Reuma Teoria ACR

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


Not infection-related

Spondylarthropathy

Gout, pseudogout


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


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


Osteoarthritis

Systemic lupus and other connective tissue diseases

Gout

Pseudogout

Sarcoidosis


Intermittent arthritis: selected causes

Gout

Pseudogout

Lyme disease

Palindromic rheumatism

Rheumatoid arthritis

Sarcoidosis

Mechanical joint problems


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


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)


Differential diagnosis of noninflammatory effusions (group

I)

Osteoarthritis

Early rheumatoid arthritis

Trauma

Osteochondritis dissecans

Osteopecrosis

Sickle-cell disease


Chronic/subsiding crystal synovitis

Lupus

Vasculitis

Scleroderma

Amyloidosis

Hypothyroidism

Differential diagnosis of inflammatory effusions (group II)



Crystalline arthritis


Viral arthritis

Lyme arthritis

Neisserial infections

Early or partially treated septic arthritis

Rheumatic fever


Differential diagnosis of purulent effusions (group III)

Pyogenic infections

Mycobacterial infections

Fungal infections

Crystalline arthritis

“Pseudo-septic” arthritis (rheumatoid arthritis)


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


Osteoarthritis: general features

Clinical

A.M. stiffness

Gel phenomenon

Joint pain and tenderness

Crepitus

Bony swelling

Angulation deformities

Functional Impairment

Laboratory

Noninflammatory

synovial fluid


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)


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


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


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.


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


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


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%


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)


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


Class IV: limited in ability to perform usual self-care, vocational, and



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


Stages of rheumatoid arthritis pathogenesis, cont’d


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


Felty’s syndrome

Seropositive rheumatoid arthritis

Splenomegaly

Leukopenia (neutropenia)

Recurrent infections

Leg ulcers


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%


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


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


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


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


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


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


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


Systemic-onset juvenile rheumatoid arthritis: clinical

features

Spiking fevers

Rash

Lymphadenopathy

Hepatosplenomegaly

Pericarditis

Pleuritis

Arthritis


Childhood malignancy and bone pain

Leukemia

Lymphoma

Neuroblastoma

Histiocytosis

Osteogenic sarcoma

Ewing’s sarcoma


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


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



Ankylosing spondylitis

Psoriatic arthritis

Reactive arthritis

Enteropathic arthritis

Crohn’s disease

Ulcerative colitis

Juvenile ankylosing spondylitis

Undifferentiated spondylarthropathies


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%


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


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


Spondylarthropathies: nonvertebral manifestations

Asymmetric peripheral arthritis

Sausage digits

Enthesopathy

Achilles yenosynovitis

Plantar fasciitis

Costochondritis

Acute anterior uveitis/iridocyclitis

Mucocutaneous lesions


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%


Psoriatic arthritis: musculoskeletal characteristics

Asymmetrical arthritis

Dactylitis/Sausage digit

Tenosynovitis

Enthesitis

Heel pain

Sacroiliitis

Spondylitis


Psoriatic arthritis: cutaneous and other manifestations

Psoriasis

Erythroderma

Nail pitting

Onycholysis

Conjunctivitis/iritis

Valvular heart disease


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


Reactive arthritis

Seronegative asymmetric arthritis following:

Urethritis or cervicitis

Infectious diarrhea

Often associated with:

Inflammatory eye disease

Enthesopathy

Circinate balanitis, oral ulceration or keratoderma


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


Systemic lupus erythematosus: 1982 classification criteria

Malar rash

Discoid rash

Photosensitivity

Oral ulcers

Arthritis

Serositis


Renal disorder

Neurologic disorder

Hematologic disorder

Immunologic disorder

Antinuclear antibody


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



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


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


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


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


Drug-induced lupus: definite drug associations

Hydralazine

Procainamide

Minocycline

Chlorpromazine

Isoniazid

Penicillamine

Methyldopa

Interferon-alpha


Drug-induced lupus: possible drug associations

Anticonvulsants

Quinidine

Propylthiouracil

Sulfonamides

Lithium

Beta-blockers

Nitrofurantoin

Sulfasalazine


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


Antiphospholipid antibody syndrome: clinical

manifestations

Arterial thrombosis

Venous thrombosis

Valvular abnormalities

Pregnancy loss and infertility

Livedo reticularis


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


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


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


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


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


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


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


Classification of scleroderma

Localized scleroderma

Morphea

Linear scleroderma

“En coup de sabre”


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


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


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


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


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


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


Sicca syndrome manifestations

Keratoconjunctivitis sicca

Ocular dryness

Corneal injury

Xerostomia

Oral dryness

Dysphagia

Dental caries

Thrush

Nasal dryness and epistaxis

Vaginal dryness

Dyspareunia

Candidiasis


Sjögren’s syndrome: associated conditions

Connective tissue diseases

SLE

RA

Systemic sclerosis

Hypothyroidism

Cryoglobulinemia

Autoimmune hepatitis


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


Approaches to the classification of vasculitis

Clinical syndromes

Vessel size

Laboratory markers

Pathology

Etiology


Vasculitis: Classification by vessel size

Small vessels (venules, arterioles)

Drug-induced and serum sickness

Henoch-Schönlein purpura

Cryoglobulinemia

Vasculitis associated with

systemic rheumatic diseases


malignancy

Hypocomplementemic urticarial

vasculitis


infections


Small and medium muscular

arteries

Classic PAN

Microscopic polyangiitis


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


Churg-Strauss vasculitis or allergic granulomatosis

Giant cell or temporal arteritis

Takayasu arteritis


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


Polyarteritis nodosa (PAN) vs. microscopic polyangiitis

(MPA)

PAN

Small/medium muscular artery

Renal vascular nephropathy

No lung involvement

ANCA negative


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


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%.


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%


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



Cryoglobulinemia

Immunoglobulins that undergo reversible precipitation in the cold

Clinical features related to cold precipitating proteins

Acral cyanosis, ulceration and necrosis


Clinical features related to immune complex deposition

Purpura

Arthralgia

Nephritis

Neuropathy


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)


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


ACR 1990 criteria for classification of polyarteritis nodosa


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%


ACR 1990 criteria for classification of Churg-Strauss

syndrome


Asthma

Eosinophilia > 10%

Neuropathy, mono or poly

Pulmonary infiltrates, non-fixed

Paranasal sinus abnormality

Extravascular eosinophils

Sensitivity 85% and specificity 99.7%.


ACR classification criteria: Wegener’s granulomatosis


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)



Polymyalgia rheumatica: differential diagnostic possibilities

Fibromyalgia

Depression

Occult infection

Occult malignancy

Hypothyroidism


Temporal arteritis

Viral myalgia


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


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


Giant cell arteritis

Clinical features

Headache

Temporal artery

abnormality

Jaw claudication

Visual loss; diplopia

Extremity claudication

PMR symptoms

Weight loss, fever

Respiratory symptoms


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


ACR 1990 criteria for the classification of giant cell

(temporal) arteritis


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



ACR classification criteria: Takayasu arteritis


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%.


Reuma Teoria ACR

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