Washington manual of rheumatology 3rd ed subspeciality consult

Assuch, it provides guidance on how to perform the musculoskeletal examination andarthrocentesis, what laboratory testing may prove useful, and which medications areappropriate including

THE WASHINGTON MANUAL™

Rheumatology Subspecialty Consult Second Edition

Professor of Internal Medicine

Washington University School of Medicine

St Louis, Missouri

Katherine E Henderson, MD

Assistant Professor of Clinical Medicine

Department of Medicine

Division of Medical Education

Washington University School of MedicineBarnes-Jewish Hospital

St Louis, Missouri

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© 2012 by De partme nt of Me dicine , Washington Unive rsity School of Me dicine

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All rights reserved This book is protected by copyright No part of this book may be reproduced in any form by any means, including photocopying, or utilized by any information storage and retrieval system without written permission from the copyright owner, except for brief quotations embodied in critical articles and reviews.

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Library of Congre ss Cataloging-in-Publication Data

The Washington manual rheumatology subspecialty consult — 2nd ed /editor, Leslie E Kahl.

p ; cm.

Rheumatology subspecialty consult

Includes bibliographical references and index.

ISBN 978-1-4511-1412-6 (alk paper) — ISBN 1-4511-1412-5 (alk paper)

I Kahl, Leslie E II Title: Rheumatology subspecialty consult.

[DNLM: 1 Rheumatic Diseases—diagnosis—Handbooks 2 Rheumatic Diseases—therapy— Handbooks WE 39]

616.723—dc23

2012004717

The Washington Manual™ is an intent-to-use mark belonging to Washington University in St Louis to which international legal protection applies The mark is used in this publication by LWW under license from Washington University.

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10 9 8 7 6 5 4 3 2 1

Samuel Grant Professor of Medicine

Department of Internal Medicine

Associate Professor of Medicine

Department of Internal Medicine

Division of Rheumatology

Washington University School of Medicine

St Louis VA Medical Center

St Louis, Missouri

Department of Internal Medicine

Associate Professor of Medicine

Department of Internal Medicine

Washington University School of Medicine

Washington Manual Subspecialty Series addresses this challenge by concisely and

practically providing current scientific information for clinicians to aid them in thediagnosis, investigation, and treatment of common medical conditions

I want to personally thank the authors, which include house officers, fellows, andattendings at Washington University School of Medicine and Barnes-JewishHospital Their commitment to patient care and education is unsurpassed, and theirefforts and skill in compiling this manual are evident in the quality of the finalproduct In particular, I would like to acknowledge our editor, Dr Leslie Kahl, andthe series editors, Drs Tom De Fer and Katherine Henderson, who have workedtirelessly to produce another outstanding edition of this manual I would also like tothank Dr Melvin Blanchard, Chief of the Division of Medical Education in theDepartment of Medicine at Washington University School of Medicine, for hisadvice and guidance I believe this Subspecialty Manual will meet its desired goal

of providing practical knowledge that can be directly applied at the bedside and inoutpatient settings to improve patient care

Victoria J Fraser, MD

Dr J William Campbell ProfessorInterim Chairman of MedicineCo-Director of the Infectious Disease DivisionWashington University School of Medicine

Preface

e have written this manual as a guide to inpatient and outpatient rheumatologyconsultations The target audience includes medical students, residents, andother medical professionals who care for patients with rheumatologic problems Inaddition, this manual could also serve as a pocket reference for medicalprofessionals specializing in rheumatology It is not intended as a compendium ofrheumatology but, rather, focuses on how to approach rheumatologic problems Assuch, it provides guidance on how to perform the musculoskeletal examination andarthrocentesis, what laboratory testing may prove useful, and which medications areappropriate (including dosages and recommended monitoring), all framed within abrief overview of the major rheumatologic diseases

The editor would like to thank the contributing authors for their participation inthis project A special thanks goes to Dr Tom De Fer for his ongoing support andoversight, and to the authors and editors of the first edition of the manual for leadingthe way

L.E.K

1 Approach to the Rheumatology Patient

Maria C Gonzalez-Mayda and Leslie E Kahl

2 Rheumatologic Joint Examination

Michael L Sams and Leslie E Kahl

3 Arthrocentesis: Aspirating and Injecting Joints and Bursa

Rebecca Brinker and Leslie E Kahl

4 Synovial Fluid Analysis

Jeffrey Sparks and Leslie E Kahl

5 Laboratory Evaluation of Rheumatic Diseases

Kristine A Kuhn and Leslie E Kahl

6 Radiographic Imaging of Rheumatic Diseases

Ashwini Komarla and Leslie E Kahl

7 Rheumatologic Emergencies

Reeti Joshi and Leslie E Kahl

8 Regional Pain Syndromes

Michael L Sams and Leslie E Kahl

9 Drugs Used for the Treatment of Rheumatic Diseases

Alfred H.J Kim and Leslie E Kahl

PART II COMMON RHEUMATIC DISEASES

10 Rheumatoid Arthritis

Richa Gupta and Prabha Ranganathan

11 Osteoarthritis

Hyon Ju Park and Prabha Ranganathan

12 Systemic Lupus Erythematosus

Alfred H.J Kim and Wayne M Yokoyama

PART III CRYSTALLINE ARTHRITIS

13 Gout

Richa Gupta and Wayne M Yokoyama

14 Calcium Pyrophosphate Dihydrate Crystal Deposition DiseaseAmy Archer and Wayne M Yokoyama

PART IV SPONDYLOARTHROPATHIES

20 Vasculitis

Alfred H.J Kim and John P Atkinson

21 Takayasu’s Arteritis

Michael L Sams and John P Atkinson

22 Giant Cell Arteritis and Polymyalgia Rheumatica

Alfred H.J Kim and John P Atkinson

Amy Archer and John P Atkinson

28 Cryoglobulinemia and Cryoglobulinemic Vasculitis

Reeti Joshi and John P Atkinson

Maria C Gonzalez-Mayda and John P Atkinson

PART VI INFECTION AND RELATED DISORDERS

32 Infectious Arthritis

Jeffrey Sparks and Prabha Ranganathan

33 Lyme Disease

Rebecca Brinker and Prabha Ranganathan

34 Acute Rheumatic Fever

Jeffrey Sparks and Prabha Ranganathan

PART VII OTHER RHEUMATIC DISORDERS

Lesley Davila and Amy Joseph

40 Mixed Connective Tissue Disease

Reeti Joshi and Amy Joseph

41 Undifferentiated Connective Tissue Disease

Rebecca Brinker and Amy Joseph

42 Adult-Onset Still’s Disease

Amy Archer and John P Atkinson

43 Relapsing Polychondritis

Lesley Davila and John P Atkinson

44 Deposition and Storage Arthropathies

Hyon Ju Park and Zarmeena Ali

45 Sarcoid Arthropathy

Lesley Davila and Leslie E Kahl

46 Amyloidosis and Amyloid Arthropathy

Rebecca Brinker and Zarmeena Ali

47 Miscellaneous Skin Conditions

Reeti Joshi, Zarmeena Ali, and Leslie E Kahl

48 Osteoporosis

Ashwini Komarla, Richa Gupta, and Zarmeena Ali

49 Avascular Necrosis

Richa Gupta, Ashwini Komarla, and Zarmeena Ali

50 Hereditary Periodic Fever Syndromes

Hyon Ju Park and John P Atkinson

Index

Approach to the Rheumatology Patient

Maria C Gonzalez-Mayda and

Leslie E Kahl

GENERAL PRINCIPLES

• Musculoskeletal conditions may be classified according to their symptompresentation, that is, inflammatory versus noninflammatory and articular versusnonarticular

• A thorough history and physical examination is necessary in order to helpnarrow your diagnosis

• Use specific ancillary tests such as radiographs, labs, and arthrocentesis tohelp confirm your initial diagnosis

• Rheumatic diseases mainly involve the musculoskeletal system.

Inflammatory disorders are often accompanied by systemic features (feverand weight loss) and other organ involvement (kidney, skin, lung, eye,blood)

Since these diseases affect multiple organ systems, they are thereforechallenging to diagnose, complicated to treat, and often humbling to study

• Musculoskeletal complaints account for a majority of outpatient visits in thecommunity

Many are self-limited or localized problems that improve with symptomatictreatment

Other conditions (e.g., septic arthritis, crystal-induced arthritis, fractures)require urgent diagnosis and treatment

• Musculoskeletal problems may also be the initial presentation of diseases such

as cancer and endocrinopathies

• Inpatient consultations usually involve patients with known diagnoses (a patientwith lupus admitted with a flare) or with multiple organ system involvementand the suspicion of a systemic rheumatic disease (a patient with respiratoryand kidney failure with positive antineutrophil cytoplasmic antibody [ANCA])

The following is an approach to patients with musculoskeletal complaints and tocommon inpatient consults Regional problems (i.e., nonsystemic musculoskeletaldisorders) are discussed in Chapter 8, Regional Pain Syndromes

Inflammatory versus Noninflammatory

The characteristics of inflammatory and noninflammatory disorders are presented in

Joint stiffness is common after prolonged rest (morning stiffness) and

improves with activity

Duration of >1 hour suggests an inflammatory condition

Noninflammatory conditions may cause stiffness usually lasting <1 hour

and the joint symptoms increase with use and weight bearing.

These distinctions are useful but not absolute

Inflammatory disorders may be immune-mediated (systemic lupus erythematosus [SLE], rheumatoid arthritis [RA]), reactive (reactive arthritis [ReA]), infectious (gonococcal [GC] arthritis), or crystal induced (gout,

pseudogout)

• Noninflammatory disorders

Characterized by absence of systemic symptoms, pain without erythema or

warmth, normal lab tests.

Osteoarthritis (OA), fibromyalgia, and traumatic conditions are commonnoninflammatory disorders

Articular versus Nonarticular

• Pain may originate from:

Articular structures (synovial membrane, cartilage, intra-articular

ligaments, capsule, or juxtaarticular bone surfaces)

Periarticular structures (bursae, tendons, muscle, bone, nerve, skin).

Nonarticular structures (i.e., cardiac pain referred to the shoulder).

• Articular disorders

Cause deep or diffuse pain that worsens with active and passive movement Physical examination may show deformity, warmth, swelling, effusion, orcrepitus

Synovitis (inflammation of the synovial membrane that covers the joint) is

a boggy, tender swelling around the joint The joint loses its sharp edges

on examination Synovitis is easy to detect in finger and wrist joints

Arthralgia refers to joint pain without abnormalities on joint examination Arthritis indicates the presence of abnormality in the joint (warmth,

swelling, erythema, tenderness)

Clinical Presentation

• The evaluation of musculoskeletal complaints should determine whether thedisorder is inflammatory or noninflammatory, whether the joints or theperiarticular structures are involved, and the number and pattern of jointsinvolved

• Number and pattern of joints involved:

The number and pattern of joints involved are important in the diagnosis

Acute monoarthritis suggests infection but gout and trauma are possible Asymmetric, oligoarticular (<5 joints) involvement, particularly of the

lower extremities, is typical of OA and ReA

Symmetric, polyarticular (≥5 joints) involvement is typical of RA and SLE Involvement of the spine, sacroiliac (SI) joints, and sternoclavicular joints

is characteristic of ankylosing spondylitis (AS)

In the hands, distal interphalangeal (DIP) joints are involved in OA

(Heberden’s nodes) and in psoriatic arthritis (PsA) but are spared by RA

Proximal interphalangeal (PIP) joint involvement is seen in OA

(Bouchard’s nodes) and RA

Metacarpophalangeal (MCP) joints are involved in RA but not in OA Acute first metatarsophalangeal (MTP) joint arthritis is classic for gout

(podagra) but is also seen in OA and ReA

Vasculitis and SLE may present with multiple organ system involvement,

often without major joint complaints

Fibromyalgia presents with diffuse pain but without arthritis.

Myositis presents with muscle weakness and rashes and occasionally

peripheral arthritis

History

• A comprehensive history and physical examination are often enough to make adiagnosis

• Ask about pain, swelling, weakness, tenderness, limitation of motion, stiffness,

as well as constitutional symptoms when presented with a musculoskeletal

complaint since these clues will help you narrow your diagnosis.

For example, joint pain that occurs at rest and which worsens with movementsuggests an inflammatory process versus pain elicited with activity andrelieved by rest usually indicates a mechanical disorder such as adegenerative arthritis

• Inquire about what medications they are taking since many have side effects

and patients on immunosuppressive drugs are predisposed to developinginfections

Some medications may cause a lupus-like syndrome (e.g., hydralazine, procainamide), myopathies (e.g., statins, colchicine, zidovudine), or

osteoporosis (e.g., corticosteroids, phenytoin).

Alcohol commonly precipitates gout and may rarely cause myopathies and

avascular necrosis (AVN)

Vasculitis, arthralgias, and rhabdomyolysis may also be seen with substance

abuse (e.g., cocaine, heroin).

• Certain areas of the United States and Europe have an increased incidence of

Lyme disease; therefore, it is important to inquire about recent trips to these

areas

• A family history may be important in AS, gout, and OA.

• The review of systems may identify other organ involvement and support the

Rashes are seen in SLE, vasculitis, PsA, dermatomyositis (DM), adult-onset

Still’s disease, and Lyme disease

Raynaud’s phenomenon is a reversible, paroxysmal constriction of small

arteries that occurs most commonly in fingers and toes and is precipitated bycold The classic sequence is initial blanching followed by cyanosis andfinally erythema due to vasodilation and is accompanied by numbness ortingling It may be idiopathic or associated with scleroderma, SLE, RA, and

Pleuritis and pericarditis may be seen in RA, SLE, MCTD, and adult-onset

Still’s disease

Gastrointestinal (GI) involvement is seen in enteropathic arthritis;

polymyositis (PM), and scleroderma The latter may be associated with

dysphagia

• Certain diseases are more frequent in specific age groups and genders.

SLE, juvenile RA, and GC arthritis are more common in the young

Gout, OA, and RA are more common in middle-aged persons

Polymyalgia rheumatica (PMR) and giant cell arthritis occur in the elderly Gout and AS are more common in men; gout is rare in premenopausalwomen

SLE, RA, and OA are more common in women

Physical Examination

• Tailor the physical examination based on the presenting complaint

• If the main complaint is arthritis or arthralgia, look for signs of inflammationsuch as swelling, warmth, erythema over the joint and surrounding structures,tenderness, deformity, limitation of motion, instability of the joint, and crepitus

• If the pain appears to be nonarticular in origin, examine the surrounding bursae,tendons, ligaments and bones for tenderness and inflammation

• If there is suspicion of systemic involvement, make sure to perform a thoroughexamination from head to toe so as not to miss any important findings that couldhelp you determine the etiology of their symptoms

Examine the hair for signs of alopecia and or rashes

The skin for any type of discoloration, rash, ulceration, blistering as well asthe nails for pitting

The nose and mouth for signs of ulceration

The heart and lungs for signs of pericardial and or pleural rubs, and/ordecreased breath sounds from underlying pulmonary fibrosis

The abdomen for signs of serositis, pain related to ischemic bowel, etc

A thorough examination of all the joints to look for the signs mentionedabove

Examine the back thoroughly at the spinous processes, paraspinal and

surrounding muscles Do not forget to examine the SI joints as well.

If there is clinical suspicion for Behçet’s disease and/or inflammatory boweldisease, a genital examination would be indicated since ulcerations can beseen

of referred pain (shoulder pain due to peritonitis or heart disease)

• The history should exclude trauma and can provide clues such as:

History of tick bite (Lyme disease)

Sexual risk factors (GC arthritis)

Colitis, uveitis, and urethritis (ReA)

• Physical examination usually distinguishes between articular and nonarticulardisorders

• Perform arthrocentesis in patients with acute monoarthritis (see Chapter 4,Synovial Fluid Analysis) Send synovial fluid for:

Leukocyte count with differential (>2000/mm3 suggest an inflammatoryprocess)

Gram stain and culture

Crystal analysis A wet mount of the fluid examined under polarizing

microscopy may identify crystals, but the presence of crystals does not

• Radiographs are useful in cases of trauma and may show OA orchondrocalcinosis in calcium pyrophosphate deposition disease.

• A patient with synovial fluid that is highly inflammatory requires empiric

antibiotic therapy until the evaluation, including cultures, is completed.

TABLE 1-2 FEATURES AND CAUSES OF MONOARTHRITIS

Approach to Polyarthritis

• Polyarthritis is one of the most common problems in rheumatology (Fig 1-2)

• The number and pattern of joint involvement suggest the diagnosis (Table 1-3)

• There are many nonarticular causes of generalized joint pain

Disorders of periarticular structures (tendons, bursae) cause joint pain butusually involve a single joint

Myopathies occasionally cause widespread pain, but muscle weakness is theprimary symptom.

PMR causes shoulder and pelvic girdle pain with morning stiffness, but there

is usually no arthritis on examination; weakness is not a feature of thisdisease

Neuropathies, primary bone diseases (Paget’s disease), and fibromyalgia canalso cause widespread pain but are distinguished by history and physicalexamination

FIGURE 1-1 Approach to monoarthritis JRA, juvenile rheumatoid arthritis;PMNs, polymorphonuclear cells (Adapted from American College of Rheumatology

ad hoc Committee on Clinical Guidelines Guidelines for the initial evaluation of the

adult patient with acute musculoskeletal symptoms Arthritis Rheum 1996;39:1.)

FIGURE 1-2 Approach to polyarthritis (Adapted from American College ofRheumatology ad hoc Committee on Clinical Guidelines Guidelines for the initial

evaluation of the adult patient with acute musculoskeletal symptoms Arthritis

Rheum 1996;39:1.)

• Rheumatoid arthritis is the prototypical polyarticular arthritis.

Given its frequency (1%), it is important to recognize and distinguish RAfrom other types of arthritis

RA usually begins with symmetric peripheral arthritis of multiple small

joints of hands, wrists, and feet which progresses over weeks to months.Morning stiffness is prominent and usually lasts >1 hour Other joints (knees,ankles, shoulders, elbows) are sequentially involved Involvement of thecervical spine, temporomandibular joint, and sternoclavicular joint may also

be seen

Lumbar spine and SI involvement is very rare

• SLE may present with polyarthritis similar to RA that may be intermittent.

Fever, rashes, serositis, and other organ involvement may accompany arthritis

in SLE

• Viral arthritis (due to parvovirus B19, hepatitis B, rubella, HIV) may have an

acute onset with fever and rashes and persist for months

TABLE 1-3 CAUSES OF POLYARTHRITIS

• Palindromic rheumatism causes recurrent attacks of symmetric arthritis that

affect hands, wrists, and knees and are self-limited over several days

• The seronegative spondyloarthropathies are characterized by spine and SI

joint involvement, enthesopathy (pain at sites of tendon insertion to bone) andvarying degrees of peripheral joint, eye, skin, and GI involvement

Peripheral joint involvement is usually asymmetric, oligoarticular, and of

the lower extremities (knee, ankle)

Dactylitis, a diffuse swelling of a digit (“sausage digit”), may be seen in

fingers and toes and is characteristic of ReA and PsA

A symmetric, polyarticular form of PsA exists that is similar to RA.

• Oligoarticular disease may also be seen with Behçet’s disease, sarcoidosis,and relapsing polychondritis

• Bacterial arthritis may be polyarticular in patients with preexisting jointdamage (RA)

• GC arthritis may be migratory and is accompanied by fever, pustular skinlesions, and tenosynovitis

• Fever and migratory arthralgias or mild arthritis may be seen in early Lyme

disease, and a persistent oligoarticular arthritis occurs months later.

• Bacterial endocarditis often presents with fever, low back pain, andarthralgias and may have a positive RF

• Gout is usually monoarticular, but polyarthritis is sometimes seen, particularlylater in disease

• Pseudogout may present as “pseudo-RA” with bilateral hand and wristinvolvement

• Rheumatic fever occurs after streptococcal infection and is characterized bymigratory arthritis of large joints, fever, and extra-articular involvement(carditis, chorea, rash)

• OA is the most common form of noninflammatory polyarthritis.

DIP, PIP, first carpometacarpal (CMC), knees, hips, and first MTP joints

are typically involved

Hemochromatosis predisposes to OA in unusual joints (second and thirdMCP)

Approach to Patients with Positive Antinuclear Antibodies

• Antinuclear antibodies (ANAs) are autoantibodies that target nucleic acid and

nucleoprotein antigens, and are usually detected by indirectimmunofluorescence (see Chapter 5, Laboratory Evaluation of RheumaticDiseases) (Figure 1-3)

• ANAs are very sensitive (their absence by current assays practically rules outSLE) but not too specific for SLE

• ANAs are present in other rheumatic diseases (e.g., scleroderma, MCTD, PM,SS), in drug-induced lupus (DIL), in some infectious diseases (e.g., HIV), and

in up to 5% of healthy individuals (in low titers).

• They are also present in many patients with chronic liver or lung disorders, and

in those with nonarticular autoimmune diseases such as thyroiditis

• An ANA test should be ordered only when you suspect a patient has an

underlying autoimmune condition such as SLE.

• A positive ANA should be followed by a complete history and physicalexamination to identify those conditions

A history of hydralazine or procainamide use suggests DIL

Myositis, skin changes, and Raynaud’s phenomenon suggest MCTD, myositis,

or scleroderma

Sicca symptoms (dry eyes and dry mouth) suggest SS

• SLE is a multisystem disease that is diagnosed clinically.

Criteria developed for the classification of lupus (see Chapter 12, SystemicLupus Erythematosus) can be used as a framework for assessing whether apatient has SLE

Certain other diseases (acute HIV infection, endocarditis, autoimmunehepatitis) may fulfill criteria for SLE

High-titer ANAs (>1:640) should be followed by assays for antibodies tocertain antigens (double-stranded DNA, SSA/Ro, SSB/La, ribonucleoprotein[RNP], Smith [Sm]) that may be more specific for SLE and other rheumaticdiseases

If no certain diagnosis is made, follow-up may be indicated

Up to 40% of patients referred to a rheumatologist for evaluation of positiveANAs who do not fulfill criteria on presentation do fulfill criteria for SLEafter months to years of follow-up

Approach to Patients with Possible Systemic Vasculitis

• The vasculitides are a heterogeneous group of disorders characterized byinflammation of blood vessels

• Vasculitis is often suspected in patients with multiple organ involvement.Vessels in the respiratory tract, kidneys, GI tract, peripheral nerves, and skinmay be involved in varying degrees depending on the category of vasculitisand the size of the blood vessel involved

• Perform a complete history and physical examination on these patients.

Patients with suspected vasculitis should be questioned about fever, rashes,arthralgias or arthritis, abdominal pain, weight loss, and any underlyingrheumatic diseases (SLE, RA).

The physical examination should identify other organ system involvement(purpura, peripheral neuropathy, joint abnormalities) that may not be obvious

on initial presentation

• ANCA should be ordered if suspecting some types of vasculitis.

ANCA is sensitive and specific for some vasculitides but may be seen ininfections (such as HIV)

Positive ANCA tends to require confirmation with more specific assays forantibodies against myeloperoxidase (MPO) and proteinase-3 (PR3) (see

Chapter 5, Lab Evaluation of Rheumatic Diseases)

• Other lab tests (ANA, complement levels, hepatitis panels, cryoglobulins,urinalysis) may be useful to establish etiology

• Chest and sinus radiographs may reveal occult respiratory tract involvement.

• Diagnosis may require pathologic examination of skin, nerve, kidney, or lungtissue

• Infection should be ruled out before treatment with corticosteroids orimmunosuppressives is considered

• Bacterial endocarditis, embolic disease and cocaine use may mimic vasculitisand therefore should also be considered

SPECIAL CONSIDERATIONS

Perioperative Considerations

• There are special considerations for patients with rheumatic diseases whoundergo elective surgical procedures, related to joint deformities that limitmobility, medications the patient is taking, and existing end-organ damage

• Joint deformities in patients with RA, juvenile RA, and AS may include

limited jaw opening and cervical spine fusion or laxity, which can contribute

FIGURE 1-3 Approach to a positive ANA dsDNA, double-stranded DNA; Sm,Smith (From Kavanaugh A, Tomar R, Reveille J, et al Guidelines for clinical use

of the antinuclear antibody test and test for specific autoantibodies to nuclear

antigens Arch Pathol Lab Med 2000;124:71–81.)

Patients with AS may also have limitations in thoracic expansion that may

complicate mechanical ventilation.

• Cystitis, skin infections, and possible sources of bacteremia such as cariesshould be treated before joint replacement to prevent seeding of the prosthesis

• Patients with SS are at risk for corneal abrasions in surgical settings and need

to receive ocular lubricants before and after surgery

NPO orders may be made more tolerable with artificial saliva

Particular care is needed with intubation given the usual poor state ofdentition in these patients

• Many patients with rheumatologic diseases are on nonsteroidal inflammatory drugs (NSAIDs), corticosteroids, disease-modifying anti-rheumatic drugs (DMARDs), and/or biologic agents (see Chapter 9, DrugsUsed for the Treatment of Rheumatic Diseases) Below are recommendations

anti-on when to hold specific medicatianti-ons perioperatively

Aspirin and other NSAIDs affect platelet aggregation and should bediscontinued 5 to 7 days before surgery Selective cyclooxygenase-2 (COX-2) inhibitors do not affect platelet aggregation and may be used until the day

of surgery

Patients who are on or have received corticosteroids in the previous yearmay be at risk for adrenal insufficiency during the stress of surgery.Hydrocortisone, 100 mg IV q8h, is the traditional “stress dose,” but lowerdoses may be sufficient Taper the corticosteroid dose to the daily dose (or

to zero in patients not previously on corticosteroids) within a few days if thepatient is stable

Methotrexate should be withheld 48 hours before surgery and restarted within

1 to 2 weeks to prevent a flare of arthritis

Nephrotoxic agents such as cyclophosphamide should also be withheldpreoperatively

Biological agents including etanercept, infliximab, adalimumab, and anakinramay be withheld for 1 week before and after surgery

• Prophylaxis against deep vein thrombosis is mandatory in joint replacementpatients

• Aggressive physical therapy is essential for rehabilitation

• Acute crystalline arthritis is common in the postoperative period, andmanagement may be difficult in patients with renal dysfunction or who cannothave oral intake In these patients, narcotic analgesics or intra-articular

corticosteroids (after excluding infection) may be an option (see Chapter 13,Gout).

FOLLOW-UP

• Many rheumatologic diseases have similar clinical pictures at onset but evolveinto distinct patterns over time and close follow-up is needed to make adiagnosis

• A patient with symmetric, peripheral polyarthritis may have a self-limited viralarthritis, but persistence for >12 weeks suggests that RA will be the finaldiagnosis

• Urgent treatment is required in cases with active infection Other patients may

be treated with NSAIDs and should be referred to a rheumatologist if diseasepersists

Rheumatologic Joint Examination

Michael L Sams and Leslie E Kahl

GENERAL PRINCIPLES

• The full joint examination is often excluded from the general complete physicalexamination because it is considered too time-consuming for minimaldiagnostic gain The joints are usually addressed only if the patient has aspecific complaint

• Musculoskeletal disorders, however, are common in patients who present tophysicians and are the most common reason for disability in the population

• The joint examination, if done consistently, can be performed with increasingefficiency and brevity

• A brief screening examination is presented in Table 2-1

it takes for it to resolve with activity

• It is also important to ascertain functional impairment, including how well thepatient can perform activities of daily living (e.g., dressing, grooming,cooking) and how the musculoskeletal disorder interferes with occupation orhobbies

Physical Examination

• In general, the examination should begin with a visual inspection noting

asymmetry, alignment, swelling, deformities, and changes in color.

• The examination continues with palpation for warmth, tenderness, and crepitus. Any specific structures of the joint that are tender should be noted, as patientswith rheumatologic disease can also have common musculoskeletalcomplaints, such as meniscal tear

Hard swelling around joints may be due to bony deformities, whereas tender,

“boggy” swelling may be due to synovial inflammation (synovitis)

• Simultaneously notice the bulk, tone, and strength of the associated musclegroups

• Assess active and passive ranges of motion, making note of any pattern ofjoint involvement

TABLE 2-1 BRIEF JOINT SCREENING EXAMINATION

• Finally, perform specific provocative tests for each joint to help identify a

source of pain A good example is Phalen’s test for carpal tunnel syndrome

• Note the patient’s gait as he or she walks into the room Is the patient using anyadaptations to protect a particular joint? How does the patient sit in a chair? Is

the patient able to get up to the examination table unassisted? Note the use ofcanes or other assistive devices Look for uneven wear on shoes.

• Perform a general physical examination, paying close attention to the skin,eyes, oropharynx, and nervous system

Examining Individual Joints

Hands

• Begin with visual inspection by instructing the patient to stretch out all fingers

with the palms down Then, palms up, have the patient make a fist and opposethe thumb to the base of the fifth finger Note any finger lag Inspect for visibleswelling or erythema Observe for deformity For example, sublimations of themetacarpophalangeal (MCP) joints with ulnar deviation of the digits are seenwith rheumatoid arthritis (RA) Lastly, note any atrophy of the lumbrical orthenar muscles, which can suggest nerve compression

• Palpate each joint for tenderness If there is finger lag with extension palpatethe flexor tendon for a snapping with extension or the presence of a nodule

Either of these findings may suggest trigger finger.

• Also note grip strength and ability to fine pinch Normal patients should beable to make a completely closed fist

• Heberden’s nodes: Hard, painless nodules on the dorsolateral aspects of thedistal interphalangeal (DIP) joints and are characteristic of osteoarthritis(OA)

• Bouchard’s nodes: Similar to Heberden’s nodes, except found on the proximal

interphalangeal (PIP) joints (also characteristic of OA)

• “Swan neck” deformity: Hyperextension of the PIP joint with fixed flexion of

the DIP joint

• “Boutonniere” deformity: Fixed flexion of the PIP joint with hyperextension

of the DIP joint

• Other findings include tophi (hard or soft uric acid deposits seen in chronic gout) , fingernail and cuticle abnormalities (seen in psoriatic arthritis,

dermatomyositis) and sclerodactyly (thin, tapered fingers with tight overlyingskin and loss of soft tissue seen in scleroderma)

Wrists

• Passively flex, extend, and deviate the wrists medially (ulnarly) and laterally(radially) Normal range of motion for flexion is 80 degrees and extension is

• Palpate along the extensor surface of the ulna and over the olecranon bursa for

rheumatoid nodules These fleshy nodules are usually firm, nontender, and

mobile with respect to the overlying skin Gouty tophi, which often occur in the

same area, are usually more firm

• Palpation of the elbow joint is easier posteriorly and laterally as less softtissue obscures the joint Tenderness over the medial or lateral epicondylesshould be noted

• Finally, observe the olecranon bursa for any swelling, warmth or erythema.

To evaluate internal rotation and adduction ask the patient to touch thecontralateral scapula by reaching behind the back

• To evaluate the rotator cuff perform both impingement and drop arm tests The Neer impingement sign can be elicited by passive forward flexion of the

internally rotated arm while the examiner stabilizes the scapula A positive testproduces pain with this maneuver The drop arm test evaluates for a tear of therotator cuff The test is performed by placing the patient’s shoulder in 90degrees abduction Have the patient slowly lower the arm to the side The test

is considered positive if the arm cannot be smoothly lowered to the side.

Neck

• Ask the patient to flex and extend the neck Normal range of motion permits thechin to touch the chest Ask the patient to turn his or her neck to the right thenthe left Normal rotation is about 60 degrees To evaluate lateral flexion, askthe patient to bring his or her ear toward each shoulder

• Palpation of the spinous processes should be performed on the basis of theclinical history to assess for tenderness that can suggest infection, fracture orjoint involvement Examine for tender points in the paraspinal musculature

• Spurling’s test may be performed to help assess if radicular pain from the neck

is present The patient’s neck is extended and rotated to the side of the pain andthen firm pressure is applied to the top of the head pushing downwards Ifradicular symptoms are present it suggests foraminal stenosis as the cause

• Finally, RA can lead to atlantoaxial subluxation Caution should be taken withpassive range of motion testing in these patients

Back

• Assess the curvature of the spine The normal spine has three curves: Lumbarlordosis, thoracic spine kyphosis, and cervical lordosis Identify the presence

of abnormal lordosis, kyphosis, scoliosis, or list (lateral tilt of the spine)

• Palpate for any tenderness along the spine and paraspinal muscles While thepatient is standing, instruct him or her to bend forward, backward, right, andleft

• If spondyloarthropathy (see Chapter 15, Undifferentiated Spondyloarthritis) is

suspected, perform the modified Schober test With the patient standing, mark

two midline points, one 10 cm above the lumbosacral junction (midlinebetween the posterior iliac spines) and one 5 cm below the junction Thepoints will be 15 cm apart Ask the patient to flex forward and measure thedistance between the two points In a normal individual, the span will increase

by ≥4 cm

Hips