You are in: eMedicine Specialties > Radiology > MUSCULOSKELETAL Rheumatoid Arthritis, HandsArticle Last Updated: May 10, 2007AUTHOR AND EDITOR INFORMATIONAuthor: Ian Y Y Tsou, MBBS, FRCR, Clinical Lecturer, Faculty of Medicine, National University of Singapore; Consulting Staff, Department of Radiology, Mount Elizabeth Medical Centre Ian Y Y Tsou is a member of the following medical societies: American Roentgen Ray Society and Royal College of Radiologists Coauthor(s): Wilfred CG Peh, MD, MBBS, FRCP(Glasg), FRCP(Edin), FRCR, MHSM, Clinical Professor, Faculty of Medicine, National University of Singapore; Senior Consultant Radiologist, Programme Office, Singapore Health Services; Michael A Bruno, MD, Associate Professor, Departments of Radiology and Medicine, Pennsylvania State University College of Medicine; Director, Radiology Quality Management Services, Milton S Hershey Medical Center, Pennsylvania State University College of Medicine Editors: Amilcare Gentili, MD, Clinical Professor of Radiology, University of California at San Diego; Consulting Staff, Department of Radiology, Thornton Hospital; Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand; Lynne S Steinbach, MD, Chief of Musculoskeletal Radiology, Professor, Department of Radiology, University of California at San Francisco; Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute; Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington Author and Editor Disclosure Synonyms and related keywords: RA, human leukocyte antigen, DR4, DRB1, joint deformity, joint fusion, metacarpophalangeal joint, MCP joint, proximal interphalangeal joint, PIP joint, thumb interphalangeal joint, IP joint, distal interphalangeal joint, DIP joint, swan-neck deformity, boutonnière deformity, button-hole deformity, inflammatory joint disease, rheumatoid nodules INTRODUCTIONBackgroundRheumatoid arthritis (RA) is a systemic inflammatory disease that results in cartilage and bone destruction. RA is characterized by a typical pattern and distribution of synovial joint involvement. Disorganization of the joint leads to deformities and loss of function. PathophysiologyRA is characterized by diffuse cartilage loss and erosion of bone and cartilage. It starts in the synovial membrane, with the initial processes of edema, neovascularization, and hyperplasia of the synovial lining. Proliferation of synoviocytes and macrophages causes thickening of the synovial lining and, together with lymphocytes, plasma cells, and mast cells, develops into pannus. Pannus is a sheet of invasive cellular tissue that is continuous with the synovial lining. As a result of the higher proportion of synoviocytes and macrophages, pannus causes erosion of bone and cartilage at the margin of joints. Prominent villous formation occurs in the synovium, as does an inflammatory joint effusion. The effusion causes capsule distention and stretching of the ligamentous tissues, resulting in laxity of the capsule. With further progression of the disease, the joint becomes unstable and begins to deform. At the cellular level, cytokines, such as interleukin 1 and tumor necrosis factor-alpha, stimulate synoviocytes to produce cartilage-degrading enzymes. Other factors involved at this stage include other interleukins and transforming growth factor-beta. Cytokines also regulate production and expression of adhesion molecules, which allow pannus to attach to cartilage and bone. Hyperplastic synovium and pannus produce several enzymes that are capable of degrading components of bone and cartilage. One important group of enzymes is the matrix metalloproteinases, which are secreted from synoviocytes and chondroblasts in response to cytokines. Other proteolytic enzymes also play a contributory role. Genetically, RA has been shown to be associated with positive human leukocyte antigen DR4; a strong association with human leukocyte antigen DRB1 has been shown as well. FrequencyUnited StatesThe prevalence of RA is approximately 1%, with a range of 0.4-2%; however, a prevalence of 5% has been reported among some groups of North American Indians, especially in the Yakima, Pima, and Chippewa tribes. InternationalSimilar rates of 0.3-1% are seen in Europe and in Asia, and the rates are slightly lower in Africa. Mortality/MorbidityThe primary effect of RA is in joint deformity and fusion, which occurs in the advanced stages.
RaceRA affects persons of all races. SexWomen are affected 2-3 times more commonly than men. In some groups, the female preponderance is greater; for example, the female-to-male ratio is as high as 9:1 in Asian Indians. AgeThe onset in women is slightly earlier than in men. RA begins in women in their mid 20s. RA rarely occurs in men younger than age 30 years. In both sexes, the incidence rises constantly, with a broad peak in individuals aged 60-70 years. AnatomyIn the hands, the metacarpophalangeal (MCP), proximal interphalangeal (PIP), and thumb interphalangeal (IP) joints are most frequently involved. The distal interphalangeal (DIP) joints are involved only in the presence of a coexisting MCP or PIP disease. Tenosynovitis of the flexor tendons causes a reduction in finger flexion and grip strength. Nodular thickening in the tendon sheath may also produce a trigger finger. As RA progresses, its characteristic deformities become apparent. These include ulnar deviation of the fingers at the MCP joints, subluxation of the MCP joints with the proximal phalanx slipping to the volar side of the metacarpal heads, hyperextension of the PIP joint with flexion of the DIP joint (swan-neck deformity), flexion of the PIP joint with hyperextension of the DIP joint (boutonnière/button-hole deformity), Z-shaped deformity of the thumb from subluxation of the first MCP joint and compensatory hyperextension of the IP joint, and drooping of the ring and little fingers resulting from rupture of the extensor tendons at the point of crossing the inflamed, eroded ulnar styloid. In the wrist, the early stages of RA cause tenosynovitis of the extensor tendons, causing swelling over the distal wrist. The ulnar styloid may become tender, which indicates inflammatory synovitis. The distal end of the ulna tends to sublux dorsally, and the carpal bones sublux anteriorly to the distal radius and ulna. Bony erosions and ankylosis of the carpal bones are also seen and appear to be prominent features in Asian patients. Clinical DetailsArthritis typically has an insidious onset, with symmetric, polyarticular involvement of the small joints in the hands and feet. Symptoms of pain and stiffness are usually present. The classic persistent aching pain tends to have a diurnal variation (ie, it is worse in the morning and eases with activity). Stiffness is also more common in the early morning after a period of inactivity. Stiffness lasting more than 1 hour is fairly specific for inflammatory joint disease. Clinical signs include joint swelling, muscle wasting, instability, malalignment, and restriction of range of motion. Joint swelling may be real or apparent, with real swelling resulting from synovial thickening and joint effusion in active synovitis and apparent swelling resulting from malalignment. In addition, RA is a systemic disease and a number of important extra-articular manifestations have been identified. Fatigue, malaise, and weight loss are prominent features and may reflect disease activity. Generalized osteoporosis involving both the appendicular and axial skeleton is common. A mild normochromic normocytic anemia is commonly present and is similar to anemia of chronic disease; however, a degree of anemia lower than 10 g/dL is unusual. Felty syndrome is the combination of neutropenia and splenomegaly in RA. Rheumatoid nodules are small, firm, nontender subcutaneous nodules that are most often found over the proximal third of the ulna and at the olecranon. Nodules may also occur at the fingers and thumbs (particularly in the dominant hand) and elsewhere in the body. Nodules are strongly associated with a positive rheumatoid factor. Pleural effusion and pleuritis are the most common pulmonary manifestations. Pulmonary rheumatoid nodules are associated with the presence of skin rheumatoid nodules and are usually peripheral. They may cavitate but rarely calcify. Multiple nodules on a background of pneumoconiosis is known as Caplan syndrome. In addition, the incidence of pulmonary fibrosis and bronchiectasis is increased in RA. Cardiac features include pericarditis and rheumatoid nodules in the heart. RA vasculitis frequently manifests as obliterative endarteritis, with proliferation of the intima in digital vessels resulting in nailfold and digital infarcts. Several nerve entrapment syndromes, such as the median nerve in carpal tunnel syndrome, ulnar nerve compression within the Guyon canal, and the posterior tibial nerve in the tarsal tunnel are more common in RA. The eyes may show keratoconjunctivitis sicca and/or scleritis. Sjögren syndrome may occur together with keratoconjunctivitis sicca. Rheumatoid factor is an immunoglobulin M antibody that is present in 60-80% of patients with RA at some stage during the disease; however, rheumatoid factor is not specific for RA and is also present in other connective tissue diseases, infection, and autoimmune disorders. In addition, rheumatoid factor is present in 1-5% of people without RA. Seropositive results are associated with nodules, vasculitis, and Sjögren syndrome. Preferred ExaminationRadiography remains the first choice in imaging RA. Magnetic resonance imaging (MRI) provides a more accurate assessment, as well as earlier detection of lesions. Ultrasonography of specific joints based on radiographs may have a role as well. Limitations of TechniquesThe mainstay of imaging RA in the hands is radiography. Radiography is cheap, is easily reproducible, and allows easy serial comparison for assessment of disease progression. The main disadvantage is the absence of specific radiographic findings in early disease, since visualization of erosions may only be seen later. MRI continues to develop as a treatment tool, with the main thrust being detection of early disease at a stage at which disease-modifying drugs can be used; however, the cost of the examination and the small size of the joints involved limit widespread use. With further experience and cheaper scans, MRI scanning for the treatment of RA may gain acceptance in the future. Patient Education: For excellent patient education resources, visit eMedicine's Arthritis Center. Also, see eMedicine's patient education articles Rheumatoid Arthritis and Understanding Rheumatoid Arthritis Medications. DIFFERENTIALS[Reiter Syndrome, Musculoskeletal] Ankylosing Spondylitis Calcium Pyrophosphate Deposition Disease Gout Juvenile Rheumatoid Arthritis Psoriatic Arthritis Other Problems to Be ConsideredScleroderma
RADIOGRAPHFindingsMorphology Usually, the earliest sign of RA is a periarticular soft-tissue swelling with a fusiform appearance (see Image 1). Normal fat planes may be obliterated, which occurs as a result of joint effusion, edema, and tenosynovitis. Juxta-articular osteopenia is another early sign, particularly during the acute inflammatory stage (see Image 2). Osteopenia subsequently becomes more generalized as the disease progresses. Initially, joint spaces in the small joints of the hands show widening as a result of effusion; however, with cartilage destruction, joint spaces narrow. Erosions usually begin at the bare area of the joint not covered by cartilage, such as the intracapsular articular margins (see Image 3). Marginal erosions occur as a result of direct mechanical action of the hypertrophied synovium and granulation tissue (see Images 4-5). The forces of tendon imbalance and capsular contraction result in joint subluxation and malalignment (see Image 6). Characteristic appearances include the boutonnière (flexion at the PIP joint and extension at the DIP joint) and swan-neck (extension at the PIP joint and flexion at the DIP joint) deformities at the IP joints. Ulnar deviation is also commonly present at the MCP joints. MCP joints may also dislocate and have overriding ends (main en lorgnette or telescoping joints). Subluxations are progressive and may occur with or without the presence of bony erosions (see Image 7). Tendinous involvement of RA may result in rupture of the tendon, causing increased disability. If significant involvement of the scapholunate joint is present, laxity or rupture may cause rotatory subluxation of the scaphoid. Fusion or joint ankylosis is common in the later stages of RA (see Image 8). Fusion usually takes place in a deformed or malaligned position (see Image 9). This further reduces the functionality of the hand and affects independence in the activities of daily living. In the end stages, extensive erosions may combine to result in resorption and tapering of the ends of the bones. Complications of therapy may be seen, such as generalized osteoporosis from steroid usage. Large bony erosions and severe osteopenia predispose patients to relatively atraumatic fractures. Long-term steroid use also predisposes patients to avascular necrosis. Distribution The hands are affected symmetrically, and the disease process is usually visible first at the second and third MCP joints and the third PIP joint. The disease continues in the remainder of the MCP and PIP joints. Varying stages may be seen in different joints at a single point in time. DIP joint involvement without proximal involvement is rare. Erosions may be detected first either in the MCP and PIP joints or at the carpal bones. Erosions may also be seen at the intra-articular portion of the distal end of the radius or within the carpal bones. Carpal bone ankylosis is a common and fairly specific sign, particularly in the Asian population, in whom it tends to occur early in the disease process. Degree of ConfidenceRA is indicated on radiographs by the overall pattern and features of joint involvement. Although the presence of bony erosions is typical of RA, erosions are not specific because of the large number of other erosive arthritides. An absence of osteophyte formation and subchondral sclerosis is also indicative of RA rather than osteoarthritis. False Positives/NegativesIn the late stage (but not in the early stage), the deformities and joint fusion of RA are usually distinguishable from other causes of erosive arthropathy. No normal variants simulate RA. CT SCANFindingsCurrently, computed tomography (CT) has a minimal role in treating RA of the hands. Although CT is useful for demonstrating bony pathology, erosions in the small joints of the hands are evaluated best by using a combination of radiography and MRI. MRI also has the added advantage of being able to visualize bone marrow edema and synovial and soft-tissue involvement. MRIFindingsMRI provides images with good delineation of soft-tissue changes, cartilaginous defects, and osseous erosions associated with RA. In particular, the ability to detect synovial hypertrophy and pannus formation before the onset of bony erosions has become more valuable with the advent of disease-modifying antirheumatic drugs. The drugs, which retard the progression of RA, are most effective in the early stages of disease. Signal intensity of the inflamed synovial lining may vary markedly on T1- and T2-weighted images. An inflamed synovial lining usually demonstrates low signal intensity on T2-weighted images, and the images may be even darker if hemosiderin or a predominantly fibrous component is present within the lining. Tenosynovitis resulting from inflammation is seen as high–signal-intensity fluid on T2-weighted sequences (see Image 10). The intravenous (IV) administration of a gadolinium-based contrast agent allows better distinction of inflammatory soft-tissue changes and can help differentiate pannus from eburnation. In addition, rapid enhancement of proliferative inflammatory synovium has been reported, compared to minimal signal change in healthy soft tissue and bone. Fat suppression and fast dynamic scanning have also been used to increase the sensitivity of synovial imaging. Gadolinium-based contrast agents (eg, gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. These diseases have occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or magnetic resonance angiography (MRA) scans. As of late December 2006, the FDA has received reports of 90 such cases. According to the FDA, over 200 cases have been reported worldwide. NSF/NFD are debilitating and sometimes fatal diseases. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape. MRI is clearly superior to radiography in the early detection of bone erosions (see Image 11, Image 13). MRI is more sensitive to bone marrow edema. Bright lesions on T2-weighted images often do not correspond to findings on radiographs or T1-weighted images (see Image 12). These lesions are believed to represent potentially reversible pre-erosive changes. Routine use of MRI in evaluating rheumatoid arthropathy in the hands and wrist has been limited by the relatively high cost, the difficulty in positioning disabled patients, and the inability to image several joints simultaneously. Degree of ConfidenceMRI has been shown to be more sensitive to early changes in RA, and in the appropriate clinical setting, it is more accurate than plain radiography in the diagnosis of RA. False Positives/NegativesImaging of early synovial disease by MRI makes early detection possible, but the findings are not specific in differentiating RA from other causes of synovitis or joint erosions. ULTRASOUNDFindingsHigh-resolution sonography with a high-frequency probe is used for evaluation of the small joints in RA (see Image 14). Joint effusion is hypoechoic, while the hypertrophic synovium is more echogenic. Rheumatoid nodules are seen as fluid-filled round cavities with sharp borders. Bone erosions may be seen as irregularities in the hyperechoic cortex. Complications of RA, such as tenosynovitis and tendon rupture, can also be visualized by using ultrasonography. This is most useful in the MCP and IP joints. The carpal bones and the carpometacarpal joints are not visualized as well because of their irregular configuration and deeper location. Sonography has been applied to the assessment of RA with the goal of improving on the current standard of conventional radiography. Ultrasonography, especially when augmented by amplitude color Doppler (ACD) imaging, has also provided clinically useful information in the assessment of RA. Originally, ACD or power Doppler scanning was developed as an improvement to existing vascular sonographic imaging techniques to delineate stenoses by imaging blood flowing in vessel lumina by virtue of higher sensitivity to flow and lack of directional dependence. ACD imaging has been applied to RA with the goal of evaluating the manifestation of hyperemia in the inflammatory joint tissues at the symptomatic sites of disease. Synovial hyperemia is a fundamental pathophysiologic feature of RA (see Image 15). Most likely, synovial hyperemia is the principal factor underlying the hallmark x-ray observation of periarticular demineralization on radiographs of patients with RA, and it is believed to occur in proportion to joint-destructive disease activity. Hyperemia in the tendon sheath is common as well (see Image 16). Two recent reports in the literature have explored a possible role for Doppler ultrasonography in evaluating arthritis, especially RA, on the basis of synovial hyperemia. In 1996, Newman et al1 used a 3-level semiquantitative approach by trained radiologists to grade Doppler flow subjectively in patients with RA, psoriatic arthritis, and calcium pyrophosphate deposition disease arthritis. The investigators found that the degree of abnormal tissue hyperemia visualized correlates grossly with the severity of local disease activity as measured by synovial-fluid white blood cell (WBC) counts and patients' subjective estimates of symptoms. The report did not include joint examinations or other physical/functional measurements. Hau et al 2 from the University Hospital of Würzburg, Germany, reported findings from ultrasound in the MCP and PIP joints of 34 patients with RA and 15 control subjects over a 6-month period. In the protocol, each patient was examined only once. Patients were stratified into 3 groups based on swelling and tenderness. Ultrasonography was performed by using a Siemens Doppler unit and a 5- to 13-MHz linear-array transducer operating at 12 MHz. The authors identified joint pannus on gray-scale images as hypoechoic soft tissue adjacent to the articular surfaces and described the pannus distribution as favoring the radial side of the joints. Velocity color Doppler (ie, conventional direction-sensitive Doppler) imaging was used to evaluate vascularization and blood flow in the rheumatoid pannus as a surrogate marker of inflammation. The quantitation method used was analogous to that used by one of the coauthors of this article in preliminary studies. A simple image-based quantification was obtained in the study by Hau et al2 by summing the color pixels within all joints in each patient classification after selection of regions of interest within each joint. The result was a unitless numerical scale that showed a greater than tenfold difference between patients with active RA and controls. From the preliminary investigations, both groups (Newman in 1996 and Hau in 1999) concluded that power Doppler ultrasonography can reflect disease activity in RA, and each group suggested further research in this area.
Degree of ConfidenceSimilar to MRI, ultrasonography serves as an early diagnostic tool and can help in evaluating the cause of joint swelling in a patient with RA. False Positives/NegativesImaging of early synovial disease by ultrasonography makes early detection possible, but sonography is not specific in differentiating RA from other causes of synovitis or joint erosions. NUCLEAR MEDICINEFindingsApart from demonstration of active disease, the results of radionuclide bone scans remain nonspecific and are used mainly to provide an overview of the distribution of disease. ANGIOGRAPHYFindingsCurrently, angiography has no defined role in assessing RA of the hands. INTERVENTIONThe primary role of imaging in RA of the hands and wrists is to exclude septic arthritis. Percutaneous ultrasonography-guided aspiration may be useful for localizing and obtaining samples of any collection that can be found. Image 17 shows ultrasonography-guided biopsy of the second MCP joint in the right hand. Medical/Legal Pitfalls
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Rheumatoid Arthritis, Hands excerpt Article Last Updated: May 10, 2007 | |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||