INTRODUCTION TO RHEUMATOLOGY

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INTRODUCTION TO RHEUMATOLOGY

• KATHRYN DAO, MD
• Arthritis Consultation Center
• July 21, 2005

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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."
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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
200453383-6. Center for Disease Control and
Prevention. MMWR 200453388.
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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?
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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

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RED FLAG CONDITIONS

• FRACTURE
• INFECTION
• ORGAN INVOLVEMENT

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Articular vs. Periarticular

• Finding ARTICULAR PERIARTICULAR
• Pain Diffuse, deep "point" tenderness
• ROM Pain Activepassive Active motion
• in all planes in few planes
• Swelling Common Uncommon

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Peri-/Non-articular Pain

• Fibromyalgia
• Fracture
• Bursitis, Tendinitis, Enthesitis, Periostitis
• Carpal tunnel syndrome
• Polymyalgia rheumatica
• Sickle Cell Crisis
• Raynauds phenomenon
• Reflex sympathetic dystrophy
• Myxedema

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Inflammatory vs Noninflammatory
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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
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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

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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

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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

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Rheumatic Review of Systems

• Fever/Constitutional septic arthritis,
  vasculitis, Stills 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

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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

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Location
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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?
Musculoskeletal Complaint

• Nonarticular Condition
• Trauma/Fracture
• Fibromyalgia
• Polymyalgia Rheumatica
• Bursitis
• Tendinitis

Is it Articular?
No
Yes
Is Complaint gt 6 wks Duration?
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?
No
Chronic

• Acute Arthritis
• Infectious Arthritis
• Gout
• Pseudogout
• Reiters Syndrome
• Initial Presentation of Chronic Arthritis

Acute
No
Yes
Chronic Inflammatory Arthritis

• Chronic Inflammatory
• Mono/oligoarthritis
• Consider
• Indolent infection
• Psoriatic Arthritis
• Reiters Syndrome
• Pauciarticular JA

Chronic Noninflammatory Arthritis
lt4
How Many Joints Involved?
Are DIP, CMC, Hip or Knee Involved?
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Chronic Inflammatory Polyarthritis
No
Yes
No

• Consider
• Psoriatic Arthritis
• Reiters 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
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Know It When You See It

• Hard bony enlargements
• Heberdens nodes at the DIP joints
• Bouchards nodes at the PIP joints
• Often have squared first CMC joint due to
  osteophytes at that joint

Osteoarthritis
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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
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Rheumatoid Arthritis Late Stages

• Deformities
• Nodules
• Tendon Rupture

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Know It When You See It
Jaccouds Deformity of SLE
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Know It When You See It

• Often associated with
• Inflammatory eye disease
• Balanitis, oral ulceration, or keratoderma
• Enthesopathy
• Sacroiliitis

Seronegative asymmetric arthritis
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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 skin

Psoriatic arthritis
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Know It When You See It

• May look like psoriasis or syphilis
• Can occur in patches or as sterile pustules

Keratoderma blennorrhagica in Reiters syndrome
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Know It When You See It

• Butterfly/Malar rash
• Involves cheeks, spares nasolabial fold

Systemic lupus erythematosus
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Know It When You See It
Dermatomyositis
Interarticular dermatitis of SLE
Both have periungual erythema
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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

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Know It When You See It
Mantle aka Shawl Sign of Dermatomyositis
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Know It When You See It

• Not usually associated with systemic disease

Linear scleroderma
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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
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Know It When You See It

• Can be 1o or 2o
• Stress/cold can trigger
• Keep extremities and body warm

Raynauds phenomenon
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Know It When You See It

• Characteristic of dermal vasculitis

Palpable purpura
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Know It When You See It

• Relapsing polychondritis
• May also occur in Wegeners granulomatosis and
  syphilis

Saddle nose deformity
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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
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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
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Know It When You See It
Polarizer
Pseudogout CPPD)
Gout (Uric Acid)
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Know It When You See It

• Usually a few lesions
• Usually found on the extremities

Skin pustule with disseminated gonorrhea
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Know It When You See It

• Tap if joint/bursa infection suspected
• Do not tap through cellulitis

Infection
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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
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Know It When You See It

• Acropachy
• Right Soft tissue swelling between joints
• Left Periosteal new bone formation

Hyperthyroidism
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Know It When You See It

• Shoulder pad sign
• The worst case you are likely to see
• Patient also has macroglossia and purpura

Amyloidosis
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Rheumatologic Assessments

• LABS DO NOT MAKE A DIAGNOSIS HP 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

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RHEUMATOSCREEN PLUS
?

• CBC differential
• Chem-20
• Uric acid
• Urinalysis
• ESR
• C-reactive protein
• RPR
• CPK
• Aldolase
• ASO titer
• Immune complexes
• TFTs 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
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Presbyterian Hosp. CheapoScreen
CBC diff 35.00 Chem-20
108.00 Urinalysis 30.00 ESR or CRP
25.30 Uric acid 40.00

• ANA
• RF

238.30
CUSHY LABS INC. YOUR INDECISION IS OUR BREAD AND
BUTTER
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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
  (lt6wks)
• Chronicity ( gt 6 weeks)

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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

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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

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Extreme Elevation of ESR
Cause ESR gt 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 1461986
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ESR Age
MAge/2 F(Age10)/2
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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 Hodgkins disease

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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.

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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?

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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 lt 30 IU/ ml)
• C3 173, C4 28, ASO 151 Todd units
• Uric Acid 6.6, CCP Ab neg
• Normal SPEP, UPEP, TFTs, TSH, Ferritin
• Others?

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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?

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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 lt 30 IU/ ml)
• C3 173, C4 28, ASO 151 Todd units
• Uric Acid 6.6
• Normal SPEP, UPEP, TFTs, TSH, Ferritin
• HBsAg (), Neg. for HCV, HAV, HIV

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DDx of Migratory Arthritis

• Viral arthritis (hepatitis B)
• Rheumatic fever
• Gonococcal arthritis
• SLE
• Behcets

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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

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What am I?