rheumatoid arthritis1

8/12/2019 Rheumatoid Arthritis1

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Overview of Rheumatoid

Arthritis

Naureen Mirza, MDMay 10, 2010


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Epidemiology

An annual incidence of approximately 0.2 per1000 in males and 0.4 per 1000 in females

A prevalence of 0.5-1% is reported in diversepopulations worldwide

Twin studies show the disease to have aheritability of 60%

Hormonal and reproductive factors contribute tothe female excess and parity

Obesity, smoking, coffee consumption, and priorblood transfusion have also been identified aspotential risk factors


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1. Morning stiffnessMorning stiffness in and around the joints, lasting at least 1

h before maximal improvement

2. Arthritis in three or

more joint areas

Soft tissue swelling or fluid (not bony overgrowth) observed

by a physician, present simultaneously for at least 6weeks

3. Arthritis of hand

joints

Swelling of wrist, MCP or PIP joints for at least 6 weeks

4. Symmetric arthritis Simultaneous involvement of the same joint areas (defined

in 2) on both sides of the body (bilateral involvement of

PIP, MCP or MTP joints is acceptable without absolutesymmetry) for at least 6 weeks

5. Rheumatoid nodules Subcutaneous nodules over bony prominences, extensor

surfaces or in juxta-articular regions, observed by a

physician

6. Rheumatoid factor Detected by a method positive in fewer than 5% of normalcontrols

7. Radiographic

changes

Typical of RA on posteroanterior hand and wrist

radiographs; it must include erosions or unequivocal

bony decalcification localized in or most marked

adjacent to the involved joints (OA changes alone do

not qualify)


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New criteria for the diagnosis of RA

Joint involvement

1 med-lg joint

0

2-10 Med-Lg joints 1

1-3 small joints 2

4-10 small joints 3

>10 small joints 5

Serology

Neither RF or Anti ccp

0

At least one + with low titer 2

Atleast one with high titer 3

Duration of synovitis

6 weeks 1

Acute phase reactants

Neither CRP or ESR abnormal

0

Abnormal CRP or abnormal ESR 1

Score >=6 indicates

definite RA


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History In RAChronological account of illness from the onset

Onset: acute or gradual, with detailsLocation of pain (local or referred): precise anatomy,

presence/absence of swelling

Pattern of joint involvement: axial, peripheral, symmetric

Type of pain: quality and character

Severity: pain threshold effects, interference with activities of daily

living, range of joint movement

Radiation of pain: local or deep referred type

Clinical course: duration, frequency, periodicity, persistence

Modifying factors: aggravating, relieving, medication effectsAssociated symptoms: fatigue, other systemic symptoms

Duration of morning stiffness: non-restorative sleep pattern

Present status: regional review of joints, extra-articular features,

functional class, activities of daily living, psychologic state


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Extra-articular features

Record presence of nodules, Raynaud's phenomenon,

digital infarcts, episcleritis, peripheral neuropathy, palmar

erythema, leg ulcers

Note tendon sheath involvement, or tendon nodules,

subluxation or rupture

Check for anemia, splenomegaly, leukopenia, pleuritis or

pericarditis, the sicca syndrome or renal involvement

Articular: measures of inflammatory activity

Check for tenderness, synovial effusion, grip strength

Articular: measures of destruction and deformity

Check for lax collaterals, subluxation, malalignment,

metatarsal prolapse, hammer toes and bone-on-bone

crepitus

Examination


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Articular manifestations

Arthritis : Joint swelling, tenderness,

warmth, limitation of motion, pain in range

of motion,

Morning stiffness

Muscle atrophy

Chronic deformitiesAnkylosis of the joint


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Rheumatoid nodules20% of RA patients with +tests for blood

rheumatoid factors and rarely in RF

most commonly on pressure areas

central fibrinoid necrosis with surrounding

fibroblastsMay occur in any organs as well

May regress with treatment , but has been

reported to increase with Methotrexate use

believed to occur as a result of small vessel

vasculitis with fibrinoid necrosis, which

forms the center of the nodule, and

surrounding fibroblastic proliferation


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Histology of rheumatoid nodule

Rheumatoid nodule with granulomatoustransformation.There is prominent central fibrinoidnecrosis, with surrounding palisading histiocytes and anouter layer of chronic fibrosing connective tissue withinflammatory cells including lymphocytes and fibroblasts.


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Hematologic abnormalities

Anemia

Thrombocytosis

Thrombocytopenia ( rare )

Eosinophilia(esp related to gold)

Lymphadenopathy ( concomitant Sjogrens syndrome may

increase the risk of Lymphoma)Splenomegaly ( clinically in 5-10 % with active RA and upto

58% by radionuclide scanning)


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Feltys syndrome

RA in combination with splenomegaly and leukopenialong-standing, seropositive, nodular, deforming RA

33%do not have active synovitisLower extremity ulcers, hyperpigmentation, bacterial

infections, ANAs, Also may have thrombocytopenia,hypocomplementemia,

an increased risk for the development of lymphomas


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Neutropenia

Anemia

Thrombocytopenia

Splenomegalylarge granularlymphocytes in thesepatients represent in

vivoactivatedcytotoxic T cells andclonality is present.

Large granular lymphocytes


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Pulmonary manifestations

Pulmonary nodules

Pleural effusions (50%)

Caplans syndrome

Diffuse interstitial

pulmonary fibrosis (28%)

Bronchiolitis obliteransorganizing pneumonia

(BOOP)

Obliterative bronchiolitis

Drug induced

Isolated pul. Arteritis (rare)

Pulmonary hypertension

(rare)

Airway obstruction (38%)


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Caplans

syndrome


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Cardiac disease in RA

Pericarditis (50%)

Premature cardiovascular disease(TNF- is produced by cardiacmyocytes and resident macrophages during cardiac stress and may help trigger andperpetuate atherosclerosis)

Myocardial and endocardial disease (clinically insignificant)

Coronary arteritis

Valvular abnormalities

Rheumatoid nodule in the aortic valve cusp


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Ocular InvolvementMost common : Keratoconjuctivitis sicca (10-35%)

Episcleritis: nodular or diffuse, appears acutely andcauses eye redness and pain

Scleritis is less common than episcleritis, but is more obviously correlatedwith vasculitis, long-standing arthritis and active joint inflammation.

Untreated scleritis may progress to scleromalacia


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Other uncommon manifestation of RA in the

eyeUveitis

Episcleral nodulosis

Corneal filamentary keratitis

Peripheral ulcerative keratitis

Retinal detachments

Macular edema

Glucocorticoids, Gold and Chloroquine

Brown's syndrome, which is defined as diplopia upon

upward and inward gaze and is believed to be the result of

inflammation and thickening of the superior tendons, and

optic neuritis


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Neurological manifestations

Nerve compressions

(Carpal tunnel, tarsal tunnel, atlantoaxial subluxation, subaxial

c-spine involvement, extradural nodules)

Stroke, seizure, hemorrhage, encephalopathy and

meningitis as a result of cerebral vasculitis, amyloidosis orrheumatoid nodules, or both, in the dura and choroid plexus

of the brain

Most patients have long-standing RA with other extra-

articular disease


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Renal Involvement

The kidneys are usually spared in RA, although a low-grade

membranous nephropathy, glomerulitis, vasculitis and secondary

reactive amyloidosis have all been described

Renal abnormalities frequently result from the agents used intreating RA, notably gold, D-penicillamine, cyclosporin and NSAIDs,

and usually manifest as membranous nephropathy and acute

interstitial nephritis, often the result of a drug hypersensitivity

reaction


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Amyloidosis

0.7-5.8% of patients with RA

Virtually every organ system may be involved in the secondary

amyloidosis that complicates RA, including the heart, kidney, liver,

spleen, intestines and skin.

Renal manifestations of amyloidosis are the most common onesPoor prognosis : 4-year survival rates of about 58% are reported


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Rheumatoid vasculitisSystemic vasculitis uncommon, usually with longstanding,

poorly controlled disease

pANCA

More in patients with feltys

Skin: Nail fold infarcts . Leg ulcers, gangrene


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Baker's popliteal cyst


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Diagnosis

CBC

RF

Anti ccpESR

X rays of affected joints

Musculoskeletal ultrasoundMRI


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Additional testing

PPD

Chest X ray

Hep B and C serologiesANA

Joint aspiration

Synovial biopsyLyme serology


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More aggressive disease

TNF inhibitors

Abatacept (CTLA 4 IgG)

Rituximab (Anti CD 20)

rheumatoid arthritis1

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