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Senoir 21-2 > RHeum > Flashcards

RHeum Flashcards

1
Q

Percentage of patients WITH the disease who have a Positive Test Result

A

Sensitivity

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2
Q

Percentage of patients Without disease with Negative Test Result

A

Specificity

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3
Q

What is the classic threshold for an elevated ESR

A

ESR > 100 generally means significant cancer, serious infection, renal or autoimmune disease

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4
Q

Is CRP relevant in Lupus

A

No

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5
Q

In chronic rheum d/o what will the complement be..

A

Hypo

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6
Q

Define spectrum of dz characterized by inflammation of the sacroiliac (SI) joints, spine, or peripheral joints

A

Spondyloarthropathies or Spondyloarthritis (SpA)

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7
Q

What is the hallmark feature of Ankylosing Spondylitis

A

Hallmark feature is LBP which improves with activity or exercise, worsens with rest

  • Awakens pt at night
  • Generally younger than 45 years of age

+Chest Pain

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8
Q

Eye S/s with ankylosing spondylitis

A

Acute anterior uveitis (inflammation of the iris, ciliary body & choroid) present in up to 50% over the disease course

Most common extra-articular manifestation

Typically abrupt, unilateral, intense pain, redness, photophobia

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9
Q

TTP over the sacral/illeal joint

Think

A

Ankylosing spondylitis

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10
Q

What would you expect to see on labs in a pt with Ankylosing Spondylitis

A

CRP elevated in 30-50% of patients with ankylosing spondylitis

Negative RF

Mild, normocytic anemia of chronic disease

Elevated alkaline phosphate
+ HLA-B27
—Not required for diagnosis

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11
Q

Pain greater than 3 months with age less than 45

HLB27 postive w/ >2 SpA features
Or
Sacroillitis and and >1 SpA feature

Think

A

Ankylosing Spondylitis

SpA features: 
Inflammatory Back Pain 
Arthritis 
Enthesitis 
Uveitis 
Dactylitis 
Psoriasis 
Crohns 
Good response to NSIADS 
FamHx 
HLBA27 
Elevated CRP
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12
Q

What is an independent RSK fx for mortality in Ankylosing spondylitis

A

Cervical spine fx is an independent predictor of inpatient mortality

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13
Q

Tx approach to Ankylosising Spondylitis

A

1) NSAIDs
- very good response
2) PT
- 1st line Tx (Active>passive)
3) Steroids
- Directed at the SI joint
4) DMARDs
- Sulfasalazine/ Methotrexate
5) Biologics
- 2nd line, TNF Inhibitors -amaubs
6) Surgery

(Refer, stop smoking, Rhemo and Ortho)

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14
Q 
HLBA 27 (Ankylosing Spondylitis) 
\+ Gastro S/s

Refer to

A

Gastro (IBD)

And Rheum

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15
Q

What is the common Gastro commorbidity with Arthritis

A

IBD

Chrons or Ulcerative Colitis

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16
Q

What is the approach to IBD associated Arthritis

A

NSAIDs avoided as first line because they may lead to exacerbations of IBD.

TNF inhibitors are recommended for active axial ankylosing spondylitis w/ concomitant IBD

Should be co-managed by rheumatology & gastroenterology

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17
Q

Pt that cant pee, cant see, cant bend the knee

A

Postinfectious Triad:

  • Conjunctivitis
  • Arthritis
  • Urethritis

Dx for Reactive Arthritis

Develops days to weeks following GI/GU infection

Typically Monoarthritis

In a young male pt mc with chlamydia

Hallmark: Enthesitis: inflammation at sites where tendons attach to bone, MC at Achilles

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18
Q

This finding in a pt with reactive arthritis

A

Keratoderma blennorrhagica:

erythematous macules and vesicles, progressing to papules, hyperkeratotic plaques, and pustules

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19
Q

Classic clinical triad of Reactive Arthritis

A

Classic Clinical Triad
Conjunctivitis (or uveitis), Urethritis, Oligoarthritis
Pts can also present with a 4th: Mucocutaneous lesions (Apthous)

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20
Q

What is the lab to help R/o septic arthritis and gout for reactive arthritis

A

Arthrocentesis should be performed to exclude septic arthritis & gout
-Usually WBC counts of 5000-50,000

-Predominantly polymorphonuclear cells and the synovial fluid is sterile (negative Gram stain & culture)

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21
Q

What is the Tx approach to Reactive Arthiritis

A

NSAIDs: First choice for articular manifestations (ibuprofen/naproxen)

Intra articular glucocorticoids may be considered for peripheral arthritis involving few joints.

Systemic steroids have limited benefit for axial symptoms

Ophthalmology referral if uveitis is present

Antibiotics: Appropriate short term therapy if infection remains active. Does not improve the arthritic symptoms.

Consider DMARD (sulfasalazine or methotrexate) for those that fail NSAIDS and glucocorticoids

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22
Q

Psoriatic Arthritis on X-ray

A

Radiographic findings of erosions, osteolytic destruction of the interphalangeal joints, and juxta-articular new bone formation

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23
Q

What is a distinguishing feature of psoriatic arthritis

A

Enthesitis is a distinguishing feature in the pathogenesis of psoriatic arthritis

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24
Q

Psoriatic Arthritis is most common to what joints

A

Fingers (DIP) with stiffness, swelling, nail pitting

Distinguishes from RA

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25
Q

What are the 5 manifestations for Psoriatic Arthritis

A

Articular involvement

Dactylitis
(uniform swelling of the digit)

Enthesitis

Skin and nail changes

Extra articular manifestations
-HTN, T2DM, Fatty liver, Occular inflammation, IBD

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26
Q

What is the most common finding in psoriatic arthritis

A

MC finding is joint –space narrowing & erosions

Distinguished from RA by the absence of juxta-articular osteopenia & presence of pathologic new bone formation

“Pencil-in-cup” (sharpened point) appearance to DIP with Arthritis Mutilans

Periostitis in MCP, MTP, phalanges (“fluffy” appearance)

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27
Q

What is the treatment approach to psoriatic arthritis

A

Treat the s/s of inflammation

-NSAIDs (1st line
-Steroids ( rheum consult)
-DMARDS (rheum consult)
MC Methotrexate)

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28
Q

RA spares what joint?

A

DIP and typically spares the spine

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29
Q

When is RA worst

A

Morning Stiffness

  • Hallmark of inflammatory arthritis and RA
  • Worse in AM or after prolonged periods of rest
  • Lasts for hours and improved with activity
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30
Q

Boutonnière deformity

A

(“knuckle being pushed through a buttonhole”)

flexion of the PIP joints and hyperextension of the DIP joints

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31
Q

Swan Neck deformity

A

hyperextension of the PIP joints and flexion at the DIP joints

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32
Q

What is the triad of Fetly Syndrome (RA)

A

RA
Splenomegaly
Leukopenia
(usually neutropenia, <2000 mm3)

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33
Q

What is the hallmark lab in RA

A

Anti-CCP antibodies: present 60-70%

Rheumatoid Factor: present ~50% at presentation

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34
Q

How do you start DMARDs for RA

A

Bridge tx

Takes 2-6 months to read peak effect

Typically 5-10mg prednisone daily while titrating DMARD to effect

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35
Q

What are the ADE of methotrexate

A

This is the anchor of Rx for RA

ADE: Bone marrow suppression and development of pneumonitis

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36
Q

Hydroxychlorquine

A

Usually used in conjunction with other synthetic DMARDS (i.e. methotrexate)
-Least toxic, but least effective as stand-alone tx
-Retinal toxicity—uncommon, but serious
-Increased risk over time—cumulative dose
—Initial eye exam and annual eye exams after 5 yrs of tx

Monitor: CBC, LFT, BMP, UA w/ micro-

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37
Q

Leflunomide

A

DMARDS

Comparable to Methotrexate
-Teratogenic, hepatic toxicity

Monitor: Pregnancy test, CBC, LFT, TB (baseline), hepatitis screening panel (baseline), blood pressure at baseline

Long half life—women need blood tests drawn prior to pregnancy even if stopped med years prior

Use cholestyramine to eliminate leflunomide if woman is considering to become pregnant

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38
Q

What are the 4 things biological DMARDs do

A

Inhibit Tumor Necrosis Factor
(anti TNF agents)

Deplete CD20+ B Cells
(rituximab)

Inhibit T-Cell costimulation (abatacept)

Block the receptor for interleukin-6 (tocilizumab)

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39
Q

What is the absolute C/I for Biological DMARDs

A

Active TB or Untreated latent TB is an absolute contraindication to use of biologic DMARDs

Reactivation of latent TB has occurred within weeks of starting anti-TNF agents

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40
Q

Tx approach to RA

A

RA should be diagnosed early and DMARD started at time of diagnosis

  • Treatment goal of remission or low activity
  • Meeting treatment goal more important than medication choice
  • Most patient will need combo DMARD therapy. Typically methotrexate + something else

NSAIDS and glucocorticioids should be used sparingly as needed or a bridge therapy

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41
Q

DONT FORGET THESE SCREENS FOR RA tx

A

Consider phone consultation first to initiate therapy until patient is seen

Test for TB, Hepatitis B, C, HIV etc

Immunizations prior to immunosuppression (pneumococcal, flu)

Bridge communication between Rheumatologist and PCM

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42
Q

Define scleroderma

A

Scleroderma = Hard Skin
-systemic autoimmune disease characterized by varying degrees of skin fibrosis, vascular damage, and a wide array of internal organ dysfunction.

Two Main Manifestations
-Fibrotic dz (skin tightening & interstitial lung dz (ILD)
—Limited cutaneous (confined to fingers, toes & face)
—Diffuse cutaneous (skin tightening extends proximal to elbows, knees & trunk)

Vascular dz (Raynaud phenomenon & pulmonary arterial HTN (PAH)

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43
Q

What are the two prominent S/S in scleroderma

A

Raynuads and ANA Abs

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44
Q

What is the most common cause of death in scleroderma

A

Most common cause of death is cardiac involvement (~30%) followed by lung dz (~25%)

All require evaluation by a rheumatologist

45
Q

What define diffuse scleroderma

A

Raynaud presentation at onset of scleroderma Dx

Skin tight ting at the proximal extremity AND TRUNCAL

More ACS, More valvular disorders, Pulm HTN group 2 and 3.

SEVERE GI DYSMOTILITY->
gut Failure

MORE MORE MORE SEVERE

46
Q

What labs are specific to diffuse scleroderma

A

ntitopoisomerase (Scl-70),

anti-RNA polymerase III

47
Q

What defines LIMITED scleroderma

A

Calcinosis!

Skin tightness DISTAL TO THE ELBOWS

And Reynauds SEVERAL YEARS!!! Before onset of scleroderma!

PULM HTN GROUP 1

LESS FREQUENT S/s
(Rare Myocardial Dysfuntion, rare renal crisis)

48
Q

What labs are specific to LIMITED scleroderma

A

Anticentromere,

anti-Pm-Scl, anti-Th/To

49
Q

What is CRESTS syndrome

A

Calcinosis,
Raynaud phenomenon, Esophageal dysmotility, Sclerodactyly, & Telangiectasis

Dx for LIMITED Scleroderma

50
Q

What is the treatment approach to Raynauds

A

Cold avoidance

CCB

If 30 years old and new onset= Scleroderma!
Screen for ANA and nail fold capillary examination

51
Q

In Sclerodrema Large pericardial effusions are associated with pulmonary arterial hypertension

Means

A

Poor prognosis

52
Q

Pulmonary S/s in Sclerodrema

A

Active interstitial lung disease (ILD) occurs in pts with early diffuse scleroderma in 1st four yrs of illness

Pulmonary hypertension occurs over time ->long-standing disease

53
Q

PFTs w/ Scleroderma

A

PFTs: 80% have restrictive pattern consistent with interstitial lung disease

High-resolution CT scanning: Interstitial fibrosis

54
Q

GI manifestations of Scleroderma

A

Dysphagia
Severe reflux/GERD

If left untreated can lead to esophagitis, esophageal ulceration with bleeding, esophageal stricture, or Barrett esophagus

55
Q

Scleroderma Renal Crisis

A

Occurs in only minority of pts
Incidence = 5% of scleroderma patients

More common in Diffuse Type Scleroderma(10-15%)

Abrupt onset of hypertension—malignant hypertension
-MOST DREADED complication of systemic sclerosis

10% present without hypertension and have poor outcome

  • Elevated creatinine, proteinuria, hematuria
  • Onset usually within 3-5 years of disease onset
56
Q

What is the treatment for Sclerodermal Renal Crisis

A

EARLY Tx with an ACE!

57
Q

Do we give Scleroderma pts get steroids?

A

NO!! Defer to specialist!!

58
Q

Pulm fibrosis is more specific to what type of scleroderma

A

Diffuse!!

59
Q

Money maker imaging for scleroderma

A

High res Chest CT

60
Q

Cardiac screening for Scelroderma

A

Cardiac – EKG / Echo / Stress testing

Function/Ischemia
-Screen for pulmonary hypertension w/ annual transthoracic echocardiogram (TTE)

61
Q

PFT f/u for scleroderma

A

Baseline and every 4-12 months depending on stage of disease and pt symptoms

62
Q

Tx for scleroderma

A

Early Skin treatment:
mycophenolate mofetil (MMF) and cyclophosphamide (CYC)
(also used to treat PULM s/s)
(may progress to pulm transplant)

Treat skin pain with: gabapentin

DO NOT USE STEROIDS!

RITUXIMAB emerging Tx!!
(very promising)

Raynauds: Avoid cold and stress,
Rx: Dihydropyridine CCB
(NOT VERAPAMIL AND DILTIZAEM!)

Encourage to eat small frequent meals to control GI S/s 
-GERD:PPI/ H2 blockers 
-Delayed Gastric Emptying: 
Metoclopramide/ erythromycin 
(Gut stimulating)

Octreotide: IBS s/s (severe)

Rotating ABX for diarrhea

And PROBIOTICS FOR ALL PTS!!
(yogurt)

Maintain MOBILITY with PT

Arthritis/ Arthralgias: Methotrexate!

63
Q

A pt presents with Raynauds, GERD, Skin changes, Swollen fingers/ calcifications, and arthralgias

What is the approach

A

Sclerodrema W/u

Negative ANA= seek other Dx

64
Q

Define Chronic inflammatory disorder mainly characterized by lymphocytic infiltration of exocrine glands prominently.

Usually presents as persistent dryness of the mouth and eyes due to functional impairment of the salivary and lacrimal glands

A

Sjogren Syndrome

65
Q

Primary vs Secondary Sjorgens Syndrome

A

Primary – SjS in absence of another rheumatologic disease

Secondary (also known as “associated” SjS) –
-SjS with another rheumatologic disease (usually RA, systemic sclerosis, or SLE)

66
Q

What is the typical pt with sjorgerns syndrome

A

Primarily affects white perimenopausal women

67
Q

What is the KEY feature in Sjorgrens

A

SICCA features: Xerostomia

Occular dryness, and eye “fatigue”

Salivary gland dysfunction and edema

68
Q

What is the endocrine co-morbid with Systemic SJS dz

A

Thyroid Dz

69
Q

What are the specific labs for primary SJS. And Secondary SJS

A

Primary: Anti Ro.

Secondary; Anti La

70
Q

CBC findings for SJS

A

Normochromic, normocytic anemia

Isolated cases of hemolytic anemia

Mild leukopenia, lymphopenia, neutropenia

Mild thrombocytopenia

71
Q

Hypergammaglobulinemia
Monoclonal band

=

A

SJS , with high incidence rate of lymphoma and cancer

72
Q

Treatment for SJS

A

DRY eye: preservatives free art. Tears

  • punctual occlusion
  • topical cyclosporine (consult rheum)

Dry Mouth: sialogogues (Pilocarpine)
Lemon Drops
-meticulous oral hygiene

AVOID ETOH AND TOBACCO
AVOID ANTIHISTAMINES

Prednisoe for arthritis
Hydroxychlorquine for mild arthritis

73
Q

Pathognomonic rash assoc with dermatomyositis

A

Heliotrope rash / Gottron sign

74
Q

Most common S/s of Inflammatroy Muscle Dz

A

PROXIMAL muscle weakness

NOT DISTAL IN ISOLATION

75
Q

What are Gattrons papules and Gattrons Sign

A

Gattron’s papules: raised, violaceous lesions on the extensor surfaces
MC on MCP, PIP & DIP
(Looks like psoriatic)

Gattron’s sign: erythematous rash involving extensor surfaces

76
Q

Define: red or purplish discoloration of eyelids

-This may appear as hyperpigmentation in dark skinned patients

A

Heliotrope Rash

ONLY WITH DERMATOMYOSITIS

77
Q

Major Dx criteria for Inflammatory Muscle Dz

A

Muslce Bx (proximal)

+ elevated muscle enzymes
(CK, Aldolase, AST/ALT/ LDH)

Abnormal EKG

And characteristic Rash
(Dermatomyositis only

78
Q

What two Anitbodies are found in Inflammatory Muscle Dz

A

Anti-Jo 1 antibodies (20-30%)

ANA (85-95%)

79
Q

What is the 1st like treatment for proximal inflammatory muscle dz

A

High dose prednisone, tapered down over a year

Caution : can cause steroid induced myopathy

80
Q

What are the add on Rx for pts that do not recover with in 2-3 months of Tx with Predinose in Inflammatory muscle Dz.

A

Azathioprine

Methotrexate (caution of ILD)

Myclophenolate mofetil

Rituximab

IVIG

Cyclophosphamide

81
Q

CANT LEAP Rx for GOUT

A 
Cyclosporine
Alcohol
Nicotinic acid (Niacin)
Thiazide diuretics
Lasix (loop diuretics)
Ethambutol
Aspirin
Pyrazinamide
82
Q

Gout on US

A

Double contour sign

Superficial, hyperechoic band on the articular cartilage

83
Q

Treatment appraoch to gout

A

Start with MAX dose of NSAIDS

Indomethacin (or other NSAIDs)

+ colchicine (1.2 mg at first flare) followed by a half some with in an hour
(Max dose of 1.8 mg/ hr)

84
Q

Prophylaxis colchicine dose for gout

A

Patients receiving prophylaxis therapy may receive treatment dosing; wait 12 hours before resuming prophylaxis dose

Prophylaxis:
0.6 mg once or twice daily; maximum: 1.2 mg/day

85
Q

Approach to Urate Lowering Tx for gout

A 
One or more tophi 
Frequent attacks
-2+ attacks a year
CKD stage 2 or worse
Nephrolithiasis

Goal of uric acid <6.0
-Maintaining the serum level at this target allows precipitated crystals to dissolve and be cleared
(Goal of less than 5 if tophi present)

Agent of choice: Allopurinol

86
Q

In gout tx. This rx is Reserved for those with severe gout with abundant tophaceous deposits.

A

Pegloticase

87
Q

What is the most common way to classify primary vasculitis

A

Vessel Size

88
Q

Pt from turkey with a vascular dz

Think

A

behcets

89
Q

What is Mono-neuritis multiplex

A

Foot drop or wrist drop

90
Q

What is the most common vasc dz in adults

A

Giant cell Arteritis

Pts are over the age of 50 and more commonly female

91
Q

A pt presents with HA and Jaw Claudication, scalp tenderness, with visual changes, and polymyalgia Rheumatica

Think

A

Giant cell Arteritis

ESR >50 (dx criteria)
Age over 50
New headache
Bx with tissue!!

Treat urgently with HIGH DOSE Steroids and admit if vision changes.
(and prophylax for j. Jevichie pneumonia)

92
Q

Tx for Giant Cell

A

If vision changes:
Admit and IV-> PO steroids (methylpredinsone)

If no vision changes:
Prednisone

Taper Steroids down after ESR returns to normal (10% taper per week)

93
Q

A pt presents with “feeling old” and muscle pain, NOT IN THE JOINT. In the neck or torso, shoulder or proximal regions of the arms, hips or proximal aspects of the thighs

ESR greater than 40.

Think

A 
Polymyalgia Rheumatica 
(DDX with GIANT CELL= NO EYE INVOLVEMNT)

Treat with prednisone

94
Q

What are the major prognosis complications for Giant Cell

A

Perm. Blindness and Thoracic Aneurysm and Aortic Dissection

95
Q

A pt presents with UE Claudication, typically a woman, arms hurt when she brushes her hair, ect

Think

A

Takayasu’s

Look for Abnormal pulse exam / unequal blood pressures

Often involved with Aortic Regurg and Angina

HIGH ESR AND CRP

96
Q

What is the treatment for Asians with Takayusu

A

High dose prednisone 1mg/kg/day x 1 month ->
Taper to 10mg/day over 4-6 months

Very effective but relapse is common

Addition of Methotrexate or Mycophenolate Mofetil to prednisone may provide added benefit

May be good candidates for Surgical revasc surgery

10 year survival rate 80-90%

97
Q

A pt presents with HLA B51, + painful aphtous ulcers of the mouth and genitals
+erythema nodosum and vision changes

Think

A

Behcet

Mc to people in turkey

Treatment start with steroids and refer

98
Q

A pt presents with necrotizing inflammatory vasculitius of the medium arteries that spares the venous circulation and capillaries

Insidious onset with a Hx of Hep B w/in 6 months

SPARES THE LUNGS (DDX with microscopic polyangitis )

A

Think Polyarteritis Nodosa

WILL BE ANCA NEGATIVE!

Test for Hep B

And Get a Bx (fibroid necrosis)

Tx with High dose steroids (+cyclophosphamide if severe)

99
Q

A pt presents with severe digital ischemia without internal organ involvement
That is a chronic smoker with ulcerations

Think

A

Thromboangitits obliterans (buergers)

  1. recognition of clinical findings-digital ischemia without other organ involvement
  2. identification of typical pattern in angiography
  3. exclusion of diseases that may mimic Buerger
  4. confirmation that ongoing tobacco exposure is present

Tx: STOP SMOKING!

100
Q

What is the 2nd MC vasculitis in childhood

A

Kawasakis

101
Q

A pt 3-5 years old presents with Bilateral nonexudative conjunctivitis, Oral changes (cracking lips, strawberry tongue, erythema of oral & pharyngeal mucosa)

Peripheral extremity changes (erythema/edema of hands/feet, periungual desquamation)

Polymorphous rash, Cervical lymphadenopathy (least common feature)

Think

A

Kawasakis

Tx with IVIG quickly + aspirin

If no improvement add cyclosporines

If all tx have failed then add of Steroids

102
Q

Two types of small vessel vasculitis

A

Immune complex vs ANCA

All with have the classic Palpable purpura

103
Q

What is the MC Vasculitis in Children

A

Henoch-schonlein purpura

Tetrad: purpura, arthritis, glomerulonephritis, and abdominal pain is often observed.

Dx Criteria: 2/4 needed

  1. Palpable Purpura
  2. Age less than 20
  3. Bowel Angina
  4. Vessel wall granulocytes on Bx
104
Q

Tx for Hosoch-Schonlein Purpura

A

DAPSONE!

No NSAIDs!

105
Q

All pts with Cryoglobinemia will have

A

Postive RF factor, and palpable purpura

Assoc with multiple myeloma and Hep C!

106
Q

A pt has cryoglobulins in the blood with abs to Hep C or Hep C RNA

Think

A

Dx of Cryoglobinemia

Treat the underlying HepC
-Ledipasvir or Ribavarin

And antivirals: RITUXIMAB

Can evolve to B cell lymphoma

107
Q

A pt presents with a necrotizing vasculitis WITH an URI, lower RI and renal manifestations

Think

A

Granulomatosis with polyangitis

(Saddle nose deformity and ANCA positive)

Order a CT

And treat with Steroids + RITUXIMAB
(Methotrexate and steroids if limited)

108
Q

All pts on prolonged steroids should get prophylaxis Rx for …

A

All should receive Pneumocystis jiroveci pneumonia prophylaxis
-Sulfamethoxazole-trimethoprim, Dapsone

109
Q
A

Aka Churg-Strauss Syndorme

ASTHMA!! (RARE renal involvement)

3 phases; asthma -> blood eosinophilia-> nec vasc

Tx with Steroids

Senoir 21-2 (77 decks)

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