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MSK > Rheumatological Conditions - Exam 2 > Flashcards

Rheumatological Conditions - Exam 2 Flashcards

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1
Q

Where do B cells develop? What is the first line to autoantibody defense?

A

bone marrow

antibodies created by B cells which would attack a body protein found in the bone marrow is destroyed

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2
Q

Where do T cells mature? What happens next?

A

thymus

in the Thymus and T cells progress to lymph nodes where the T Cell will meet and activate a similar B cell.

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3
Q

What is the second line of defense against autoantibody?

A

Without T Cell activation of the B Cells, the body will not proliferate the autoantibody

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4
Q

What are the broad overviews of the role of the T and B cells?

A

T cells recognize harmful pathogens and rally other cell types to come defend

B cells produce antibodies designed to attack specific antigens

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5
Q

What are the 4 broad causes of AI?

A

genetics

infection

stress

medication SE

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6
Q

T/F: All AI disease are multiorgan and thus need to be carefully monitored by a specialist

A

FALSE, some AI dz only involve one organ ( Hashimoto’s) but some are involve multiple organs (Lupus, Crohn’s)

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7
Q

What is the best way to dx rheumatologic conditions?

A

through PATTERNS of the pts history, PE, labs and imaging

all 4 components are needed to make the dx

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8
Q

What is a highly sensitive test? What is the drawback?

A

highly sensitive test: will catch the majority of sick pts but also has a higher probability of a FALSE POSITIVE

aka the car alarm that is super sensitive and will go off for a slight gust of wind even though no one is trying to steal the car

SNOUT rules out if the test is negative

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9
Q

What is a highly specific test?

A

has a lower threshold for excitement and will pick up all the sick people but will miss some sick people in the process, rate of FALSE NEGATIVES

aka if you highly specific test is positive result indicates you have the disease but if your highly specific test is negative you still also might still have the disease

SPIN rules in if the test is positive

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10
Q

What lab test would you start with if you suspect an AI dz? What does it stand for? What is it checking for?

A

ANA

antinuclear antibody

checking the body for autoantibodies that attack self

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11
Q

**What is considered a positive ANA? Does the pt always have to be symptomatic?

A

Positive if titer is ≥ 1:160

NO!! 5% of the healthy population has a titer level that is above this level and are completely fine

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12
Q

**What does a higher ANA titer level indicate? **What does it NOT indicate?

A

Greater likelihood of autoimmune disease

higher titer does NOT indicate worse symptoms

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13
Q

How would you describe ANA in terms of sensitivity and specificity?

A

Fairly High Sensitivity and Low Specificity

if the test is negative you do NOT have AI but more tests are needed to determine specific diseases

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14
Q

Once a pt tests + for ANA, what should you NOT do again? What if the ANA test is negative?

A

do NOT need to consistently keep repeating ANA level, it will not tell you any additional information that you did not already know

if the -ANA then it is acceptable to repeat ANA later on down the road if you suspect AI dz

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15
Q

How sensitive is a +ANA test for the following diseases?

Drug Induced lupus
SLE
scleroderma
Sjorgen Syndrome
Dermatomyositis/Polymyositis
RA

A
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16
Q

Memorize the lab studies for which test charts* DO IT!! Do not need to know middle column

A
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17
Q

What is an ENA test stand for? What does a positive result indicate? What 3 diseases should you be thinking of?

A

Leads the clinician to a more specific disease in the presence of possible Connective Tissue Disease

SLE, Systemic Scleroderma, and polymyositis.

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18
Q

Rheumatoid Factor indicates ____ in the blood that is produced by the _____

A

proteins

immune system

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19
Q

What 8 diseases will RF be positive in?

A

Rheumatoid Arthritis
Sjogren’s Syndrome
Juvenile Arthritis
Scleroderma
Mononucleosis
TB
Leukemia
Multiple Myeloma

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20
Q

RF is not _____. What does this mean?

A

not specific

It can be found in completely healthy patients and patients with rheumatoid disease may not be RF (+)

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21
Q

What are the top 3 diseases that will have a +RF? give percentages

A

Sjogrens: 75-95%

RA: 70-90%

SLE: 15-35%

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22
Q

What does a higher RF indicate?

A

higher levels = worse prognosis but it may NOT be present in early disease

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23
Q

**_____ is the newer and better lab test for RA

A

Anti-CCP: Anti cyclic citrullinated peptide

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24
Q

CRP is best at identifying (acute/chronic) inflammation. Where is it produced?

A

CRP = ACUTE inflammation

produced in the liver and is NOT a reliable test in pts with liver failure

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25
ESR is best at identifying (acute/chronic) inflammation. What does a normal test rule out?
ESR: Chronic inflammation normal ESR rules out temporal arteritis and polymyalgia rheumatica
26
What are some natural reasons why an ESR may be elevated?
↑ w/ age, pregnancy (2nd/3rd TM), medications (OCP)
27
What does an ANCA test stand for? What are the 2 types?
Antineutrophil Cytoplasmic Antigen p-ANCA (Perinuclear pattern) c-ANCA (Cytoplasmic pattern)
28
When will a p-ANCA test be positive?
(+) SLE, Sjogren's, RA, Polymyositis, Dermatomyositis, and many more inflammatory, autoimmune, and infectious conditions
29
When will a c-ANCA test be positive?
(+) Wegener Granulomatosis & Churg-Strauss Syndrome
30
______ A chronic, systemic, multi organ autoimmune disease of connective tissues secondary to antibody formation and immune complex deposition.
Systemic Lupus Erythematosus (SLE)
31
How common is SLE? What gender? What age range? What race?
1/1000 in USA 9:1 female: male ration 65% of patients diagnosed from 16-55 years of age Native American > African American > Asian (Chinese, Filipino) > Hispanic and Japanese > Caucasian
32
What are the 5 precipitating factors for SLE?
Sun exposure Stress Infections Pregnancy Surgery
33
**What is the classic triad of SLE?
Low grade fever Joint Pain Malar (Butterfly) Rash that SPARES THE nasolabial folds
34
** What is important to note with regards to the rash of SLE?
Malar (Butterfly) Rash that covers teh cheeks and nasal bridges that SPARES the nasolabial folds
35
What is the MC tool used to dx SLE? What are the criteria? Do all the signs have to be present at the same time?
The most used tool is the American College of Rheumatology Criteria from 1997. 4 out of 11 common signs and symptoms must be present for the diagnosis to be made. the s/s do NOT have to be present at the same time to count
36
What are the 11 s/s of lupus? How many do you need to make the dx?
+ANA malar rash discoid rash photosensitivity oral ulcers arthritis kidney disease neurologic dz serositis hem disorders immunologic abnormalities need 4/11 to make the dx
37
What is the sensitivity and specificity of dx lupus with the criteria from the American College of Rheumatology Criteria from 1997?
96% Sensitivity and Specificity with this diagnostic tool
38
What are the kidney dz requirements for SLE dx? What are the neuro?
>500mg protein in 24 hrs. ≥3+ protein UA dipstick Casts: Red, Hgb, granular, tubular, mixed seizures or psychosis in the absence of electrolyte abnormalities or medication SEs
39
What are the serositis options in the dx of lupus?
Pericarditis - evidenced by EKG, rub, pericardial effusion OR Pleuritis - Convincing hx of pleuritic pain or pleural rub or pleural effusion
40
What are the hem disorders for the dx of lupus?
Hemolytic Anemia w/ reticulocytosis Leukopenia <4000 on 2 or more occasions Lymphopenia <1500 on 2 or more occasions Thrombocytopenia <100,000
41
What are the immunologic options that would meet the criteria for immunologic portion of dx criteria?
Abnormal ANA at any point in absence of drugs known to cause (+) ANA Anti-dsDNA in abnormal titer Anti-Smith Positive Antiphospholipid Antibody
42
What are the 3 criteria for dx lupus that would qualify for a positive Antiphospholipid Antibody? **What is the highlighted finding?
An abnormal serum level of IgG or IgM anticardiolipin antibodies OR A positive test result for lupus anticoagulant using a standard method OR A false-positive serologic test for syphilis known to be positive for at least 6 months and confirmed by Treponema pallidum immobilization or fluorescent treponemal antibody absorption test **false-positive serologic test for syphilis should think lupus**
43
_____ is the second dx tool to dx lupus. What ANA level does it require in order to use it?
ACR/EULAR 2019 criteria ANA ≥ 1:80 mainly used to enlist pts into research studies
44
Discoid rash in SLE
45
discoid rash in SLE
46
What are the derm manifestations that you will commonly seen in SLE? How common will pts present with them?
Malar Rash Discoid Rash Photosensitivity Oral / Nasopharyngeal Ulcers Raynaud’s Alopecia Dermatologic Manifestations (>80%)
47
Describe the arthritis present with SLE? How common will they present with MSK manifestations?
≥2 joints Migratory / Symmetric Often peripheral, esp. hands myalgias MSK Manifestations (>90%)
48
How common are renal complaints in lupus? How do you prove it?
Renal (16-38%): Hematuria Proteinuria Elevated Creatinine May be asymptomatic Biopsy proven Lupus Nephritis guarantees SLE Dx
49
How common are neuro manifestations in SLE?
Neuro (10-80%) Cognitive dysfunction Stroke Neuropathy Transverse myelitis HA Delirium Depression
50
What are some heme manifestations of SLE? What % of patients?
Hemolytic Anemia (+ Coombs) or leukopenia or lymphopenia or thrombocytopenia Anemia of Chronic Disease Lymphadenopathy Antiphospholipid Syndrome Venous & Arterial thrombosis Recurrent Miscarriages 36%
51
What are 2 very common SLE categories that do NOT count towards one of the 4/11/
GI and systemic: fatigue, fever, malaise, weight changes, weakness
52
What is the tx for drug induced lupus? What organ systems are usually NOT involved? ____ has a 100% sensitivity. _____ has 95%
discontinuation of offending agent CNS/Kidney/Alopecia ANA+ Anti-Histone Antibodies
53
**What medications are in the HIGH risk category for drug induced lupus? _____ is in the moderate risk category
Procainamide Hydralazine Penicillamine quinidine
54
How often should you monitor a pt with SLE that is completely stable? _____ for most patients
Every 6 months for completely stable 3-4 months for most patients and possibly much less during an active flare
55
What are the lab monitoring requirements for SLE?
CBC CMP UA w/ micro
56
What CBC finding is often the first sign of an acute flare?
thrombocytopenia Anemia is also often present
57
Pts with lupus need to use ___ sunscreen. ____ and ___ will need to be supplemented in their diet
SPF ≥55 vit d and daily multivitamin
58
What are the non-pharm management for SLE?
exercise!! prevent fatigue and joint stiffness smoking cessation immunization avoid pregnancy during active flares avoid: sulfa abx and minocycline
59
Which vaccines are safe in STABLE lupus pts?
Influenza Pneumococcal HPV HBV
60
Why should a lupus pt avoid pregnancy during a flare? **What medications should a lupus pt AVOID?
(Increased miscarriage and difficult pregnancy) Encourage “quiescence” for at least 6 months before attempting pregnancy. avoid: sulfa abx and minocycline
61
T/F: Medications which cause drug induced SLE are generally safe.
TRUE!!
62
What is considered mild SLE? What is the tx?
Mild Manifestations Skin joint and mucosal Hydroxychloroquine, (+/-) NSAID <7.5 mg prednisone daily if needed
63
What is considered moderate SLE? What is the tx?
Moderate Manifestations Significant but non-organ threatening disease -Hydroxychloroquine + NSAID -5-15 mg prednisone daily which is tapered -Immunosuppressive (Methotrexate or Azathioprine)
64
What is considered severe SLE? What is the tx?
Severe Manifestations Renal/CNS involvement -Hydroxychloroquine + NSAID -High dose IV prednisone -Immunosuppressive agent (Belimumab, Rituximab, Cyclosporine)
65
What drug class is hydroxychloroquine? What are the indications?
DMARD Malaria, SLE, and RA
66
_____ is the mainstay of tx for SLE. Where is it excreted? What does it help reduce?
Hydroxychloroquine or Chloroquine Mainly excreted through Liver Reduces flares and risk of thrombotic complications
67
_____ is needed at baseline for hydroxychloroquine. What is needed after? **What is the potential finding?
Ophthalmic Exam at Baseline Annual eye exams after 5 years unless significant risk factors necessitate sooner Chloroquine Retinopathy - “Bull’s Eye Retinopathy” caused by drug toxicity
68
Chloroquine Retinopathy - “Bull’s Eye Retinopathy” caused by drug toxicity of hydroxychloroquine
69
____ or ____ is used to treat the joint symptoms of SLE. Which one should you NOT use? Why?
naproxen!! (not ibuprofen) or prednisone celebrex if risk of ulcer Ibuprofen ->Associated with aseptic meningitis in SLE population
70
_____ is used in SLE if hydroxychloroquine and NSAIDs not enough. Addition of ____ may allow for lower doses
prednisone addition of Methotrexate may allow for lower doses of steroid therapy
71
_____ this multisystem disease can be appreciated as thickened and tightening of the skin and internal connective tissues and organs. How common is it? What age range? What sex?
scleroderma 1-2/100,000 in USA 10:1 female to male dx 30-50 years old
72
**What is the super highlighted skin finding that is associated with scleroderma?
**puffiness that doesn't respond to diuretics sclerodactyly
73
What are the 4 subsets of scleroderma? Which one is rare?
Limited Cutaneous Systemic Sclerosis Diffuse Cutaneous Systemic Sclerosis Systemic Sclerosis Sine Scleroderma - RARE Systemic Sclerosis with Overlap Syndrome
74
______ Most disease is distal to the elbows and the face and neck. Many manifest CREST.
limited cutaneous systemic sclerosis
75
_____ Disease progresses to include proximal and even truncal manifestations.
diffuse cutaneous systemic sclerosis
76
______ All of the other manifestations present without skin manifestations
Systemic Sclerosis Sine Scleroderma
77
______ Any of the above with overlap features of another systemic rheumatologic disease (ex: SLE, Sjogren’s, RA, etc…)
systemic sclerosis with overlap syndrome
78
_____ is the limited form of scleroderma? How common? What does it primarily affect?
CREST syndrome (systemic is other 20% and includes trunk and proximal extremities) 80% Primarily Head and Hands
79
______ is limited cutaneous systemic sclerosis. What are the components?
Though esophageal most common, 90% of Scleroderma pts. have GI symptoms including: GERD Choking Cough after swallowing Bloating Early Satiety Dysphagia so really just think GI not specifically esophageal
80
What am I? What dx?
calcinosis cutis CREST syndrome
81
What am I? What dx?
Sclerodactyly CREST syndrome
82
What labs should you order in CREST syndrome? What are the likely results?
83
**All pts with scleroderma/CREST should have what 3 things at baseline? What are you looking for?
1. PFTs 2. Echo 3. CT of Chest **Interstitial Lung Disease** is present and also looking for systemic s/s that you cannot see on the outside
84
What is the tx for scleroderma/CREST syndrome?
CREST is the less severe form of scleroderma NO DEFINITVE cure!! just treat the organ system based
85
What is the tx for arthritis in scleroderma/CREST?
Start with NSAID as long as kidney function stable With Inflammatory (RA), add to NSAIDs in a stepwise approach: Low dose steroids Hydroxychloroquine Methotrexate Biologics Don’t forget physical therapy and weight loss!
86
What is the tx in scleroderma/CREST with diffuse skin sclerosis or organ involvement?
Methotrexate
87
______ chronic autoimmune inflammatory disorder characterized by diminished lacrimal and salivary gland function with resultant dryness of the eyes and mouth. How common is it? What sex? What age?
Sjӧgren’s Syndrome 4 million cases: one of the more common AI disorders 10:1 female to male dx between 50-60
88
Sjӧgren’s Syndrome primarily attacks _______. Give 4 examples
glands!!! aka everything is dry!!!! mouth, eyes, vagina, trachea (chronic cough), dry itchy skin
89
What is the difference between primary and secondary Sjogren's syndrome?
Primary: No other disease involvement Secondary: Overlap - often with RA, SLE
90
What are the some dry mouth s/s associated with Sjӧgren’s Syndrome?
Halitosis, insatiable thirst, cavities - often at the gum line, dysphagia without liquid
91
Which will be elevated in Sjogren's syndrome ESR or CRP? _____ gene is found in 85% of patient's with Sjogren's syndrome
ESR often elevated, CRP is not HLA-DR52
92
What AI labs should you order in SS? What % are positive?
Anti-SSA (Anti-Ro) / Anti-SSB (Anti-La) - 60-80% of pts with primary disease are (+); be leary of weakly positive tests
93
What type of bx can confirm Sjogren's Syndrome? _____ test can confirm those with eye symptoms
Labial salivary gland biopsy may confirm suspected disease Schirmer Test
94
What is considered a negative Schirmer Test?
>10mm is considered NEGATIVE
95
What is the tx for Sjogren's syndrome? _____ is used to increase salivation and lacrimation. What is xerostomia?
pilocarpine dry mouth
96
_____ are idiopathic inflammatory myopathies, characterized by the shared features of proximal skeletal muscle weakness and evidence of muscle inflammation. How common are they? What is the female/male ration? When are each usually dx?
Dermatomyositis (DM) and polymyositis (PM) 1:100K dermatomyositis 0.5 :100K polymyositis 3 females to 1 male DM: childhood or adult PM: adult
97
How will polymyo and dermatomyo present? What muscle groups are usually spared? may have _____
Insidious onset of symmetric, progressive, painless proximal weakness eye and facial muscles spared may have dysphagia
98
What are some movements that pts with PM or DM may have a hard time doing? **Increased risk of ______ 3-5 years after onset especially with _____
difficulty rising from a chair, climbing steps, combing hair, overhead movements **cancer **dermatomyositis has 5-7 greater risk
99
Dermatomyositis has an increased cancer risk, which ones specifically?
Ovarian breast colon melanoma Non-hodgkin’s
100
What am I? What dx?
Gottron’s Papules Dermatomyositis
101
_____ raised, violaceous scaly eruptions on knuckles. What dx?
Gottron's papules Dermatomyositis
102
What am I? What dx?
Heliotrope Eruption (Blu-purple) Dermatomyositis
103
What am I? What dx?
mechanic hands: hyperkeratotic with dirty appearance Dermatomyositis
104
**What am I? What dx?
**Shawl Sign Dermatomyositis*
105
What should be included in your work-up of a pt with PM/DM? **What is important to note?
CK > 10X upper limit of normal in active disease and >5x upper limit of normal in rhabdo
106
What is the tx for PM/DM?
STEROIDS!!!!
107

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