David B. Hellmann
Bursitis
General Considerations
Bursitis, the inflammation of a bursal sac, is a common problem. Bursal sacs are structures lined with a synovial membrane that secretes and absorbs liquid. The bursae provide a lubricating mechanism between structures such as bones, ligaments, tendons, muscles, and skin. Although they usually are isolated, occasionally they are in communication with a joint space. There are more than 150 such structures in the body, but the number is not fixed. A new bursa may appear whenever there is friction between structures.
Bursitis usually is easy to diagnose and treat in the office. Occasionally, clinicians face the challenge of distinguishing bursitis from arthritis or other conditions. Also, it is important to quickly recognize and treat the few patients who have septic bursitis. When the diagnosis remains uncertain or the usual treatments are unsuccessful, referral to an orthopedist or rheumatologist may be necessary.
Causes
The most common cause of bursitis is minor trauma, as may occur to the subdeltoid bursa from repetitively throwing a baseball or to the prepatellar bursa from prolonged kneeling on a concrete floor. Because the development of bursitis is a function of both physical stresses and the condition of the bursa and surrounding tissues, bursitis is rare before age 20 years; it is particularly common in middle-aged and older people. Less often, bursitis is caused by systemic disorders such as rheumatoid arthritis, polymyalgia rheumatica, or gout. Septic bursitis after trauma is of special concern in patients with superficial bursitis (e.g., olecranon or prepatellar bursitis). Basic calcium phosphate crystals are associated with calcific periarthritis, tendinitis, and bursitis, but little is understood about the exact mechanism of calcium crystal deposition.
Manifestations
Patients with acute bursitis experience abrupt onset of localized pain that is exquisitely tender and usually is aggravated by any movement of the structures adjacent to the bursae. The pain usually is described as a deep aching discomfort. How well the patient can localize the discomfort depends on whether the bursa is superficial or deep to the skin and whether the affected region has extensive or exiguous sensory nerve fibers. Thus, when asked “Where does it hurt?,” the patient with anserine bursitis often uses a fingertip to pinpoint the location, whereas a patient with trochanteric bursitis usually waves the entire hand over the general region of pain.
Bursitis near the knee, hip, elbow, or shoulder can mimic arthritis of those structures. Features that suggest bursitis include sudden onset after some repetitive physical activity; swelling, redness, or tenderness localized to the bursa and not the joint (see below); absence of pain on passive motion of the joint; and, if necessary, a normal joint x-ray film or magnetic resonance imaging.
Infection is suggested by fluctuant swelling of the bursa in association with redness and heat of the overlying skin (1). Location is an important consideration because the vast majority of bursal infections involve the olecranon and prepatellar bursae. Some comorbid conditions, notably diabetes, alcoholism, intravenous drug use, and immunosuppression, increase the likelihood of septic bursitis. Fever should always suggest infection, but it also can complicate gouty bursitis. The absence of fever does not exclude infection; only 40% of patients with septic bursitis demonstrate fever (1). A large bursal swelling can compress other structures or even rupture, thus explaining why bursitis around the knee or the hip can present as diffuse leg swelling (mimicking thrombophlebitis), an inguinal hernia, or a pelvic mass (2).
P.1214
Aspiration of Bursae
Aspiration usually is easily accomplished by the generalist, especially when the bursa is superficial (Table 74.1). The chief indication for aspirating a bursa is to rule out infection. Analysis of bursa (or joint) fluid can also reveal whether the fluid is inflamed or contains crystals (usually from gout).
|
TABLE 74.1 Technique for Aspiration of Superficial Bursae (or Joints) and Analysis of Bursal (or Synovial) Fluid |
|
|
The laboratory tests performed on bursal (or joint) fluid depend on the clinical questions (Table 74.2). A bursal or joint fluid white blood cell count >1,000 indicates inflammation; <1,000 white blood cells is characteristic of traumatic fluid (Table 74.2). Bursal infection tends to give very high white blood cell counts, averaging approximately 75,000 for olecranon bursitis (1). Rheumatoid arthritis and gout also occasionally produce markedly elevated bursal fluid white blood cell counts. Gram stains and cultures are the specific tests for infection. Similarly, examining the fluid with a microscope equipped with polarizing lenses is the only way to identify the crystals that are specific for gout (seeChapter 76). Therefore, most bursal fluid can be characterized by the white blood cell count, Gram stain plus culture, and analysis for crystals. Glucose, protein, mucin clot, and other (e.g., enzymes, complement) determinations are not specific and are not needed.
|
TABLE 74.2 Patterns of Bursal (or Joint) Fluid Findings in Common Problems |
||||||||||||||||||||||||||||||||||||
|
||||||||||||||||||||||||||||||||||||
Treatment
Treatment of septic bursitis requires administering antibiotics and draining the bursal sac. Gram stain of the bursal fluid (see below) should guide initial antibiotic choice. More than 75% of cases of septic bursitis are caused by Staphylococcus aureus, but Gram stain is positive in only 65%. If the Gram stain shows no bacteria or if gram-positive cocci are found, a penicillinase-resistant antistaphylococcal drug should be used. Vancomycin should be used initially if the patient is acutely ill or has recently been in a facility or lives in a region where methicillin resistant S. aureus is prevalent. If gram-negative organisms are found, blood cultures should be obtained and an extrabursal site of infection sought. The antibiotic choice should be based on the organism most likely causing the extrabursal infection. The need for hospitalization depends on both the severity of the infection and the degree of host compromise. Patients with high fever and chills, intense surrounding cellulitis, deep bursal involvement, extrabursal infection, or suspicion of an uncommon organism should be hospitalized for administration of parenteral antibiotics. Hospitalization also should be considered for patients who are compromised by age, alcoholism, diabetes mellitus, or immunosuppression, almost regardless of the intensity of bursal inflammation. Patients who are less ill and less frail can be treated with oral antibiotics on an outpatient basis. Repeat bursal aspiration also is required until the fluid stops accumulating. For example, successful treatment of septic olecranon bursitis requires an average of two to three aspirations over 1 or 2 weeks. The timing of these repeat aspirations is based solely on the clinical response (or absence thereof) of the patient. The duration of antibiotic therapy averages 2 to 4 weeks but should be individualized according to the clinical response and the character of aspirated fluid. In cases requiring repeated bursal aspiration, antibiotics should be continued for at least 5 additional days after the bursal fluid has become sterile or for a total of 2 weeks (whichever is longer).
Gout, pseudogout, orrheumatoid arthritis should be treated with anti-inflammatory agents (see Chapters 76 and 77). Usually traumatic bursitis resolves spontaneously
P.1215
if the area of inflammation is rested. However, spontaneous resolution requires several weeks, and therapy shortens this period considerably. Therefore, if sepsis and gout are ruled out, the treatment outlined in Table 74.3 is indicated.
|
TABLE 74.3 Treatment of Bursitis |
|
|
First-line therapy for nonseptic bursitis can be either a nonsteroidal anti-inflammatory agent (see Chapter 77) or an injection into the bursa of a mixture of lidocaine and depoglucocorticoid (Table 74.4). Injection usually is the better initial choice when the patient has severe pain (which often intensifies at night) and when the clinician has experience with soft-tissue injections. Cellulitis of skin overlying the bursa contraindicates bursal injection. Systemic anticoagulation with warfarin or heparin is a relative contraindication to bursal injection. Immediate and dramatic but transient relief of pain secondary to lidocaine indicates that the proper site has been injected. The anti-inflammatory effect of the steroid injection is seen in approximately 72 hours. If the response is not satisfactory, the bursa can be reinjected in approximately 2 weeks. Waiting 2 weeks before reinjection provides ample time to rule out iatrogenic sepsis, which occurs rarely after a steroid injection. Depending on the location, other modalities such as ultrasound, heat or cold application, and physical therapy can be used as adjuncts. If a bursitis does not respond to two steroid injections, rheumatologic consultation to rule out associated systemic disease may be necessary. Rarely, definitive treatment by surgical excision of the bursa is necessary.
|
TABLE 74.4 Methods of Injection of Bursae or Joints with Lidocaine or Depoglucocorticoid Preparations |
||
|
Specific Forms
Several forms of bursitis are particularly common, and their unique aspects are described.
Olecranon Bursitis
This common form of bursitis—also called student's or miner's elbow—is characterized by a goose egg–like swelling located just behind the olecranon process of the ulna (see Chapter 69). An effusion of the elbow joint itself, in contrast, causes diffuse swelling. Another feature that distinguishes olecranon bursitis from elbow joint inflammation is the near lack of pain with passive extension and flexion of the elbow when olecranon bursitis is the only problem. Important features of olecranon bursitis are that it often is associated with systemic disease, such as rheumatoid arthritis or gout; symptoms often are chronic, in that they have been present for 2 or 3 weeks before a patient sees a physician; and the olecranon is a common site for septic bursitis after trauma and may be associated with surrounding cellulitis. If rheumatoid arthritis or gout is present, it is important to realize that sepsis may coexist. Traumatic olecranon bursitis usually is hemorrhagic, although xanthochromic fluid may be present.
Swelling in the area of the olecranon bursa should be aspirated if symptomatic (Tables 74.1 and 74.2). Gram stain, culture, white blood cell count, and crystal identification with polarizing microscopy should be obtained.
Therapy
Patients with olecranon bursitis should be taught to avoid leaning or resting on their elbows. More specific therapy depends on the characteristics of the fluid (3). If monosodium urate crystals are present, specific therapy for gout is indicated (see Chapter 76). Traumatic bursitis responds to simple removal of fluid; however, if the fluid reaccumulates, a steroid injection (Table 74.4) should be given. If sepsis is identified, the patient should be treated with an antibiotic and bursal aspiration as outlined above.
Prepatellar Bursitis
Prepatellar bursitis (housemaid's or carpenter's knee) is a common form of bursitis, easily recognized by its location
P.1216
overlying the inferior portion of the patella (see Chapter 72). It is particularly common in carpet layers, plumbers, and carpenters and most often is caused by trauma from kneeling. Because it also may be a site of sepsis, usually the bursa should be aspirated.
Anserine Bursitis
The anserine bursa is fan shaped and lies between the confluence of tendons of the sartorius, gracilis, and semitendinosus muscles and the tibia at the anterior medial aspect of the knee just below the joint space (see Chapter 72). Anserine bursitis is seen most in patients with arthritis, especially overweight middle-aged women with osteoarthritis of the knee. It is recognized by its location; the pain typically is produced when the knee is flexed and is particularly troublesome at night. The patient often seeks comfort by sleeping with a pillow between the thighs.
If there is surrounding erythema or if the patient is febrile, aspiration should be attempted because sepsis, although uncommon, may be present. Therapy depends on the findings (Tables 74.2 and 74.3). When injection therapy is used, the solution should be injected in a fan-shaped pattern so that the entire bursa is treated.
Ischial Bursitis
The ischial bursa is located over the ischial tuberosity, close to the sciatic nerve and the posterior femoral cutaneous nerve. When a person is sitting, the ischial bursae are covered only with subcutaneous tissue and skin. When a person is standing, the gluteus maximus also covers the bursa.
The most common reason for inflammation of this bursa is trauma, as may occur in bicycling. It is rarely a site of sepsis. Usually the inflammation results in an abrupt onset of pain, but occasionally the onset is more insidious. The patient often has exquisite pain when sitting or lying. Because of the close proximity of the sciatic nerve to the bursa, there may be an associated neuritis resulting from pressure on the nerve, which causes sciatic pain to radiate into the leg (see Chapter 71). Direct pressure over the ischial tuberosity causes sharp pain, and the patient may hold the painful buttock elevated when sitting. In addition, the pain is intensified when the patient is supine and the hip is passively flexed. The patient has difficulty standing on tiptoe on the affected side.
The differential diagnosis of symptoms suggestive of ischial bursitis includes lumbar spine disease, thrombophlebitis, and inflammatory back disease or sacroiliitis (see Chapter 78). Localization of the pain over the ischial tuberosity and the finding of induration near the ischial tuberosity on rectal examination establish the diagnosis of ischial bursitis.
Aspiration of the bursa, even when it is inflamed, is discouraged because it often is difficult to localize, and the surrounding structures, especially the sciatic nerve, may be injured. If aspiration is indicated because there is associated fever and, therefore, the possibility of septic bursitis, the patient should be referred to an orthopedist for immediate evaluation.
The patient may obtain some comfort when sitting by using a pillow that allows pressure to be eliminated from the ischial tuberosity underlying the inflamed bursa. Standard therapy (Table 74.3) with a nonsteroidal anti-inflammatory agent usually provides dramatic improvement within 2 to 3 days; however, if there has been associated leg pain or weakness from sciatic nerve inflammation, those symptoms may persist for several months. Ultrasound therapy, administered by a physical therapist, may be effective and should be considered if initial therapy has not relieved the symptoms within several days.
If the diagnosis is unclear, there is a question of sepsis, and if the patient does not respond to therapy within 1 week, consultation with a rheumatologist or orthopedist is recommended. If the diagnosis is confirmed, the consultant may aspirate the bursa and, if sepsis is ruled out, inject it with lidocaine and depo glucocorticoid, which often results in dramatic improvement.
Semimembranosus Gastrocnemius Bursitis (Baker Cyst)
The semimembranosus gastrocnemius bursa, commonly called a cyst, lies in the posterior medial aspect of the knee behind the femoral condyle and in 50% of patients is continuous with the knee joint (see Chapter 72). The cyst is best seen and palpated when the patient is standing. Swelling of this bursa is commonly associated with other knee problems, such as internal derangements, rheumatoid arthritis, or degenerative arthritis. The bursa is rarely infected. Often, a Baker cyst is asymptomatic. The patient with a large cyst may note a vague discomfort or fullness behind the knee. Many Baker cysts produce symptoms only when they rupture, causing acute swelling, pain, and redness of the calf and lower leg (syndrome of pseudothrombophlebitis). The possibility that a patient with acute calf swelling has pseudothrombophlebitis from a ruptured Baker cyst is strongly suggested by finding swelling of the knee joint. When in doubt, a study to exclude thrombophlebitis should be done (see Chapter 57). Sonography, magnetic resonance imaging, and arthrography can visualize a ruptured cyst but usually are not needed if the clinical picture is consistent and thrombophlebitis has been excluded. Rarely, an unruptured Baker cyst can compress deep veins and cause thrombophlebitis.
The differential diagnosis of a posterior knee fullness includes an aneurysm of the popliteal artery. Therefore, it is important to palpate any fullness for pulsations.
P.1217
Management of an uncomplicated Baker cyst includes aspiration and instillation of a corticosteroid–anesthetic mixture. Usually, this can be accomplished by aspirating and injecting the joint space. Rarely, the cyst must be aspirated or injected from the posterior approach. Because of the important structures in the popliteal fossa (artery, nerve, and vein), aspiration of the bursa posteriorly should be done by an orthopedist. Weight-bearing should be minimized for several days. The response to this therapy usually is excellent. Management of a ruptured cyst consists of bed rest, heat, and elevation. Although no data confirm this recommendation, an elastic bandage around the knee when treating a Baker cyst is not recommended because it may cause the cyst to compress more severely the venous system, increasing the chance of a thrombophlebitis. Instillation of corticosteroids into the joint that has an effusion may be helpful. Nonsteroidal anti-inflammatory agents may help; after symptoms have started to abate, ambulation can be increased slowly.
Iliopectineal Bursitis
The iliopectineal bursa lies anterior to the hip joint, with which it communicates in approximately 15% of people. It lies between the inguinal ligament and the iliopsoas muscle just lateral to the femoral artery. It may be inflamed from running or other similar trauma. Pain in the anterior pelvis, groin, and thigh is the most common manifestation of iliopectineal bursitis; swelling may result in a bulge resembling a femoral hernia (see Chapter 97) below the inguinal ligament. Bursitis may be present in conjunction with intrinsic inflammatory joint disease such as rheumatoid arthritis. Extension of the hip (e.g., during walking) intensifies the pain, so the patient often limits the stride of the affected side. The anterior crural nerve (the largest branch of the lumbar plexus) lies just below the bursa and may be irritated from bursal inflammation. The resulting neuritis causes pain in the thigh, which often is also intensified by walking. Weakness of anterior muscles of the thigh also may be present. When a bursa is enlarged, it may compress the femoral vein, resulting in edema in the affected leg.
If a hernia can be ruled out (see Chapter 97), the bursa should be aspirated by an orthopedist. Aspiration and injection with lidocaine and usually a corticosteroid result in lasting improvement.
Trochanteric Bursitis
The trochanteric bursa lies in the lateral aspect of the thigh over the greater trochanter of the femur and is closely associated with tendons of the glutei muscles (4). The problem primarily affects older people. Most cases are of unknown cause, although many are thought to result from osteoarthritis. Infection of the bursa is rare. The onset of pain may be abrupt, subacute, or chronic. Patients with trochanteric bursitis often misinterpret their lateral buttock pain to be caused by hip arthritis (which more characteristically produces groin pain). At times, the discomfort mimics that of lumbar spine disease (see Chapter 71). Occasionally, trochanteric bursitis causes pain that radiates to the knee or even to the groin. Discomfort is intensified by movement from the sitting to the standing position, going up and down stairs, or sleeping on the affected side.
On examination, point tenderness over the bursa with reproduction of the pain is observed. The Patrick test (external rotation of the hip combined with abduction) often is painful, whereas internal rotation, flexion, and extension of the hip usually are pain free. Any remaining concern about the knee or lumbar spine causing the pain is eliminated at examination of these structures. X-ray films of the bursa, which sometimes reveal calcifications, usually are not necessary.
Therapy, as outlined in Table 74.3, usually is effective. In addition, the patient should sleep with a small pillow under the involved buttock to keep weight shifted off the bursa.
Subdeltoid Bursitis
Chapter 69 discusses subdeltoid bursitis.
Tenosynovitis
As with bursae, there are many sites of potential tenosynovial inflammation. Tendinitis and tenosynovitis generally occur simultaneously. The synovial-lined tendon sheath usually is the site of maximal inflammation.
Inflammation of a sheath of a tendon is a common problem. For the most part, only long tendons have sheaths. Tenosynovitis most often occurs from exercise, especially when a tendon has been used repetitively in an improperly conditioned person (see Chapter 72). Tenosynovitis also may be part of a generalized inflammatory process. Sometimes tenosynovitis is the first manifestation of this process.
Tenosynovitis often affects the dorsal extensor tendons of the wrist. It manifests most commonly as pain that is intensified with hand extension. In the acute stage, swelling and pain over the dorsal aspect of the wrist or over the dorsal radioulnar joint may occur. Occasionally, a friction rub is felt or heard when the appropriate muscle is contracted.
When tenosynovitis is identified in the absence of trauma, a systemic disease should be suspected and, if present, specific therapy administered. Gonorrhea should be suspected in sexually active people if inflammation involves the tendons of the ankle or wrist in the setting of a monarthritis, fever, and skin rash. Using Transgrow media,
P.1218
culture of the endocervical canal and rectum in women and of the urethra in men is indicated (see Chapter 37).
If the tenosynovitis has developed because of trauma, such as exercise or overuse, or for unknown reasons, nonspecific therapy with a nonsteroidal anti-inflammatory agent, as for bursitis (Table 74.3), is appropriate. The fingers should be splinted in the position of function. If symptoms persist after 3 or 4 days of conservative therapy, the peritenon (loose tissue surrounding the tendon) should be injected with lidocaine and a corticosteroid (e.g., Celestone, Aristocort, or Kenalog) (Table 74.4). To avoid injuring the tendon or causing skin atrophy, only small doses of depo glucocorticoid (i.e., approximately 0.5 mL of steroid mixed with an equal amount of lidocaine depending on the site) should be injected. To avoid injecting directly into the tendon, one should never inject if the syringe plunger cannot be depressed easily. Occasionally, symptoms are recurrent, in which case referral to a rheumatologist or orthopedist is indicated. Chapters 68, 69, 72 and73 discuss tenosynovitis and tendinitis involving specific tendons.
Stenosing Tenosynovitis
Stenosing tenosynovitis is not a complication of tenosynovitis; rather, it occurs primarily when trauma is severe and localized. In stenosing tenosynovitis, either a nodule forms on a tendon or an actual stenosis of a tendon sheath of a long tendon develops. As a result, the affected part sticks in a fixed position that sometimes is painful. When this condition affects the flexor tendons of the fingers, it is calledtrigger finger. The patient is unable to flex a digit fully or, once it is flexed, the digit locks and literally must be straightened by external force until it suddenly snaps free. When stenosing tenosynovitis is present in the abductor or extensor tendon of the thumb, it is called de Quervain disease, the most common form of stenosing tenosynovitis. In this problem, the thumb usually still has motion and does not always lock like a trigger finger. To evaluate for de Quervain tenosynovitis, the patient should make a fist by curling the fingers over the flexed thumb, then passively deviate the fist in an ulnar direction. Exquisite pain over the base of the thumb with this maneuver (Finkelstein test) confirms the diagnosis. Stenosing tenosynovitis usually is caused by repeated trauma (e.g., prolonged use of a screwdriver) but occasionally is seen in association with rheumatoid arthritis, amyloidosis, pregnancy, and myxedema.
The treatment is identical to that of bursitis as outlined in Tables 74.3 and 74.4. Splinting is especially helpful in the treatment of stenosing tenosynovitis of the hands and fingers. If the patient does not respond to several weeks of conservative therapy, surgical release may be necessary. When a nodule is palpable, it should be injected with a small amount of corticosteroid (e.g., 10 mg Celestone, Aristocort, or Kenalog) and lidocaine. Often, the condition resolves in several days to weeks.
Dupuytren Contracture
The palmar fascia may undergo nodular hypertrophic fibroplasia of unknown cause. This results over many years in the development of a flexion contracture (Dupuytren contracture). The process causes the skin to be fixed to the underlying fascia by adhesive bands, resulting in a fixed puckered appearance. A Dupuytren contracture almost always is painless but may result in significant functional disability. Although all digits may be involved, the fourth and fifth digits are affected most commonly. The condition affects primarily middle-aged or older men and is bilateral in nearly 40%. It is more common in epileptics and alcoholics, but most affected patients have neither condition. Once the condition is present, passive extension of the fingers does not retard the process and, in fact, may accelerate it. If functional disability is present, the patient should be referred to an orthopedic surgeon for consideration for surgery. Oral anti-inflammatory agents and local cortisone injections are not effective in retarding the process.
Ganglions
Ganglions, cystic swellings arising from the synovium of a joint or tendon sheath, are the most common benign tumors of the hand. They tend to occur more often in women from the teens through age 50 years. Onset may be sudden or gradual, and the cyst may change over time, sometimes disappearing and then recurring. The swellings usually are smooth, tense, and fixed to the deep tissues. The most common site is the dorsum of the wrist between the extensor tendon of the thumb and the extensor tendon of the index finger. They also may occur on the volar aspect of the foot (the tarsal area) and ankle. Aching or weakness of the involved area may be associated. Nonoperative treatment in symptomatic patients (generally asymptomatic ganglions are left untreated), consisting of aspiration or cortisone injection, may be successful. Recurrences may require treatment by operative excision.
Fibromyalgia
Fibromyalgia (also called fibrositis) is a syndrome of widespread chronic musculoskeletal pain, chiefly affecting young women, that is unaccompanied by any objective findings except for increased tenderness at specific anatomic sites, known as tender points (5). The diagnosis
P.1219
is purely clinical; no laboratory tests establish the diagnosis of fibromyalgia. The cause of fibromyalgia is unknown, but speculations center on disturbances in rapid eye movement sleep, chronic viral infections, psychological disorders, neuroendocrine abnormalities, and abnormalities of muscle metabolism (5). Usually fibromyalgia is primary, occurring in the absence of other medical conditions. Some patients have secondary fibromyalgia, which is diagnosed when the syndrome accompanies another disorder, most commonly hypothyroidism, rheumatoid arthritis, hepatitis C, or Lyme disease (5).
Fibromyalgia is important to recognize for three reasons. First, fibromyalgia is common, affecting 3% to 10% of the general population and accounting for 20% to 30% of all patients referred to rheumatologists. Second, fibromyalgia responds, albeit imperfectly, to treatment. Third, failure to diagnose and treat fibromyalgia often subjects the patient to multiple consultations and needlessly expensive laboratory testing.
Manifestations
Ninety percent of patients with fibromyalgia are women. Most experience the gradual onset of symptoms between the ages of 20 and 45 years. The cardinal features of fibromyalgia are widespread soft-tissue aching and stiffness that occur essentially daily. The symptoms vary in severity, often involving the axial skeleton, shoulder, and pelvic girdles, with duration of more than 3 months. Typically the pain is chronic, persisting for years. The condition may be aggravated by fatigue, tension, excessive work, immobilization, and weather changes. Although pain is the predominant symptom, most patients with fibromyalgia have many other symptoms, including fatigue, recurrent headaches, sore throat, depression, fitful sleep, difficulty concentrating, alternating constipation and diarrhea, numbness, swollen glands, and subjective sense of fever or joint swelling. In fact, a careful history to rule out depression (see Chapter 24) and somatization (seeChapter 21) is important.
Characteristically in fibromyalgia, the rich history of complaints contrasts starkly with the poverty of physical findings. Objective adenopathy, weakness, and joint inflammation or weakness are notably absent. Indeed, the only physical finding in fibromyalgia is tender points. Although the exact number and location of these tender points are somewhat controversial, the nine paired tender points most commonly found, along with four control points, are illustrated in Fig. 74.1. Nearly 90% of patients with fibromyalgia have tenderness of at least 11 of the 18 (nine paired) tender point sites (5,6).
|
FIGURE 74.1. The tender point locations in fibromyalgia are remarkably constant from patient to patient. Multiple locations have been described; the nine paired tender points shown represent frequently occurring points in a wide distribution. Most patients with fibrositis usually have 11 or more tender points. Control points are not unduly tender; their examination should be interspersed with that of the tender points. (Modified from Bennett RM. The fibromyalgia syndrome. In: Kelly WN, Harris ED, Ruddy S, et al., eds. Textbook of rheumatology. 5th ed. Philadelphia: WB Saunders, 1997, 2001:513 , with permission.) |
Laboratory tests in primary fibromyalgia are normal. The purpose of the laboratory tests and physical examination is to exclude other causes of diffuse musculoskeletal pain, which include polymyalgia rheumatica (a condition of elderly people), Parkinson syndrome, polymyositis, endocrine disorders (especially hypothyroidism, but also hyperthyroidism, Addison disease, hyperparathyroidism, and panhypopituitarism), cancer, renal tubular acidosis, and chronic fatigue syndrome (considered by many experts to be a variant of fibromyalgia). Fibromyalgia appears to be slightly more common in patients with hepatitis C and, infrequently, may be triggered by Lyme disease (5). Laboratory testing need not be extensive. Basic laboratory tests should include complete blood cell count, erythrocyte sedimentation rate, thyroid function tests, and
P.1220
muscle enzyme levels. Tests for hepatitis C or Lyme disease should be considered in patients with exposures to those infections. By definition, all these studies are normal in patients with primary fibromyalgia, so any abnormalities should warrant a further search for an underlying disorder.
Table 74.5 summarizes the approach to diagnosis of fibromyalgia. The two criteria for diagnosis—widespread pain and mild or greater tenderness in 11 or more of 18 tender point sites—are 88% sensitive and 81% specific. The diagnosis of fibromyalgia should not be made too quickly or too slowly. Because the diagnosis depends on excluding objective abnormalities, rarely is it wise to diagnose primary fibromyalgia on the patient's first visit. Documenting the absence of fever, weight loss, or any other abnormality takes time and increases the certainty of the diagnosis. Because fibromyalgia rarely begins after age 50 years, great caution should be exercised when considering the diagnosis in older patients. On the other hand, there is no benefit to delaying the diagnosis in a typical host with a compatible clinical picture. Indeed, such delays often prompt unnecessary consultations and redundant laboratory investigations.
|
TABLE 74.5 Initial Examination of a Patient with Possible Fibromyalgia Syndrome |
||||||||||||
|
||||||||||||
Treatment
Once the diagnosis of fibromyalgia is confirmed and comorbid conditions (e.g., sleep disorders, depression, or hypothyroidism) have been excluded, the first step and cornerstone of treatment is education. Explaining to the patient that a distinct recognizable syndrome is present significantly reduces the frustrations that may have built up over months, and sometimes years, of previously unproductive medical evaluations. Patients also take solace in learning that fibromyalgia does not shorten life or cause crippling deformities and that the cause and treatment of fibromyalgia are under active investigation.
Step 2 of treatment is to consider initiating both nonpharmacologic and pharmacologic treatment. Although no drug has been specifically approved by the U.S. Food and Drug administration for managing fibromyalgia, controlled trials have demonstrated that several treatments can modestly improve symptoms (5). For example, the tricyclic antidepressants, such as amitriptyline (e.g., Elavil, Endep, or generic, 10–50 mg at bedtime), and cyclobenzaprine compounds, which are structurally related to the tricyclic antidepressants (e.g., Flexeril or generic, 5–30 mg at bedtime), can improve fibromyalgia symptoms (5,7). Fluoxetine (Prozac), a selective serotonin reuptake inhibitor, also has been demonstrated to be effective at 20 mg/day (5,8). Nonsteroidal anti-inflammatory medications are generally not effective. Corticosteroids and narcotics have no role in the treatment of fibromyalgia. Because of the modest efficacy of pharmacologic therapy, treatment often may include other modalities as well, such as cognitive behavioral therapy and an exercise program to maintain a good general level of aerobic fitness (5,9,10). Attempts should be made to modify other aggravating factors, such as mechanical, physical, and psychologic stresses. Should steps 1 and 2 be ineffective, then consideration should be given to referring the patient to specialists (e.g., rheumatologists, psychiatrists, or psychologists) (5).
Myofascial Pain Syndromes
Related problems, possibly distinct from fibromyalgia, are the myofascial pain syndromes (MPS), which are characterized by the presence of deep tender points. However, in MPS, the tender point is termed a trigger point because firm palpation of the point produces pain in a referred distribution. A second feature distinguishing MPS from fibromyalgia is the presence of just one or a regional clustering of points in MPS, in contrast to the widespread distribution
P.1221
of symptoms and tender points in any given patient with fibromyalgia. A wide array of clinical syndromes in many different anatomic areas have been ascribed to MPS.
The pathogenesis of MPS is unknown. Patients with MPS may be helped by passive stretching of involved muscles after injection of the trigger point with a local anesthetic or use of a vapocoolant spray (e.g., ethyl chloride). Attention should be directed to elimination of any possible aggravating factors, such as overuse or repetitive injury to involved muscle areas.
Raynaud Phenomenon
Definition
Raynaud phenomenon is a syndrome characterized by episodic vasospasm of the digital vessels in response to cold or emotional stress. Classically, a triphasic response occurs. First cutaneous pallor extends from the fingertips to the middle of the fingers, followed rapidly by mottling of the skin and cyanosis as venous blood refluxes back into an empty cutaneous capillary bed (this pallor or cyanosis persists until rewarming of the digits). Finally, the recovery phase occurs over 15 to 20 minutes, resulting in intense hyperemia (11,12). Vasospasm usually is triggered by an abrupt change in ambient temperature, so it may occur even in the summer when a patient moves into air-conditioned or refrigerated areas. Attacks may begin in one finger before spreading to other digits in both hands. The feet and other acral parts (e.g., the ears or nose) may be involved. Patients often do not describe spontaneously the classic phases of Raynaud phenomenon but commonly note deep cyanosis associated with numbness, a pins-and-needles sensation, or frank pain on cold exposure.
Causes and Prevalence
Raynaud phenomenon most commonly occurs in an idiopathic or primary form, called Raynaud disease, in which no underlying abnormality can be defined. Raynaud phenomenon also can occur secondary to a defined vascular abnormality or in association with a specific disease process (Table 74.6). Primary Raynaud phenomenon is thought to be common, occurring principally in women aged 20 to 30 years. The true prevalence of Raynaud phenomenon is unknown but is estimated to be 2% to 6% in the general population and up to 20% in the selective population of young women. Patients with primary Raynaud phenomenon are otherwise healthy, generally have infrequent attacks (one to four episodes weekly), and rarely develop local cutaneous complications, such as digital pitting, ulcerations, or loss of hand function. Patients with primary Raynaud phenomenon usually have an uncomplicated course, with gradual decrease in the frequency of episodes over a number of years. Estimates suggest that 8% to 19% of patients believed initially to have the primary form will develop a defined secondary form (usually a connective tissue disease).
|
TABLE 74.6 Classification of Raynaud Phenomenon |
In approximately 40% of patients who present to a general physician with Raynaud phenomenon, the phenomenon is secondary to an underlying condition, the most common being a connective tissue disease (Table 74.6). Raynaud phenomenon occurs in >90% of patients with systemic sclerosis and may be the initial symptom, preceding the other features of the disease by years. Approximately 40% of patients with systemic lupus erythematosus and 10% of patients with rheumatoid arthritis
P.1222
may have associated Raynaud phenomenon. Raynaud phenomenon may be a presenting feature in patients who have a systemic vasculitis. Disturbances of the axillary or cervical neurovascular bundle can lead to Raynaud phenomenon. Patients with neurovascular compression syndromes (cervical rib, scalenus anticus syndrome) and proximal vascular lesions (atherosclerosis) may present with unilateral Raynaud phenomenon. Hematologic abnormalities, such as cryoglobulinemia, paraproteinemia, or cold hemagglutinins, may present as typical Raynaud phenomenon. Ergot-containing drugs, such as ergotamine, and β-blocking agents may be causative or potentiating agents. Occupational injury (vibration white finger syndrome) causing the syndrome occurs in a high proportion of workers who operate vibratory tools (e.g., lumberjacks, shipyard workers, or meat cutters).
Initially, determining whether a patient has a primary or secondary form of Raynaud phenomenon may be difficult. Clues that suggest a secondary form include onset during childhood, male sex, onset in a woman after age 30 years, unilateral Raynaud, Raynaud affecting a single digit, fingertip ulcers, digital gangrene, or symptoms or signs of a systemic disorder.
Evaluation
Patients with Raynaud phenomenon should have a focused history (including a drug review) and physical examination to determine whether their condition is primary or secondary. An extension of the examination, evaluation of the nail bed with an ophthalmoscope (using approximately a 20+ diopter), may reveal small telangiectasias or abnormal cutaneous capillary loops. These small vessel changes may be the earliest findings in an associated underlying connective tissue disease, primarily systemic sclerosis (13). The presence of ulcers on the digits indicates that the Raynaud is very severe and that the patient likely has a secondary form of Raynaud phenomenon. Patients with unilateral Raynaud phenomenon should be carefully evaluated for a local vascular lesion, including bilateral blood pressure determination, auscultation over major vessels to determine the presence or absence of vascular bruits, and assessment of the peripheral pulses. Special testing for possible neurovascular compression syndrome (see Chapter 69) and consideration for chest x-ray film, magnetic resonance angiography, and noninvasive evaluation of the peripheral circulation (Doppler studies or digital plethysmography) are appropriate when only unilateral Raynaud phenomenon is present. Angiography may be necessary in cases where a correctable occlusive vascular lesion is strongly suspected. Carpal tunnel syndrome has been implicated in both unilateral and bilateral Raynaud phenomenon, and nerve conduction studies may be appropriate when the history and physical examination suggest this nerve compression syndrome (see Chapter 92).
If the patient is a woman with onset between the ages of 15 and 30 years who has mild bilateral Raynaud, does not have nail fold capillary changes, has no ulcerations or other ischemic finger tip changes, and exhibits no other manifestations of a connective tissue disease, primary Raynaud is the likely diagnosis (12). For such a patient, laboratory evaluation can be limited to verifying a normal complete blood count, a negative antinuclear antibody test, and ordering renal and liver function tests to screen for collagen disease affecting these organs. If the patient is suspected of having secondary Raynaud, determinations of disease-specific autoantibodies (e.g., anti–double-stranded DNA antibodies for systemic lupus erythematosus, and anti-centromere or anti–SCL-70 antibodies for scleroderma), cryoglobulins, Westergren erythrocyte sedimentation rate, and serum protein electrophoresis are indicated. Referring the patient to a rheumatologist for any additional evaluation is appropriate. Even when a systemic illness is not identified, the patient should be followed expectantly because an underlying illness may emerge several years later.
Treatment
The principal mode of treatment in patients in whom a correctable cause cannot be found is avoiding the cold and staying warm (11). This includes wearing mittens or gloves and avoiding a general chill of the body by wearing a hat and loose-fitting warm clothing in winter months. Smoking aggravates Raynaud phenomenon and should be stopped (see Chapter 27). Drugs that promote vasoconstriction (Table 74.6) should be stopped or avoided. Emotional stress should be assessed and controlled by appropriate measures (see Chapter 20). Patients with mild Raynaud phenomenon often improve with education about the cause and nature of these episodes. Unfortunately, temperature biofeedback therapy is ineffective. Table 74.7 summarizes the nonpharmacologic treatment of patients with Raynaud phenomenon. Most patients, particularly those with primary Raynaud phenomenon, do not need, and should not be treated with, drugs. Rather, pharmacologic treatment should be limited to patients with repeated attacks who limit their daily activities or who have developed digital ulceration or other ischemic changes. There is no evidence that decreasing the number of episodes of Raynaud phenomenon will alter the progressive changes that may occur in patients with scleroderma or another connective tissue disease.
|
TABLE 74.7 Nonpharmacologic Management of Patients with Raynaud Phenomenon |
|
|
A wide variety of vasoactive agents has been used in patients with Raynaud phenomenon, but few agents have proved to be definite benefit. Many of the patients are young women of child-bearing potential; therefore, unproven treatment that may have a teratogenic effect should be avoided.
P.1223
Calcium channel blockers are the cornerstone of drug therapy for Raynaud (11,14). Nifedipine has been most extensively used and its efficacy best documented, but other preparations (including amlodipine, isradipine, nicardipine, nisoldipine, and felodipine) also may be effective (11,15). These agents relax smooth muscle, reduce peripheral vascular resistance, and increase peripheral blood flow. Patients with primary Raynaud respond better than patients with Raynaud phenomenon secondary to a connective tissue disease, especially systemic sclerosis. The major side effects of these agents are secondary to their vasodilatory activity and include hypotension, dizziness, headache, and peripheral edema. Approximately 40% to 50% of patients experience some light-headedness caused by hypotension and flushing on initiation of calcium channel blocker therapy. However, these side effects usually are transient, can be minimized by starting with low doses, and do not require discontinuation of the medication. For this reason, the orthostatic blood pressure should be periodically measured. Calcium channel blockers should never be used during pregnancy or in a patient planning to become pregnant because these agents have been shown to be teratogenic in animal models.
The calcium channel blocker that has been most extensively studied, and the drug of first choice, is nifedipine. The initial dosage usually is 30 mg of a sustained-released preparation once daily. The patient should be encouraged to resume usual activities and to keep a diary of the number and intensity of Raynaud attacks. The patient should be monitored for important orthostatic hypotension (symptomatic decrease in systolic pressure ≥20 mm Hg below baseline or a fall <90 mm Hg). If needed to improve control of Raynaud phenomenon, the dosage of nifedipine can be increased by 30 mg sustained-release preparation every 2 weeks to a maximum of 90 mg sustained-release preparation daily. Thereafter, monitoring every 2 to 4 months is important because the initial response may be transient, and side effects, such as esophageal reflux, may limit the usefulness of the drug. If the patient does not respond to nifedipine or the drug is not tolerated, amlodipine beginning at 5 mg/day can be initiated (11). Patients generally have resolution or a dramatic reduction in the intensity and number of episodes of Raynaud phenomenon in the summer. For this reason, medication should be discontinued unless repeated cold exposure or active Raynaud phenomenon is documented.
Other drugs have been used for treatment of Raynaud phenomenon. Intravenous iloprost has been shown to be moderately effective for severe Raynaud in patients with scleroderma, but this agent is no longer available in the United States (11,16). Orally administered prostaglandins have not been shown to be effective (14). Losartan, an angiotensin II type 1 receptor antagonist, has shown promise in treating primary and secondary forms of Raynaud phenomenon (17). Topical nitroglycerin paste applied to the digits or nitroglycerin patches have been used with some success, but their indications are not established, and consultation with a rheumatologist is suggested before such agents are prescribed. Prazosin (Minipress) occasionally is of benefit if it is tolerated (2–8 mg/day in two or three divided doses) (18). Nutritional antioxidants have not been shown to be effective.
Surgical sympathectomy once was a popular treatment of Raynaud phenomenon but now is rarely performed, primarily because of the high relapse rate (40%–50%) and frequency of significant postural hypotension. Selective digital sympathectomy may be performed in centers where microsurgery is available; however, long-term controlled studies of this procedure are lacking. Sympathectomy should be considered only for short-term relief from an intractable course complicated by digital ulceration that has failed medical treatment. All patients who have had such a severe course or have ulcers on their digits should be seen by a vascular surgeon or rheumatologist. Local digital block performed by a vascular surgeon or rheumatologist has been used for temporary treatment of significant digital tissue compromise. A good response may predict which patient may have a good effect from digital or cervical sympathectomy. A patient who has severe disease with digital ulcers is susceptible to developing secondary soft-tissue infection. Local débridement and antimicrobial treatment may be necessary if ischemic ulcerations become infected. Whirlpool treatment is the most effective method of ulcer débridement. In cases of secondary complications, consultation with a vascular surgeon or a rheumatologist is advised.
P.1224
Specific References*
For annotated General References and resources related to this chapter, visit http://www.hopkinsbayview.org/PAMreferences.