Rheumatoid arthritis can be diagnosed in some cases simply by inspecting the hand, even when no nodules are present. First, there may be interosseus atrophy, best noted by inspecting the dorsum of the hand in oblique lighting, but with practice, discernible in direct light. Weakness of the interossei results in inability to forcefully extend the interphalangeal joints, which is necessary for finger-flicking movements. Second, there is ulnar deviation of the hand and the fingers. The best fingers to check for ulnar deviation are the third and index fingers, as many normal older persons will appear to have mild ulnar deviation of the other fingers. In severe carpal rheumatoid arthritis, however, all the fingers show a clear ulnar deviation.
It is worth remembering that rheumatoid arthritis of the wrist tends to spare the radial side of the joint. In fact, a severe arthritis of the wrist that spares the ulnar side and vigorously attacks the radial side is likely to be osteoarthritis (degenerative joint disease). Also remember that classic rheumatoid arthritis never attacks the distal interphalangeal (DIP) joints.
Other manifestations of rheumatoid arthritis include (a) loss of grip strength; (b) limited flexion in one or more fingers due to nodular tenosynovitis (felt as a small movable nodule in the flexor tendon as you passively flex and extend the joint); (c) vasculitic lesions, which may herald serious systemic vasculitis (Weiss, 1984
); and (d) Haygarth nodes (actually not nodes but fusiform synovial swellings of the proximal interphalangeal [PIP] joints).
Dr Gerry Rodnan of Pennsylvania made the diagnosis of rheumatoid arthritis by squeezing the metacarpal heads during a handshake (Rodnan et al., 1973
). This can be quite painful for the patient. If you suspect this diagnosis, it is better not to shake hands but simply to ask what would happen if you were to squeeze the patient’s hand.
The American College of Rheumatology criteria for the diagnosis of rheumatoid arthritis are given in Table 24.2
. Although the criteria focus on joint manifestations, remember that rheumatoid arthritis is a systemic disease (see Chapter 10
for eye findings).
Generalized symptoms such as fatigue, weight loss, malaise, and depression are also common (Lee and Weinblatt, 2001
TABLE 24.1 Diseases associated with subcutaneous nodules similar to those found in rheumatoid arthritisa
Rheumatic and immunologic diseases
Systemic lupus erythematosus
Discoid lupus erythematosus
Anarthritic rheumatoid syndrome
Degenerative joint disease (Heberden nodes)
Agammaglobulinemia with polyarthritis
Erythema elevatum diutinum
Acrodermatitis chronic atrophicans
Discoid lupus erythematosus
Basal cell carcinoma
Tuberous xanthomatosis (see Chapter 7)
Basal cell carcinoma
Leprosy (Hansen disease)
Ganglions of the hand or wrist
Foreign body reactions
Delayed local reaction to tuberculin
aConditions in bold face have fooled the clinician most consistently. From Kaye BR, Kaye RL, Bobrove A. Rheumatoid nodules: Review of the spectrum of associated conditions and proposal of a new classification, with a report of four seronegative cases. Am J Med. 1984;76:279-292, with permission.
Although the patient’s and the doctor’s attention may be focused on the peripheral joints, the cervical spine is affected early in the course of rheumatoid arthritis, even within the first two years (see Chapter 25
). This is for some reason strongly correlated with involvement of the carpal and metacarpophalangeal (MCP) joints (K. Smith, personal communication
, 2009) and with erosions in the bones of the hands and feet (Winfield et al., 1983
). Wasting of the forearm and hand muscles may result from compression of the anterior spinal artery at upper and mid cervical levels (Mathews, 1998
TABLE 24.2 Diagnostic criteria for rheumatoid arthritis
In/around joints, lasting at least 1 hour before maximal improvement
Arthritis of three or more joint areas
At least three areas simultaneously have had soft tissue swelling or fluid; the 14 possible areas are R/L PIP, MCP, wrist, elbow, knee, ankle, and MTP joints
Arthritis of the hand joints
At least one area swollen in the wrist, MCP, or PIP joint
Bilateral PIP, MCP, MTP without absolute symmetry acceptable
Subcutaneous nodules over bony prominences, extensor surfaces, or in juxta-articular regions
Serum rheumatoid factor
By any method in which result has been positive in <5% of normal control subjects
PA hand/wrists X-ray with erosions or bony decalcification in or adjacent to involved joints
Four or more criteria needed to make diagnosis; must have 1-4 for at least 6 weeks.
PIP, proximal interphalangeal; MCP, metacarpophalangeal.
Source: From Mayeaux EJ Jr. 2000 Revised ARA Criteria for Classification of Rheumatoid Arthritis. Intern-in-the-middle-of-the-night series. Shreveport, LA: Louisiana State University Health Sciences Center. Available at: lib-sh.lsumc.edu/fammed/intern/ra.html. Accessed: June 18, 2004, with permission.
This “degenerative,” seronegative arthritis produces characteristic nodules of the DIP joints called Heberden nodes. These feel like two little dried split peas placed subcutaneously on the lateral and medial dorsal surface of the finger at the DIP. When they are present on the PIP joint, they are called Bouchard nodes. Osteoarthritis tends to spare the MCP joints.
The American College of Rheumatology criteria for the diagnosis of progressive systemic sclerosis require the presence of
proximal scleroderma (skin tightness) or the presence of two of three other criteria. These criteria are
Proximal is defined as above the wrists by the American College of Rheumatology, and above the elbows by various others. The criteria were designed for research and thus exclude individuals in whom the disease is not fully expressed. Scleroderma skin changes above the MCP or metatarsophalangeal (MTP) joints are 91% sensitive and 99.8% specific for definite scleroderma (Wigley, 2001
Nail-fold capillary changes are described in Chapter 7
Patients with sclerodactyly alone have some or all of the features of the CREST syndrome, and it has been argued that this syndrome should be called “limited scleroderma.” The CREST syndrome includes calcinosis, the Raynaud phenomenon (see Chapter 18
), esophageal dysmotility, sclerodactyly, and telangiectasia.
TABLE 24.3 Diagnostic criteria for systemic lupus erythematosus
Malar rash (see Fig. 9-9)
Fixed malar erythema, flat or raised, tending to spare the nasolabial folds
Discoid rash (see Fig. 7-10)
Erythematous raised patches with adherent keratotic scaling and follicular plugging
Skin rash as a result of unusual sensitivity to sunlight
Oral or nasopharyngeal ulceration, usually painless
Nonerosive arthritis involving peripheral joints, with tenderness, swelling, or effusion
Pleuritis—convincing history of pleuritic chest pain or rub or evidence of pleural effusion OR pericarditis—documented by ECG, rub, or evidence of pericardial effusion
Persistent proteinuria, >0.5 g/day or >3+ if quantification is not performed OR cellular casts—may be red cell, hemoglobin, granular, tubular, or mixed
Seizures OR psychosis in the absence of offending drugs or known metabolic derangements (uremia, ketoacidosis, or electrolyte imbalance)
Hemolytic anemia—with reticulocytosis OR leukopenia: <4,000 total at least twice OR lymphopenia: <1,500 at least twice times OR thrombocytopenia: <100,000 in absence of offending drugs
Positive antiphospholipid antibody OR anti-DNA OR anti-SM OR false-positive serologic test for syphilis
Positive antinuclear antibody (ANA), at any point in time, and in the absence of known drugs to be associated with “drug-induced lupus” syndrome (see Table 24.4)
Diagnosis requires four or more manifestations present serially or simultaneously. Remember that these are simply “criteria.” Always use your clinical judgment in evaluating a patient for potential lupus.
From Gill JM, Quisel AM, Rocca PV, et al. Diagnosis of systemic lupus erythematosus. Am Fam Physician. 2003;68: 2179-2186 and Callegari PE, Williams WV. Laboratory tests for rheumatic diseases: When are they useful? Postgrad Med. 1995;97(4):65-74, with permission.
New criteria have been proposed for early diagnosis and classification on the basis of advances such as the identification of scleroderma (anticentromere) autoantibodies and other advances (LeRoy and Medsger, 2001
). Disease manifestations are variable and complex. The use of a term such as “undifferentiated connective disease with features of scleroderma” has been suggested as a hedge against overdiagnosis and overly aggressive treatment (Wigley, 2001
Systemic Lupus Erythematosus
Systemic lupus erythematosus (SLE) is a multisystem disease diagnosed by a combination of clinical and laboratory criteria. The American College of Rheumatology diagnostic criteria are given in Table 24.3
. Always remember that diseases do not necessarily fit precisely into authoritatively defined pigeonholes. In particular, there are many more neurologic manifestations, both central and peripheral, that have been attributed to lupus. These include demyelinating disorders, chorea, Guillain-Barré syndrome, myasthenia gravis, and autonomic disorders (Ruiz-Irastorza et al., 2001
TABLE 24.4 Drugs associated with systemic lupus erythematosus
Atenolol, labetalol, timolol
Diclofenac, ibuprofen, sulindac
From Greenberg B, Michalska M. Systemic lupus erythematosus. Postgrad Med. 1999;106:213-223, with permission.
Almost all patients experience arthralgias and myalgias, and most develop intermittent arthritis. Pain is often out of proportion to physical findings. Characteristically, there is symmetric fusiform swelling of joints, most frequently the PIP and MCP joints and the wrists and knees. Diffuse puffiness of hands and feet, and tenosynovitis, may be seen. In contrast to rheumatoid arthritis, joint deformities are unusual, with only 10% of patients developing swan-neck deformities of the fingers and ulnar drift at MCP joints. Erosions are rare. Subcutaneous nodules occur.
A careful drug history is mandatory. Drugs that have been associated with SLE are listed in Table 24.4
. The syndrome is rare except with procainamide (the most common offender) and hydralazine, and is probably related to genetically determined drug acetylation rates.
Tophi occur more frequently on the ears and feet, and when present on the hand (Fig. 24-1
), though highly suggestive of gout, could be initially confused with a rheumatoid nodule. Definitive diagnosis is made by joint aspiration and demonstration of urate crystals (see Chapter 28
). Presence of a tophus is 33% sensitive and 93% specific for gout.
Patients with hemochromatosis are prone to a degenerative type of arthritis that can affect the hands and fingers as well as the joints of the lower extremities. The PIP joints of the middle and ring fingers may be affected, mimicking the Haygarth fusiform swelling of rheumatoid arthritis confined to those joints.