Necrotising myositis: a surgical emergency that may have minimal changes in the skin.

Necrotising myositis is a surgical emergency. It is underappreciated that it may present without changes in the skin. Diagnosis is therefore often delayed. We describe a case of necrotising myositis necessitating glenohumeral disarticulation. Remarkable features were the absence of skin signs and the rapidity with which the patient became extremely septic. A review of the literature has shown the importance of early diagnosis and quick decision making to minimise mortality.

Osteoporosis and bone morbidity in cardiac transplant recipients.

PURPOSE: To evaluate the incidence and etiology of osteopenia and pathologic fractures in cardiac transplant recipients. PATIENTS: Thirty-one adult male cardiac transplant recipients and 14 adult men with congestive heart failure (CHF) awaiting cardiac transplantation. METHODS: Assessment of indices of bone and mineral metabolism and of bone mineral density (BMD) by dual-energy x-ray absorptiometry. RESULTS: BMD in the proximal femur was below normal in both groups compared to that in age-matched control subjects, whereas BMD in the lumbar spine was normal. There was no significant difference in BMD at any site between the two groups. No clinical parameter predicted BMD. In all patients, laboratory indices of bone mineral metabolism, except parathyroid hormone (PTH) levels, were normal and not statistically different between the two groups. CHF patients had a trend toward elevations of PTH, 1,25-dihydroxyvitamin D, and urinary calcium excretion compared to transplant patients. Eight of 31 transplant patients and 2 of 14 CHF patients had vertebral compression fractures (c2 = 11.8, p < 0.0006). Transplant recipients with fractures had twice as many rejection episodes as did transplant patients without fractures, but did not differ in cumulative dose of steroids. Two patients developed avascular necrosis of the femoral head following transplantation. CONCLUSIONS: Cardiac transplant recipients and patients with CHF awaiting transplantation had decreased hip BMD, but normal spine BMD. Although immunosuppressive therapy did not appear to influence bone mass, loop diuretics prior to transplantation may have stimulated a mild secondary increase in PTH that could have differentially caused loss of bone density at the hip in both groups. Pulse corticosteroids used in treating rejection may have contributed to the increased incidence of vertebral fractures in transplant patients. These data suggest that severe CHF with its associated diuretic use and decreased activity are primary contributors to osteopenia in these patients.

Management of complications of glucocorticoid therapy.

Corticosteroids are commonly employed in the treatment of a wide variety of pulmonary disorders, including asthma, chronic obstructive pulmonary disease, pulmonary fibrosis, sarcoidosis, and pulmonary vasculitides; as well as in the lung transplant population. Frequently, steroid-induced complications contribute significantly to morbidity and diminished quality of life, often overshadowing the impact of the underlying lung disease. This article will discuss the more commonly encountered complications of corticosteroids in the context of the method of delivery, events occurring with chronic use, and events related to withdrawal.

Soluble urate in sera and synovial fluids from patients with different joint disorders.

OBJECTIVE: To evaluate simultaneous serum and synovial fluid (SF) urate levels in various inflammatory and noninflammatory joint disorders and to correlate SF white blood cell (WBC) counts with serum and joint fluid urate levels. METHODS: Sixty-three paired samples of sera and SF from 58 patients including 25 patients with inflammatory arthropathies, 18 patients with gout and 15 patients with noninflammatory joint disorders, were measured for urate concentrations by a UV enzymatic method. RESULTS: In inflammatory arthropathies other than gout, urate concentrations in SF were significantly lower than in paired sera (p < 0.0001). There was no difference between the SF and serum urate levels in noninflammatory arthropathies and in gout. In gout, however, SF urate occasionally were found to be considerably higher than in sera. This phenomenon was observed in fluids with massive amounts of monosodium urate crystals. There was no correlation between SF WBC counts and serum of SF urate levels in any of the disease groups studied. CONCLUSIONS: Serum and synovial fluid levels vary more than previously recognized. SF urate levels tend to reflect serum levels in gout and noninflammatory arthropathies but not in inflammatory joint disorders. Disturbed purine metabolism in inflammatory arthropathies may reflect a component in the pathophysiology of inflammation. The elevations of SF urate levels seen in gout are unique for this disease and most likely reflect crystal dissolution in joints.

Measurement of lower limb blood flow in patients with neurogenic claudication using positron emission tomography.

STUDY DESIGN: Ten subjects (seven with neurogenic claudication and three control subjects) underwent examination of lower limb muscle blood flow before and after exercise using positron emission tomography. OBJECTIVES: To investigate the hypothesis that lower limb muscle ischemia was the origin of symptoms in neurogenic claudication. BACKGROUND: Patients with neurogenic claudication secondary to spinal stenosis experience lower limb discomfort after exercise similar to that of ischemic claudication. However, they do not have clinical evidence of peripheral vascular disease. The authors postulated that the lower limb discomfort in patients with neurogenic claudication may arise from muscle ischemia due to inadequate dilatation of arterioles in response to exercise, this itself arising secondary to sympathetic dysfunction due to spinal stenosis. METHOD: Using O15-labeled water and positron emission tomography measured thigh and leg muscle blood flow response to exercise bilaterally in seven patients with unilateral neurogenic claudication and three control subjects were measured. RESULTS: The average values obtained for mid-thigh and mid-calf muscle perfusion at rest were 2.57 ml/min/100 g tissue (2.23-3.90) and 2.39 ml/min/100 g tissue (2.03-3.46), respectively. The average values obtained from mid-thigh and mid-calf perfusion after exercise were 4.41 ml/min/100 g tissue (2.8-6.0) and 4.87 ml/min/100 g (2.2-11.7). We found no difference in muscle perfusion between symptomatic and asymptomatic limbs in this group of patients. CONCLUSION: These studies suggest that muscle ischemia is not the origin of symptoms in most patients with neurogenic claudication.

Lyme disease: diagnosis & management.

Lyme disease is the most common vector borne illness occurring in the United States. Most cases occur in the East. Prompt recognition of typical features and understanding the limitations of laboratory testing are essential in order to provide appropriate management. Outcome in cases treated early is uniformly good.

Polymerase chain reaction as a diagnostic tool.

Although the method of polymerase chain reaction (PCR) is technical, the concept is simple: PCR is a sequential process that explores a sample for the presence of a known sequence of DNA or RNA. Chapter sections cover specific applications of PCR to infectious diseases with rheumatic manifestations.

Clinically significant gastropathy associated with nonsteroidal antiinflammatory drug use in children with juvenile rheumatoid arthritis.

OBJECTIVE: To estimate the frequency of documented clinically significant gastrointestinal (GI) side effects secondary to nonsteroidal antiinflammatory drugs (NSAID) therapy and to describe the adverse events. METHODS: Computerized medical records of 702 patients with juvenile rheumatoid arthritis (JRA) administered NSAID were searched for the occurrence of clinically significant gastropathy (esophagitis, gastritis, peptic ulcer disease). RESULTS: Five children were identified who had a total of 10 events of gastropathy documented by either barium swallow or endoscopy, and thought to be attributable to NSAID therapy. Each child had at least 2 separate events of clinically significant gastropathy. CONCLUSION: Although mild GI disturbances are frequent side effects associated with NSAID therapy, the number of children with JRA who experience clinically significant gastropathy appears to be low.

Rheumatic symptoms associated with hypothyroidism in children.

We describe five children with varied rheumatic manifestations, including fibromyalgia and arthralgias, ultimately proved to be associated with hypothyroidism. All musculoskeletal symptoms improved after thyroid replacement therapy. We conclude that rheumatic manifestations of hypothyroidism can be as varied in children as in adults.

Novel gastrointestinal tract manifestations in juvenile dermatomyositis.

We report a case of juvenile dermatomyositis in which a dilated atonic esophagus was associated with delayed gastric emptying and intestinal mucosal thickening, resulting in a radiographic "stacked coin" appearance. These findings, which can also occur in infectious, neoplastic, or other immune-mediated diseases, broaden the spectrum of gastrointestinal tract manifestations in juvenile dermatomyositis. Physicians should be alert for these treatable manifestations in children with myositis who present with unexplained gastrointestinal symptoms, which are reversible with immunosuppressive therapy.

Discontinuation of methotrexate treatment in juvenile rheumatoid arthritis.

OBJECTIVE: Children with juvenile rheumatoid arthritis (JRA) treated with methotrexate (MTX) were examined for their course after the discontinuation of the drug to define the relapse and remission rates and to identify predictors of relapse. METHODOLOGY: A retrospective chart review of all patients with JRA was conducted in two pediatric rheumatology centers. A total of 101 patients being treated with MTX were identified. Dose, response to the drug, and length of time until reaching a state of complete control were noted. The outcome of patients with a complete response in whom the drug was discontinued was examined with regards to length of time to relapse or continued remission. RESULTS: In 25 patients, MTX was discontinued after reaching complete control of the disease. There were no statistically significant predictors of response to MTX identified. Of 25 whose MTX was discontinued, relapse occurred in 13 (52%) after a mean of 11 months after discontinuation. There was no significant difference among patients who relapsed or those who remained in remission as to sex, subtype of JRA, number of months to complete control, or number of months in complete control until discontinuing MTX. Patients younger than 41/2 years at diagnosis were found to be more likely to relapse than patients diagnosed at a later age. In 10 of the patients who relapsed, complete control was induced within a mean of 7 months after restarting MTX. CONCLUSION: The optimal time for discontinuing MTX in children with JRA who have achieved complete control is unknown. Relapse occurred in approximately half of the patients in whom MTX was discontinued. Because response to reinstitution of the drug is good, it is reasonable to discontinue MTX after prolonged complete control. It remains to be seen whether the relapse rate can be improved by waiting for longer periods of time in complete control before its discontinuation.

Prevalence and outcome of uveitis in a regional cohort of patients with juvenile rheumatoid arthritis.

OBJECTIVE: To determine the prevalence and outcome of chronic uveitis in patients with juvenile rheumatoid arthritis (JRA). METHODS: A retrospective analysis of 760 patients with JRA followed in 4 pediatric rheumatology centers. Patients with chronic uveitis were identified and their medical and ophthalmologic records were reviewed. RESULTS: Seventy-four patients with uveitis were identified. The prevalence of uveitis was 9.3%. The mean interval from the onset of JRA to the onset of uveitis was 21 months, and 90% of the patients who developed uveitis did so within the first 4 years of their disease. Visual complications (synechiae, band keratopathy, cataract, or glaucoma) developed in 31% of the patients with uveitis. Complications were more common in patients who presented with uveitis early in the course of their JRA. Complications were also more common in antinuclear antibody (ANA) negative than in ANA positive patients. Visual loss to 20/50 or worse occurred in only 11% of patients with uveitis, and no patient became blind. CONCLUSION: In a very large cohort of patients with JRA, uveitis was uncommon and poor visual outcome was rare. Visual complications did not necessarily result in a poor outcome.

Sleep and periodic limb movement in sleep in juvenile fibromyalgia.

OBJECTIVES: Fibromyalgia has been recently recognized in children and adolescents as juvenile fibromyalgia (JF). In adult fibromyalgia, subjective complaints of nonrestorative sleep and fatigue are supported by altered polysomnographic findings including a primary sleep disorder known as periodic limb movements in sleep (PLMS) in some subjects. Although poor sleep is a diagnostic criterion for JF, few reports in the literature have evaluated specific sleep disturbances. Our objectives were to evaluate in a controlled study the polysomnographic findings of children and adolescents with JF for alterations in sleep architecture as well as possible PLMS not previously noted in this age group. METHODS: Sixteen consecutive children and adolescents (15.0 +/- 2.6 years of age) diagnosed with JF underwent overnight polysomnography. Polysomnography was also performed on 14 controls (14.0 +/- 2.2 years of age) with no history of an underlying medical condition that could impact on sleep architecture. Respiratory variables, sleep stages, and limb movements were measured during sleep in all subjects. RESULTS: JF subjects differed significantly from controls in sleep architecture. JF subjects presented with prolonged sleep latency, shortened total sleep time, decreased sleep efficiency, and increased wakefulness during sleep. In addition, JF subjects exhibited excessive movement activity during sleep. Six of the JF subjects (38%) were noted to have an abnormally elevated PLMS index (>5/hour), indicating PLMS in these subjects. CONCLUSION: Our study demonstrated abnormalities in sleep architecture in children with JF. We also noted PLMS in a significant number of subjects. This has not been reported previously in children with this disorder. We recommend that children who are evaluated for JF undergo polysomnography including PLMS assessment. juvenile fibromyalgia; periodic limb movement in sleep; restless legs syndrome.

Sustained improvement over two years in physical function, structural damage, and signs and symptoms among patients with rheumatoid arthritis treated with infliximab and methotrexate.

OBJECTIVE: To evaluate the efficacy and safety of repeated administration of infliximab plus methotrexate (MTX) over a 2-year period in patients with rheumatoid arthritis (RA) who previously experienced an incomplete response to MTX. METHODS: Four hundred twenty-eight patients were randomly assigned to receive MTX plus placebo or infliximab at a dose of 3 or 10 mg/kg plus MTX for 54 weeks, with an additional year of followup. The protocol was later amended to allow for continued treatment during the second year. Of 259 patients who entered the second year of treatment, 216 continued to receive infliximab plus MTX for 102 weeks. Ninety-four of these 259 patients experienced a gap in therapy of >8 weeks before continuing therapy. Infusions were administered at weeks 0, 2, and 6, followed by treatment every 4 weeks or every 8 weeks (alternating with placebo infusions in the interim 4-week visits) at a dose of 3 or 10 mg/kg for a total of 102 weeks (including the gap in therapy). For safety and efficacy assessments, data on the patients who were randomized to receive treatment, irrespective of whether treatment was administered for 102 weeks, were evaluated using all actual observations available. The efficacy measures included the Health Assessment Questionnaire (HAQ) (physical function), Short Form 36 health survey (SF-36) (health-related quality of life), total radiographic scores (structural damage), and the American College of Rheumatology 20% improvement criteria (ACR20) (signs and symptoms). RESULTS: The infliximab plus MTX regimens resulted in significantly greater improvement in HAQ scores (P < or = 0.006) and SF-36 physical component summary scores (P < or = 0.011) compared with the MTX-only group. There also was stability in the SF-36 mental component summary score among patients who received the infliximab plus MTX regimens. Median changes from baseline to week 102 in the total radiographic score were 4.25 for patients who received the MTX-only regimen and 0.50 for patients who received the infliximab plus MTX regimen. The proportion of patients achieving an ACR20 response at week 102 varied from 40% to 48% for the infliximab plus MTX groups compared with 16% for the MTX-only group. CONCLUSION: Throughout 102 weeks of therapy, infliximab plus MTX provided significant, clinically relevant improvement in physical function and quality of life, accompanied by inhibition of progressive joint damage and sustained improvement in the signs and symptoms of RA among patients who previously had an incomplete response to MTX alone.