1. INTRODUCTION TO RHEUMATOLOGY
KATHRYN DAO, MD
Arthritis Consultation Center
July 21, 2005

2. I am the size of a rabbit with fur as smooth as an otter.
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 legs
My name means "flat feet." 1

3. Why even care? 3500 rheumatologists
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.

4. Musculoskeletal Complaint
Joint Pain
Joint Swelling
Diffuse/Systemic Sxs
Initial Rheumatic History and Physical Exam to Determine:
1. Is it articular
2. Is it acute or chronic?
3. Is inflammation present?
4. How many/which joints are involved?
5. Are there RED FLAGS?

5. 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

6. RED FLAG CONDITIONS
FRACTURE
INFECTION
ORGAN INVOLVEMENT

7. Articular vs. Periarticular
Finding ARTICULAR PERIARTICULAR
Pain Diffuse, deep "point" tenderness
ROM Pain Active+passive Active motion
in all planes in few planes
Swelling Common Uncommon

8. 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

9. Inflammatory vs Noninflammatory

10. Formulating a Differential Dx
Condition
Articular
Nonarticular
Inflammatory
Septic
Gout
Rheumatoid arthritis
Psoriatic arthritis
Bursitis
Enthesitis
PMR
Polymyositis
Noninflammatory
Osteoarthritis
Charcot Joint
Fracture
Fibromyalgia
Carpal tunnel
RSD

11. 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

12. 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

13. 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

14. 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

15. 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

16. Location

17. Osteoarthritis Rheumatoid Arthritis Musculoskeletal Complaint
Initial Rheumatic History and Physical Exam to Determine:
1. Is it articular
2. Is it acute or chronic?
3. Is inflammation present?
4. How many/which joints are involved?
Nonarticular Condition
Trauma/Fracture
Fibromyalgia
Polymyalgia Rheumatica
Bursitis
Tendinitis
Is it Articular? No
Yes
Is Complaint > 6 wks Duration? No
Yes
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?
Acute Arthritis
Infectious Arthritis
Gout
Pseudogout
Reiter’s Syndrome
Initial Presentation of Chronic Arthritis
Acute
Chronic No
Yes
Chronic Inflammatory Arthritis
Chronic Inflammatory
Mono/oligoarthritis
Consider:
Indolent infection
Psoriatic Arthritis
Reiter’s Syndrome
Pauciarticular JA
Chronic Noninflammatory Arthritis
<4
How Many Joints Involved?
Are DIP, CMC, Hip or
Knee Involved? 4+
Chronic Inflammatory
Polyarthritis No
Yes No
Consider:
Psoriatic Arthritis
Reiter’s Syndrome
Is it Symmetric?
Unlikely to be
Osteoarthritis
Consider:
Osteonecrosis
Charcot Arthritis
Osteoarthritis
Yes
Consider:
SLE
Scleroderma
Polymyositis
Rheumatoid
Arthritis
Are PIP, MCP or MTP
Joints Involved? No
Yes
Adapted from J. Cush, MD

18. Know It When You See It Osteoarthritis 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 3

19. Know It When You See It Rheumatoid arthritis 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 4

20. Rheumatoid Arthritis: Late Stages
Associated deformities: Zeta thumb, swan neck, buttonierres, subluxation, ulnar deviation
Deformities
Nodules
Tendon Rupture 5

21. Know It When You See It Jaccoud’s Deformity of SLE
Associated deformities: Zeta thumb, swan neck, buttonierres, subluxation, ulnar deviation
Jaccoud’s Deformity of SLE 5

22. Know It When You See It Seronegative asymmetric arthritis
Often associated with:
Inflammatory eye disease
Balanitis, oral ulceration, or keratoderma
Enthesopathy
Sacroiliitis
Seronegative asymmetric arthritis 10

23. Know It When You See It Psoriatic arthritis
Inflammation of the DIP joints
Sausage fingers
Joint involvement shows radial pattern
Nail changes
Psoriatic patches
Arthritis may start before the skin
Psoriatic arthritis 7

24. 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 9

25. Know It When You See It Systemic lupus erythematosus
“Butterfly”/Malar rash
Involves cheeks, spares nasolabial fold
Systemic lupus erythematosus 12

26. Know It When You See It Dermatomyositis
Interarticular dermatitis of SLE
Both have periungual erythema 13

27. Know It When You See It Periungual 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 15

28. Know It When You See It “Mantle” aka “Shawl” Sign of Dermatomyositis17

29. Know It When You See It Not usually associated with systemic disease
Linear scleroderma 18

30. 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 19

31. Know It When You See It Can be 1o or 2o Stress/cold can trigger
Keep extremities and body warm
Raynaud’s phenomenon 19

32. Know It When You See It Palpable purpura
Characteristic of dermal vasculitis
Palpable purpura 20

33. Know It When You See It Saddle nose deformity Relapsing polychondritis
May also occur in Wegener’s granulomatosis and syphilis
Saddle nose deformity 21

34. Relapsing polychondritis
Know It When You See It
Relapsing polychondritis
Left: Ear changes with inflammation in the cartilage and swelling
Right: Loss of ear cartilage in late stages
Relapsing Polychondritis 22

35. Know It When You See It Gout Tophi appear rather late in gout
Prick the tophus with a needle. Put the drop of material on a slide
Gout 24

36. Know It When You See It
Polarizer
Pseudogout CPPD)
Gout (Uric Acid) 25

37. Know It When You See It Usually a few lesions
Usually found on the extremities
Skin pustule with
disseminated gonorrhea 27

38. Know It When You See It Infection
Tap if joint/bursa infection suspected
Do not tap through cellulitis
Infection 28

39. Know It When You See It Ehlers-Danlos syndrome
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 33

40. Know It When You See It Acropachy
Right: Soft tissue swelling between joints
Left: Periosteal new bone formation
Hyperthyroidism 32

41. Know It When You See It Amyloidosis Shoulder pad sign
The worst case you are likely to see
Patient also has macroglossia and purpura
Amyloidosis 31

42. 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

43. 
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
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
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
CUSHY LABS INC. “YOUR INDECISION IS OUR BREAD AND BUTTER”

44. Presbyterian Hosp. CheapoScreen
CBC & diff $35.00
Chem $108.00
Urinalysis $30.00
ESR or CRP $25.30
Uric acid $40.00
ANA + RF $
CUSHY LABS INC. “YOUR INDECISION IS OUR BREAD AND BUTTER”

45. 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)

46. 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

47. Erythrocyte Sedimentation Rate
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

48. Extreme Elevation of ESR
Cause
ESR > 100 (%)
ESR 75 –99 (%)
Infection
14 (33)
6 (16)
Renal Dz
7 (17)
4 (11)
Neoplasm
Inflammatory
6 (14)
Miscellaneous
4 (9.5)
Unknown
17 (46)
Total
42 (100)
37 (100)
RME Fincher, Arch Int Med 146:1986

49. ESR & Age
M=Age/2
F=(Age+10)/2

50. 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

51. 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.
C/O fatigue. Denies fever, rash

52. 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?

53. 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?

54. 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?

55. Case 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

56. DDx of Migratory Arthritis
Viral arthritis (hepatitis B)
Rheumatic fever
Gonococcal arthritis
SLE
Behcets

57. 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

58. What am I?