Musculoskeletal problems and injuries - part 4 ppt

cost in 2004 of $1300–1400 per month) and have common local and systemic side effects. 9 Leflunomide has been shown to be superior to methotrexate and sulfasalazine for symptomatic relief of rheumatoid arthritis, with seri- ous adverse effects taking place in 0% to 2% of users. 15 It is terato- genic, and female patients who want to become pregnant after having taken the drug must take cholestyramine to bind the drug. Without the cholestyramine, the medication can be present in the body for up to two years. 9 Surgery When pharmacological therapy and all other modalities have been attempted, surgery sometimes provides relief of pain. Ninety percent of elderly patients with severe incapacitating rheumatoid joint disease can expect excellent pain relief and satisfactory motion following total hip or knee replacement. 16 Experimental Treatment and Therapy of the Future Because of the chronicity, lack of a cure, and associated disability of RA, patients are vulnerable to many unproved “alternative” therapies. There is definitely a need for better therapies, making RA an intensely active area of current research. Borrowing an idea from oncology to suppress immune responses maximally, combination therapy with DMARDs is being actively investigated. Other experimental therapies include high-dose intra- venous prednisolone, total lymphoid irradiation, interferon- ␥ , inter- leukin-1 (IL-1) inhibitors, cyclosporine, monoclonal antibody antagonists against T-cell receptors, and phenytoin. Tetracyclines have been investigated for years based on the theory that infectious agents such as Mycoplasma or Chlamydia may cause RA. 17 The anti- body-absorbing column Prosorba is used with plasmapheresis, and is Food and Drug Administration (FDA) approved for moderate to severe RA in patients refractory to methotrexate. It is expensive, and long-term efficacy is unknown. Juvenile Rheumatoid Arthritis Juvenile rheumatoid arthritis (JRA), a heterogeneous group of dis- eases formerly known as Still’s disease, is clinically distinct from RA in adults. The cause is unknown. Hypotheses concerning etiology include infection, hypersensitivity, an autoimmune reaction, or a com- bination of these factors. Fortunately, at least 75% of children with 5. Rheumatoid Arthritis and Related Disorders 111 JRA eventually have long remissions without significant residual deformity or loss of function. About 5% of patients with adult RA have symptoms beginning in childhood. Clinical manifestations of JRA fall into three major categories: pau- ciarticular (40–50%), polyarticular (25–40%), and systemic (10–20%). These classifications are helpful for determining diagnosis, treatment, and prognosis in children with chronic arthritis. Consideration of other possibilities for arthritis, including mechanical or degenerative disor- ders, septic arthritis, reactive arthritis to extra-articular infection, con- nective tissue diseases, neoplastic disorders, endocrine disorders (type 1 diabetes mellitus, hyperthyroidism, hypothyroidism), and idiopathic pediatric joint pain syndromes is recommended. Diagnosis may be dif- ficult without persistent, objective joint swelling. JRA is largely a diag- nosis of exclusion. In pauciarticular-onset disease, children have four or fewer joints involved during the first six months of symptoms. Large joints are pri- marily affected, often asymmetrically. Pauciarticular type I JRA affects girls 80% of the time, usually before age 4. Pauciarticular type II JRA affects boys 90% of the time, usually at age 8 or older, and many go on to develop spondyloarthropathies such as ankylosing spondylitis. There is a 10% to 30% risk of chronic iridocyclitis with this disease, and many authorities recommend frequent slit-lamp examinations to prevent scarring and loss of vision. Polyarticular-onset JRA occurs mostly in girls, involving multiple joints including small joints. RF-positive polyarticular JRA tends to be more severe than RF-negative disease, both acutely and with long- term risk of severe arthritis. Systemic-onset JRA is characterized by high intermittent fevers (Ͼ102°F), rash, hepatosplenomegaly, lymphadenopathy, arthralgias, and leukocytosis. Arthritis becomes chronic but the systemic symp- toms generally dissipate with time. The NSAIDs are generally used as first-line treatment for JRA as concerns about Reye’s syndrome have discouraged use of salicylates, the drugs of choice in the past. As in adults, low-dose methotrexate is being used more frequently as the second-line drug of choice. Sulfasalazine has also been used with success. Gold compounds and antimalarials are probably less effective in JRA than in adult RA and are used less commonly. Long-term systemic glucocorticoids are effective for symptom relief but do not prevent joint damage and are best avoided in children if possible. Topical steroids are used for asso- ciated iridocyclitis. Physical and occupational therapy are important for protecting joint mobility; this is particularly important in JRA as children often do not complain of pain but simply stop using affected 112 Joseph W. Gravel Jr., Patricia A. Sereno, Katherine E. Miller joints. The ultimate goal is to utilize these various treatments to encourage children with JRA to live active, normal lives. The family physician coordinates care with other members of the treatment team and offers necessary support to the child and family. Systemic Lupus Erythematosus Systemic lupus erythematosus (SLE) is a complicated rheumatological disorder with a broad range of presentations. The incidence of SLE has more than tripled in the past 30 years, from 1.5/100,000 in 1950–79 to 5.6/100,000 in 1980–92. 18 The incidence among female patients is three times that of male patients, resulting in a prevalence of 1 in 700 for women between the ages of 20 and 64 years. 19,20 The disease incidence in African-American and Hispanic women in the same age group is higher than in their Caucasian counterparts. The pathogenesis of SLE is not completely understood. Current theories include polyclonal B-cell activation and antigen stimulation resulting in the immune response that characterizes this complex dis- order. Studies have pointed to a genetic factor contributing to the development of SLE. Twin studies have revealed a concordance rate among monozygotic twins to be as high as 30% to 50%. An associa- tion with human leukocyte antigen (HLA) groups DR2, DR3, DR4, and DR5 has also been found. Laboratory Findings Detection of antinuclear antibodies is a highly sensitive screening test for SLE, although it is not specific for SLE. A marginally elevated anti- nuclear antibodies titer is found in 2% to 5% of normal individuals. 20 About 95% of SLE patients have positive antinuclear antibodies titers that are more than two times higher than the normal limit identified by any given laboratory. Other antibodies identified in SLE patients include anti-double-stranded DNA, anti-DNA-histone complex, anti- Sm (Smith antigen), and anti-Ro (Robert antigen). Antibodies to dsDNA and Sm antigen are specific for SLE and have been associated with more severe cases. Anti-Ro antibodies are associated with various dermatological manifestations of SLE. Anti-single-stranded DNA is not specific for SLE and therefore plays no role in diagnosis. Up to 30% of SLE patients also have circulating antiphospholipid antibodies. These antibodies, known as the “lupus anticoagulant” may result in prolonged partial thromboplastin (PTT) and prothrombin (PT) times yet paradoxically result in an increased risk of thrombotic events. When counseling female patients who have circulating 5. Rheumatoid Arthritis and Related Disorders 113 antiphospholipid antibodies, it is important to discuss the increased risk of spontaneous abortions. In fact, a history of recurrent sponta- neous midtrimester abortions should trigger testing for antiphospho- lipid antibodies. Systemic lupus erythematosus is characterized by a wide variety of presentations. The SLE classification system, revised in 1982 and updated in 1997, identifies 11 symptoms of the disease or systems affected in SLE patients. To confirm a diagnosis of SLE, patients must have at least 4 of the 11 criteria present either serially or simultane- ously (Table 5.4). Mucocutaneous Manifestations The classic malar butterfly rash is present in only one third of patients. It usually presents abruptly after exposure to sunlight and lasts for several days or weeks. More commonly, patients have a patchy macu- lopapular rash on sun-exposed areas. Subacute cutaneous lupus erythe- matosus presents with a unique rash characterized by photosensitivity and superficial, nonindurated, nonscarring lesions. One third to two thirds of SLE patients are markedly photosensitive, and sun exposure not only results in rash but also may induce a flare of systemic manifestations. Seventy percent of patients with photosensi- tivity have anti-Ro antibodies. 21 Discoid lesions are raised plaques that may result in scarring. Other skin manifestations include alopecia, hyperpigmentation, and hives. Biopsy shows immunoglobulin deposition at the dermoepidermal junction. This finding is known as the lupus band test. 22 Arthritis Arthralgias are the most common complaint of SLE patients and are often present at the time of initial diagnosis. Up to 76% of patients develop arthritis associated with disease activity. It is difficult to dif- ferentiate the joint complaints of SLE patients from those of RA patients, but SLE patients usually present with pain out of proportion to the degree of synovitis. Also, in contrast to RA patients, SLE patients have soft tissue involvement that can result in joint deformity without evidence of cartilage involvement (Jaccoud arthropathy). Tendon rupture can also occur. Serositis SLE patients can have inflammation of the pleural, pericardial, and peritoneal membranes. Exudative pleural effusions are common but 114 Joseph W. Gravel Jr., Patricia A. Sereno, Katherine E. Miller 5. Rheumatoid Arthritis and Related Disorders 115 Table 5.4. 1982 American College of Rheumatology Revised Criteria for Classification of Systemic Lupus Erythematosus (updated 1997) Criterion Definition 1. Malar rash Fixed erythema, flat or raised, over the malar eminences, tending to spare the nasolabial folds 2. Discoid rash Erythematous raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions 3. Photosensitivity Skin rash as a result of unusual reaction to sunlight, by patient history or physician observation 4. Oral ulcers Oral or nasopharyngeal ulceration, usually painless, observed by physician 5. Arthritis Nonerosive arthritis involving two or more peripheral joints, characterized by tenderness, swelling or effusion 6. Serositis Pleuritis—convincing history of pleuritic pain or rub heard by a physician or evidence of pleural effusion OR Pericarditis—documented by electrocardiogram or rub or evidence of pericardial effusion 7. Renal disorder Persistent proteinuria greater than 500 mg/24 hours or greater than 3ϩ on dipstick OR Cellular casts—may be red cell, hemoglobin, granular, tubular or mixed—on urine sediment 8. Neurological disorder Seizures or psychosis in the absence of offending drugs or known metabolic derangements 9. Hematological disorder Hemolytic anemia with reticulocytosis OR Leukopenia—less than 4000/mm 3 on 2 or more occasions OR Lymphopenia—less than 1500/mm 3 on 2 or more occasions OR Thrombocytopenia—less than 100,000/mm3 in the absence of offending drugs (continued) 116 Joseph W. Gravel Jr., Patricia A. Sereno, Katherine E. Miller Table 5.4. (continued). Criterion Definition 10. Immunological disorder Anti-DNA in abnormal titer OR Anti-Smith antibody OR Positive finding of antiphospholipid antibodies based on: 1. An abnormal serum level of IgG or IgM anticardiolipin antibodies 2. A positive test result for lupus anticoagulant using a standard method 3. A confirmed false-positive serologic test for syphilis known to be positive for at least 6 months 11. Positive ANA test An abnormal titer of ANA by immunofluorescence or an equivalent assay at any point in time and in the absence of drugs known to be associated with drug-induced lupus syndrome. For classification of SLE at least 4 of these 11 criteria must be met, either serially or simultaneously, during any interval of obser- vation. ANA ϭ antinuclear antibody. are usually small and therefore of little clinical importance. 22 Up to 29% of SLE patients have symptoms of pericarditis including pain, friction rub, and electrocardiographic changes. Renal Disease Renal involvement occurs in as many as 75% of patients, and the resulting glomerulonephritis is a major determinant of morbidity and mortality 23 (see Reference 37, Chapter 97). Immune complex deposi- tion along the glomerular basement membrane results in an inflam- matory response resulting in the characteristic glomerular findings. SLE patients should have an annual urinalysis to look for early evi- dence of proteinuria. Because of renal compensatory mechanisms, even urinalysis and measurement of serum creatinine or creatinine clearance may underestimate actual parenchymal damage. Renal biopsy obviously can document the type of lesion present in sympto- matic patients, but biopsy results have not been useful for predicting disease progression. A persistently elevated serum creatinine level (Ͼ2 mg/dL) is the best predictor of future renal morbidity. 19 Neurological Disorder Neurological complaints among SLE patients vary from headache to seizures. Neuropsychiatric findings in SLE patients can be the result of direct injury from immune complexes and thrombotic events or of other organ dysfunction. Cognitive dysfunction has been documented in 20% to 70% of patients. Progressive decline in cognitive function leading to dementia has been reported but is rare. Laboratory studies in symptomatic patients are significant for antineuronal antibodies. Most patients have normal cerebrospinal fluid (CSF) findings, but CSF studies are important to exclude infectious causes of neurological manifestations. Immunological CSF studies (i.e., oligoclonal bands) are not specific in SLE patients. Studies comparing magnetic reso- nance imaging (MRI) to computed tomographic (CT) in symptomatic SLE patients have met with conflicting results. CT is more than ade- quate for diagnosing mass lesions or intracranial hemorrhage. MRI is more sensitive for picking up the signs of chronic vascular injury but may also identify incidental or clinically insignificant lesions. 23 Hematological Disorder It is not surprising that most SLE patients have anemia of chronic dis- ease. Up to 25% of SLE patients may also have an autoimmune throm- bocytopenia; and 5% of these patients have severe thrombocytopenia 5. Rheumatoid Arthritis and Related Disorders 117 with a platelet count as low as 20,000 cells/mm. 3,23 Furthermore, SLE patients have higher incidences of both arterial and venous thromboem- bolic events. Some studies have shown up to a fiftyfold increase in risk of myocardial infarction among reproductive-aged women with SLE, compared to age-matched controls. 18 Some investigators have recom- mended prophylactic aspirin or other anticoagulation treatment for all SLE patients, regardless of antiphospholipid status, 24 and aggressive anticoagulation with warfarin [with a goal international normalized ratio (INR) of 2.5–3.5] for patients with known antiphospholipid antibodies. 18 Treatment Treatment of SLE is best tailored to individual patients and their specific symptoms. Current treatment regimens have in general been successful in decreasing the morbidity and mortality associated with SLE. Current data indicate that more than 90% of patients survive at least 15 years. All SLE patients are encouraged to minimize sun exposure and to use sunscreen. The most common complaint, arthralgias, can often be ade- quately treated with NSAIDs. Patients being treated long term with NSAIDs should be monitored periodically for renal and hepatic side effects. Glucocorticoids have also been used to treat severe symptoms, but they increase the risk of side effects. Cutaneous manifestations of SLE generally respond well to treatment with antimalarials (i.e., hydroxychloroquine or quinacrine). For treatment of skin disease resist- ant to either of these agents, small studies have used dapsone, azathio- prine, gold, intralesional interferon, and retinoids. Methotrexate is more effective than placebo for moderate SLE without renal involvement. 18 The foundation of treatment of SLE patients with renal disease has long been glucocorticoid therapy; however, this practice is being chal- lenged. Patients with mild disease can often be managed with low- dose prednisone; patients with diffuse proliferative or severe focal proliferative glomerulonephritis may require a two-month course of high-dose prednisone (1 mg/kg) followed by a prolonged taper. However, studies using cyclophosphamide have shown this drug to be effective as either a single agent or in combination with glucocorti- coids, and more effective than glucocorticoids alone. 18,23 Like treatment of renal disease, the mainstay for treating thrombocy- topenia has long been glucocorticoids. Patients with disease resistant to glucocorticoid treatment may respond to splenectomy, but prior to con- sideration of splenectomy providers must weigh the benefits against the risks posed by decreased immune function. Cyclophosphamide and chemotherapeutic agents such as vincristine and procarbazine have also been used in patients with severe disease. 23 118 Joseph W. Gravel Jr., Patricia A. Sereno, Katherine E. Miller Reiter Syndrome Reiter syndrome, a form of reactive arthritis, is defined as “an acute, sterile synovitis associated with a localized infection elsewhere in the body”—generally a venereal infection. 25 The hallmark of Reiter syn- drome is the triad of arthritis, conjunctivitis, and urethritis. Other symptoms found in patients with Reiter syndrome include sacroiliitis, enthesopathic symptoms with the most common sites being the Achilles’ tendon and planter fascia, dactylitis, mucocutaneous lesions including stomatitis, circinate balanitis, and nail lesions. The exact prevalence and incidence of Reiter syndrome is unknown. It has a five- to ninefold higher incidence in men and the prevalence is increased in patients positive for HLA-B27. 26 Laboratory findings in Reiter syndrome patients are usually nonspe- cific and do not confirm the diagnosis. There is no cure for Reiter syn- drome, but the underlying illness should be treated. Raynaud’s Disease During the late nineteenth century Maurice Raynaud described digital vasospasm that seemed to be cold-induced. He believed that this phe- nomenon, now known as Raynaud’s disease, was due to changes in the CNS control over vascular innervation. Raynaud’s phenomenon is classically described in patients who develop extremity blanching and numbness with cold exposure, followed by cyanosis and then ery- thema on rewarming. The fingers are affected most commonly, but the toes and ears may also be involved. Raynaud’s disease has been divided into primary and secondary forms. Primary Raynaud’s is more common than the secondary form, occurring in 3% to 16% of the general population. 27 Secondary Raynaud’s disease is far less common, developing in only 3% to 9% of patients; it is defined as Raynaud’s phenomenon associated with the development of a connective tissue disease (most commonly scle- roderma). Evaluation of a patient in whom Raynaud’s disease is suspected includes a thorough history and physical examination. Changes con- sistent with the disease can be reproduced in the office by immersing the patient’s affected extremity in ice water. Antinuclear antibodies are positive in 17% to 26% of patients but do not predict disease pro- gression. 28 Investigations into the pathophysiology of Raynaud’s disease have led to identification of a number of abnormalities, but the complete 5. Rheumatoid Arthritis and Related Disorders 119 mechanism has not been fully established. Studies have shown that patients with this disorder have an abnormal adrenergic response. Neuropeptide release (possibly due to sensory nerve system damage) and endothelial factors have also been identified. 27–29 It is important to discuss with patients the role of behavior modifica- tion. Conservative approaches to treatment include warm socks or mit- tens and cold avoidance. Patients are encouraged to stop smoking and to avoid vasoconstrictive drugs, such as amphetamines, cocaine, and over-the-counter decongestants. Caffeine may also exacerbate symp- toms by causing a rebound vasoconstriction after an initial vasodilata- tion. In patients with vasospasm associated with emotional stress, relaxation and stress management strategies have also been helpful. When conservative strategies fail, patients may respond to calcium channel blockers. Nifedipine has been the most widely studied at doses of 10 mg sublingually for immediate treatment of acute vasospasm or 30 to 60 mg of nifedipine taken on a chronic basis, although care must be taken to avoid symptomatic hypotension. Scleroderma Scleroderma, or systemic sclerosis, is a connective tissue disorder whose hallmark is tissue fibrosis. Systemic scleroderma is character- ized by progressive fibrosis of the skin, lungs, heart, gastrointestinal tract, and kidneys. An association of limited skin involvement and late visceral involvement is known as CREST syndrome (calcinosis, Raynaud’s phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias). A localized form of scleroderma, known as linear scleroderma or morphea, exists when fibrotic changes are localized to the skin; it does not involve the GI tract. 30 As with many other connective tissue diseases, scleroderma affects women three times as often as men, and the incidence in the United States is estimated to be 1/100,000 persons per year. The incidence peaks in women between the fifth and sixth decades of life. 31 Clinically, patients with systemic involvement present earlier in the disease course with mostly skin complaints. The histopathologic features found in patients with scleroderma include diffuse small artery and arteriolar vasculitis with fibrinoid necrosis, intimal thickening, and mucopoly–saccharide deposition. The exact mechanism responsible for excess deposition is unknown. Because there is no cure for scleroderma, treatment goals are to optimize function of involved organ systems. The skin is involved in most patients, so it is important that patients use moisturizers to help 120 Joseph W. Gravel Jr., Patricia A. Sereno, Katherine E. Miller [...]... drugs Arthritis Rheumatol 1991; 34: 1353–60 12 Simon LS, Weaver AL, et al Anti-inflammatory and upper gastrointestinal effects of celecoxib in rheumatoid arthritis: A randomized controlled trial JAMA 1999;282;1921–8 13 Bello CS, Garrett, SD Therapeutic and adverse effects of glucocorticoids U.S Pharmacist Continuing Education Program no 43 0-0 0 0-9 9028-H01, August 1999 14 Van der Heijde DM, van Leeuwen... NSAIDs for nonnarcotic pain relief, low-dose tricyclic antidepressants, and membrane-stabilizing anticonvulsants such as gabapentin. 14 Other medications that have resulted in some success include short-term use of corticosteroids, calcium channel blockers, - and ␤-adrenergic antagonists, and intramuscular calcitonin Useful topical regimens include lidocaine patches and capsaicin cream 0.025% applied four... Med 2000;160(13):2 042 –8 25 Hughes R, Keat A Reiter’s syndrome and reactive arthritis: A current view Semin Arthritis Rheum 19 94; 24( 3):190–210 26 Kirchner J Reiter’s syndrome Postgrad Med 1995;97:111–21 27 Kahaleh B, Matucci-Cerinic M Raynaud’s phenomenon and scleroderma Arthritis Rheum 1995;38:1 4 28 Adee A Managing Raynaud’s phenomenon: A practical approach Am Fam Phys 1993 ;47 (4) :823–9 29 Dowd P Raynaud’s... pregnancy and hormonal therapy, morbidity and mortality, and pathogenesis Ann Intern Med 1995;123 :42 –53 22 Osial T, Cash J, Eisenbeis C Arthritis associated syndromes Prim Care 1993;20:857–82 23 Boumpas D, Austin H, Fessler B, et al Systemic lupus erythematosus: emerging concepts Part 1 Renal, neuropsychiatric, cardiovascular, pulmonary, and hematologic disease Ann Intern Med 1995;122: 940 –50 24 Wahl DG,... the Musculoskeletal System Jeffrey G Jones and Doug Poplin Problems of the Soft Tissues Fibromyalgia Fibromyalgia (FM) is a common musculoskeletal syndrome characterized by generalized pain, fatigue, and a number of associated symptoms The condition has formerly been called fibrositis and psychogenic rheumatism The condition is confusing in that there is considerable overlap between FM symptoms and. .. presence of a “taut band,” and a twitch response Palpation of the trigger point characteristically produces pain referred beyond it.11 The syndrome may be acute and is often seen after strain or trauma to a muscle Nonmusculoskeletal symptoms, such as fatigue, are unusual The pathophysiology is not well understood The terminology used to describe this and related problems is not standardized, adding to... cool skin, decreased hair and nail growth, hyperhidrosis, decreased range of motion, and atrophy Behavioral and emotional changes are often seen during this phase and are often related to protecting the painful body part The atrophic phase may be associated with decreased pain but is generally associated with irreversible changes in skin, subcutaneous tissue, and muscle Fibrosis and contractures may develop... roentgenographic changes (e.g., with a three-phase bone scan or on plain radiographs); and response to neural blockade Referral to an anesthesiologist for a trial of blockade, when accompanied by objective verification of a successful block and associated decrease in signs and symptoms, can be helpful from both diagnostic and treatment points of view.15 1 34 Jeffrey G Jones and Doug Poplin Management Treatment... Saf 2000;22 :40 5– 14 18 Ruiz-Irastorza G, Khamashta MA, Castellino G, et al Systemic lupus erythematosus Lancet 2001;357:1027–31 19 Mills J Systemic lupus erythematosus N Engl J Med 19 94; 330:1871–9 5 Rheumatoid Arthritis and Related Disorders 125 20 Condemi J The autoimmune diseases JAMA 1992;268:2882–92 21 Boumpas D, Fessier B, Austin H, et al Systemic lupus erythematosus Part 2 Dermatologic and joint... of 20 and 50 Patients usually present with local pain or functional dysfunction related to the location of the tumor About half of these tumors occur in the knee Radiographs reveal the typical, large lytic “soap-bubble” lesions The cell of origin of these tumors is not clear, and the biological behavior is somewhat 140 Jeffrey G Jones and Doug Poplin Fig 6 .4 Young woman with persistent foot pain and . patients include anti-double-stranded DNA, anti-DNA-histone complex, anti- Sm (Smith antigen), and anti-Ro (Robert antigen). Antibodies to dsDNA and Sm antigen are specific for SLE and have been associated with. high-dose intra- venous prednisolone, total lymphoid irradiation, interferon- ␥ , inter- leukin-1 (IL-1) inhibitors, cyclosporine, monoclonal antibody antagonists against T-cell receptors, and. Bello CS, Garrett, SD. Therapeutic and adverse effects of glucocorti- coids. U.S. Pharmacist Continuing Education Program no. 43 0-0 0 0-9 9- 028-H01, August 1999. 14. Van der Heijde DM, van Leeuwen