Rheum Flashcards

1
Q

Define Sensitivity

Define Specificity

What is a good example of one of these types of tests

What is a good examples of these tests

A

Probability of correctly Dx PT w/ Dz

Probability of correctly r/o Dz in PTs w/out a Dz

Sen: ANA lupus
Spec: Anti-dsDNA lupus

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2
Q

What process causes the formation of acute phase responses?

What result is significant

When are ESR values more useful?

A

IL-6, cytokines stimulate liver to synthesize proteins

> 100= Ca, infection, renal/autoimmune dz

Normal (sensitivity test)

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3
Q

What are two presentations that may be normal variants with an increased ESR

When do ESRs have minimal use?

A

Obesity
Pregnancy

Nephrotic syndromes
End stage RDz

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4
Q

What lab result is better than ESR for inflammation/infection indications?

What is an example when a CRP result would be necessary and vital?

CRP is particularly good for tracking ? Dzs

A

CRP- better when accuracy is vital

Steroid tapers

GCA, RA

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5
Q

What are three Dzs that would have a minimal or no CRP elevation?

There is no elevated CRP in Lupus unless ? is present

? Dx can have an elevated CRP w/out any inflammation

A

Poly/Dermatomyositis
Scleroderma

Serositis, Synovitis

Renal failure

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6
Q

What are 3 examples of acute phase reactants?

Which one was used to track progression of COVID?

Define Complement System and number of proteins

A

Ferritin Fibrinogen Platelet

Ferritin, D-dimer

Aids immune system w/ inflammatory/immune response; >30 proteins

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7
Q

What are the functions of the complement system?

What are the 3 pathways of the Complement System

A
Chemotaxis
Opsonization
Ab response
Leukocyte activation
Cell lysis
Clearing apoptotic cells

Classic Alternative
Mannose binding Lectin

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8
Q

Complement system tends to be reduced in chronic autoimmune and inflammatory states such as ?

These Complement measurements are usually used to monitor ? via ? results

Which part of the system screens for deficiency of the classical pathway?

A
Cryoglobinemia
Serum sickness
SLE
Vasculitis
Glomerlonephritis
Subacute endocarditis

SLE w/ C3, C4

CH50- total deficiency= complete deficit early in cascade

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9
Q

Although rare, a complete deficiency of C3 is seen in ? PTs

ANA can detect autoantibodies against ?

A

Peds w/ recurrent pyogenic organisms

Histones, DNA, RNP via fluorescence microscopy

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10
Q

What are ANA Titers indicative of

What test is 97% specific for SLE and is rarely present in other conditions or healthy PTs

This result does not appear in drug induced lupus except for ?

A

High Sen, Low Spec:
>1:160- Pos
<1:80- Neg

Anti-dsDNA

Penicillamine
Minocycline
Antitumor necrosis factors

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11
Q

When is the indication to order an Anti-dsDNA

Define an Anti-Smith (Sm)

Define an Anti-U1-RNP

A

DDx of SLE and pos ANA

Abs recognize nuclear proteins that bind to RNA; specific for SLE

Abs recognize complex proteins and nuclear RNA in SLE, MCTDz

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12
Q

When are Anti-Sm and Anti-U1-RNP tests ordered

When are Anti-Ro (SS-A) or Anti-La (SS-B) ordered

When would these be ordered after a negative ANA

A

Concern of Mixed CT Dz

Primary concern for SLE or Sjogrens

Suspected subacute cutaneous lupus or PTs w/ recurrent photosensitive rashes

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13
Q

Mother of children w/ neonatal cutaneous lupus and congenital heart blocks may be ASx but need to be tested for ?

This also needs to be ordered for ? female PTs

A

SLE or Sjogrens

PTs w/ SLE and considering pregnancy

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14
Q

Anticentromere Abs occur in ?

What type of test is Anticentromere Abs

Rheumatoid factor is an Ab directed against ?

A

Scleroderma, CREST

98% specific

Fc region of IgG

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15
Q

Detection of IgM Rheumatoid Factor titers mean ?

Define Sero-Negative RA

A

> 1:40= latex fixation
20 or more= nephelometry

Ab against IgM

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16
Q

What non-RA conditions can have a positive RF?

A
Cryoglubulinemia
Hep B/C
Elderly PTs
SLE/Sjogrens
Sarcoidosis
Primary biliary cirrhosis
Malignancy
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17
Q

Define Anti-CCP Ab

What is Anti-CCP Ab as strong predictor for?

What combo of lab results is nearly 100% indicative PT will develop RA?

A

Anti-Cyclic Citrullinated peptide Ab; rules RA in earlier than RF in true RA

RA progresstion, joint erosion in PTs w/ early onset, undifferentiated inflammatory arthritis

+ RF and Anti-CCP

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18
Q

What is encompassed in Anti-Neutrophilic Cytoplasmic Ab lab tests

These are highly specific but need ? test to confirm a ? Dx

A

Proteinase-3: GPA
Myloperoxidase- EGP, MPA

Biopsy for GPA Dx

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19
Q

Proteinase 3 is to ?

Myloperoxidase is to ?

A

Cytoplasmic

Perinuclear

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20
Q

Other autoantibodies that are associated w/ ?:

Scl 70
Anti-centromere
Anti-Jo-1
Anti-Mi-2
Anti-Histone
A

Scl: diffuse scleroderma

AC: limited scleroderma/CREST

AJ: dermatomyositis and polymyositis

AM: dermatomyositis

AH: drug induced lupus

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21
Q

Antiphospholipid Abs are AKA ?

What are these Abs associated w/

These contribute to the acceleration of ? and are ordered during ? work up

A

Lupus anticoagulant

Antiphospholipid syndrome- A/V thrombus
Possible SLE

Atherosclerosis
Hypercoagulable

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22
Q

Synovial fluid analysis is performed to exclude what three Dxs

Do not perform this procedure if ?

These results are not usefule for ?

A

Monoarthritis
Infections
Crystal induced arthritis

Bleeding risk
Overlying cellulitis/rash

Polyarthritis

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23
Q

What are the tests included when ordering synovial fluid analysis?

What does it mean if results come back w/ <2K cells, >2K cells or hemorrhagic?

A
Gram stain
Culture
Cell count w/ diff
Polarized microscopy
Glucose/LDH/protein
<2K: OA
>2K: +crystal= pseudo/gout
- culture= RA AS PsA SLE
\+ Gram/Culture= SA
Hem: Tumor TB Trauma
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24
Q

What lab tests would be useful for monitoring SLE and are AKA the “Five fingers of Lupus?

What one would not be useful?

A
Chemistry 
UA 
CBC 
Complement 
AutoAb (ds DNA)
((ESR C3 C4 Anti-ds DNA))

ANA

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25
Pt w/ back stiffness, blurred vision, photophobia and uveititis needs ? lab ordered? PT w/ suspected SLE from Sxs/PE needs ? test ordered w/ ANA for confirmation
HLA-B27- Ankl Spondyl Anti-Smith Ab and, Anti-ds DNA
26
# Define Seronegative Spondyloarthropathy This group is referred to as such based on the absence of ? What are the 4 conditions within this category
Dzs characterized by inflammation of: Uveitis Sacroiliitis Enthesitis Spondylitis No RF or Abs associated w/ conditions Psoriatic arthritis Ankylosing spondylitis IBS associated arthritis Reactive arthritis
27
What is the epidemiology of Ankylosing Spondylitiis What PT populations are at increased risk for developing this?
White M, possible Native American/Eskimo, w/ HLA-B27 w/ insidious onset in late teen/early 20s 1st degree relative w/ AS
28
How does Ankylosing Spondylitis present What condition is rarely seen in PTs w/ Ankylosing Spondylitis What are extra-articular manifestations that may be seen in Ankylosing Spondylitis
Worse w/ rest, pain at night Abnormal FABER/Schober Transient peripheral arthritis- hip, shoulder, knee Cauda Equina Ant uveritis- presenting Sx AV blocks- MC 1*
29
What will be seen on lab results in PTs w/ suspected Ankylosing Spondyltitis What causes the 'bamboo spine' appearance?
Inc ESR Anemia + HLA-B27 90% Syndesmophytes- bony growth from in ligament, leads to vertebral fusion
30
PT is Dx w/ Ankylosing Spond if one clinical and one radiological parameter including ? What are the 4 grades for classifying Ankylosing Spondylitis
``` Low back pain/stiff x 3mon Dec lumbar movement Limited 4th ICS expansion Sacroiliitis- unilateral 3-4 Bilateral grade 2-4 ``` 1: Irregular SI/facets 2: Erosion, sclerosis 3: Erosion, sclerosis w/ widening SI joints 4: ankylosis of both SI joints
31
How is Ankylosing spondylitis Tx
First line- NSAIDs DMARDs: Early dz w/ peripheral joint involvement- Sulfasalazine TNF-I: Etanercept I/A/G-umab (PT refractory/two NSAID trials)
32
? Ankylosing Spondylitis presentation has a worse prognosis What environmental factor has large prevalence w/ AS back pain? What is the phrase for Reactive Arthritis due to their post-infectious triad these presentation of ?
Hip dz <2yrs of onset Tobacco cessation Conjunctivitis- cant see Arthritis- cant bend knees Urethritis- cant pee
33
What are the essentials of Dx for Reactive Arthiritis Where in the body/how do these cases present
Inflammatory arthritis triggered by GI/GU infection Dactylitis Oligoarthritis- MC in LE Mucocutaneous lesion- variable Enthesitis
34
? is the MC cause of inflammatory arthritis in young men How does gender ratio differ for reactive arthritis epidemiology What would be seen on joint fluid analysis?
Reactive arthritis STI: M>F, chlamydia Enteric: M>F, Campylobacter Salmonella Dysentery Yersinia Ecoli Shigella Bacterial Ags, not organisms
35
How long after infection does it take for Sxs to present and where Reactive arthritis frequently has ? common recurrence and ? if frequent recurrence ? is the prominent feature of reactive arthritis and where/how is it seen on PE
<28d after Dysentery/Chlamydia LE: knees, ankle, feet Common: joint arthritis Frequent= spondyl/sacroiitis Enthesitis- Plantar fascia Achilles Pelvis w/ warm TTP
36
What causes Dactylitis in Reactive Arthritis Dactylitis is a feature of spondyloarthropathies usually seen in ? What are the three mucocutaneous features of Reactive Arthritis
Sausage digit- toe*/finger synovitis and enthesitis Reactive/Psoriatic arthritis Circinate balanitis- characteristic; painful inflammatory lesion of glans/shaft Keratoderma blennorrhagica- papular waxy rash on palms/soles Aphthous ulcer- shallow painless on mucosa
37
What microbes are tested for in STI etiology of Reactive Arthritis What microbes are tested for GI etiologies
G/C, HIV Salmonella Shigella Yersinia Campylobacter
38
How is Reactive Arthritis Tx What meds are considered if PT fails initial medical therapy What part of Reactive Arthritis may relapse years later?
Ibuprofen: first line Injections- peripheral joints Systemic steroids- axial Sxs Sulfasalazine or Methotrexate Urethritis Uveitis Arthralgia
39
# Define Psoriatic Arthritis What would be seen on PE In almost all cases ? precedes that arthritis
Chronic inflammation of skin/nail w/ DIP arthritis Dactylitis- MC in toes Nail dystrophy- characteristic Psoriasis
40
What are the 5 patterns of Psoriatic Arthritis
DIP- distinguishes from RA Arthritis mutilans Psoriatic spondylitis Asymmetric oligo- hand/feet (MC) Symmetric polya- mimics RA
41
What will be seen on Psoriatic Arthritis x-rays? How is Psoriatic Arthritis Tx The use of ? med in psoriatic arthritis can precipitate severe flare ups
DIP w/ arthritis mutulans MCP MTP Phalange- periostitis NSAIDs- first step/mild case DMARDs- second line: Methotrexate, Sulfasalazine TNF inhibitors- CAGI-umab/Etanercept Systemic glucocorticoids w/ Rheum/Derm supervision
42
What is the difference in quality of arthritis between PA and RA Define Enteropathic Arthritis What are the two types of Enteropathic Arthritis
Less in PA than RA Arthritis associated w/ Crohns>UC Peripheral- acute, early; MC- knee Flare ups parallel IBD severity Spondylitis- indistinguishable from AS based on Sx/X-ray Severity differs from IBD
43
How is Enteropathic Arthritis Tx What needs to be avoided in these PTs What is the prognosis for PTs Dx w/ Enteropathic Arthritis
Tx IBD Persistent- Sulfasalazine Sulfa failure- Anti-TNF NSAID- exacerbates IBD Excellent for arthritis Poor for IBDz
44
# Define Rheumatoid Arthritis What part of the body does it cause destruction in? This form of arthritis is the MC ?
Chronic systemic inflammatory dz targeting synovium Juxta-articular erosion Inflammatory arthritis in 1% of world population
45
What age prevalence does RA affect the most? PTs w/ RA are at risk for ? Why do these PTs have higher mortality rate?
M: 50-70s F: 30-40s Septic arthritis Osteoporosis Lymphoma, NH/B-cell CV dz
46
Genetic factors that cause RA mostly involve ? Associated risks w/ Abs against ? What are external triggers that could cause RA?
T-cell activation from cytokine signaling Citrullinated protein epitopes Citrullination- modified conversion arginine to citrulline Smoking Infection Periodontitis
47
Since RA has a prevalence in twins, what type is more likely to develop it? What autoimmune factors play a role in it's development? Once established in the body, what type of damage does RA cause?
Monozygotic>Dizygotic RF/Anti-CCP Abs Activated T/B cells and complement in synovium Pannus- hypertrophy/inflamed synovium of RA
48
Direct synovial infections by ? microbes can cause RA? How does RA present What part of the body does it effect first?
``` Parvovirus B19 Retrovirus Enteric bacterial infection Mycoplasma/bacterial EBV ``` Symmetric polyarthritis w/ insidious onset and strong FamHx component PIP MCP and MTP (sero negative- in DIPs)
49
What PE finding is essential for RA Dx What is the sequence of synovitis involvement of RA What is the hallmark of RA
Bilateral symmetric hand/wrist arthritis worse in AM Wrist Knee Elbow Ankle Hip Shoulder Morning stiffness x hrs Improve w/ movement
50
What are late complications of RA usually seen in the hands? Why is the risk for neck involvement w/ RA so important to ID pre-op?
Boutonniere Ulnar deviation- MCPs Swan neck deformity Cord injury w/ intubation Prevent AAD/C1-2 subluxation
51
Tendon positioning of Swan Neck deformity Tendon positioning of Boutonniere deformity Define Felty's Syndrome triad
DIP flexion/bent PIP hyper extension DIP hyper extension PIP flexion RA Splenomegaly Luekopenia (neutro) <2000
52
What type of lab results would be seen in PTs w/ RA induced Sjogrens Syndrome? RA PTs are at increased risk for ? post-op complication They are also at risk for ? spontaneous infection especially if on ? med
Anti-Ro/La (SSA/SSB) negative Knee/hip joint infections Septic arthritis, A-TNFs
53
What are the old criteria used for Dx RA What is the new criteria used?
``` 4 of 7, first 4 x 6wks: Serum RF Morning stiffness Arthritis x 3 joint areas Symmetric arthritis Hand arthritis Rheumatoid nodules Radiographic changes ``` ``` 6 out of 9 points across four domains: Joint involvement-5 Acute phase reactant-1 Serology- 3 Synovitis duration-1 ```
54
What type of RA has a better prognosis Felty's Syndrome must be distinguished from ? Felty's PTs also have increased ? and ? related to leukopenia
Seronegative RF Large Granular Lymphocyte Syndrome- indolent leukemia Bacterial infections Non-healing ulcers
55
What would be seen on PE of RA PTs What labs are ordered What x-rays should be ordered?
Synovitis Nodules Splenomegaly RF Anti-CCP CBC ESR/CRP ANA- r/o lupus Hand/wrist
56
What do lab results for PTs w/ RA look like When/how often are images needed for these PTs? What do PTs need to do prior to starting DMARD medical therapy
RF Anemia- proportional to Dz Thrombocytosis Synovial fluid w/ 5-50K WBCs Onset, Q12mon Update vaccines: Zoster Influenza Yellow fever
57
How is RA Tx medically What drug is the standard of care and approved for monotherapy in RA Tx What med can be taken w/ monotherapy to reduce s/e?
Two total, DMARD and: NSAIDs GCCS- Prednisone bridge x 2-6mons PPI if >65 and on NSAID Methotrexate PO Folate
58
What monitoring is done how often during RA Tx regimes? What systemic dz needs to be r/o prior to starting Tx What adverse effects occur during RA Tx
CBC BMP LFT SrCr q2-4wks initial, q12wks TB Hep B/C HIV Marrow suppression Pneumonitis
59
What synthetic DMARDs can be used for Tx for RA
Hydroxychloroquine- Least toxic/effective as mono Retinal toxiciity over time Monitor CBC Minocycline: Early seropositive Dz effective Unknown MOA Use >2yrs= hyper pigmentation Sulfasalazine: Combo w/ Metho/Hydroxy Monitor: CBC LFT UA BMP ``` Leflunomide: Comparable to Methotrexate Teratogenic, hepatic toxicity TB LFT CBC BP Preg Hepatitis Use Cholestyramine to eliminate if considering pregnancy ```
60
# Define a DMARD What are the top four meds and their MOAs DMARDs must be administered ? routes
Bioengineered by recombinant DNA to target cytokine surface molecules/receptors Block IL-6: Tocilizumab Inhibit T-cell- Abatacept Deplete CD20- Rituximab Inhibit TNF SQ/IV infusion
61
All Pts need to be screened for ? prior to beginning a biologic because ? ? are absolute c/is for DMARDs Biologics are not recommended for PTs w/ solid malignancy or non-melanoma skin Ca Tx <5yrs, Hx of Tx skin melanoma or lymphoproliferative malignancy except for ?
Latent TB Inc risk for infections Active/UnTx Latent TB NYHA Class 3,4 HF or EF<50%- no anti-TNF Rituximab
62
4 categories of biological DMARDs used for RA Tx
TNF- A inhibitors: CAGI-mab/Etanercept IL-1 receptor antagonist- Anakinra Other: Rituximab, inhibits CD20 Tocilizumab, inhibits IL-6 Selective Costimulation mod- Abatacept, alters T-cell activation
63
Biologic DMARDs inc risk for ? infections There is a possible increased risk for ? What lab results is used to correlate Dz activity and Tx efficacy
TB HF exacerbation MS flare up Listeria/Histoplasma Lymphoma ESR/CRP
64
Before starting ImmSupp therapy for RA Tx, what screenings are recommended? How early do RA PTs need to start DMARDs? ? is ubiquitous in PTs w/ RA
TST/Interferon G release Hep B/C HIV At Dx Osteoporosis
65
+RF test in PTs w/ suspected RA means ? What joint would not be commonly involved w/ RA? What are four correct statements about Methotrexate
More severe case of RA, Anti-CCP is specific Sacroiliac Monitor CBC/LFT C/i w/ active hepatitis Weekly dose, start 7.5mg Suppressed marrow
66
SLE has a predilection for ? PT populations Define SLE What are the common manifestations of SLE
Female of child bearing age Autoimmune dz characterized by multi-system involvement and production of autoantibodies Serositis Fatigue Rash, photosensitive Polyarthritis
67
What issue may occur during SLE flares? What are the three types of Lupus?
Hypocomplenentemia Drug-induced Neonatal SLE
68
What are the Lupus specific cutaneous involvements? What are the Lupus non-specific cutaneous involvements?
ACLE- local/general rash SCLE- torso/limb, no face; MOST photosensitive CCLE- discoid plaque on scalp-neck Livedo reticularis Bullous Chilblain Periungual
69
Chronic systemic damage from SLE is usually mediated by ? What causes organ destruction How are RA and SLE similar
Abs to cells and soluble IgG/phospholipid Ags Direct Ab binding Immune complexes Monozygotic twin prevalence
70
What genes are associated w/ SLE The presence of ? will precede PTs clinical illness What are SLE predisposing RFs for women
HLA Type DR2/3- encode complement pathway Autoantibodies High FSH/LH OCP/hormone replacement Low free androgen Early menarche
71
What are the environmental factors that place PTs at risk for Lupus Consider SLE in PTs w/ multisystem Dz and ? SLE PTs w/ Lupus induced hair loss need ? education
Drug- hydralazine isoniazid procainamide UV- exacerbates, not trigger EBV- mimicry Tobacco- inc anti-dsDNA Pos ANA Regrows 6-8wks after Dz activity/or d/c medication
72
11 criteria for SLE
Neuro Dz Heme d/o ANA+ Malar rash Arthritis Immunologic abnormals (anti-Sm/dsDNA) Renal Dz Photosensitivity Oral ulcer (painless) Discoid rash- in PO, painful Serositis
73
When compared to other forms of lupus, SCLE is usually induced by ? What type of Abs are associated w/ SCLE What hematological d/os are seen in lupus
Meds- hydrochlorothiazide, terbinafine Anti-Ro/SSA Thrombocyto <100K ACDz Leuko <4K Lympho <1500
74
What can SLE PTs develop w/ or w/out a Hx of steroid therapy ? lupus complication can lead to life threatening hemodynamic complications Define Libman-Sacks Endocarditis
Osteonecrosis Pericarditis Mitral/Aortic valve thickening
75
What is a rare cardiovascular manifestation of SLE What does SLE cause that can exacerbate the above rare manifestation What parts of the body are MC involved w/ SLE related lymphadenopathy
CADz Accelerated atherosclerosis Cervical, Axillary
76
What is the MC pulm manifestation of SLE What are three rare but life threatening pulmonary manifestation of SLE What would be seen on PFTs for PTs w/ SLE
Pleural involvement Alveolar hemorrhage/Lupus pneumonitis Interstitial lung dz Vanishing lung syndrome Restrictive w/ low diffusion
77
What SLE manifestations are most difficult to assess What test and images will be ordered for any suspected SLE PT w/ neuro involvement Almost all SLE PTs will have ? but only half will it be clinically significant
Neuro, Central>Peripheral LP, MRI Lupus nephritis- develops first 36mon w/ hematuria/proteinuria
78
Why does advancing renal dz make it difficult to track SLE progression How often are SLE PTs screened because of this How are these cases classified
Falsely inc ESR/CRP Q3mon w/ 24hr urine Renal biopsy
79
SLE can cause ? types of abdominal pain SLE Lab results would show ? What lab results will correlate w/ SLE activity but ? is more accurate?
Peritonitis Pseudo obstruction Rarely: pancreatitis, mesenteric vasculitis Chem: HyperK LFT: Hep/Pancreatitis UA: hematura proteinuria casts Correlate: ESR but can also be d/t anemia/Renal failure Better: complement/ds-DNA
80
What is sensitive but not specific for SLE and which one is spec but not sens? Anti-dsDNA correlates w/ the development of ? ? Abs are associated w/ development of neonatal lupus and heart blocks?
Sen- ANA Spec- Anti-smit/ds-DNA Lupus nephritis Anti-Ro/La (SSA/SSB)
81
What is a clinical indicator SLE is seen on lab results during flares and used as an indicator? Female SLE PTs w/ ? anti-phospholipid Abs are at risk for ? What images will be ordered for SLE Pts
Dec C3 C4 CH50 Lupus anticoagulant Anti-Cardiolipin AB Miscarriage CXR- initial pleuritis CT- pneumonitis, alverolar hemorrhage, fibrosis
82
What complication screenings may fall to the primary level in SLE PTs What low, med and high potency meds are used for cutaneous SLE
CAD- HTN/HLD Vaccine- Flu/PPV-23 Bone- Vit D/Ca Annual cervical Ca screen First- low HCN Limbs/trunk: Triamcinol/Betamethasone High- hypertrophic regions- Clobetasol Caution: adrenal suppresion
83
What meds are second line agents for acute, SCLE and DLE? Only use steroids on face for ? and preferably use ? base ? is used for scalp lesions or discoid lupus on scalp?
Tacro/Picrolimus Retinoids <2wks, Ointment Lesions: Steroid lotions Discoid: triamcinolone injection
84
What med is the TxOC for systemic Lupus due to its ability to tx ? aspects What is the most feared s/e of this Tx and needs ? steps taken What are two additional benefits of using this TxoC
Hydroxychloroquine- Cutaneous Arthritis Fatigue Serositis Retinopathy, baseline and annual eye exams Anti-thrombotic Lipid lowering
85
What drug is used for Lupus PTs that don't respond to Hydroxy? What medication is especially useful for bullous lupus When/why would systemic CCS be used
Chloroquine Dapsone CNS/Nephritis
86
What medication or its alternative is the standard of care for proliferative lupus nephritis What ImmSupp drugs may be used for lupus as steroid-sparing drugs? What is the first and second line drug used for associated arthralgia/arthritis
IV Cyclophosphamide qMon Mycophenolate mofetil ``` Belimumab Cyclosporine Rituximab Azathioprine Methotrexate ``` NSAIDs Fail= steroid/HydroxyChlor
87
What meds are used to control HTn and renal complications in Lupus? Most PTs w/ Lupus end up dying from ? bimodal issues? What is the criteria for PT to have met lupus remission
ACEI/ARB Early- Infection, SLE Late- atherosclerosis, Ca >5yrs w/ no clinical/lab evidence and no Tx
88
What is drug-induced Lupus classically associated with Which one is associated with Subacute Cutaneous Lupus
``` Anti-TNFs Chlorpromazine Hydralazine Isoniazid Methyldopa/Minocycline Procainamide Interferon Alpha Quinidine ``` Hydrochlorothiazide
89
How does drug induced lupus present There is increased risk for poor maternal/fetal outcomes if ? coexists What is the PATH acronym for Lupus and pregnancy outcomes
Fever Myalgia Polyarthritis Serositis w/ anti-histone Abs Antiphospholipid syndrome Active lupus nephritis ``` During first trimester: Proteinuria Antiphospolipid syndrome Thrombocytopenia HTN ```
90
? contraception is safe for PTs w/ SLE When can OCPs be used? ? anticoagulants need to be recommended for SLE PTs that are pregnant and have Antiphospholipid Syndrome
Levongestrel IUD Stable low dz and documented negative antiphospholipid Abs Heparin, ASA
91
Antiphospholipid Syndrome is a ? d/o and means PTs have ? Abs to phospholipids What type of anticoagulation issue can PT w/ APS have? This is usually associated w/ ? cardiac Dx
Acquired, usually w/ SLE- Anticardiolipid (IgG/IgM) B2-glycoprotein 1 Prolonged aPTT Libman Sacks endocarditis
92
How is APS Dx
Antiphospholipid Abs or clinical presence of lupus anticoagulant and, Vascular thrombosis Pregnancy morbidity
93
How are APS Tx What needs to be avoided All PTs w/ antiphospholipid Abs should have aggressive management of ? three things
Heparin INR goal 2-3 Rivaroxaban Estrogen contraception Solo ASA therapy HTN Cholesterol Smoking
94
# Define Scleroderma Scleroderma has a strong association w/ ? When/who is it seen
Heterogenous rheumatic dz w/ thick skin and lung/kidney lesions Raynaud's Phenomenon 50y/o Choctaw Native American females in OK
95
If AfAm have Scleroderma, there is higher prevalence of ? compared to Caucasians that have ? PTs w/ Scleroderma usually have 1* relatives w/ ? What is considered the strongest RF for this Dz
AfAm: younger w/ lung dz Cau: Milder, limited Dz ``` Thyroiditis Raynauds Interstitial lung dz MS RA ``` FamHx
96
Rheum Classification of scleroderma includes ? criteria What are the two classification forms and their associated syndromes
``` Thickened skin proximal to MCP joint or, Two of: Bibasilar fibrosis Digital pitting Sclerodactyly ``` Systemic- Limited/Crest, Diffuse Localized- Morphea, Linear
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What is the difference in location of Diffuse or Local Scleroderma What is the unique exception to this rule? ? Abs are associated with each of these types?
Limited- below elbow/face, slow Diffuse- above elbow, rapid Limited w/ PA-HTN Limited- Anti-centromerase Diffuse- Scl-70, anti-RNA polymerase 3
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What viruses are thought to cause scleroderma? What is the sub-group of limited scleroderma?
CMV ``` CREST: Calcinosis cutis Raynauds Esophageal dysmotility Sclerodactyly Telangectasis ```
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? type of scleroderma has a better prognosis Where does calcinosis appear Where does the Telangectasis appear
CREST Extensor surface of forearm Palmar hand
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Scleroderma PTs w/ ? are more likely to develop telangectasis Skin Morphea is more likely to present in ? type What is the MC Tx for Raynauds
PHTN Localized Cold avoidance Topical CCBs
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Pts >30y/o w/ first Raynauds should have ? What are the two forms of pulmonary issues seen with different types of scleroderma
ANA Nail capillary exam (primary- normal capillaries, no abs) Diffuse- inflammatory alveolitis leading to pulmonary fibrosis/interstitial lung dz; in first 4yrs of dz Limited/CREST- vascular dz leading to PAHTN Seen w/ long standing dzs; morbidity
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What is the MC cause of scleroderma related death What would be seen on PFTs in PTs w/ diffuse sclerdoerma induced interstitial lung dz What monitoring do these PTs need?
Diffuse scleroderma PHTN Restrictive pattern PFTs Echos
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What type of upper GI issues do scleroderma PTs have What lower GI issues can they have? What renal issue can present in a minority of sclerodermis PTs and how is it Tx
GERD w/ dysphagia Alternating diarrhea/constipation Sclerodermal Renal crisis- more common in Diffuse as sudden onset HTN/malignant HTN Tx w/ Captopril, ACEI
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What are the 3 high RFs for developing sclerodermal renal crisis What type of MSK issues is common in diffuse scleroderma Define Sicca Complex
Early diffuse scleroderma CSS RNA Polymerase 3 Abs Tendon friction rub- ankle knee wrist Dry eyes/mouth seen in Scleroderma PTs
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Diffuse Scleroderma will have ? presentations
Rapid onset Weight loss Dyspnea w/ exertion Skin texture change- pain edema pruritis
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What lab results help confirm clinical Dx of Scleroderma What two images are ordered for these PTs How often do they need PFTs
ANA- 95% Antipoisomerase 1 Abs (Anti-SCL 70)- specific, diffuse Anticentromere- limited* Chest CT- activity/severity EKG/Echo- PHTN screening Baseline Q4-12mon
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When does a Scleroderma PTs PFT suggest PHTN has developed? What PT education goes w/ Tx goals What meds are used for Scleroderma to protect internal organs and which ones are avoided?
Isolated reduction of DLCO Reduction OOP to decline of FVC No proven medication Skin worst by 24mon, improves from there Cyclophosphamide- only one, protects pulmonary; Avoid CCS
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What meds are given for scleroderma induced GI issues w/ PPIs What motility agents and ABX are used? What is the best predictor of prognosis
Metoclopramide Domperidone Octerotide- motility Rotate- Tetracycline Metronidazole No severe organ invovlement in first 3-5yrs of Dx
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What are the most frequent Sxs of Scleroderma from most to least? What is the best lab test to differentiate between diffuse and limited sclerosis? What lab finding is MC associated w/ scleroderma?
``` Raynauds GERD Skin Finger swelling Arthralgia ``` Anticentromere antibodies + ANA
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# Define Sjogren's Syndrome What is the difference between Primary and Secondary What is the key feature of a Sjogrens presentation w/ ? subjective Sx/complaint
Chronic inflammatory d/o of lymphocytic infiltration of exocrine glands P: no other rhem dz S: associated, RA, systemic sclerosis, SLE Xerostomia Keratoconjunctivitis Sicca/xerophthalmia
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Often PTs have Primary Sjogrens w/ ? What is the common MSK complaint? Sjogren's PTs are more likely to develop ? form of Cutaneous Vasculitis if they are ? Pos
Fibromyalgia Arthralgia CLV, Ro/SSA+
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What is the MC cardiovascular manifestation PTs w/ Sjogrens can develop? What are the two types of pulmonary complications Sjogrens PTs can develop Since it rarely presents, PTs that have pleurisy may have ?
Raynauds Bronchial>Interstitial SLE
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What GI issue may be associated w/ Sjogrens? What is the MC Neuro complaint? What is the MC complaints for the Dz overall? Sjogrens PTs have a 44x greater risk for developing ?
Primary Biliary Cirrhosis Peripheral neuropathy Fatigue Lymphoma
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Sjogrens have an association w/ ? thyroid dz or ? Psych d/o What may be seen on seroogical results in these TPs What are the MC serological findings?
Subclinical hypothyroid Dep/Anx ANA Pos RF Pos Anti Ro/La*(SSA/SSB) Cytopenia- normocytic anemia, leuko/thrombocytopenia Inc ESR Hypergammglobulinemia- monoconal IgG
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How is Sjogrens Dx What meds can be used for Sx Tx of Dry eyes What can be done for Sx relief of dry mouth
``` Schirmer test- pos dye stain Abnormal scintigraphy, sialography, sialometry Dry eyes/mouth x 3mon Pos Biopsy Pos SSA/SSB ``` Topical cyclosporine/glucocorticoids Pilocarpine Cevimeline Lemon drops
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What 3 drugs are used for potentially severe scenario Txs Prognosis w/ Sjogrens is good except for the development of ? How are inflammatory muscle dzs characterized
Hydroxychloroquine- fatigue/arthralgia Prednisone- arthritis Prednisone and Methotrexate- internal organs Lymphoma Bilateral proximal muscle weakness over wks/months
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Dermatomyositis has a significant increased risk for ? two malignancy Since the etiology is unknown, what would be seen on muscle biopsies of these Dzs ? is pathognemonic for dermatomyositis
Hematologic lymphoma Malignant tumors Lymphocytic infiltrates Perifascular atrophy Gottron sign Perifascicular atrophy
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What is the MC presenting Sx of Dermatomyositis Define Heliotrope Rash Define Mechanics Hands
Symmetric prox muscle weakness- Neck Pelvic Thigh Shoulders Eyelid rash of red/violet color Covers areas spared by Lupus, over joints Cracked skin w/ dirty lines, especially PTs w/ Antisynthetase Syndrome
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PTs w/ Antisynthetase Syndrome have autoimmune myopathy and ? features What type of pulmonary involvement do most PTs have
``` One Ab and Two of: Non-erosive arthritis Fever Interstitial lung dz Raynauds Mechanic hands ``` Weakness/inflammation= Interstitial (inflammation) pneumonia Aspiration (weakness) pneumonia
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What is the classic presentation of Dermatomyositis How is it Dx What Neuro test can help confirm Dx
Proximal muscle weakness Heliotrope Gottron Sign Muscle biopsy Rash (Dermatomyositis) Elevated CK Aldolase LDH AST/ALT Abnormal EMG
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Inflammatory Muscle Dz related Anti-Jo Abs are more likely to be in ? PTs What other serological result is likely to be seen How are suspected Inflammatory Muscle Dzs evaluated in order?
Interstitial lung Dz + ANA Hx/Physical Labs EMG MRI Biopsy
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During an Inflammatory Muscle work up, why would GGT finding be significant What are two different MRI findings for Inflammatory Muscle Dz that mean different time frames Why would this imagine be needed for final Dx
GGT released w/ AST/ALT from liver Dz, not muscle damage Muscle edema- active inflammation Fatty infiltration- chronic dz Site for biopsy- deltoid or biopsy w/ 4/5 strength
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What is the mainstay of therapy for PTs w/ Inflammatory Muscle Dz What is the s/e When is the prognosis of Inflammatory Muscle Dz poor
Prednisone: 1-2mg/kg/day x 28d, taper Steroid induced myopathy Underlying malignancy
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# Define Gout This is characterized by ? Gout is more common in men until women reach ? age
Heterogenous metabolic d/o of elevated uric acid Monoarthritis Extra-articular complication Deformity 65
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What do WBC ranges from synovial fluid analysis correlate to Gout is the MC ? Hyperuricemia starts at ? lab level
Norma: <150 Non-inflamm: <3K Inflamm: >3K Septic: >50K Cause of inflammatory arthritis in men >40 6.8mg
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Gout is caused by what two issues What are the 3 stages of gout What leads to bone/cartilage erosion in gout and what are they AKA ?
Under secretion/Over production of uric acid from purine metabolism 1: ASx hyperuricemia, no arthritis 2: acute gouty arthritis, intercritical gout 3: Continuous arthritis w/ attacks, tophi Chronic inflammatory response Macrophages cytokine/enzymes- Rat Bite lesions
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What type of kidney issue can develop due to hyperuricemia This risk doubles at ? level
Nephrolithiasis >13mg
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PTs that are over producers are classified as ? and is due to ? enzyme deficiencies How are under excretors classified? What drugs can cause gout?
Metabolic classification: HGP- Lesch Nyhan G6PD- von Gierkes Dz Majority, Renal CANT LEAP Cyclosporine Alcohol Nicotinic acid Thiazide diuretic Lasix Ethamutol ASA Pyrazinamide
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What is the most susceptible joint to gouty attacks that presents w/ half of first attacks? What are the next most likely joints to be involved Almost all gout PTs will have a second attack w/in ?
MTP- podagra Ankle Knee Wrist Finger Elbow 2yrs
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What types of extra-articular mainfestations can occur from gout? Initial podagra attacks have dramatic responses to ? Tx meds What 3 medications are known for causing gout
Nephrolithiasis Tophi- finger elbow great toe Skin desquamation w/ pruritus NSAID, Colchicine Niacin ASA HCTZ/Lasix
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How is a definitive Dx of gout made? PTs having a gout flare will have ? level serum uric acid? When are these serum levels most important?
Negative birefringent, needle sodium-urate crystals in neutrophils Low or Norm Monitoring urate lowering therapy
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Lab results of an acute gout attack will show elevation in ? 3 things? How long do PTs need to use medical therapy for gout flares? What is the level PTs want to keep uric acid at to prevent further attacks
WBC ESR CRP 2-3 days after flares resolve 6mg or less <5mg if tophi present
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What drug is used for acute gout attacks and prophylaxis What medication is used for PTs who are refractory to or c/i to taking NSAIDs or prophylaxis drug What drugs can be used for injections
Colchicine- 1.2mg first Sx, 0.6mg 1hr later Max 1.8mg in 60min Wait 12hrs for prophylaxis (max 1.2mg/day) CCS- prednisone Methylpred/Triamcinolone
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What are the indications PTs w/ gout need urate lowering therapy What drugs are used for lowering therapy
Tophi 2+ attacks/yr CDK Stage 2 or worse Nephrolithiasis XOIs: Allopurinol- DOC, rash means stop med Febuxostat- safe for PTs w/ renal insufficiency/Allopurinol reactions Uricosurics, only for <65y/o: Probenecid, if renal function preserved Inc Methotrexate/Ketorolac to toxic levels
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What drug is reserved for gout PTs w/ severe cases and abundent tophi? Destructive arthropathy is rarely seen in PTs that have first gout attack after age ? Leukocyte count >5K and rhomboid shaped crystal in synovial fluid means ?
Pegloticase 50y/o Pseudogout (Ca Pyrophosphate Dihydrate Deposition Dz)- weakly positive birefringent
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What part of the body is pseudogout likely to infect? This Dx is commonly associated w/ what two Dzs? Pseudogout is MC associated w/ acute medical illnesses and ? and needs to be considered ?
Knee, Wrist Hemochromatosis Hyperparathyroidism Hypothyroid Surgeries Elderly hospitalized PT w/ new acute monoarthritis post-op/ortho/MI
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How is Pseudogout Tx What drug is used for resistant cases Define Vasculitis
NSAIDs/Colchicine GCSS if intolerant to PO meds Methotrexate Heterogenous d/os of inflammation and destruction of vessels
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What are the MC forms of primary vasculitis Why are these d/os one of the great Dx challenges of medicine Vasculitis is associated w/ ? Ab and due to ? infection
GCA Granulomatosis w/ polyangitis Microscopic polyangitis Non-sepcific Sxs Slowly developing ANCA, Hep B/C
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What are the large vessel vasculitis'? What drives the pathophysiology of these conditions? What are the medium vessel dzs
Takayasus GCA/PMR Behcet dz T-cell mediated Ag driven PAN Buergers Dz, Thromboangitis Obliterans Kawasakis Dz- MLNS
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What are the two categories of small vessel vasculitis
Immune mediated: Cutaneous (hypersensitive) HSP Cryoglobin ANCA- GPA (Wegeners) EGP (Churg Strauss Synd) Microscopic
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Behcets Dz is found in ? country Polyarteritis Nodosa is associated w/ ? infection In vasculitis what happens when the inflammatory cells invade the vessel?
Turkey Hep B/C Fibrinoid necrosis, panmural destruction
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What is the name of the type of damage that occurs from leukocytes in vasculitis Since so many vasculitis criteria overlap, what is needed for definitive Dx
Leukocytoclasis Biopsy/Radiological evidence (tawasakus)
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# Define Mononeuritis Multiplex What are the characteristic derm findings?
Vascular neuropathy: peripheral neuropathy due to damage to sciatic/peroneal nerve Palpable purpura Urticaria x 24hrs Punched out ulcers Livedo Reticularis (precedes ulcers)
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Half of GCA PTs also have ? What PE finding is found in a quarter of GCA PTs What is needed for Dx? When does the biopsy need to be done by?
Polymyalgia rheumatica- ache/stiff shoulder, neck, hip girdle Aortic involvement, asymmetric pulses 3 of 5: >50y/o HA Abnormal artery ESR elevated Abnormal biopsy Within 2wks of steroids
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Why would PMR and GCA Dxs be confused/mixed What is a differentiator? Since PMR can mimic GCA and RA stiffness, what med is used for these PTs
15% of PMR have abnormal temporal artery biopsies No blindness w/ pure PMR Prednisone 15-20mg/day
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When does a Dx of PMR need to be reconsidered? What are two late complications seen in GCA
No improvement after one week on steroid Thoracic aortic aneurysms Aortic dissections
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# Define Tkayasu Arteritis How does it present Most PTs respond to ? med
Granulomatos vasculitis of aorta in young Asian women Bruit Absent pulse Claudication HTN FOUO Prednisone +/- Methotrex or Mycophenolate Relapse is common though
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Behcet Syndrome is common in ? ethnic backgrounds due to ? gene What does this type of inflammatory vasculitis involve What is this conditions hallmark presenting feature
Asian Turkish MidEast 25-35 HLA B51 Both art/vein of all sizes Painful aphthous ulcers on mouth/genitals Eye ball pain Erythema nodosum that ulcerates
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What Neuro Dz can Behcet syndrome mimic What two TNF inhibitors are effective for Tx
MS due to white matter involvement Inflixi/Adalimumab
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# Define Polyarteritis Nodosa What part of the body does this Dz spare What unique constitutional Sx does it present w/
Necrotizing inflammatory vasculitis of medium arteries and muscular arterioles Venous circulation/capillaries Post-randial pain (intestinal angina)
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How is Polyarteritis Nodosa Dx What would be seen as lacking on lab results? This Dz can be associated w/ ? infection
Angiogram Biopsy (lung sparing) Absent granulomatous inflammation Hep B Appears <6mon from infection
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What PE finding is common w/ Polyarteritis Nodosa True PAN cases are ? negative What would be seen on biopsy and angiography results
Mononeuritis multiplex- foot drop Digital gangrene ANCA negative B: fibrinoid necrosis A: microaneurysms
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How is Polyarteritis Tx if PT is refractory to GCSS or major organ involvement Small Vessel vasculitis Dzs include ? structures How are these broadly classified
Cyclophosphamide Arteriole Venule Capillary Immune complex mediated ANCA
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What are the pathological hallmarks for Henoch-Schonlein Purpura and it's MC? What tetrad may be seen? Nearly all cases occur in ? PTs w/ ? rare complication
Vasculitis w/ IgA depositions MC vasculitis in Peds Glomerulonephritis Abdominal pain Purpura Arthritis Peds, IgA glomerulonephritis in adults
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HSP can follow viral URIs or ? ? is a mandatory PE finding for Dx Henoch-Schonlein Purpura How is it Tx
``` EBV Mycoplasma Varicella Parvo Strep/Staph ``` Palpable purpuric rash NSAIDs, avoid if Renal Dz Steroids for arthralgia/GI Sxs Severe/RF: admit, methylpred or, Pred w/ Azathioprine/Cyclophos
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What is a poor prognosis sign for PTs w/ HSP and the silver lining ? is the MC organ involved in Cryoglobin w/ nearly all PTs being _Pos What causes this organ involvement to start
Perisistent renal involvement No ESRDz progression Skin, RF IgG/IgM Abs from low temps <37*C Cryoglobulins binds w/ Ag forms, immune complex leading to IC mediated vasculitis
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What 3 PTs are less likely to survive a Cryoglobulinemia Dx What type is primarily caused by malignancy and manifests w/ hyperviscosity syndromes How does Essential/Primary Cryoglobulinemia differ from other cases?
Male >60 w/ renal involvement Type 1- plasma cell d/os Non-Hep C Others associated w/ SLE and Sjogrens
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What is the classic triad of Cryoglobulinemia? How is Cryoglobulinemia Tx What is used for PTs w/ essential cryoglobulinemia
Myalgia Arthralgia Purpura Harvoni for Hep C w/ Rituximab Rituximab
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# Define GPA What are the hallmarks When/who does the usually infect
Necrotizing vasculitis w/ respiratory and renal manifestations Necrosis Vasculitis Granulomatous inflammation White, northern Europeans during 4-5th decade
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90% of PTs w/ Granulo w/ Poly have ? upper respiratory dz How is this condition Dx What type of image can help since most PTs will have ? finding
Nasal involvement +cANCA highly specific biopsy Chest CT, pulm lesions
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How is Granulo w/ Poly Tx All PTs should receive ? for prophylaxis What meds are used for remission agents?
``` Severe: Rituximab w/ GCSS or Cyclophosphomide w/ GCSS Limited: Methotrexate and GCSS ``` PCP/TMP-SMX Rituximab Azathioprine or Methotrex if Cyclophos was used for remission induction
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What are the 3 hallmarks for Eosinophil Granulo w/ Poly Half will have EGPA w/ ? Abs Why is this useful for lab Dx
Systemic vasculitis Eosinophilia Asthma, adult onset ANCA w/ specificity for myeloperoxidase P-ANCA pattern on fluorescence testing
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PT Dx w/ EGP should have ? HEENT exam done What are the 3 clinical phases of this Dz What is less common in this Dz compared to GPA
Nasal- almost all have allergic rhinitis w/ polyps Prodromal- allergic dz Eosinophilic- blood eosinophilia and infiltration Vasculitic- necrotizing Less renal involvement
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What is the MC cardiac manifestation of EGP What neuro manifestations are seen in more than half of cases? How is it Dx
CHF Peripheral neuropathy Mono multiplex Biopsy- vascular and extravascular for pANCA
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How is EGPA Tx What drug is used for remission induction strategy
GCSS alone Azathioprine, Methotrexate or Mycophenolate added Cyclophos for vasculitic neuoropathy, glomerulonephritis or cardiac involvement Cyclophosph
166
# Define Microscopic Poly This it the MC cause of ? syndrome What PE finding is more prominent and unique w/ this form
Renal and Pulm involved systemic vasculitis Pulm-renal syndrome of alveolar hemorrhage and glomerulonephritis Hemoptysis
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How is Microscopic Poly Dx How is it Tx What is the drug of choice if PTs cant take/don't respond to the previous Tx regime
Biopsy of lung kidney or skin w/ pANCA pos Cyclophosph and GCSS Cyclophosph
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How is Microscopic Poly maintained in remission Define Hyperalgia Define Allodynia
Cyclophos induced, switch to Azathiprine of Methotrexate Excessive severe pain from noxious stimulus Pain from innoculous stimulus, inc tenderness to light pressure
169
Fibromyalgia frequently co-exists w/ ? conditions This Dx needs to be reconsidered if PTs are older than ? What is believed to be the reason for increased pain in these PTs
Depression IBS Chronic fatigue syndrome Interstitial cystitis >55 Glutamate Substance P
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Fibromylagia PTs nearly all have ? Sx that's more bothersome than the pain How many pressure points are needed on exam for Dx
Fatigue 11 out of 18 w/ enough pressure to blanch nails
171
What labs are ordered for fibromylagia work up What is the most effective Tx If needed, what meds are used
CBC CMP ESR CK TSH Non-pharm therapy ``` NSAIDs Acetaminophen Amitriptyline at bed Nortriptyline Tramadol ```
172
What two med classes need to be avoided when Tx fibromyalgia What med may be used for Tx that is also beneficial w/ Psych issues What is the alternate to the above that is cheaper
Steroids Opioids Duloxetine Milnacipran
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FDA Approved drugs for fibromylagia Tx How is Rosacea malar rash different from ACLE malar rash? How is Seborrheic Dermatitis rash different from ACLE rash
Duloxetine- SNRI Milnacipran- SNRI Pregabalin- anti-convulsant, GABA analogue Rosacea- stinging telangiectasis and pustules, worse w/ heat/ETOH Scaly plaques on nasolabial folds
174
Why are Lupus PTs at increased risk for coagulation issues? ? is the MC manifestation of SLE Tobacco can cause ? result to be high
Anti=phospholipid Abs- IgG/IgM anti-cardiolipin Lupus anticoagulant Arthritis anti-DNA
175
What can cause false-pos of syphilis Takayasu PTs w/ cardiac involvement tend to have ?
SLE Lyme AR Angina from stenosis at coronary artery ostia
176
HSP needs to have what two DDx r/o
GMP | Microvasvular