introduction to rheumatology kathryn dao, md arthritis consultation center july 21, 2005
TRANSCRIPT
INTRODUCTION TO RHEUMATOLOGY
KATHRYN DAO, MD
Arthritis Consultation Center
July 21, 2005
What am I?
I am the size of a rabbit with fur as smooth as an otter. I have a spongy beak covered with sensitive skin.
I am a monotreme.I protect myself with poisonous spurs from my hind legsMy name means "flat feet."
Why even care?
• 2002 CDC reported arthritis as the leading cause of disability in the US.
• 55.4 million have chronic joint symptoms lasting for more than 3 months
• 21.5 million have not seen a physician• 2 million have activity limitations• 25% will be unable to work within 7 years of disease onset• Direct and indirect costs are estimated at 1% of the US
gross domestic product = $86.2 billion
Center for Disease Control and Prevention. MMWR 2004;53:383-6.Center for Disease Control and Prevention. MMWR 2004;53:388.
3500 rheumatologists
Musculoskeletal Complaint
Initial Rheumatic History and Physical Exam to Determine:
1. Is it articular2. Is it acute or chronic?3. Is inflammation present?4. How many/which joints are involved?5. Are there RED FLAGS?
Joint Pain
Joint Swelling
Diffuse/Systemic Sxs
Goals of Assessment• Identify “Red Flag” conditions
Conditions with sufficient morbidity/mortality to warrant an expedited diagnosis
• Make a timely diagnosis• Common conditions occur commonly• Many SkM conditions are self-limiting• Some conditions require serial evaluation over time
to make a Dx• Provide relief, reassurance and plan for evaluation and
treatment
RED FLAG CONDITIONS
• FRACTURE
• INFECTION
• ORGAN INVOLVEMENT
Articular vs. Periarticular
Finding ARTICULAR PERIARTICULAR
Pain Diffuse, deep "point" tenderness
ROM Pain Active+passive Active motion
in all planes in few planesSwelling Common Uncommon
Peri-/Non-articular Pain
• Fibromyalgia• Fracture• Bursitis, Tendinitis, Enthesitis, Periostitis• Carpal tunnel syndrome• Polymyalgia rheumatica• Sickle Cell Crisis• Raynaud’s phenomenon• Reflex sympathetic dystrophy• Myxedema
Inflammatory vs NoninflammatoryFeature Inflammatory Noninflammatory
Pain (worse when?) Yes (morning) Yes (night)
Swelling Soft Tissue (+ effusion) Bony
Erythema Sometimes Present Absent
Warmth Sometimes Present Absent
Morning Stiffness Prominent ( > 1 hr.) Minor ( < 45 min.)
Systemic Features+ Sometimes Present Absent
Elevated ESR or CRP* Frequent Uncommon
Synovial Fluid WBC WBC > 2,000 /mm3 WBC < 2,000 /mm3
Examples Septic arthritis, RA, Gout,Polymyalgia rheumatica
Osteoarthritis, AdhesiveCapsulitis,Osteonecrosis
+ fever, rash, weight loss, anorexia, anemia * ESR: erythrocyte sedimentation rate; CRP: C-reactive protein
Formulating a Differential DxCondition Articular Nonarticular
Inflammatory Septic
Gout
Rheumatoid arthritis
Psoriatic arthritis
Bursitis
Enthesitis
PMR
Polymyositis
Noninflammatory Osteoarthritis
Charcot Joint
Fracture
Fibromyalgia
Carpal tunnel
RSD
Mono/Oligo vs Polyarticular
Less than 4 joints• Osteoarthritis• Fracture• Osteonecrosis• Gout or Pseudogout• Septic arthritis• Lyme disease• Reactive arthrtis• Tuberculous/Fungal
arthritis• Sarcoidosis
4 or more joints• Osteoarthritis• Rheumatoid arthritis• Psoriatic arthritis• Viral arthritis• Serum Sickness• Juvenile arthritis• SLE/PSS/MCTD
History: Clues to Diagnosis• Age
• Young: JRA, SLE, Reiter's, GC arthritis• Middle: Fibromyalgia, tendinitis, bursitis, LBP RA• Elderly: OA, crystals, PMR, septic, osteoporosis
• Sex • Males: Gout, AS, Reiter's syndrome• Females: Fibrositis, RA, SLE, osteoarthritis
• Race• White: PMR, GCA and Wegener's • Black: SLE, sarcoidosis• Asian: RA, SLE, Takayasu's arteritis, Behcet's
Rheumatic Review of Systems
• Constitutional: fever, wt loss, fatigue• Ocular: blurred vision, diplopia, conjunctivitis, dry eyes• Oral: dental caries, ulcers, dysphagia, dry mouth• GI: hx ulcers, Abd pain, change in BM, melena, jaundice• Pulm: SOB, DOE, hemoptysis, wheezing• CVS: angina/CP, arrhythmia, HTN, Raynauds• Skin: photosensitivity, alopecia, nails, rash• CNS: HA, Sz, weakness, paraesthesias• Reproductive: sexual dysfunction, promiscuity, genital lesions,
miscarriages, impotence• SkM: joint pain/swelling, stiffness, ROM/function, nodules
Rheumatic Review of Systems
• Fever/Constitutional: septic arthritis, vasculitis, Still’s disease• Ocular: Reiters, Behcets, Sjogrens, Cataracts (steroids)• Oral: Sjogrens, Lupus, GC, myositis, drugs• GI: Reactive arthritis, IBD, hepatitis, Polyarteritis, Scleroderma • Pulm: SLE, RA lung, Churg-Strauss, Wegeners, Scleroderma • CVS: Vasculitis, PSS, Raynauds, antiphospholipid syndrome• Skin: SLE, psoriatic, vasculitis, Kawasaki syndrome• CNS: lupus carpal tunnel, antiphospholipid, vasculitis• GYN/GU: antiphospholipid, SLE, Reiters, Behcets, CTX• Musculoskeletal: Gout, RA, OA, fibromyalgia, fracture
Onset & Chronology• Acute: Fracture, septic arthritis, gout, rheumatic
fever, Reiter's syndrome
• Chronic: OA, RA, SLE, psoriatic arthritis, fibromyalgia
• Intermittent: gout, pseudogout, Lyme, palindromic rheumatism, Behcet's, Familial Mediterranean Fever
• Additive: OA, RA, Reiter's syndrome, psoriatic
• Migratory: Viral arthritis (hepatitis B), rheumatic fever, GC arthritis, SLE
Location
Musculoskeletal Complaint Initial Rheumatic History and Physical Exam to Determine:1. Is it articular2. Is it acute or chronic?3. Is inflammation present?4. How many/which joints are involved?
Is it Articular?
Is Complaint > 6 wks Duration?
Nonarticular Condition• Trauma/Fracture• Fibromyalgia• Polymyalgia Rheumatica• Bursitis• Tendinitis
ChronicAcuteAcute Arthritis• Infectious Arthritis• Gout• Pseudogout• Reiter’s Syndrome• Initial Presentation of Chronic Arthritis
Is Inflammation Present?1. Is there prolonged AM stiffness?2. Is there soft tissue swelling?3. Are there systemic symptoms?4. Is the ESR or CRP elevated?
Chronic Inflammatory Arthritis
Chronic Noninflammatory ArthritisHow Many Joints Involved?
Chronic InflammatoryMono/oligoarthritisConsider:• Indolent infection• Psoriatic Arthritis• Reiter’s Syndrome• Pauciarticular JA
Are DIP, CMC, Hip orKnee Involved?
Unlikely to be OsteoarthritisConsider:• Osteonecrosis• Charcot Arthritis
Osteoarthritis
Chronic InflammatoryPolyarthritis
Is it Symmetric?
Are PIP, MCP or MTP
Joints Involved?
Consider:• SLE• Scleroderma• Polymyositis
Consider: • Psoriatic Arthritis• Reiter’s Syndrome
Rheumatoid Arthritis
Yes
No
Yes
Yes
No
No
No
Yes
Yes
NoNo Yes
<4
4+
Adapted from J. Cush, MD
Know It When You See It
Hard bony enlargements Heberden’s nodes at
the DIP joints Bouchard’s nodes at
the PIP joints Often have “squared” first
CMC joint due to osteophytes at that joint
Osteoarthritis
Know It When You See It Soft synovial swelling Synovitis and volar
subluxation at the MCP joints
Synovitis of the wrists Synovitis of the PIP joints
with early swan neck deformities
Rheumatoid arthritis
Rheumatoid Arthritis: Late Stages
• Deformities
• Nodules
• Tendon Rupture
Know It When You See It
Jaccoud’s Deformity of SLE
Often associated with:• Inflammatory eye disease• Balanitis, oral ulceration, or
keratoderma• Enthesopathy• Sacroiliitis
Know It When You See It
Seronegative asymmetric arthritis
Know It When You See It Inflammation of the DIP
joints Sausage fingers Joint involvement shows
radial pattern Nail changes Psoriatic patches Arthritis may start before
the skinPsoriatic arthritis
Know It When You See It
May look like psoriasis or syphilis
Can occur in patches or as sterile pustules
Keratoderma blennorrhagica in Reiter’s syndrome
Know It When You See It “Butterfly”/Malar rash Involves cheeks,
spares nasolabial fold
Systemic lupus erythematosus
Know It When You See It
Both have periungual erythema
Interarticular dermatitis of SLEDermatomyositis
Know It When You See ItPeriungual changes
Seen in lupus erythematosus, dermatomyositis, and scleroderma
Thickening of capillary loops Dropout of capillary
loops Hemorrhage in the nail fold may
also be present
Know It When You See It
“Mantle” aka “Shawl” Sign of Dermatomyositis
Know It When You See It Not usually
associated with systemic disease
Linear scleroderma
Know It When You See It Appears in a broad-
based interrupted pattern in systemic vasculitis, including SLE
May occur as a fine, connected, lacy pattern in normals
Livedo reticularis
Know It When You See It
Can be 1o or 2o
Stress/cold can trigger Keep extremities and
body warm
Raynaud’s phenomenon
Know It When You See It
Characteristic of dermal vasculitis
Palpable purpura
Relapsing polychondritis
May also occur in Wegener’s granulomatosis and syphilis
Know It When You See It
Saddle nose deformity
Relapsing polychondritis
Left: Ear changes with inflammation in the cartilage and swelling
Right: Loss of ear cartilage in late stages
Know It When You See It
Relapsing Polychondritis
Know It When You See It• Tophi appear rather late in
gout• Prick the tophus with a
needle. Put the drop of material on a slide
Gout
Know It When You See It
Polarizer
Gout (Uric Acid) Pseudogout CPPD)
Usually a few lesions Usually found on the
extremities
Know It When You See It
Skin pustule with disseminated gonorrhea
Know It When You See It
Infection•Tap if joint/bursa infection suspected
•Do not tap through cellulitis
Know It When You See It A true connective-tissue
disease Left: Hypermobility of
joints. Can touch thumb to volar surface of forearm
Right: Hyperelasticity of skin
Associated with vascular abnormalities
Ehlers-Danlos syndrome
Acropachy Right: Soft tissue
swelling between joints
Left: Periosteal new bone formation
Know It When You See It
Hyperthyroidism
Shoulder pad sign The worst case you
are likely to see Patient also has
macroglossia and purpura
Know It When You See It
Amyloidosis
Rheumatologic Assessments
• LABS DO NOT MAKE A DIAGNOSIS; H&P DOES!
• How can labs lead you astray?• ESR/CRP: Origins and associations• Serologies (RF, ANA, CCP, APL, ANCA): when to
do & in what OTHER diseases are they positive?
• Arthrocentesis for diagnosis
RHEUMATOSCREEN PLUS CBC & differential Chem-20 Uric acid Urinalysis ESR C-reactive protein RPR CPK Aldolase ASO titer Immune complexes TFT’s w/ TSH EBV titers
IgM- RF ANA ENA (SSA, SSB,
RNP, Sm) dsDNA-Crithidia Scl-70, Jo-1 Histone Abs Ribosomal P Ab Coombs C3, C4 CH50 Cryoglobulins West Nile Ab
Lupus anticoag. Cardiolipin Ab c-ANCA anti-PR3, -MPO anti-GBM SPEP Lyme titer HIV Chlamydia Ab. Parvovirus B19 HBV, HCV, HAV HLA typing CCP Ab
CUSHY LABS INC. “YOUR INDECISION IS OUR BREAD AND BUTTER”
ANA+
RF
CBC & diff $35.00
Chem-20 $108.00
Urinalysis $30.00
ESR or CRP $25.30
Uric acid $40.00
$ 238.30
CUSHY LABS INC. “YOUR INDECISION IS OUR BREAD AND BUTTER”
Presbyterian Hosp. CheapoScreen
Further Investigations• Many conditions are self-limiting• Consider when:
• Systemic manifestations (fever, wt.loss, rash, etc)• Trauma (do exam or imaging for Fracture, ligament
tear)• Neurologic manifestations• Lack of response to observation & symptomatic Rx
(<6wks)• Chronicity ( > 6 weeks)
Acute Phase Reactants• Erythrocyte Sedimentation Rate (nonspecific)• C-Reactive Protein (CRP)• Fibrinogen• Serum Amyloid A (SAA)• Ceruloplasmin• Complement (C3, C4)• Haptoglobin• Ferritin• Other indicators: leukocytosis, thrombocytosis,
hypoalbuminemia, anemia of chronic disease
• ESR : Introduced by Fahraeus 1918• Mechanisms: Rouleaux formation
• Characteristics of RBCs• Shear forces and viscosity of plasma• Bridging forces of macromolecules. High MW fibrinogen
tends to lessen the negative charge between RBCs and promotes aggregation.
• Methods: Westergren method• Low ESR: Polycythemia, Sickle cell, hemolytic anemia,
hemeglobinopathy, spherocytosis, delay, hypofibrinogen, hyperviscosity (Waldenstroms)
• High ESR: Anemia, hypercholesterolemia, female, pregnancy, inflammation, malignancy,nephrotic syndrome
Erythrocyte Sedimentation Rate
Extreme Elevation of ESRCause ESR > 100
(%)ESR 75 –99
(%)
Infection 14 (33) 6 (16)
Renal Dz 7 (17) 4 (11)
Neoplasm 7 (17) 4 (11)
Inflammatory 6 (14) 6 (16)
Miscellaneous 4 (9.5) 0
Unknown 4 (9.5) 17 (46)
Total 42 (100) 37 (100)
RME Fincher, Arch Int Med 146:1986
ESR & Age
0
10
20
30
40
50
60
ES
R m
m/h
r
<30 30-39 40-49 50-59 60-69 70-79 80-89
Age (years)
M=Age/2F=(Age+10)/2
ACP Recommendations for Diagnostic Use of Erythrocyte Sedimentation Rate
• The ESR should not be used to screen asymptomatic persons for disease
• The ESR should be used selectively and interpreted with caution....Extreme elevation of the ESR seldom occurs in patients with no evidence of serious disease
• If there is no immediate explanation for an increased ESR, the physician should repeat the test in several months rather than search for occult disease
• The ESR is indicated for the diagnosis and monitoring of temporal arteritis and polymyalgia rheumatica
• In diagnosing and monitoring patients with rheumatoid arthritis, the ESR should be used prinicipally to resolve conflicting clinical evidence
• The ESR may be helpful in monitoring patients with treated Hodgkin’s disease
Case• 28 yr. old WF presents with sudden onset of knee
swelling and pain 7 days ago. Two days later, knee resolved but both wrists began to swell. On day 7, the wrists improved but all PIPs were swollen and tender.
• By day 10 She visits her PCP who examines her and orders “Rheumatoscreen Plus” and XRAYs.
• He sends her home on OTC ibuprofen, tylenol and Vicks Vapo-Rub.
• she complained of arthritis in PIPs, wrists, knees and ankles. + Tenosynovitis L wrist. AM stiffness was 4 hours.
Case• Day 14 she returns to PCP with
low grade fever, pruritic rash on the trunk and extremities.
• Exam: symmetric polyarthritis in an RA-like distribution. Tenosynovitis has resolved. Urticarial lesions over trunk and extensor surface of arms. (+)2 cm nontender, left axillary LN. No malar rash, nodules, acne, or Raynauds phenomena.
• Investigations?
Case• WBC = 11.2• H/H = 13.7 / 38.9 MCV = 89• ESR = 123 mm/hr• SMA-12 WNL, except albumin = 3.3, AST-67, ALT 77• ANA negative• RF 57 IU/ml (nl < 30 IU/ ml)• C3 173, C4 28, ASO = 151 Todd units• Uric Acid = 6.6, CCP Ab neg• Normal SPEP, UPEP, TFT’s, TSH, Ferritin• Others?
Case• She returns after 1 wk for LN Bx results
(negative) • Pt. states her rash and arthritis have nearly
resolved.• Exam confirms only mild swelling in knees• However, her sclera are definitely icteric.• Next?
• WBC = 11.2• H/H = 13.7 / 38.9 MCV = 89• ESR = 123 mm/hr• SMA-12 WNL, except albumin = 3.3• ANA negative• RF 31 IU/ml (nl < 30 IU/ ml)• C3 173, C4 28, ASO = 151 Todd units• Uric Acid = 6.6• Normal SPEP, UPEP, TFT’s, TSH, Ferritin• HBsAg (+), Neg. for HCV, HAV, HIV
Case
DDx of Migratory Arthritis• Viral arthritis (hepatitis B)
• Rheumatic fever
• Gonococcal arthritis
• SLE
• Behcets
Hepatitis B Associated Arthritis• Arthritis and urticaria part of the “prodrome”
• Manifestations due to immune complex deposition• Before the Jaundice• Usually while LFTs elevated
• Acute onset• Additive (RA like) or migratory (ARF like) arthritis• Often with tenosynovitis• Synovial fluid: inflammatory• Arthritis disappears with onset of Jaundice
What am I?