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?