RC
`927_
`M346
`2000
`LIFE
`SCIENCE
`
`

`

`Manual of Rheumatology
`and Outpatient Orthopedic Disorders
`Diagnosis and Therapy
`Fourth Edition
`
`Associate Editor
`Paul Pellicci, M.D.
`Associate Professor of Surgery
`(Orthopedic)
`Weill Medical College of Cornell
`University;
`Associate Attending Surgeon
`Hospital for Special Surgery
`New York, New York
`
`Forewords. by
`John L. Decker, M.D.
`National Institutes of Health
`Bethesda, Maryland ·
`
`Charles L. Christian, M.D.
`Physician-in-Chief Emeritus
`Hospital for Special Surgery
`New York, New York
`
`Editors
`
`Stephen A. Paget, M.D.
`Joseph P. Routh Professor of
`Medicine
`Weill Medical College of Cornell
`University;
`Physician-in- Chief
`Attending Rheumatologist and Chair
`Division of Rheumatology
`Hospital for Special Surgery;
`New York Presbyterian Hospital
`New York, New York
`
`Allan Gibofsky, M.D., J.D.
`Professor of Medicine and Public
`Health
`Weill Medical College of Cornell
`University;
`Professor of Law, Fordham
`University;
`Attending Rheumatologist
`Division of Rheumatology
`Hospital for Special Surgery;
`New York Presbyterian Hospital
`New York, New York
`
`John F. Beary, III, M.D.
`Clinicill Professor of Medicine
`University of Cincinnati;
`Attending Physician
`Rheumatology Division
`Veterans Administration Medical
`Center
`Cincinnati, Ohio
`
`~~ LIPPINCOTT WILLIAMS & WILKINS
`
`•
`
`A Wolters Kluwer Company
`Philadelphia • Baltimore • New York • London
`Buenos Aires • Hong Kong • Sydney • Tokyo
`
`

`

`Acquisitions Editor: Richard Winters
`Developmental Editor: Michelle LaPlante
`Production Editor: Emily Lerman
`Manufacturing Manager: Colin J. Warnock
`Cover Illustrator: Patricia Gast
`Compositor:· Circle Graphics
`Printer: R.R. Donne/ley, Crawfordsville
`
`© 2000 by LIPPINCOTT WILLIAMS & WILKINS
`530 Walnut Street
`Philadelphia, PA 19106 USA
`LWW.com
`
`All rights reserved. This book is protected by copyright. No part of this book may be repro(cid:173)
`duced in any form or by any means, including photocopy, recording, or utilized by any informa(cid:173)
`tion storage and retrieval system without written permission from the copyright owner, except for
`brief quotations embodied in critical articles and reviews. Materials appearing in this book'pre(cid:173)
`pared by individuals as part of their official duties as U.S. govemment employees are not covered ·
`by the above-mentioned copyright.
`
`Printed in the USA
`
`Library of Congress Cataloging-in-Publication Data
`
`Manual of rheumatology and outpatient orthopedic disorders : diagnosis and therapy I
`editors, Stephen A. Paget, Allan Gibofsky, John F. Beary, III; associate editor, Paul
`Pellicci; forewords by John L. Decker, Charles L. Christian.-4th ed.
`p.; em.
`Includes bibliographical references and index.
`ISBN 0-7817-1576-8 (alk. paper)
`·
`1. Rheumatology-Handbooks, manuals, etc. 2. Orthopedics-Handbooks, manuals, etc.
`I. Paget, Stephen A.
`II. Gibofsky, Allan.
`III. Beary, John F.
`[DNLM:
`1. Rheumatic Diseases-diagnosis-Handbooks. 2. Rheumatic
`Diseases-therapy-Handbooks. 3. Ambulatory Care-Handbooks. 4. Bone
`Diseases-Handbooks. WE 39 M294 2000]
`RC927 .M346 2000
`616.7'23-dc21
`
`99-045307
`
`Care has been taken to confirm the accuracy of the information presented and to describe gen(cid:173)
`erally accepted practices. However, the authors, editors; and publisher are not responsible for
`errors or omissions or for any consequences from application of the information in this book and
`make no warranty, expressed or implied, with respect to the currency, completeness, or accuracy
`of the contents of the publication. Application of this information in a particular situation remains
`the professional responsibility of the practitioner.
`The authors, editors, and publisher have exerted every effort to ensure that drug selection and
`dosage set forth in this text are in accordance with current recommendations and practice at the
`time of publication. However, in view of ongoing research, changes in govemment regulations,
`and the constant flow of information relating to drug therapy and drug reactions, the reader is
`urged to check the package insert for each drug for any change in indications and dosage and for
`added wamings and precautions. This is particularly important when the recommended agent is a
`new or infrequently employed drug.
`Some drugs and medical devices presented in this publication have Food and Drug Adminis(cid:173)
`tration (FDA) clearance for limited use in restricted research settings. It is the responsibility of
`the health care provider to ascertain the FDA status of each drug or device planned for use in
`their clinical practice.
`
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`28. RHEUMATOID ARTHRITIS
`
`Dror Mevorach and Stephen A. Paget
`
`Rheumatoid arthritis (RA) is a chronic, systemic, autoimmune, inflammatory disorder
`in which an erosive, symmetric joint disorder maintains the center stage accompanied
`by a variable, but at times prominent, degree of extraarticular involvement. The term
`was introduced by Garrod in 1859 and relates today to a disorder occurring in about
`1% of the world's population, with a twofold to threefold female predominance before
`60 years of age and greater equity between the sexes thereafter. Rheumatoid factor
`(RF), an immunoglobulin M (IgM) auto-antibody against the Fe portion of an IgG mol(cid:173)
`ecule first described by Waaler in 1940, is the main serologic marker, found in 75% to
`80% of patients.
`During the past 10 years, epidemiologic studies have unearthed ~disturbing infor(cid:173)
`mation about the true potential of this disease, which now guides our modern thera(cid:173)
`peutic approach; RA is a chronic disease that leads to joint damage within the first 2
`years, causes marked functional limitation and a 30% loss of work within the first
`5 years, and shortens life by 5 to 7 years. This aggressive disorder demands the early
`institution of an equally aggressive therapeutic approach. A treatment plan should be
`individually crafted that is based on patient-specific clinical and functional parame(cid:173)
`ters and employs a wide range of effective medications and physical therapeutic
`modalities aimed at altering the disease course and maintaining function.
`As our concepts about the pathogenesis and clinical realities of RA have crystallized
`and been coupled with refined methods derived from biotechnology, promising biologics
`have been developed. Monoclonal antibodies, recombinant cytokines, cytokine receptor
`fusion proteins, and other biologics have moved from the status of novel reagents stud(cid:173)
`ied in phase I trials to validated therapeutic tools in widespread use. With the develop(cid:173)
`ment of these extraordinary agents, a new era in the focused treatment ofRA has begun.
`
`I. Epidemiology. Whereas the worldwide prevalence rate ofRA is about 1%, higher
`rates are found in certain groups, such as 5.3% in certain Native-American tribes.
`RA is two to three times more common in women than in men, but in persons over
`50 years of age, the disease frequency becomes more equal. Although the onset of
`disease is most common between the ages of 40 and 60, in one-third of patients,
`RA develops after the age of 60.
`II. Genetic factors in RA are important in defining disease susceptibility and
`severity.
`A. Family studies have demonstrated an increased risk for disease in siblings
`of persons affected with RA. Concordance has been found to be 12% to 15% in
`monozygotic and 4% in dizygotic twins, strong evidence for a major influence
`of genetic factors in disease causation.
`B. The :major histocompatibility complex (MHC) is a region of genes whose
`MHC I and II products provide a system for displaying antigenic peptides to
`T cells. RA was shown to be associated with the HLA-DR4 and -DR1 haplo(cid:173)
`types; on further molecular characterization, the association was confined to
`a short sequence in the HLA-DRB1 gene that codes for the RA epitope in
`amino acid positions 67 through 74. Some of the HLA-DRB1 alleles (HLA(cid:173)
`DRB1 *0401, *0404, and *0408 in general populations and some others in spe(cid:173)
`cific ethnic populations) are RA-associated alleles. These MHC genes are
`related not only to the initiation of the disease but also to its course and sever(cid:173)
`ity. For instance, patients with non-DR4 disease-associated genes have milder,
`seronegative disease, and patients with two (homozygous) DRB1 *04 alleles
`have more severe and extraarticular disease.
`C. Other genetic factors are not as well defined, but because the HLA asso(cid:173)
`ciation represents less than 30% of the genetic risk, several candidate genes
`coding for cytokines, chemokines, and signal transduction factors may influ(cid:173)
`ence disease initiation, severity, and progression.
`
`192
`
`-
`
`

`

`28. Rheumatoid Arthritis 193
`
`III. Pathogenesis
`A. No clear etiology has been defined. Although there are no convincing stud(cid:173)
`ies demonstrating a specific infectious etiology in RA, some studies support
`the possibility that an infectious agent may be responsible for the disease in
`a genetically predisposed host. Immune responses initially generated against
`such immunogens would be sustained by cross-reactivity to host antigens
`within the synovial joints, leading to a breakdown of normal immunologic
`tolerance and a chronic destructive autoimmunity. Candidate infectious
`agents include viruses (e.g., parvovirus B19, Epstein-Barr virus), Mycoplasma,
`and other bacteria (e.g., streptococci). Possible auto-antigens include type
`2 collagen, proteoglycan, chondrocyte antigens, heat shock proteins, and
`immunoglobulins.
`B. Histopathology. In the early months of RA, edema, angiogenesis, hyper(cid:173)
`plasia of synovial lining, and inflammatory infiltrate are already present .
`. Once the disease enters a more chronic phase, massive hyperplasia, mainly
`of type A synovial cells, and subintimal mononuclear cell infiltration are
`prominent. The synovium ofRA assumes the appearance of a reactive lymph
`node because of the extensive infiltration by plasma cells, macrophages, and
`lymphocytes in the form oflarge lymphoid follicles. The histologic appearance
`ofthe synovium in RA, however, is not specific, as a similar picture is seen
`in other inflammatory arthritides, such as psoriatic arthritis and Reiter's
`syndrome. One characteristic feature of RA is the invasion of and damage to
`cartilage, bone, and tendons by an infiltrating inflammatory synovial tissue
`mass called the pannus.
`C. Cellular i:nun.unity. CD4+ T lymphocytes in the form of aggregates or diffuse
`infiltrates are found in the subintimal area. B cells, although in low numbers,
`evolve into plasma cells and produce RF. Activated macrophages and dendritic
`cells are intermixed with other immunoreactive cells and are thought to be
`important in antigen presentation. Neutrophils, mainly localized to the syn(cid:173)
`ovial fluid and not the synovial membrane, are prominent effectors of inflam(cid:173)
`mation, and cartilage is damaged by the various enzymes they release.
`D. Cytokines, chern.okines, growth factors, enzyrn.es, and other soluble
`rn.ediators. Cytokines and growth factors bind to cell surface receptors and
`transmit a signal to the cell, with a resultant shift in activation. In this way,
`they play an integral role in the initiation and perpetuation of synovitis.
`Interleukin-1 (IL-l), IL-6, tumor necrosis factor-alpha (TNF-a), and colony
`stimulating factor-1 (CSF-1) are produced by macrophages and fibroblasts;
`they have broad effects on many cells that lead to cell proliferation, in(cid:173)
`creased release of prostaglandins and matrix-degrading proteases, fever,
`and bone resorption. IL-2, IL-3, IL-4, IL-6, and interferons are produced in
`T cells and lead to activation and amplification of cellular and humoral
`immune responses. Enzymes such as metalloproteinases (collagenase, stro(cid:173)
`melysin), which degrade matrix proteins, and complement proteins, which
`participate in acute inflammation, are effector molecules and as such have
`the ability to alter the environment directly. The relative contribution of
`each arm probably depends on the degree of chronicity, extent of therapeu(cid:173)
`tic interventions, and other poorly defined factors. Because of the highly
`complex interrelationships between these cells, it is unlikely that therapies
`aimed at only one aspect of the axis will succeed in all patients.
`E. Auto-antibodies. RFs are anti-globulin antibodies that bind to the F~ por(cid:173)
`tions of IgG. The mechanisms initiating RF secretion and its exact role in
`disease pathogenesis have not been established. RF is found in the serum
`of 75% to 80% of RA patients, is locally produced in rheumatoid synovial
`tissue, and may be present in the serum of patients with other disease char(cid:173)
`acterized by B-cell or immune hyperreactivity, such as systemic lupus ery(cid:173)
`thematosus (SLE) and bacterial endocarditis. The presence of high RF titers
`is associated with severe, erosive disease, a worse functional outcome, rheu(cid:173)
`matoid nodules, other extraarticular disease manifestations, and HLA-DR4
`positivity.
`
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`

`194
`
`III. Diagnosis and Therapy
`
`IV. Clinical presentation
`A. Disease criteria. The 1987 revised American Rheumatology Association
`criteria for the classification of RA were developed for epidemiologic pur(cid:173)
`poses. However, because of the high sensitivity and specificity of these crite(cid:173)
`ria in the classification of RA, they are useful to consider at the time of diag(cid:173)
`nosis. Of the seven criteria, the presence of four is sufficient for classifying a
`patient as having RA. The first four criteria must be present for at least
`6 weeks. They include the following:
`1. Morning stiffness or stiffness after rest lasting longer than 1 hour.
`2. Polyarthritis of at least three joints in 14 areas, including right and left
`proximal interphalangeal (PIP) joints, metacarpophalangeal (MCP) joints,
`wrists, elbows, knees, ankles, and metatarsophalangeal (MTP) joints.
`3. Arthritis of hands, wrists, MCP joints, or PIP joints; symmetric arthritis.
`4. Simultaneous arthritis in both sides ofthe body.
`5. Subcutaneous rheumatoid nodules.
`6. SerumRF.
`7. Hand radiographic changes typical ofRA, including erosions or periartic(cid:173)
`ular osteoporosis.
`B. Joint, tendon, and bursal involvement. Symmetric polyarthritis with
`variable degrees of damage and inflammation of the hands and feet, mainly
`the wrists, MCP joints, PIP joints, MTP joints, elbows, knees, ankles, and
`shoulders is characteristic of RA. Early changes include ulnar styloid promi(cid:173)
`nence, and later deformities resulting from combinations of joint and tendon
`damage may evolve, including ulnar deviation, boutonniere and swan neck
`deformities. Flexor tenosynovitis can lead to triggering of the fingers and may
`eventuate in rupture of tendons. Extensor tenosynovitis is seen as swelling
`over the dorsum of the wrist, and flexor tenosynovitis can lead to carpal tun(cid:173)
`nel syndrome from median nerve entrapment. Olecranon bursitis often pre(cid:173)
`sents as swelling at the tip of the elbow; synovial extensions, known as Baker's
`cysts, appearing from the knee to the medial calf region may mimic phlebitis.
`Spinal disease is limited to the cervical region and, in patients with severe dis(cid:173)
`ease, may lead to atlanto-axial subluxation and even cord compromise.
`C. Disease presentation and course. The gradual onset of symmetric poly(cid:173)
`arthritis is most common, occurring in at least 50% of patients; a sudden onset
`is seen in 10% to 25% of patients. Other patterns of presentation include
`monarticular disease; palindromic (short-lived and episodic) disease; extraar(cid:173)
`ticular features, such as nodules; and a proximal type resembling polymyalgia
`rheumatica. Whatever the onset is, the subsequent course may be brief or
`episodic, prolonged and progressive, or something intermediate. A monocyclic
`course is a single cycle with remission for at least 1 year, seen in 10% of
`patients. A polycyclic course is seen in 70% of patients, with either intermit(cid:173)
`tent or continuing subtypes. The latter group shows smoldering activity with
`incomplete remission or progression. A progressive pattern with increasing
`joint damage and extraarticular manifestations is seen in about 10% of
`patients. Included in this group are patients with malignant RA, a polyarteri(cid:173)
`tis nodosa-like disorder.
`D. Extraarticular presentation. Although the joint disease dominates the
`clinical picture, constitutional symptoms such as fatigue and extraarticular
`features such as serositis, sclerosis, subcutaneous nodules, and rheumatoid
`vasculitis may be prominent, dominant, or life-threatening. Subcutaneous
`nodules appear in 20% to 30% of seropositive patients. Nodules develop mostly
`in pressure areas such as the elbows, finger joints, Achilles tendon, and occip(cid:173)
`ital scalp and are associated with active and more severe disease. Interest(cid:173)
`ingly, methotrexate treatment may cause an increase in nodulosis, especially
`in the fingers. N ailfold infarcts may be seen when rheumatoid vasculitis devel(cid:173)
`ops. Pulmonary involvement is common, with pleurisy, pleural effusion,
`parenchymal nodules, interstitial alveolitis, fibrosis, and bronchiolitis obliter(cid:173)
`ans organizing pneumonia. Cardiac manifestations include pericarditis,
`myocarditis, valvulitis, nodule formation with arrhythmia, amyloidosis, and
`
`

`

`28. Rheumatoid Arthritis 195
`
`vasculitis. Ocular keratoconjunctivitis sicca is the most common eye abnor(cid:173)
`mality, but sclerosis and scleromalacia may be associated with extensive dis(cid:173)
`ease activity. The median, ulnar, and posterior tibial nerves may become
`entrapped by neighboring sites of joint inflammation or damage. Peripheral
`neuropathies and central nervous system disease can be manifestations of
`rheumatoid vasculitis. Felty's syndrome (granulocytopenia, splenomegaly,
`recurrent infection) and Sjogren's syndrome may coexist with RA and often
`occur in patients with active, systemic disease.
`V. Laboratory studies and imaging
`A. Laboratory studies. IgM RF is detected in the serum of about 75% to 80% of
`RA patients. High titers are associated with more severe and extraarticular
`disease. The fact that 20% of RA patients are seronegative highlights the fact
`that the diagnosis ofRA is based on clinical, not laboratory, data. An elevated
`erythrocyte sedimentation rate (ESR) and levels ofC-reactive protein are com(cid:173)
`monly found in RA and correlate well with disease activity in most patients.
`Normochromic normocytic anemia of chronic disease is frequently seen in
`active RA; it may be complicated, however, by other conditions, such as drug(cid:173)
`induced suppression of bone marrow and blood loss. Thrombocytosis is a fre(cid:173)
`quent correlate of active RA; thrombocytopenia and leukopenia may be seen
`in drug-induced bone marrow suppression or in Felty's syndrome. Elevated
`alkaline phosphatase is common in severe disease, but elevation of other liver
`enzymes more likely is related to treatment with nonsteroidal antiinflamma(cid:173)
`tory drugs (NSAIDs), steroids, or methotrexate.
`B. Imaging techniques are employed in defining the diagnosis, severity,
`progression, extent of disease, response to therapy, and presurgical state
`ofRA patients. The most common plain radiographic findings are soft-tissue
`swelling, periarticular osteoporosis, marginal erosions, joint space narrow(cid:173)
`ing, and joint deformities.
`VI. Diagnosis. A careful clinical examination (history plus physical examination)
`is a powerful diagnostic tool; it should focus on the pattern and severity of joint
`inflammation and damage, the presence and extent of constitutional and extra(cid:173)
`articular manifestations, modifying medical and psychosocial factors, coexistent
`medical illnesses, and family history.
`A. Differential diagnosis
`1. Systemic lupus erythematosus and other connective tissue dis(cid:173)
`orders. The symmetric joint inflammation of RA and SLE may be indis(cid:173)
`tinguishable. However, in SLE, erosions do not develop, and the joint dis(cid:173)
`ease is commonly accompanied by such manifestations of SLE as fever,
`serositis, nephritis, dermatitis, cytopenias, and antinuclear antibody (ANA)
`and anti-DNA seropositivity. Other connective tissue disorders, such as
`scleroderma and the vasculitides, may present with an RA-like polyarthri(cid:173)
`tis, or this may develop later.
`2. Polymyalgia rheumatica. Especially in the elderly, it is at times very dif(cid:173)
`ficult to differentiate between late-onset (>60 years) RA that is seronega(cid:173)
`tive and polymyalgia rheumatica. At times, RA may present with promi(cid:173)
`nent soreness and stiffness in the shoulder and pelvic girdles. The presence
`oftemporal arteritis symptoms or signs supports the diagnosis ofpolymyal(cid:173)
`gia rheumatica.
`3. Seronegative spondyloarthropathies characteristically present with
`asymmetric inflammatory disease of the large joints of the lower extremi(cid:173)
`ties, often with low-back disease and telltale manifestations such as psori(cid:173)
`asis, urethritis, uveitis, or inflammatory bowel disease.
`4. Crystal deposition arthritis. Both gout and pseudogout may present
`in a polyarticular, RA-like fashion. Careful history, radiographs, andjoint
`fluid analysis are helpful in defining these diagnoses.
`5. Osteoarthritis. In the setting of severe, RA-related joint damage, sec(cid:173)
`ondary osteoarthritis may develop and be a contributing factor to joint
`dysfunction and the need for hip or knee replacement. Osteoarthritis itself
`can easily be differentiated from RA by a late age of onset, localization in
`
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`196
`
`III. Diagnosis and Therapy
`
`the distal and proximal interphalangeal joints, monarticular involvement
`of a single hip or knee, the propensity to involve the neck and low back,
`and the absence of joint inflammation and constitutional features.
`6. Infectious arthritis
`a. Viral arthritides may present as a self-limited, RA-like disorder. This
`is particularly true in the setting of rubella infection or immunization
`and parvovirus B19 infection. Associated symptoms and signs, sero(cid:173)
`logies, and course establish the diagnosis. Hepatitis C-associated RA(cid:173)
`like disease is another consideration. The finding of RF in hepatitis
`C-associated cryoglobulinemia makes it distinguishable only on docu(cid:173)
`mentation of hepatitis C infection. Early hepatitis B may present as a
`self-limited RA-like disease in the pre-icteric phase.
`b. Spirochetal arthritis. Lyme disease rarely presents in an RA-like
`fashion. Early Lyme disease may present with arthralgias and myal(cid:173)
`gias in the setting of the erythema chronicum migrans rash and his(cid:173)
`tory of tick bite. Late (tertiary) Lyme presents with a waxing and wan(cid:173)
`ing monarticular synovitis or oligoarthritis (four or fewer joints) that
`often involves the knees.
`c. Bacterial arthritides. Whipple's disease may present as a periph(cid:173)
`eral, symmetric, migratory, non-deforming seronegative polyarthritis,
`simultaneously with or long before gastrointestinal manifestations
`and lymphadenopathy.
`d. Reactive arthritis associated w"ith group A f3-hemolytic strep(cid:173)
`tococcal infection. Rheumatic fever must be considered in chil(cid:173)
`dren and adults of all socioeconomic strata. The telltale clinical pre(cid:173)
`sentation is a ~nigratory polyarthritis, particularly in the setting of a
`recent sore throat. Almost invariably, the patient will have an elevated
`anti-streptolysin 0 titer and sedimentation rate.
`VII. Global assessment of functional status and disease. To craft an optimal
`therapeutic plan and establish baseline data for later comparison, the initial col(cid:173)
`lection of pertinent clinical, laboratory, and functional information is mandatory.
`Apart from the traditional detailed history and clinical evaluation, a number of
`self-report questionnaires [Health Assessment Questionnaire (HAQ), Arthritis
`Impact Measurement Scale (AIMS)], as well as physicianjoint count and radio(cid:173)
`graphic scores, have been devaloped to assess functional capacity, performance
`of daily activities, disease severity and progression, and response to therapy.
`Clinical evaluation of the amount of joint damage is based on range of motion,
`instability, mal-alignment, subluxation, crepitus, and radiographic changes.
`Negative prognostic markers (e.g., high RF titers; development of erosions, par(cid:173)
`ticularly early in the disease course; early or severe functional limitation; family
`history of severe RA; development of extraarticular disease) should be entered
`into the clinical and therapeutic equations.
`VIII. Treatment
`A. Treatment goals in rheumatoid arthritis
`1. Control of the immunologic and inflammatory disease process.
`2. Prevention of joint damage and normalization of function and life span.
`3. Complete relief of symptoms and return to normal performance of activi(cid:173)
`ties of daily living.
`4. Avoidance of complications of the disease and its treatment.
`5. Education, counseling, and physical and occupational therapy.
`B. General approach to the patient with rheumatoid arthritis
`1. Patient and family education is important in the management of
`rheumatoid disease. Strong emphasis must be placed on the crucial role
`played by the patient in minimizing disability. Both the patient and the
`family must be taught what RA is and how it differs from other forms of
`arthritis. The patient should be told that RA can be a chronic, lifelong dis(cid:173)
`ease but that a variety of measures can lead to disease control. Such emo(cid:173)
`tional support may help the patient to maintain employment or an optimal
`activity schedule. In addition to familiarizing patients with the concepts
`
`

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`28. Rheumatoid Arthritis 197
`
`of chronic disease and its management, specific points must be stressed
`regarding individual drug and physical medicine therapies used, nutri(cid:173)
`tional information, quackery, and the social services available to the
`patient with arthritis. Such an education program can take the form of frank
`discussions between the physician and the patient and family, supple(cid:173)
`mented by literature dispensed by the doctor. An optimal setting is an
`established patient education program that employs lecture and audio(cid:173)
`visual material. Sex and vocational counseling should be part of the com(cid:173)
`prehensive approach to the RA patient.
`2. Exercise and rest. It is important for the RA patient to maintain a bal(cid:173)
`ance between resting and exercising joints that falls short of causing sig(cid:173)
`nificant pain or fatigue. Systemic and articular rest are both important.
`Although the classic recommendations for short rest periods during the
`day (1 hour of bed rest at mid-morning and mid-afternoon) remain, they
`are incompatible with the work requirements of most people. At times, hos(cid:173)
`pitalization may become necessary to impose a strict balance of rest and
`activity that cannot be followed by the patient at home. Articular inflam(cid:173)
`mation may be decreased by adequate rest of the affected joints with either
`bed rest or splints. The purpose of splints is to provide rest for inflamed
`joints, relieve spasm, and prevent deformities or reduce deformities already
`present. Wrist splints are particularly useful during bouts of acute wrist
`synovitis and for the management of carpal tunnel syndrome.
`3. Regular active exercise with instructions by the physician or physical
`therapist is important. Exercise is most successful after heat application .
`A 15-minute early-morning shower or a bath at 98° to 100° will help decrease
`morning stiffness. Unless advanced deformity, significant joint pain, or
`muscle wasting is present, slow and deliberate active or active-resistive
`exercises should be performed twice daily for approximately 15 minutes.
`These exercises should involve the fingers, wrists, shoulders, and knees,
`which are the areas most vulnerable to deformity and functional disabil(cid:173)
`ity (see Chapters 56 and 57). As tolerance for exercise increases and the
`activity of the disease decreases, progressive resistive exercises are indi(cid:173)
`cated for the improvement of muscular function. Static quadriceps exer(cid:173)
`cises should be performed to strengthen the muscular, ligamentous, and
`tendinous support of the knees. Initial therapy is 10 to 20 exercise sets in
`each thigh twice daily.
`4. Principles of joint protection include maintenance ofmuscle strength
`and range of motion, avoidance of positions of deformity, use of the strongest
`joints possible for a given task, utilization of joints in the most stable ana(cid:173)
`tomic planes, avoidance of continuous use of muscles and joints in a fixed
`position, and avoidance of activities beyond the patient's muscular capac(cid:173)
`ity. Active exercises in the form of activities that interest the patient should
`be provided (e.g., sculpting, clay modeling, weaving).
`5. Activities of daily living. Instruction by the occupational therapist
`should include self-help devices, resting and functional splints, and the
`demonstration of alternate methods for task performance aimed at avoid(cid:173)
`ing positions that cause joint deformity. This often includes the use of
`simulated kitchens, bathrooms, and workplace environments that allow
`for improvement in functional capacity in sites germane to the patient's
`life-style.
`C. Medication and therapy with biologics: general information
`1. Rationale for the modern therapeutic approach
`a. RA is an aggressive disorder that demands equally aggressive and
`early treatment.
`b. A lack of evident joint inflammation (e.g., no evidence of disease) is
`highly correlated with an absence ofjoint damage.
`c. Disease- and damage-modifying medications with acceptable safety
`profiles are available.
`d. Early treatment can prevent joint damage, maintain function, prolong
`life, and alter the natural history ofRA.
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`III. Diagnosis and Therapy
`
`e. Combination therapy is usually both effective and safe and is focused
`on multiple pathogenetic sites.
`f. Careful clinical profiling can effectively guide therapy.
`g. Careful choice of medications and monitoring for side effects improves
`outcome.
`2. General therapeutic concepts
`a. A well-crafted treatment regimen should begin soon after the diagno(cid:173)
`sis and be guided by the patient profile.
`b. The regimen, its rationale, potential side effects, and monitoring must
`be discussed with the patient.
`c. The aggressiveness of the regimen should match that of the disease.
`d. Patient comorbidities should be factored into the choice of medications.
`e. Disease activity and severity and the patient's functional status should
`be measured at baseline and at regular intervals.
`f. Lack of reasonable disease control demands a change in the treatment
`regimen.
`g. Multidrug regimens are state-of-the-art and include the following:
`(1) A nonsteroidal antiinflammatory drug (NSAID).
`(2) One or more disease-modifying antirheumatic drugs (DMARDs) or
`biologic agents.
`(3) Short courses of an oral or intraarticular steroid, as needed to reset
`the level of inflammation, improve function, and act as a bridge for
`other treatments. Regular monitoring is mandatory for each med(cid:173)
`ication and drug combination. Side effects or drug intolerance de(cid:173)
`mands a change in the treatment approach. Collaboration with other
`professionals should be sought as needed to improve the outcome.
`3. Definition of the patient profile. This should be established at base(cid:173)
`line and at regular intervals. Initial and future therapeutic decisions
`should be based on the following data:
`a. Quantification of the level of inflammation
`(1) Intensity and extent of joint inflammation (i.e., number and inten(c