DIAPOSITIVAS QUE USO EL DR. ACHURRA DE LA
AMERICAN COLLEGE OF RHEUMATOLOGY
Es solo la parte teórica y es puntual, así que si quieren ampliar
vayan a los libros o artículos =)
Acute monarthritis: selected causes
Noninflammatory
Trauma
Hemarthrosis
Sickle-cell disease
Avascular necrosis
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Inflammatory
Septic arthritis
Gout
Pseudogout
Lyme disease
Spondylarthropathy
Juvenile chronic arthritis
Palindromic rheumatism
Chronic monarthritis: selected causes
Infection related
Mycobacterial
Fungal
Lyme disease
Pyogenic bacterial
Mycoplasma
Adjacent osteomyelitis
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Not infection-related
Spondylarthropathy
Gout, pseudogout
Juvenile chronic arthritis
Hemophilia
PVNS
Synovial sarcoma
Neuropathic arthropathy
Osteoarthritis
Avascular necrosis
Polyarthritis: selected causes
Noninfectious causes
RA/Juvenile rheumatoid arthritis
SLE and other connective tissue
diseases
Spondylarthropathies
Gout and pseudogout
Vasculitis
Wegener’s granulomatosis
PAN/microscopic polyangiitis
Drug-induced/ serum sickness
Henoch-Schonlein pupura
Sickle-cell disease
Osteoarthritis
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Infectious causes
Bacterial sepsis
Viral arthritis
Parvovirus
HIV
Hepatitis
C/cryglobulinemia
Lyme arthritis
Subacute bacterial endocarditis
Rheumatic fever (migratory
pattern)
Chronic polyarthritis: selected causes
Rheumatoid arthritis, juvenile rheumatoid arthritis
Spondylarthropathies
Osteoarthritis
Systemic lupus and other connective tissue diseases
Gout
Pseudogout
Sarcoidosis
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Intermittent arthritis: selected causes
Gout
Pseudogout
Lyme disease
Palindromic rheumatism
Rheumatoid arthritis
Sarcoidosis
Mechanical joint problems
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Back pain: selected causes
Infectious
Vertebral osteomyelitis
Septic discitis
Epidural abscess
Septic sacroiliitis
Noninfectious/noninflammatory
Osteoarthritis
DISH
Vertebral compression fracture
Muscular/ligamentous strain
Malignancy
Retroperitoneal fibrosis
Aortic aneurysm
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Noninfectious/inflammatory
Ankylosing spondylitis
Reactive arthritis
Psoriatic spondylitis
Spondylitis associated with
inflammatory bowel disease
Tophaceous gout
Classification of joint effusions
Type of Fluid Special Features Leukocytes/mm3
Normal Clear, colorless,
viscous
200
Noninflammatory Clear, yellow, viscous 200-2000
Inflammatory Cloudy, yellow,
decreased viscosity
2000-100,000
Septic Purulent, markedly
decreased viscosity
Usually >50,000
(>95% PMNs)
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Differential diagnosis of noninflammatory effusions (group
I)
Osteoarthritis
Early rheumatoid arthritis
Trauma
Osteochondritis dissecans
Osteopecrosis
Sickle-cell disease
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Chronic/subsiding crystal synovitis
Lupus
Vasculitis
Scleroderma
Amyloidosis
Hypothyroidism
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Differential diagnosis of inflammatory effusions (group II)
Rheumatoid arthritis
Spondylarthropathies
Crystalline arthritis
Juvenile chronic arthritis
Viral arthritis
Lyme arthritis
Neisserial infections
Early or partially treated septic arthritis
Rheumatic fever
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Differential diagnosis of purulent effusions (group III)
Pyogenic infections
Mycobacterial infections
Fungal infections
Crystalline arthritis
“Pseudo-septic” arthritis (rheumatoid arthritis)
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Differential diagnosis of hemorrhagic effusions (group IV)
Trauma
Neuropathic arthropathy
Bleeding disorders
Hemophilia
Scurvy
Thrombocytopenia
Von Willebrand’s disease
Anticoagulation
Pigmented villonodular synovitis
Synovial sarcoma
Prosthetic joint
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Osteoarthritis: general features
Clinical
A.M. stiffness
Gel phenomenon
Joint pain and tenderness
Crepitus
Bony swelling
Angulation deformities
Functional Impairment
Laboratory
Noninflammatory
synovial fluid
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Radiographic
Osteophytes
Joint space narrowing
Subchondral
Cysts and sclerosis
Malalignment
Secondary osteoarthritis: causes
Dysplastic
Chondrodysplasias
Epiphyseal dysplasias
Congenital dislocation of the hip
Developmental disorders
Leg-length inequality
Posttraumatic
Acute
(e.g., fracture through joint)
Repetitive
(e.g., occupational injury)
Postoperative
(e.g., meniscectomy)
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Skeletal Failure
Osteonecrosis
Osteochondritis
Paget’s disease
Postinflammatory
Infection
Inflammatory arthropathies
(e.g., rheumatoid arthritis)
Secondary osteoarthritis: causes, cont’d
Endocrine and Metabolic
Acromegaly
Ochronosis
Hemochromatosis
Crystal deposition disorders
Connective Tissue
Hypermobility syndromes
Mucopolysaccharidoses
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Endemic
Kashin-Beck disease
Mselene disease
Miscellaneous
Frostbite
Hemoglobinopathies
Rheumatoid arthritis: epidemiology
Prevalence 1% in varied ethnic groups
Female predominance
Associated with HLA-DR4 and the DR– associated alleles, DRB1*0401 and
DRB1*0404
Variable age of onset
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ACR 1987 criteria for the classification of acute rheumatoid
arthritis
Need at least four of seven criteria:
1. Morning stiffness lasting at least 1 hr
2. Soft- tissue swelling or fluid in at least 3 joint areas simultaneously
3. At least one area swollen in a wrist, MCP, or PIP joint*
4. Symmetric arthritis*
5. Rheumatoid nodules
6. Abnormal amounts of serum rheumatoid factor
7. Erosions or bony decalcification on radiographs of the hand and
wrist
* For classification purposes, a patient shall be said to have rheumatoid
arthritis if he/she has satisfied at least 4 or these 7 criteria. Criteria 1
through 4 must have been present for at least 6 weeks.
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Classification of progression of rheumatoid arthritis
Stage I, Early
1. No destructive changes on radiographs
2. May have radiographic evidence of osteoporosis
Stage II, Moderate
1. Radiographic evidence of osteoporosis; may have slight
subchondral bone or cartilage destruction
2. No joint deformities; limitation of joint mobility may be present
3. Adjacent muscle atrophy
4. Nodules and tenosynovitis may be present
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Classification of progression of rheumatoid arthritis, cont’d
Stage III, Severe
1. Radiographic evidence of osteoporosis as well as cartilage and
bone destruction
2. Joint deformity without ankylosis
3. Extensive muscle atrophy
4. Nodules and tenosynovitis may be present
Stage IV, Terminal
1. Fibrous or bony ankylosis
2. Criteria of stage III
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ACR Preliminary definition of improvement in rheumatoid
arthritis (ACR-20, 50, 70)
Required
* Improvement in tender joint count
* Improvement in swollen joint count
* Improvement in 3 of the 5 following measures
Patient pain assessment
Patient global assessment
Physician global assessment
Patient self-assessed disability
Acute-phase reactant (ESR or CRP)
* 20%, 50%, or 70%
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ACR Preliminary criteria for clinical remission in rheumatoid
arthritis
Five criteria present for 2 months and no vasculitis, pericarditis, pleuritis,
myositis, weight loss, or fever
Morning stiffness not exceeding 15 minutes
No fatigue
No joint pain (by history)
No joint tenderness or pain on motion
No soft-tissue swelling in joints or tendon sheaths
ESR < 30 mm/hr (women) or 20 mm/hr (men)
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ACR 1991 revised criteria for global functional status in
rheumatoid arthritis
Class I: performs usual activities of daily living
Class II: performs usual self-care and vocational activities, but limited in
avocational activities
Class III: performs usual self-care activities, but limited in vocational and
avocational activities
Class IV: limited in ability to perform usual self-care, vocational, and
avocational activities
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Stages of rheumatoid arthritis pathogenesis
Stage Symptoms Findings
Stage 1: Antigen
presentation to T cells
None None
Normal x-ray
Stage 2: T and B cell
proliferation,
angiogenesis in
synovium
Malaise, mild joint
stiffness, swelling
Swelling or pain of small
joints, wrists, knees
Normal x-ray
Stage 3: SF PMN
accumulation, synovial
cell proliferation
Joint pain, swelling,
AM stiffness,
malaise, weakness
Warm, swollen joints, Inc
SF, soft tissue
proliferation, limited ROM,
nodules, soft tissue
swelling on x-ray
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Stages of rheumatoid arthritis pathogenesis, cont’d
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Stage Symptoms Findings
Stage 4: Pannus
invasion, chondrocyte
activation, enzyme
activation
Same as stage 3 Same as stage 3
Periarticular osteopenia,
proliferative pannus on
MRI
Stage 5: Subchondral
bone erosion, pannus
invasion of cartilage
stretched ligaments
Same as stage 3
Plus loss of
function deformity
Same as stage 3
Plus instability, flexion
contractures, extra-
articular disease, early
erosions and joint space
narrowing on x-ray
Radiographic features of rheumatoid arthritis
Periarticular soft-tissue swelling
Juxtaarticular osteopenia
Marginal erosions
Joint-space narrowing
Symmetric involvement
Deformities in advanced disease
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Felty’s syndrome
Seropositive rheumatoid arthritis
Splenomegaly
Leukopenia (neutropenia)
Recurrent infections
Leg ulcers
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Felty’s syndrome: extraarticular features
Rheumatoid nodules 75%
Weight loss 70%
Sjögren’s syndrome 55%
Lymphadenopathy 35%
Leg ulcers 25%
Pleuritis 20%
Skin pigmentation 15%
Neuropathy 15%
Episcleritis 10%
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Large granular lymphocyte (LGL) syndrome
LGL syndrome may be a cause of neutropenia and splenomegaly in RA
Clinical features may mimic Felty’s syndrome (“pseudo” Felty’s)
Neutropenia with normal or increased total WBC due to lymphocytosis
Clonal populations of LGL in some patients may represent neoplastic
proliferation and some patients ultimately develop leukemia or
lymphoma
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Criteria for classification of adult-onset Still’s disease
Need more than 5 criteria, including more than 2 major criteria
Major Criteria
Fever > 39oC for > 1 week
Arthralgias for > 2 weeks
Salmon-pink rash, often with fevers
Leukocytosis > 10,000/mm3 and > 80% granulocytes
Minor Criteria
Sore throat
Lymphadenopathy and/or spenomegaly
Liver dysfunction
Negative RF and negative ANA
Exclusions
Infection
Malignancies
Other rheumatic diseases
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Rheumatoid factor
Antibody directed against the Fc portion of IgG
Present in approximately 80% of RA patients
May be involved in disease pathogenesis
Higher levels tend to be associated with poorer prognosis
Found in other rheumatologic and non-rheumatologic conditions
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Classification criteria for juvenile rheumatoid arthritis
Age at onset <16 years
Arthritis defined as articular swelling/effusion or the presence of two or
more of the following signs:
Limitation of range of movement
Joint tenderness on palpation
Pain on joint movement
Increased heat over joint
Duration of arthritis > 6 weeks
Exclusion of other causes of arthritis
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Juvenile rheumatoid arthritis subtypes
Onset type F:M ANA RF
Systemic 1:1 Neg Neg
Polyarticular
RF neg 6:1 25%+ Neg
RF pos 8:1 75%+ Pos
Pauciarticular 8:1 >80% Neg
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Juvenile rheumatoid arthritis subtypes, cont’d
Onset type Extraarticular manifestations
Systemic High spiking (quotidian) fever, severe anemia, rash,
serositis, organomegaly leukocytosis, pharyngitis
Polyarticular
RF neg Low-grade fever, mild anemia, malaise
RF pos Low-grade fever, mild anemia, malaise, nodules
Pauciarticular Chronic iridocyclitis in 40%, increased incidence with
+ANA
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Juvenile rheumatoid arthritis subtypes, cont’d
Onset type Prognosis
Systemic 50% deforming arthritis
Polyarticular
RF neg 50% deforming arthritis
RF pos 90% deforming arthritis
Pauciarticular Deforming arthritis uncommon
Iridocyclitis 20% risk of blindness
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Systemic-onset juvenile rheumatoid arthritis: clinical
features
Spiking fevers
Rash
Lymphadenopathy
Hepatosplenomegaly
Pericarditis
Pleuritis
Arthritis
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Childhood malignancy and bone pain
Leukemia
Lymphoma
Neuroblastoma
Histiocytosis
Osteogenic sarcoma
Ewing’s sarcoma
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Clinical signs of malignancy
Child appears miserable
Low-grade fevers
Night pain
Pain out of proportion to physical findings
Pain in both bones and joints
Pallor, petechiae
Hepatosplenomegaly
Lymphadenopathy
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Kawasaki disease: diagnostic guidelines
Must have fever and 4 out of 5 other criteria.
1. Spiking fever for at least 5 days
2. Bilateral conjunctival congestion
3. Oropharyngeal involvement:
Diffuse oropharyngeal erythema, strawberry tongue, or redness,
dryness and fissures of lips, polymorphous erythematous rash
4. Cervical lymphadenopathy
5. One or more of the following signs:
Indurative edema of hands and feet
Erythema of palms and soles
Desquamation of fingers and toes about 2 weeks after onset
Transverse grooves in nails 2 to 3 months after onset
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Spondylarthropathies
Ankylosing spondylitis
Psoriatic arthritis
Reactive arthritis
Enteropathic arthritis
Crohn’s disease
Ulcerative colitis
Juvenile ankylosing spondylitis
Undifferentiated spondylarthropathies
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Criteria for classification of spondylarthropathy
Inflammatory spinal pain or synovitis (asymmetric or predominantly in
lower limbs) plus more than 1 of the following:
Positive family history
Psoriasis
Inflammatory bowel disease
Urethritis, cervicitis, or acute diarrhea < 1 mo. before arthritis
Buttock pain alternating between right and left gluteal areas
Enthesopathy
Sacroiliitis
Sensitivity 78.4% and specificity 89.6%
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New York criteria for diagnosis of ankylosing spondylitis
Definite ankylosing spondylitis If: Criterion 4 or 5 plus 1, 2, or 3.
1. Limited lumbar motion
2. Low back pain for 3 months improved with exercise not relieved by
rest
3. Reduced chest expansion
4. Bilateral, grade 2 to 4, sacroiliitis on X-ray
5. Unilateral, grade 3 to 4, sacroiliitis on X-ray
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Ankylosing spondylitis: characteristics of back pain
Onset of back discomfort before age 40
Insidious onset
Duration longer than 3 months
Associated with morning stiffness
Improvement with exercise
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Spondylarthropathies: nonvertebral manifestations
Asymmetric peripheral arthritis
Sausage digits
Enthesopathy
Achilles yenosynovitis
Plantar fasciitis
Costochondritis
Acute anterior uveitis/iridocyclitis
Mucocutaneous lesions
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Nail involvement
Fatigue, weight loss
Amyloidosis
Apical pulmonary fibrosis
Immunoglobulin A nephropathy
Cardiac involvement
HLA-B27 disease associations
Ankylosing spondylitis > 90% (white males)
with uveitis or aortitis ~100%
Reactive arthritis 50-80%
with sacroiliitis or uveitis 90%
Juvenile spondylarthropathy 80%
Inflammatory bowel disease
Peripheral Not increased
Axial
Crohn’s disease 50%
Ulcerative colitis 70%
Psoriasis
Peripheral Not increased
Axial 50%
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Psoriatic arthritis: musculoskeletal characteristics
Asymmetrical arthritis
Dactylitis/Sausage digit
Tenosynovitis
Enthesitis
Heel pain
Sacroiliitis
Spondylitis
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Psoriatic arthritis: cutaneous and other manifestations
Psoriasis
Erythroderma
Nail pitting
Onycholysis
Conjunctivitis/iritis
Valvular heart disease
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Psoriatic arthritis: radiographic characteristics
Erosive arthritis (usually asymmetric)
Pencil-in-cup deformity
Ray phenomenon
Arthritis mutilans
Bony ankylosis
Spurs/periosteal reaction
Non-marginal asymmetric syndesmophytes
Asymmetric sacroiliiti
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Reactive arthritis
Seronegative asymmetric arthritis following:
Urethritis or cervicitis
Infectious diarrhea
Often associated with:
Inflammatory eye disease
Enthesopathy
Circinate balanitis, oral ulceration or keratoderma
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Criteria for reactive arthritis: sensitivity and specificity
Method Sensitivity Specificity
1. Arthritis > 1 mo. 84.3% 98.2%
with urethritis and/or
cervicitis
2. Arthritis > 1 mo. and 85.5% 96.4%
either urethritis or cervicitis,
or bilateral conjunctivitis
3. Episode of arthritis, 50.6% 98.8%
conjunctivitis, and urethritis
4. Episode of arthritis of
more than 1 month, urethritis, 48.2% 98.8%
and conjunctivitis
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Systemic lupus erythematosus: 1982 classification criteria
Malar rash
Discoid rash
Photosensitivity
Oral ulcers
Arthritis
Serositis
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Renal disorder
Neurologic disorder
Hematologic disorder
Immunologic disorder
Antinuclear antibody
Systemic lupus erythematosus: 1982 classification criteria
definitions
Malar rash Fixed erythema, flat or raised, sparing the nasolabial folds
Discoid rash Raised patches, adherent keratotic scaling, follicular plugging; older lesions may cause scarring
Photosensitivity Skin rash from sunlight
Oral or nasopharyngeal Usually painless
ulcers
Arthritis Nonerosive, inflammatory in two or more peripheral joints
Serositis Pleuritis or pericarditis
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Systemic lupus erythematosus: 1982 classification criteria
definitions, cont’d
Renal disorder Persistent proteinuria or cellular casts
Neurologic disorder Seizures or psychosis
Hermatologic Hemolytic anemia, leukopenia (<4,000/mm3), lymphopenia (<1,500/mm3), or thrombocytopenia (<100,00/mm3)
Immunologic disorder Antibodies to dsDNA or SM or positive antiphospholipid antibodies (IgG or IgM antibodies, lupus anticoagulant, or false- positive serologic test positive serologic test for syphilis)
Antinuclear antibody test Positive
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Systemic lupus erythematosus: classification of nephritis
Normal glomeruli
a) Nil by all techniques
b) Normal by light but deposits on EM or IF
Mesangial glomerulonephritis
Focal glomerulonephritis
Diffuse glomerulonephritis
Diffuse membranous glomerulonephritis
Advanced sclerosing glomerulonephritis
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Systemic lupus erythematosus: nervous system
manifestations
Seizures Ataxia
Headache Rigidity, tremor
Stroke syndromes Chorea
Subarachnoid hemorrhage Hemiballismus
Transverse myelitis Coma
Multiple sclerosis-like disorder Dementia
Optic neuritis Psychiatric disorders
Aseptic meningitis
Peripheral neuropathy
Cranial neuropathy
Mononeuritis multiplex
Guillain-Barré syndrome
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Autoantibody-disease associations: SLE and drug-induced
lupus
Antigen SLE Drug-Induced LE
dsDNA 40% No
ssDNA 70% 75%-80%
Histone 70% >95%
Sm antigen 30% No
Nuclear RNP 30% No
Ribosomal RNP 10%
SS-A/Ro 35% No
SS-B/La 15% No
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Drug-induced lupus: definite drug associations
Hydralazine
Procainamide
Minocycline
Chlorpromazine
Isoniazid
Penicillamine
Methyldopa
Interferon-alpha
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Drug-induced lupus: possible drug associations
Anticonvulsants
Quinidine
Propylthiouracil
Sulfonamides
Lithium
Beta-blockers
Nitrofurantoin
Sulfasalazine
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Diltiazem
Hydrazine
Interferon-gamma
TNF inhibitors
Preliminary classification criteria for antiphospholipid
syndrome
APS is present if more than 1 clinical and 1 lab criteria met.
Vascular thrombosis
> 1 arterial, venous, or small-vessel thrombosis and
Thrombosis confirmed by imaging or Doppler or histopathology and
Without evidence of inflammation in vessel wall on histpathologic confirmation
Pregnancy morbidity
> 1 fetal death > 10th week gestation or
> 1 premature birth > 34th week with preeclampsia or placental insufficiency or
> 3 consecutive spontaneous abortions
< 10th week gestation
Laboratory criteria
Anticardiolipin antibody IgG or IgM present medium or high titers > 2 times at
least 6 weeks apart or
Lupus anticoagulant present > 2 times at least 6 weeks apart
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Antiphospholipid antibody syndrome: clinical
manifestations
Arterial thrombosis
Venous thrombosis
Valvular abnormalities
Pregnancy loss and infertility
Livedo reticularis
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Neurologic complications
cerebrovascular thrombosis
chorea
Catastrophic syndrome
Thrombocytopenia
Antiphospholipid antibody syndrome: tests
Partial thromboplastin time (PTT)
Lupus anticoagulant
Anticardiolipin antibodies by ELISA
Anti-B2-glycoprotein 1 antibody
False-positive biologic test for syphilis
Russell viper venom test
Platelet count
Antinuclear and anti-DNA antibodies
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Proposed diagnostic criteria for polymyositis and
dermatomyositis
PM diagnosed as definite with 4 out of 5 of the below criteria or probable
with 3 out of 5
DM diagnosed as definite with rash plus 3 out of 4 of the below criteria or
probable with rash plus 2 out of 4 criteria
Symmetric proximal muscle weakness
Elevated muscle enzymes (CPK, aldolase, transaminases, LDH)
Myopathic EMG abnormalities
Typical changes on muscle biopsy
Typical rash of dermatomyositis
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Polymyositis: differential diagnosis
Polymyositis and dermatomyositis
Hypothyroidism
Drug-induced myopathies
Corticosteroids, colchicine, HMG-CoA reductase inhibitors, zidovudine,
hydroxychloroquine, alcohol
Infections
Viral, toxoplasmosis, trichinosis, bacterial pyomyositis
Connective tissue disorders
Lupus, scleroderma, MCTD
Systemic vasculitis
PAN, Wegener’s granulomatosis
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Polymyositis: differential diagnosis, cont’d
Metabolic myopathies
Disorders of carbohydrate and lipid metabolism
Electrolyte disturbances
Hypernatremia, hyponatremia, hypokalemia, hypophosphatemia,
hypocalcemia
Inclusion body myositis
Sarcoid myopathy
Amyloid myopathy
Neurologic disorders
Myasthenia gravis, motor neuron disease, muscular dystrophy
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Inclusion body myositis
Males affected more than females
Age of onset usually greater than 50
Slowly progressive
Distal and asymmetric muscle weakness
Myopathic and neuropathic changes on EMG
Mononuclear cell infiltrates and vacuoles containing amyloid on
muscle biopsy
Responds poorly to corticosteroids
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Myositis-specific antibodies
ANTIBODY DISEASE ASSOCIATION PREVALENCE
Anti-tRNA synthetases
(Jo-1)
Dermatomyositis,
interstitial lung disease,
“mechanic’s hands”
20%
Anti-SRP (signal
recognition protein)
African-American women,
poor prognosis
Rare
Anti-Mi-2
Older women, “shawl
sign,” good prognosis
5%
PM/SCL
Polymyositis/scleroderma
overlap
Rare
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ACR systemic sclerosis: preliminary classification criteria
Major criterion or two minor criteria for diagnosis
Major criterion
Proximal scleroderma
Minor criteria
Sclerodactyly
Digital pitting or scars or loss of substance from finger pad
Bibasilar pulmonary fibrosis
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Classification of scleroderma
Localized scleroderma
Morphea
Linear scleroderma
“En coup de sabre”
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Systemic sclerosis
Diffuse cutaneous
Limited cutaneous
CREST syndrome
Systemic sclerosis sine
scleroderma
Overlap syndromes
Scleroderma-like syndromes
Scleroderma-like syndromes
Toxin- or drug-induced scleroderma
Vinyl chloride
Organic solvents and epoxy resins
Eosinophilic myalgia syndrome (L-tryptophan)
Toxic oil syndrome
Bleomycin
Vibration injury
Scleromyxedema
Scleredema
Eosinophilic fasciitis
Graft-versus-host disease
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Mixed connective tissue disease: proposed classification
criteria
The diagnosis of MCTD can be made if 3 (one of which must be myositis
or synovitis) of 5 clinical criteria and anti-RNP are present
Serologic criteria
anti-RNP antibodies
Clinical criteria
swollen hands, synovitis, clinical or biopsy-proven myositis, Raynaud’s
phenomenon, acrosclerosis with or without proximal systemic
sclerosis
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Raynaud’s phenomenon
Episodic, reversible digital skin color change
white to blue to red
well-demarcated
Due to vasospasm
Usually cold-induced
Primary (Raynaud’s disease) and secondary forms
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Causes of secondary Raynaud’s phenomenon
Connective tissue diseases
Scleroderma, systemic lupus erythematosus, MCTD, undifferentiated
CTD, Sjogren’s syndrome, dermatomyositis
Occlusive arterial disease
Atherosclerosis, anti-phospholipid antibody syndrome, Buerger’s
disease
Vascular injury
Frostbite, vibratory trauma
Drugs and toxins
Beta blockers, vinyl chloride, bleomycin, ergot, amphetamines, cocaine
Hyperviscosity/cold-reacting proteins
Paraproteinemia, polycythemia, cryoglobulinemia, cryofibrinogenemia,
cold agglutinins
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Criteria for the classification of Sjögren’s syndrome
Ocular Symptoms
Oral Symptoms
Ocular Signs
Schirmer test 5 mm
Rose Bengal score 4
Histopathology 1 agglomeration of 50 or more mononuclear cells/4mm
tissue (focus score)
Objective evidence of salivary gland involvement
Autoantibodies
SSA/Ro, SSB/La, ANA, RF
(4 or > high sensitivity and specificity)
Exclusions: lymphoma, sarcoid, GVH, acquired immune deficiency
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Sjögren’s syndrome: clinical features
Exocrine gland dysfunction
Sicca syndrome
Salivary gland enlargement
Hypergammaglobulinemic purpura
Central and peripheral neurologic manifestations
Nephropathy
Distal renal tubular acidosis
Interstitial nephritis
Pseudolymphoma and lymphoma
Pulmonary disease
Joint symptoms
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Sicca syndrome manifestations
Keratoconjunctivitis sicca
Ocular dryness
Corneal injury
Xerostomia
Oral dryness
Dysphagia
Dental caries
Thrush
Nasal dryness and epistaxis
Vaginal dryness
Dyspareunia
Candidiasis
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Sjögren’s syndrome: associated conditions
Connective tissue diseases
SLE
RA
Systemic sclerosis
Hypothyroidism
Cryoglobulinemia
Autoimmune hepatitis
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Sjögren’s syndrome: evaluation
Schirmer test
Rose Bengal or fluorescein stain
Slit lamp exam
Salivary flow
Scintigraphy
Dental evaluation
Minor salivary gland (lip) biopsy
Serologic tests (SSA(Ro), SSB(La), ANA, RF)
SPEP, cryoglobulins
Lymph node biopsy
Evaluation for renal tubular acidosis
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Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Approaches to the classification of vasculitis
Clinical syndromes
Vessel size
Laboratory markers
Pathology
Etiology
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Vasculitis: Classification by vessel size
Small vessels (venules, arterioles)
Drug-induced and serum sickness
Henoch-Schönlein purpura
Cryoglobulinemia
Vasculitis associated with
systemic rheumatic diseases
Vasculitis associated with
malignancy
Hypocomplementemic urticarial
vasculitis
Vasculitis associated with
infections
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Small and medium muscular
arteries
Classic PAN
Microscopic polyangiitis
Wegener’s granulomatosis
Churg-Strauss vasculitis
Kawasaki syndrome
Rheumatoid vasculitis
SLE
Large arteries
Giant cell or temporal arteritis
Takayasu arteritis
Vasculitis: Clinical syndromes
Leukocytoclastic or hypersensitivity vasculitis
Classic polyarteritis nodosa
Kawasaki syndrome
Microscopic polyangiitis
Wegener’s granulomatosis
Churg-Strauss vasculitis or allergic granulomatosis
Giant cell or temporal arteritis
Takayasu arteritis
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When to suspect vasculitis: clinical features
Multisystem disease
Unexplained constitutional signs and symptoms
Skin lesions (palpable purpura)
Ischemic vascular changes (gangrene, claudication, Raynaud’s
phenomenon, livedo)
Glomerulonephritis
Mononeuritis multiplex
Myalgia, arthralgia/arthritis
Abdominal (intestinal angina) or testicular pain
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Polyarteritis nodosa (PAN) vs. microscopic polyangiitis
(MPA)
PAN
Small/medium muscular artery
Renal vascular nephropathy
No lung involvement
ANCA negative
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MPA
Small/medium arteries, arterioles,
venules, capillaries
Glomerulonephritis
Lungs involved in up to one third
ANCA positive
Conditions that mimic systemic vasculitis
Atheroembolic disease
Cardiac myxoma
Thrombotic disorders
Anti-phospholipid antibody syndrome
Thrombotic thrombocytopenic purpura
Drug-induced vascular damage
Ergot derivatives
Cocaine
Amphetamines
Infective endocarditis
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ACR 1990 criteria for classification of hypersensitivity
vasculitis
Must have at least 3 of the five criteria present.
Age at disease onset > 16 years
Medication taken at disease onset
Palpable purpura
Maculopapular rash
Bx of arteriole and venule showing granulocytes in perivascular or
extravascular area
Sensitivity 71.0% and specificity 83.9%.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Criteria for diagnosis of Behçet’s disease
Recurrent oral ulceration plus two of the following:
Recurrent genital ulceration
Eye lesions (anterior/posterior uveitis or cells in vitreous or retinal
vasculitis)
Skin lesions (E. Nodosum, pseudofolliculitis, papulopustular lesions or
acneiform nodules)
Positive pathergy test
Sensitivity 91% and specificity 96%
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ACR classification criteria: Henoch-Schönlein purpura
Must have at least 2 of the 4 criteria present.
Palpable purpura
Age < 20 years at disease onset
Bowel angina (abnormal pain after meals or bowel ischemia usually
with bloody diarrhea)
Granulocytes in walls of arterioles or venules on biopsy
Sensitivity 87.1% and specificity 87.7%
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Cryoglobulinemia
Immunoglobulins that undergo reversible precipitation in the cold
Clinical features related to cold precipitating proteins
Acral cyanosis, ulceration and necrosis
Raynaud’s phenomenon
Clinical features related to immune complex deposition
Purpura
Arthralgia
Nephritis
Neuropathy
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Cryoglobulins: type I, II, III
Type I
Monoclonal
Usually IgM
Represent about 10% of all cryos
Associated with hematologic malignancies (Waldenstrom’s macroglobulinemia, myeloma, lymphoma)
Raynaud’s phenomenon
Digital ischemia
Type II
Mixed cryoglobulins consisting of monoclonal immunoglobulin complexed with polyclonal IgG
Hepatitis C associated in 50%
Also may be seen with other infections, hematologic malignancies, and systemic rheumatic diseases
Type III
Polyclonal IgM complexed with polyclonal IgG or IgA
Usually present in low quantity
Often associated with chronic infections, systemic rheumatic diseases, other inflammatory disorders
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ACR 1990 criteria for classification of polyarteritis nodosa
Must have at least 3 of the 10 criteria present.
Weight loss > 4 kg
Livedo reticularis
Testicular pain or tenderness
Myalgias, weakness, or leg tenderness
Mononeuropathy or polyneuropathy
Diastolic BP > 90
Elevated BUN/creatinine
Hepatitis B virus
Arteriographic abnormality
Biopsy of small or medium artery containing PMN
Sensitivity 82.2% and Specificity 86.6%
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ACR 1990 criteria for classification of Churg-Strauss
syndrome
Must have at least 4 of the 6 criteria present.
Asthma
Eosinophilia > 10%
Neuropathy, mono or poly
Pulmonary infiltrates, non-fixed
Paranasal sinus abnormality
Extravascular eosinophils
Sensitivity 85% and specificity 99.7%.
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ACR classification criteria: Wegener’s granulomatosis
Must have at least 2 of the 4 criteria present.
Nasal or oral inflammation (oral ulcers or bloody nasal drainage)
Abnormal chest radiograph (nodules, fixed infiltrates, cavities)
Urinary sediment (>5 RBC/hpf or RBC casts
Granulomatous inflammation on biopsy (in wall of artery or arteriole,
perivascular, or extravascular)
Sensitivity 88.2% and specificity 92.0%
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Polymyalgia rheumatica: differential diagnostic possibilities
Fibromyalgia
Depression
Occult infection
Occult malignancy
Hypothyroidism
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Temporal arteritis
Viral myalgia
Rheumatoid arthritis
Polymyositis
Multiple myeloma
Osteoarthritis
Rotator cuff disease
Polymyalgia rheumatica: epidemiology
Onset after age 50 and usually after 60
Highest incidence in individuals of Northern European extract
Female:male ratio 2:1
Incidence approaches 1% in older populations
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Polymyalgia rheumatica: clinical and laboratory features
Pelvic and shoulder girdle aching
Morning stiffness
Rapid response to low doses of corticosteriods
Anemia
Elevated ESR and C-reactive protein
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Giant cell arteritis
Clinical features
Headache
Temporal artery
abnormality
Jaw claudication
Visual loss; diplopia
Extremity claudication
PMR symptoms
Weight loss, fever
Respiratory symptoms
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Laboratory features
Elevated ESR
Elevated CRP
Other acute-phase reactants
Anemia
Elevated alkaline phosphatase
Relationship of polymyalgia (PMR) to giant cell arteritis
(GCA)
40-60% of patients with GCA have PMR symptoms; in about half of these
individuals, PMR is their first manifestation of GCA
10-15% of patients with PMR have GCA
PMR symptoms can occur before, with, or after GCA symptoms in
patients with GCA
GCA can develop long after onset and treatment of PMR
Treatment of GCA requires larger doses of corticosteroids than does
treatment of PMR
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ACR 1990 criteria for the classification of giant cell
(temporal) arteritis
Must have at least 3 of the 5 criteria present.
Age > 50 years at disease onset
New headache
Temporal artery abnormality (tender or decreased pulse)
Elevated Westergren ESR > 50 mm/hr
Abnormal artery biopsy: mononuclear cell infiltrate, granulomatous
inflammation, usually multinucleated giant cells
Sensitivity 93.5% and specificity 91.2%
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ACR classification criteria: Takayasu arteritis
Must have at least 3 of the 6 criteria present.
Age < 40 years at disease onset
Claudication of extremities
Decreased brachial artery pulse
BP difference > 10 mm Hg between arms
Bruit over subclavian arteries or aorta
Arteriogram abnormality: occlusion or narrowing in aorta or main
branches
Sensitivity 90.5% and specificity 97.8%.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
Copyright © 1972-2004 American College of Rheumatology Slide Collection. All rights reserved.
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