognosis

A

chronic disease that’s relapsing-remitting. 20% with self-limited disease. Vascular involvement generally progressive.

66
Q

Reactive Arthritis (ReA): describe this condition (KNOW)

A

AKA Reiter’s Syndrome or seronegative spondyloarthropathy

=Asymmetric polyarthritis that develops after a GI or GU infection, typically of large lower extremity joints

67
Q

Reactive Arthritis Epidemiology/risks:

A
  • Relatively rare (about 4/100,000)
  • Male >female
  • Typically young adults (most common in 3rd decade)
  • Strong association with HLA-B27 gene (60-80% of patients)
68
Q

Reactive arthritis Pathophysiology: what kind of infection and MC pathogens associated

A
  • GI infection (Shigella, Salmonella, Yersinia, Campylobacter, Escherichia coli, Clostridium difficile)
  • –>Inflammation in gut compromises wall integrity and activate proinflammatory cytokines –> microlesions–> cytokines migrate to joint/entheses

-Sexually transmitted infection (Chlamydia, Ureaplasma urealyticum)

69
Q

Those with ______ are predisposed to developing inflammatory enthesopathy, which is triggered by exposure to certain infectious agents

70
Q

Enthesopathy=

A

refers to a problem with the attachment of tendons, ligaments or components of a joint onto the bone. People with enthesopathy typically experience pain and may have stiffness or difficulty moving the affected joint or area of the body.

71
Q

Clinical Sx/s reactive arthritis

A
  • Sx of diarrhea or urethritis (1-4 weeks earlier)
  • ->May have asymptomatic GU infection
  • **Asymmetric arthritis usually involving large weight bearing joints (knee, ankle) which may persist
  • (+/-) axial arthritis- spine or SI joint (less common)
  • (+/-) enthesitis–> Often seen as swelling at heel
  • (+/-) dactylitis
72
Q

enthesitis=

A

inflammation of the entheses, the sites where tendons or ligaments insert into the bone.

73
Q

Dactylitis=

A

severe inflammation of the finger and toe joints

74
Q

Clinical Manifestations-extraarticular for Reactive Arthritis

A
  • General: Fever, fatigue, weight loss
  • Eye: Conjunctivitis, anterior uveitis
  • Mucocutaneous: Balanitis, stomatitis, keratoderma blennorrhagicum
  • Nails: Mimics psoriasis
75
Q

Balanitis=

A

inflammation of the skin at the end of the penis

76
Q

keratoderma blennorrhagicum=

A

skin lesions commonly found on the palms and soles but which may spread to the scrotum, scalp and trunk

77
Q

DDx Reactive arthritis

A

-Disseminated gonococcal infection
-Rheumatoid arthritis
-Ankylosing spondylitis
-Psoriatic arthritis
-Behҫet disease
-Gout/pseudogout
-Viral gastroenteritis
-Rheumatic fever
Septic arthritis

78
Q

Labs: Reactive Arthritis

A
  • (+/-) elevated CRP/ESR
  • (+/-) positive stool culture or urethral swab
  • Synovial fluid analysis: elevated WBC, mostly neutrophils
  • -Won’t recover causative organism
79
Q

Dx: ReA

A
  • Clinical dx
  • -No single diagnostic test
  • Musculoskeletal findings
  • Pt has a Preceding infection
  • No more compelling explanation for the arthritis
80
Q

Tx: reactive arthritis

A
  • Treat the underlying infection
  • -When pts are given antibiotics for STIs at time of dx, it reduces chance of ReA.
  • Arthritis treatment:
  • *NSAIDs–> Mainstay/initial therapy–Naproxen, diclofenac, or indomethacin for approx. 2 weeks
  • If no response to NSAIDs:
  • Intraarticular glucocorticoids (triamcinolone)
  • PO glucocorticoids (prednisone)
  • DMARD: sulfasalazine or methotrexate
81
Q

Prognosis/Rehab/Referral: Reactive Arthritis

A
  • Most patients have self-limiting disease
  • Typically lasts 3-5 months
  • Most patients remit within 6-12 months
  • May require PT
  • Refer to rheumatology if needed
82
Q

Sjӧgren Syndrome: describe this disease (and 2 MC symptoms)

A

=Systemic autoimmune disease that commonly affects the lacrimal and salivary glands, resulting in mouth **(xerostomia) and eye dryness **(keratoconjunctivitis sicca)

83
Q

Sjӧgren Syndrome: are other organs affected?

A
  • Other organs may be involved as well

- -***Can be primary or secondary to another autoimmune disease

84
Q

Epidemiology/Risk Factors: Sjӧgren Syndrome

A
  • Women>men 10:1
  • Peak is in middle-aged females
  • Seen in all races, Caucasian slightly more common
  • Estimated to affect 0.1-0.6% of adult females

-Tricky to truly estimate, because prevalence depends on classification criteria used.
-Genetic component
HLA-DQ and HLA-DB

85
Q

Clinical Manifestations-Ocular–> Sjӧgren Syndrome

A

keratoconjuntivitis sicca:

  • Diminished aqueous tear production
  • Eyes feel dry, gritty, sandy, foreign body sensation, burning, itching, intolerance to contacts, photophobia, eye fatigue
  • Conjunctival or corneal damage seen on Rose Bengal, lissamine green, or fluorescein staining
  • Schirmer test showing decreased tear production
  • May see mucus filaments, dilation of bulbar conjunctival vessels, or lacrimal gland enlargement
86
Q

What can keratoconjuntivitis sicca progress to?

A

Can progress to corneal ulceration,
bacterial keratitis, eyelid infection with
with destruction of corneal epithelium

87
Q

What is the objective test used to assess for dry eyes?

A

schirmer test (stick a piece of paper in their eye and see how dry their eyes are)

88
Q

What is the objective test used to assess dry mouth?

A

Saxon test or sialometry

89
Q

Clinical Manifestations-Mouth: Sjӧgren Syndrome

A
  • *Xerostomia:
  • “cotton mouth” sensation, dysphagia (esp. dry foods), trouble with prolonged speaking, loss of taste
  • Dental caries, (esp. at gum line), gingival recession, oral candidiasis common, laryngotracheal reflux, chronic esophagitis
  • Salivary gland enlargement can occur
  • –Parotid most obvious
  • Saxon test or sialometry
90
Q

Describe the Saxon test

A

Pt chews on the sponge for 2 minutes (weigh the sponge before and after)

91
Q

Clinical Manifestations of Sjӧgren Syndrome (constitutional, Skin, MSK, Lungs)

A

Constitutional: Fatigue, low-grade fever

Skin: Xerosis, purpura, Raynaud phenomenon, cutaneous vasculitis, angular chelitis, annular erythema

Musculoskeletal: Arthralgia, symmetric, nondeforming, intermittent. Mild myopathy with weakness

Lungs:

  • Dry cough, excessive throat clearing, rhinitis, sinusitis, hoarseness
  • Non-specific interstitial pneumonitis (NSIP) , lymphocytic interstitial pneumonitis (LIP), usual interstitial pneumonitis (UIP), cryptogenic organizing pneumonia (COP)
92
Q

Clinical Manifestations of Sjӧgren Syndrome (cardiac, GI, Renal)

A

Cardiac: Rare, but can have pericarditis, heart block

GI: Atrophic chronic gastritis, celiac disease more common than general population, abnormal LFTs, primary biliary cirrhosis, autoimmune hepatitis

Renal: Chronic interstitial nephritis most common.
Glomerular disease (less common)
Membranoproliferative glomerulonephritis (MPGN) and membranous nephropathy (MN)
93
Q

Clinical Manifestations of Sjӧgren Syndrome (Urogenital, neurologic and psychiatric )

A

Urogenital: Vaginal dryness, dyspareunia, pruritis
Dysuria, urinary frequency, urinary urgency, nocturia in absence of UTI

Neurologic and Psychiatric:
Painful peripheral neuropathy, ataxia, trigeminal neuropathy, mononeuritis multiplex, multiple cranial neuropathies
CNS involvement with focal lesions, chorea, aseptic meningitis, diffuse brain lesions, spinal cord involvement, cognitive dysfunction “brain fog”
-Affective disorders common, especially depression

94
Q

Labs Sjӧgren Syndrome

A
  • **Most patients have positive ANA
  • **Many have positive antibodies for SS-A (anti-Ro) and SS-B (anti-La)
  • **Many positive for RF

-Others: centromere antibodies, anti-CCP, antimitochondrial antibodies
Mild anemia, cytopenia, hypergammaglobulinemia

-May have elevated ESR. CRP can be normal or elevated

-May have abnormal LFTs
If impaired renal function, may have ↑Cr and/or ↓ bicarb.

95
Q

Dx test for Sjӧgren Syndrome

A

-**Salivary Gland Biopsy–> Long considered gold standard
In real life, often saved for patients when diagnosis is unclear

  • ≥4 salivary gland lobules are removed from incision on inner lip
  • Positive if focus score of ≥1 per 4 mm2
  • –A focus is a cluster of ≥50 lymphocytes
96
Q

DDx: Sjӧgren Syndrome

A 
Age-related sicca syndrome
Medication side effects
Chronic Hepatitis C
Sarcoidosis
HIV patients
Sialadenitis
Head and neck radiation
97
Q

How to make a dx of Sjӧgren Syndrome

A
  1. ) The patient has an objective test of dry eye (Schirmer or abnormal surface staining) or xerostomia (Saxon or whole sialometry).
    - Or imaging consistent with glandular abnormalities seen in SS
  2. ) The patient has anti-Ro/SSA and/or anti-La/SSB antibodies, a positive lip biopsy, or an established rheumatic disease
    - Or anticentromere antibodies
    - Or ANA ≥1:320 + positive RF
98
Q

Tx oral Sx: Sjӧgren Syndrome

A
  • Symptomatic
  • Artificial saliva

Mouth lubrication/oral care:

  • Maintain good hydration
  • Sugar free gum & hard candies
  • Pilocarpine or cevimeline
  • Fluoride treatment, regular dental visits, aggressive mouth care

Immunosuppresants-mixed results in studies

99
Q

Tx Ocular Sx: Sjӧgren Syndrome

A

-Environmental management (if they can avoid dry, windy areas)

  • Regular use of artificial tears:
  • -Every 2-4 hours
  • -At night, ocular ointments are helpful
  • If no relief with above, ocular cyclosporine 0.05%–>Can use with artificial tears
  • Topical steroids may be used if fail above, have ocular inflammation, and work with ophthalmologist
  • Punctal occlusion/plugs (block tears from getting out– last case scenario)
100
Q

Tx-Systemic Manifestations: Sjӧgren Syndrome

A

Immunosuppressive therapy:

  • Hydroxychloroquine
  • Methotrexate

You will probably have referred to rheumatology at this point if the patient has systemic disease

101
Q

Sjӧgren Syndrome- Prognosis/referral?

A
  • Depends on extent of involvement
  • Severe exocrine involvement–> higher lymphoma risk–>Usually B cell non-Hodgkin lymphomas
  • At higher risk of other autoimmune diseases
  • Those with extraglandular disease do have a higher M&M
  • Refer if:
  • -Systemic signs & symptoms
  • -Ocular dryness not responding to artificial tears
102
Q

Rheumatoid Arthritis (RA): describe this condition

A

Chronic, systemic inflammatory disorder with synovitis of the joints that can lead to joint destruction, deformity, and disability

103
Q

RA risk factors/epidemiology

A
  • Women>men 3:1
  • About 0.5-1% of adults

-Peak onset:
Women: 4th-5th decade
Men: 6th-8th decade

  • Significant genetic component
  • Smoking significantly increases risk
104
Q

RA Pathophysiology

A
  • **Genetics: HLA-DR MHC genes are the most important.
  • “shared epitope”
  • Synovial tissues are the main target for the autoimmune process–>
  • T cells, B cells, macrophages, synovial cells go to synovial tissues–>
  • Synovial tissue proliferates (synovitis), there is excess synovial fluid, and a pannus forms –>
  • Pannus invades nearby bone and cartilage, destroying them and stretching the joint capsule, causing destruction & deformity.
105
Q

Clinical Manifestations-Joints RA

A
  • Usually insidious onset
  • ***Morning stiffness >30 minutes
  • May recur after daytime inactivity
  • Symmetric swelling of many joints
  • Tender, painful joints
  • **Joints most often affected: PIPs fingers, MCPs, wrists, ankles, knees, MTPs
  • May have synovial cysts, tendon rupture, or entrapment syndrome
106
Q

Clinical Manifestations-Joints of Hands RA

A
  • Ulnar deviation of MCP joints is classic
  • Swan neck deformity
  • Boutonnière deformities
  • Z deformity
107
Q

Describe a swan neck deformity

A

Hyperextension of PIP, flexion of DIP

108
Q

Describe Boutonnière deformities

A

Flexion of PIP, extension of DIP

109
Q

Describe Z deformity

A

Hyperextended interphalangeal joint

110
Q

Clinical Sx/s: RA

  • General?
  • skin?
A

General: Fatigue, weight loss, low-grade fever

Skin: 
Rheumatoid nodules:
-Subcutaneous nodules
--**Almost only in those RF positive
-Often on extensor surfaces (esp. forearms), over joints, or pressure points
-Firm, not tender
-May also be in lungs, sclerae, other tissues
-Vasculitis
111
Q

Clinical Sx/s: RA

-ocular?

A
  • Keratoconjunctivitis sicca (secondary Sjӧgren Syndrome)

- Scleritis/episcleritis, scleromalacia

112
Q

Clinical Sx/s: RA

-pulmonary?

A

Pleural effusions
Rheumatoid nodules
Interstitial lung disease

113
Q

Clinical Sx/s: RA

A
  • Chronic inflammation increases risk for CV disease
  • Pericardial effusions
  • Pericarditis
114
Q

Felty Syndrome:

classic triad

A
  • Splenomegaly
  • Neutropenia–> Could be asymptomatic or have recurrent bacterial infections
  • RA–>Typically seropositive, erosive, and severe
115
Q

Labs: Felty Syndrome

A

-**Anti-CCP antibodies–>Most specific bloodwork for RA. Associated with aggressive disease.

  • **Rheumatoid Factor (RF)–> Associated with more severe disease
  • About 15% of patients are seronegative.
  • **Elevated ESR/CRP
  • May see mild anemia, thrombocytosis, WBC normal or mild leukocytosis

-Synovial fluid: inflammatory effusion, leukocytes usually 1500-25,000/cubic mm, PMNs predominate

116
Q

Imaging studies: RA

  • early on in the disease? vs
  • Findings late in the disease?
A

-Radiographs are most specific
–If early in disease, likely normal
-Initial: soft tissue swelling, osteopenia around joint
Earliest changes show in wrists or feet

-Later: Joint space narrowing and erosions

117
Q

What is the most sensitive imaging study for RA?

A
  • MRI (More sensitive than radiography)–>May detect erosions earlier in the disease course
  • Ultrasound: Also more sensitive than radiographs
118
Q

DDx RA

A 
Osteoarthritis
PMR
Psoriatic arthritis
Chronic tophaceous gout
CPPD deposition disease
SLE
Rheumatic fever
Reactive Arthritis
119
Q

Dx: RA

A
  • Inflammatory arthritis involving ≥ 3 joints
  • Positive RF and/or anti-CCP
  • ->If seronegative, can still diagnose RA if you have excluded other causes and all other characteristics met.
  • Elevated ESR and/or CRP
  • Duration of ≥ 6 weeks
  • You have excluded other causes
120
Q

RA treatment goals

A
  • Control pain and inflammation
  • Preserve function
  • Prevent deformity
  • ***Early diagnosis and initiation of DMARDs (Disease-modifying antirheumatic drugs) is important!!
  • Target of treatment is remission or low disease activity
  • Rheumatologist involvement
  • Patients often need combinations of DMARDs
  • ->**MTX + TNF inhibitor most common
121
Q

RA: pretreatment screening

A

Baseline CBC, Cr, LFTs, ESR, CRP
Ophthalmic screening
Check for latent TB
Baseline radiographs

122
Q

NSAIDs as tx for RA? y/n?

A

Help with symptoms, but don’t alter disease course

  • -Not for monotherapy
  • All are about equal
123
Q

Corticosteroids for RA tx

A
  • Very helpful for both Sx relief and slowing the rate of joint damage
  • Not recommended for monotherapy or long-term use
  • **Good as a bridge while starting a DMARD.
  • Taper as soon as able.
  • **Start with prednisone 5-20 mg/day, depending on how severe the Sx
124
Q

Conventional DMARDs (list examples)

A

**Methotrexate
*Sulfasalazine
*Hydroxychloroquine
Leflunomide
Gold (intramuscular & oral)
Azathioprine
Minocycline
Cyclosporine
Penicillamine

125
Q

Biologics (ex’s)

A

Infliximab (Remicade)
Adalimumab (Humira)
Golimumab (Simponi)
Certolizumab (Cimzia)

Abatacept (Orencia)
Rituximab (Rituxan)
Tocilzumab (Actemra)

126
Q

What is the initial DMARD of choice for tx in RA pts?

A

Methotrexate

127
Q
  • Starting dose of Methotrexate
  • S/E?
  • all Pts on methotrexate MUST take ____ ____
A
  • Normal starting dose is 7.5 mg PO weekly
  • Max dose 20-25 mg/wk
  • Usually note improvement within 2-6 weeks.
  • Contraindicated: pregnancy (teratogenic), liver disease, heavy alcohol use, severe renal impairment

-S/E: GI upset, stomatitis

Needs close monitoring:

  • CBC to monitor for cytopenias
  • LFTs for hepatotoxicity

-**All should take folic acid 1 mg PO daily or leucovorin calcium 2.5-5mg weekly 1 day after taking MTX to prevent hematologic & other s/e.

128
Q

TNF inhibitors (which one is 1st line, and when should they be prescribed?)

A

-SQ or IV
-Expensive!!
-Well tolerated
Have a much higher risk of serious bacterial infections, as well as granulomatous infections (esp. reactivation of TB)
-MUST screen for latent TB prior to starting
-Can worsen CHF
-Work really well, esp. for those with severe disease
-Etanercept generally 1st choice.

129
Q

Follow-up: RA pts

A
  • Assess Sx and functional status at all visits
  • Variety of scales that can be used. Pick one, and be consistent
  • Monitor lab work for disease activity and potential toxicities of medication
  • **Follow radiographs every 2 years
130
Q

Prognosis: RA

A

-Prior to use of MTX, very poor prognosis: morbidity, mortality, and financial loss

  • With successful treatment options, less deformity, joint surgeries, morbidity & mortality
  • Patients will have disease flares
  • Higher mortality of those with RA attributed to cardiovascular disease from chronic inflammation

-**Poor prognostic factors: RF or anti-CCP positive, extraarticular disease, functional limitation, erosions on radiograph

131
Q

Polyarteritis nodosa (PAN): describe this condition

A

***Systemic necrotizing vasculitis that usually affects medium-sized or small muscular arteries

132
Q

PAN: which organs are affected? which are spared?

A
  • Can affect any organ, but skin, muscle, peripheral nerves, kidneys, GI tract, heart are commonly affected.
  • **Lungs usually spared
133
Q

Epidemiology/Risk Factors: PAN

A
  • Slightly more common in males
  • Can occur at any age, but more common in 40-60 year olds
  • No racial group especially affected
  • Incidence 1/100,000 people yearly
  • *Associated with Hepatitis B
134
Q

Pathophysiology: PAN

A
  • Most cases are idiopathic
  • -Some associated with Hep B, Hep C, and hairy cell leukemia–>Called secondary PAN in these cases
  • The inflamed wall of the vessel thickens and lumen narrows –>less blood flow, thrombosis more likely–> ischemia or infarction of tissue
  • Inflammation can also weaken the vessel and result in aneurysm
  • ***Veins are not involved
135
Q

Systemic Sx: PAN

A
  • Fatigue, arthralgias, fever, weight loss, weakness

- Pain in the extremities is often an early feature

136
Q

Classic symptoms of PAN: skin

A
  • **Lower extremity ulcerations classic
  • Subcutaneous nodules
  • Livedo reticularis
  • Bullous or vesicular eruption
  • Palpable purpura
  • May see infarction or digital gangrene
137
Q

Renal Sx: PAN

A
  • **Kidney is the MOST commonly affected organ
  • **Hypertension and renal insufficiency
  • If severe, renal infarct
  • Perirenal hematoma from rupture of renal arterial aneurysms
138
Q

GI Sx: PAN

A

-**Abdominal pain common
Esp. after meals. “intestinal angina”
-Nausea/vomiting/diarrhea
-Infarction can lead to acalculous cholecystitis, bowel infarction, perforation, necrotizing pancreatitis, etc.

139
Q

Cardiac Sx: PAN

A
  • MI

- CHF from uncontrolled HTN or from ICM

140
Q

Lungs Sx: PAN

A

not involved

141
Q

MSK Sx: PAN

A
  • Pain in extremities, esp. early in disease process
  • Arthralgia, myalgia
  • Muscle weakness
142
Q

Eye Sx: PAN

A

Retinal hemorrhage
Visual impairment
Optic ischemia

143
Q

Nervous System Sx: PAN

A
  • Mononeuritis multiplex
  • Foot drop
  • Usually sensory problems 1st, then motor deficits
  • CNS involvement (less common): encephalopathy, seizure, stroke

Other: Ischemic orchitis

144
Q

Mononeuritis multiplex=

A

nerve damage to 2 different nerves in different parts of the body

145
Q

Ischemic orchitis:

A

Ischemic orchitis may result from damage to the blood vessels of the spermatic cord during inguinal herniorrhaphy, and may in the worst event lead to testicular atrophy.

146
Q

DDx: PAN

A

-Infectious diseases causing vascular inflammation or mimicking vasculitis–> Hep B/C, infectious endocarditis, HIV, mycotic aneurysm with embolization

-Systemic vasculidities:
Wegener’s, HSP, drug-induced vasculitis, Churg-Strauss, microscopic polyangitis

-Other diseases:
Atherosclerosis, thrombotic disorders, embolic disease, vasculitis from a connective tissue disease (ie: RA, SLE)

147
Q

Labs: PAN

A 
-Elevated CRP/ESR
May have abnormal LFTs/Hepatitis panel
- (+/-) elevated Cr (due to renal fx impaired)
-Anemia
-CK may be elevated
148
Q

Dx PAN (KNOW!!)

A
  • **Biopsy of an involved organ
  • -> “Necrotizing inflammation of medium-sized arteries”**

Angiogram: Many small aneurysms. **“rosary sign”
-Irregular constrictions of larger vessels and occlusion of smaller vessels

-CT and MR angiography also helpful to show extent of organ involvement

149
Q

Tx: PAN

-mild disease?

A

Does the patient have viral hepatitis?
If so, treat with antiviral, limit length of steroids.

Mild disease:
-Corticosteroids alone–> Prednisone 1 mg/kg daily (max 60-80 mg/day). Taper after 4 weeks if improved
If resistant/intolerant to steroids, methotrexate, azathioprine also used

150
Q

Tx PAN: Mod-severe disease

A

Initial: high-dose corticosteroids- Prednisone + immunosuppressant (cyclophosphamide) (IV or oral)

  • If life- or organ-threatening, IV methylprednisone
  • Switch off cyclophosphamide when possible to another immunosuppressant. Max 12 months.
151
Q

Prognosis: PAN

A
  • Without treatment, poor prognosis (10%- 5 year survival)
  • With treatment, 80%
  • If successfully treated, relapses less common (20%)

Worse prognosis:

  • CKD with Cr >1.6
  • Proteinuria >1g/dL
  • GI ischemia
  • CNS disease
  • Cardiac involvement

Clin Med semester 2 (24 decks)

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