CLM rheum Flashcards
S/sx of rheumatic disease (think Systemic)
- Arthralgias, myalgias,
- Fatigue, generalized weakness,
- HA, Fever, Wt change, easy bruising
- Depression, sleep disturbance, GI,
- Facial and skin features,
- Hair loss, fetal loss
Ddx Rheumatic disease: Polyarthralgia
- RA, OA, fibromyalgia
- Gout and Pseudogout, SLE
- Polymyositis, polymyalgia rheumatica
- Polyarteritis nodosa, Malignancy
- Sacoirdosis, MCtD
- Sjogrens, ankylosing spondylitis
Ddx Rheumatic Disease: Fatigue
- Depression, DM, Malignancy
- Med SE, Anemia, Hypothyroidism
- HyperCa, HIV, HF, COPD
- Connective Tisse dz/Rheum dz
Approach to pt with suspected Rheum disease
- Thorough Hx
- Complete PE
- Serologic studies to SUPPORT (caution in ordering “rheum panel” – false + and needless pt anxiety)
What are the Lab tests in rheumatology
- ESR
- CRP
- RF
- Anti-CCP
- ANA
- Anti dsDNA
- Anti ssDNA
- Anti-smith antibodies
- Antiphospholipids: Anticardoplipin antibodies and Lupus anticoagulants
- ANA subtypes:
- Anti SS-A (anti-Ro)
- Anti SS-B (anti-La)
- Anti SS-C - Human Leukocyte antigen B27 (HLAB27)
Antibodies + in SLE
ANA (most sensitive screen), SLE prep, dsDNA (most specific), ssDNA, anti-DNP, SS-A
Antibodies + in drug induced SLE
Anti histone
Sjogren syndrome antibodies
RF, ANA, SS-A, SS-B
MCTD antibodies
ANA, RNP, RF, ssDNA
RA antibodies
RF, ANA, RANA, RAP
Primary biliary cirrhosis antibodies
AMA
Thyroiditis antibodies
Antimicrosomal antihyroglobulin
Chronic active hepatitis antibodies
ASMA
Scleroderma Antibodies
ANA, Scl-70, RNA, dsDNA
Non specific inflammatory marker tests
ESR, CRP
What is ESR
- 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”
What is CRP
- 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)
What is the best screening test for SLE, who should be screened, and what results suggest SLE?
• 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)
What is the most specific test for SLE and how does it reflect disease activity
- 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
How does anti ssDNA relate to SLE
- 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
What antibody is specific for SLE, only found in 20-30% of pt, and may be used dx when DNA tests are undetectable
• Anti smith antigen
What are antiphospholipid antibodies? What are associated risks? Antiphospholipid syndrome?
• 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
What antibodies are present in drug induced SLE? What drugs can be the cause?
- 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
How should we manage pt with SLE
• ACR recommends: PCP to consult rheumatologist if you suspect characteristic SLE or seronegative disease
Physical/lab findings associated with RA
- Swan Neck deformity, Boutonniere
* +RA, +anti CCP, elevated ESR and CRP
What is Rheumatoid Factor useful for? Not useful for?
• 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
RF titer of what is considered +? Overall summary points of RF include?
- 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
What is anti-CCP useful for
- 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
What is Sjogren’s syndrome
• 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
What are lab findings associated with Sjogrens disease
- 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
Anti SS-A aka?
Anti SS-B aka?
Anti SSC?
What are they associated with?
- 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
What findings are associated with secondary Sjogren (ie secondary to RA)
• +Anti SSC
Buzz words for Sjogrens (primary)
• Ro, La, Speckled pattern, schirmer test, lip biopsy, salivary gland biopsy, lymphoid foci
What is Anti SSC useful for
• Differentiating Primary from secondary Sjogrens
What are the usual sx (suggested diagnostic criteria) of polymyalgia Rheumatica
• >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
What helps indicate risk for Ankylosing spondylitis? And when is testing for that useful?
- 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)