CLM rheum

Question 1

S/sx of rheumatic disease (think Systemic)

Answer 1

• Arthralgias, myalgias,
• Fatigue, generalized weakness,
• HA, Fever, Wt change, easy bruising
• Depression, sleep disturbance, GI,
• Facial and skin features,
• Hair loss, fetal loss

Question 2

Ddx Rheumatic disease: Polyarthralgia

Answer 2

• RA, OA, fibromyalgia
• Gout and Pseudogout, SLE
• Polymyositis, polymyalgia rheumatica
• Polyarteritis nodosa, Malignancy
• Sacoirdosis, MCtD
• Sjogrens, ankylosing spondylitis

Question 3

Ddx Rheumatic Disease: Fatigue

Answer 3

• Depression, DM, Malignancy
• Med SE, Anemia, Hypothyroidism
• HyperCa, HIV, HF, COPD
• Connective Tisse dz/Rheum dz

Question 4

Approach to pt with suspected Rheum disease

Answer 4

1. Thorough Hx
2. Complete PE
3. Serologic studies to SUPPORT (caution in ordering “rheum panel” – false + and needless pt anxiety)

Question 5

What are the Lab tests in rheumatology

Answer 5

1. ESR
2. CRP
3. RF
4. Anti-CCP
5. ANA
6. Anti dsDNA
7. Anti ssDNA
8. Anti-smith antibodies
9. Antiphospholipids: Anticardoplipin antibodies and Lupus anticoagulants
10. ANA subtypes:
    - Anti SS-A (anti-Ro)
    - Anti SS-B (anti-La)
    - Anti SS-C
11. Human Leukocyte antigen B27 (HLAB27)

Question 6

Antibodies + in SLE

Answer 6

ANA (most sensitive screen), SLE prep, dsDNA (most specific), ssDNA, anti-DNP, SS-A

Question 7

Antibodies + in drug induced SLE

Answer 7

Anti histone

Question 8

Sjogren syndrome antibodies

Answer 8

RF, ANA, SS-A, SS-B

Question 9

MCTD antibodies

Answer 9

ANA, RNP, RF, ssDNA

Question 10

RA antibodies

Answer 10

RF, ANA, RANA, RAP

Question 11

Primary biliary cirrhosis antibodies

Answer 11

AMA

Question 12

Thyroiditis antibodies

Answer 12

Antimicrosomal antihyroglobulin

Question 13

Chronic active hepatitis antibodies

Answer 13

ASMA

Question 14

Scleroderma Antibodies

Answer 14

ANA, Scl-70, RNA, dsDNA

Question 15

Non specific inflammatory marker tests

Answer 15

ESR, CRP

Question 16

What is ESR

Answer 16

• Measure of distance RBC fall in column over 1 hr; elevated in inflammation, infection, necrosis, neoplasia
• These increase PRO content of plasma RBCs stack up and settle to bottom faster
• ESR considered “inflammatory marker”

Question 17

What is CRP

Answer 17

• Non Specific “acute phase reactant” PRO
• Detects inflam d/o, bacterial infections, tissue necrosis
• Synthesized in liver during inflammatory process
• More sensitive and rapidly responding than CRP
• High sensitivity CRP may be marker for CAD –inflammatory plaques (high risk >3 mg/dL)

Question 18

What is the best screening test for SLE, who should be screened, and what results suggest SLE?

Answer 18

• ANA, + in almost all SLE, but can be + in others (Scleroderma, Sjogrens, RA, fibromyalgia)
• Test for ANA only if ACR criteria: >2 unexplained s/sx
• ANA neg SLE requires rheumatologist
• + results reported in titer AND nuclear staining pattern (reflects intracellular target of ANA)
o Stain: *Homogenous, **Rim and Speckled = SLE
o Higher titer = higher likelihood of SLE (>1:320)

Question 19

What is the most specific test for SLE and how does it reflect disease activity

Answer 19

• Anti dsDNA: 97% specific, 60% sensitive for SLE
• Found in low freq in other rheum diseases
• Fluctuates with disease activity: titers rise with flares can help monitor disease activity - Lupus nephritis

Question 20

How does anti ssDNA relate to SLE

Answer 20

• Anti ssDNA is derived from kidneys of pt with lupus nephritis, less specific than anti dsDNA, also found in RA, drug related lupus and other rheum dz
• Limited usefulness in dx of SLE

Question 21

What antibody is specific for SLE, only found in 20-30% of pt, and may be used dx when DNA tests are undetectable

Answer 21

• Anti smith antigen

Question 22

What are antiphospholipid antibodies? What are associated risks? Antiphospholipid syndrome?

Answer 22

• Anticardiolipin antibody & Lupus anticoagulant (not assoc with bleeding tendencies) – replaced +LE prep
• You can test + for one and not the other or + for one or both and not have SLE
• 40% SLE pt have antiphospholpid antibodies risk for thrombosis, abortions, neuropsych disorders, thrombocytopenia
• ANTIPHOSPHOLIPID ANTIBODY SYNDROME: thrombosis + miscarriage + thrombocytopenia
o These pt do not necessarily have SLE
o Eval all pt with recent unexplained CVA, MI or miscarriage

Question 23

What antibodies are present in drug induced SLE? What drugs can be the cause?

Answer 23

• Anti-histone antibodies + (anti dsDNA and antiSm antibodies are negative)
• Drugs: HIP! Hydralazine, isoniazid, Procainamide, methyldopa, quinidine, chlorpromazine etc
• Suspect if pt on these drugs causes arthralgias, myalgias, rash, fever, serositis

Question 24

How should we manage pt with SLE

Answer 24

• ACR recommends: PCP to consult rheumatologist if you suspect characteristic SLE or seronegative disease

Question 25

Physical/lab findings associated with RA

Answer 25

* Swan Neck deformity, Boutonniere | * +RA, +anti CCP, elevated ESR and CRP

Question 26

What is Rheumatoid Factor useful for? Not useful for?

Answer 26

• Present in about 80% of pt with RA • Can be + in pt with SJogren’s cryoglobulinemia, MCTD and some infectious dz • Most useful when dx of RA is suspected: o Clinical suspicion is low, then test is unlikely to be helpful, high incidence of false + in general population • Not useful for following progression of disease • 20% of pt with RA are seroneg, 40% may be seroneg early

Question 27

RF titer of what is considered +? Overall summary points of RF include?

Answer 27

* titer >1:80 = +RF * poor screening test, only order if high index of suspicion for RA (higher titer = higher prognostic value) * Remember seronegative RA is possible * 66% sensitive and 82% specific

Question 28

What is anti-CCP useful for

Answer 28

* Useful in dx of RA; very specific, rarely + in other dz * Especially useful when RF is negative * Pt with early RA may be seronegative (ie neg RF) but if anti CCP is elevated, dx of RA likely * 67% sensitive, 99% specific

Question 29

What is Sjogren’s syndrome

Answer 29

• Autoimmune disorder • Chronic dysfunction of exocrine glands o dry eye, mouth, mucous membrane o Keratoconjunctivtiis sicca: inadequate tear production; lymphocytic and plasma cell infiltration of Lacrimal glands • Assoc w/ ofther rheum conditions (ie have RA and sjog) • Female to male 9:1

Question 30

What are lab findings associated with Sjogrens disease

Answer 30

* CBC: mild anemia, leukopenia, eosinophelia * Polyclonal Hypergammaglobulinemia * +RF (70%) * +ANA (95%) * + Schirmer test – measure quantity of tears * Lip biopsy * Salivary Gland Biopsy – only for atypical pt, lymphoid foci = + for Sjogren

Question 31

Anti SS-A aka? Anti SS-B aka? Anti SSC? What are they associated with?

Answer 31

* Anti SSA = Ro * Anti SSB = La * Anti SSC, just that * These are subtypes of ANA * Ro and La strongly associated with SJOGRENS (70-95%) * Ro and La = SPECKLED immunofluorescent pattern * +RO and +La = CONFIRMATORY for Sjogren * Anti SSC + in 75% of pt with RA who have secondary Sjogren SSC differentiate primary from secondary sjogrens

Question 32

What findings are associated with secondary Sjogren (ie secondary to RA)

Answer 32

• +Anti SSC

Question 33

Buzz words for Sjogrens (primary)

Answer 33

• Ro, La, Speckled pattern, schirmer test, lip biopsy, salivary gland biopsy, lymphoid foci

Question 34

What is Anti SSC useful for

Answer 34

• Differentiating Primary from secondary Sjogrens

Question 35

What are the usual sx (suggested diagnostic criteria) of polymyalgia Rheumatica

Answer 35

• >50yo • Bilateral aching and morning stiffness x1mth and involving at least 2/3 areas: neck or torso, shoulder or proximal arms, hips or proximal thighs (shoulder girdle, hip girdle) • ESR >40 Associated with Temporal Arteritis (risk for vascular problems, blindness) Both characterized by elevated ESR

Question 36

What helps indicate risk for Ankylosing spondylitis? And when is testing for that useful?

Answer 36

* HLA-B27: Antigen that indicates risk for spondyloarthropathies * Occurs in >90% ankylosing spondylitis * Occurs in 80% pt with Reiters syndrome * Also seen in psoriatic arthritis and IBD associated arthritis * THUS testing only useful when ankylosing spondylitis strongly suspected (may also see elevated ESR)