A case of secondary Sjögren's syndrome with a swollen thigh.
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Biomedical subjects
Publications and source records attributed to R M Bennett.
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OBJECTIVE: To determine the prevalence and determinants of self-reported work disability in persons with fibromyalgia (FM). METHODS: A longitudinal, multicenter survey of 1604 patients with FM from 6 centers with diverse socioeconomic characteristics was begun in 1988. Assessments were by self-report questionnaire and telephone contact, and included work and disability events that occurred before and after 1988. Comparative analyses were performed on the entire data set and, separately, on the Wichita data set. RESULTS: More than 16% of patients reported receiving US Social Security disability (SSD) payments (highest center rate 35.7%; lowest center rate 6.3%) compared to 2.2% of the US population (US Social Security Administration data) and 28.9% of patients with rheumatoid arthritis seen at the Wichita outpatient rheumatology clinic. Overall, 26.5% reported receiving at least one form of disability payment when SSD and other sources of disability payments were considered. In Wichita, less than 25% of SSD awards were made specifically for FM, but after 1988 that figure increased to 46.4%. Work disability was greatest at the San Antonio and Los Angeles centers. Multivariate predictors (correlates) included pain, Health Assessment Questionnaire disability, and unmarried status. In addition, more than 70% of patients reporting being disabled did receive disability payments. On the other hand, 64% reported being able to work all or most days, and more than 70% were employed or were homemakers. CONCLUSION: Although most patients (64%) report being able to work, we found high rates of self-reported work disability awards among persons with FM followed in 6 rheumatology centers. But we also found great variability among centers as to awards and as to self-reported work ability. Center differences in work disability might reflect clinic referral patterns, physician beliefs, or socioeconomic status.
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OBJECTIVE: To investigate the serum levels of insulin-like growth factor-I (IGF-I) in patients with fibromyalgia (FM) compared to healthy controls and patients with other rheumatic diseases, and to explore possible etiologic mechanisms of low IGF-I levels in patients with FM. METHODS: Five hundred patients with FM and 152 controls (74 healthy blood donors, 26 myofascial pain patients and 52 patients with other rheumatic diseases) were studied. All had measurements of acid extracted serum IGF-I. A subset of 90 patients with FM were evaluated for clinical features that might explain low IGF-I levels. Twenty-five patients with FM underwent growth hormone (GH) provocation testing with l-dopa and clonidine. RESULTS: The mean serum IGF-I level in patients with FM was 138 +/- 56 ng/ml and in controls 215 +/- 86 ng/ml (p = 0.00000000001). Low levels of IGF-I were not due to depression, tricyclic medications, nonsteroidal antiinflammatory drugs, poor aerobic conditioning, obesity, or pain level. Patients with focal myofascial pain syndromes had normal IGF-I levels (236 +/- 68), as did most patients with other rheumatic disorders, unless they had concomitant FM. Patients with FM with initially normal levels often had a rapid decline of IGF-I over 1 to 2 years. Most patients with FM with low IGF-I levels failed to secrete GH after stimulation with clonidine and l-dopa. CONCLUSION: Many, but not all, patients with FM have low levels of IGF-I that cannot be explained by clinical associations. These results suggest that low IGF-I levels in patients with FM are a secondary phenomenon due to hypothalamic-pituitary-GH axis dysfunction.
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There are three realities that need to be understood by both clinicians and patients: (1) fibromyalgia is a complex chronic pain condition and current treatment is palliative rather than curative; (2) the major aim of treatment is to improve function, not abolish pain; and (3) a cycle of chronic pain, stress, and psychological arousal often generates a set of secondary symptoms. These secondary symptoms provide a positive feedback loop that is amenable to modification by cognitive-behavioral techniques. Multidisciplinary group treatment programs are especially suited to such techniques; their aim should be to maximize subsequent clinician-patient interactions. Thus, a current concept of optimal management is a blend of multidisciplinary group therapy and individualized clinician-based treatment.
Fifty years ago, Wegener's granulomatosis was a uniformly fatal condition; the diagnosis was usually made post-mortem. Today, many patients survive, with a 93% complete remission rate reported 4 years after diagnosis. Four medical advances over the past 50 years have enhanced our understanding and management of this rheumatologic disorder. They are a rational classification of the vasculitides, development of a diagnostically useful serologic assay, successful treatment with cyclophosphamide, and novel ideas as to etiology.
OBJECTIVE: To evaluate the impact of a 6 month group therapy program on the treatment of patients with fibromyalgia (FM). Since FM is a common problem in rheumatology practice, a program was designed to treat patients in a group setting using allied health professionals. METHODS: The FM group (15 to 25 patients) met once a week for 6 months. The average session lasted 90 min and consisted of formal lectures, group sessions emphasizing behavior modification, stress reduction techniques, strategies to improve fitness and flexibility and support sessions for spouses/significant others. The 2 major outcome measures were the fibromyalgia impact questionnaire (FIQ) and the total tender point score. Secondary outcome measures were the quality of life scale, questionnaires to assess coping strategies and attitudes to illness, an index of aerobic conditioning, flexibility, distance walked in 6 min., Beck depression and Beck anxiety questionnaires. Patients were followed 2 years and compared to a group of patients outside the program. RESULTS: Between 1989 and 1993 170 patients were evaluated and 104 patients completed the program. At the end of the 6 months 73 (70%) of patients had <11 tender points and FIQ improved 25%. Having a pain profile on MMPI or having major depression, did not predict poorer outcome. Thirty-three patients followed for 2 years after the program continue to show improvement. A control group of 29 patients who never entered the program showed no significant improvement. CONCLUSION: The group treatment of patients with FM in an outpatient setting shows promise; a more formal controlled study is needed to confirm this impression.
We describe a 53-year-old white woman whose first manifestation of ovarian adenocarcinoma was a sudden onset of severe Raynaud's phenomenon, which rapidly progressed to fixed ischemic changes in her thumb. After surgical removal of the tumor and chemotherapy, the vasospastic features regressed. To our knowledge this is only the 2nd such reported case.
FM affects approximately six million Americans, four million are women. It is a chronic muscle pain syndrome with poorly understood associations with many other conditions. Although there is no distinctive pathophysiological basis for the syndrome, these patients are readily recognized by their history of widespread body pain and multiple tender-point areas. Failure to recognize these patients results in much frustration, both in the physician and in the patient, and often results in unnecessary investigations. Treatment of FM patients has to be individualistic and demands a holistic approach; this requires time, empathy, and interaction with other specialists. Providing effective treatment to these patients is a true test of a physician's skill.
OBJECTIVE: Fibromyalgia patients often describe the presence of dry eyes and dry mouth. Conversely there is an increasing recognition that many patients with Sjögren's syndrome (SS) have fibromyalgia (FM). We decided to investigate this association. METHODS: Seventy-two patients with FM were screened with a Schirmer's test. All patients with an abnormal test had a minor salivary gland biopsy. RESULTS: Thirty-eight percent (n = 28) had a Schirmer's test of < 15 mm wetting at 5 minutes, however sicca symptoms were noted in only 19% of patients. Salivary gland biopsy in these 28 patients showed a focus score of > or = 1 in 5; a positive antinuclear antibody test (ANA) was found in 4, a positive rheumatoid factor in 3 and anti-SSA SSB antibodies in 2. Another 8 patients had abnormal salivary gland lymphocytic foci, but there were < 50 cells or the density was < 1 focus/4 mm2; all 8 of these patients had a positive ANA. None of these patients have developed systemic features of SS over a 6 year period of followup. CONCLUSION: There is a subgroup of patients presenting with FM who, on further testing, have findings consistent with primary SS. The prevalence of this association was 6.9% for probable SS and 11% for possible SS. These figures are probably an overestimation due to tertiary center referral bias. The etiologic and management implications of these observations are unclear.
OBJECTIVE: To determine if patients with fibromyalgia syndrome (FMS) are more susceptible to activity-induced muscle damage than are healthy subjects. METHODS: Eleven FMS patients and 10 healthy subjects performed concentric and eccentric exercise with their dominant and nondominant forearms, respectively. 31P magnetic resonance spectroscopy (to assess inorganic phosphate [P(i)] and phosphocreatine [PCr]) and dolorimetry (to assess pain) were performed before and 20 minutes after exercise and at 4 subsequent 24-hour intervals. RESULTS: Neither group exhibited increased P(i)/PCr ratios or reduced dolorimetry scores following the exercise protocols. FMS patients did display a phosphodiester resonance at a higher rate than healthy subjects (37% versus 12%), but this was not related to the exercise. CONCLUSION: Unchanged P(i)/PCr ratios and dolorimetry scores following acute exercise provide evidence against the hypothesis that FMS patients are more susceptible to activity-induced muscle damage than are healthy subjects, although P(i)/Pcr and pain may not adequately document such damage. The frequent occurrence of phosphodiester in the spectra of FMS patients may indicate a sarcolemmal abnormality in these subjects.
PURPOSE: This study investigated the relationships among four methods of detecting depression in patients with fibromyalgia. METHODS: Data were obtained from 100 women (mean age 43 years) who had been diagnosed with fibromyalgia. Instruments included a computerized Diagnostic Interview Schedule (C-DIS), Beck Depression Inventory (BDI), an adjusted "disease-free" BDI (BDI-A), and Minnesota Multiphasic Personality Inventory depression subscale (MMPI-D). Chance-corrected concordance, sensitivity, specificity, and accuracy among the four methods were calculated. RESULTS: The C-DIS detected 22% and BDI-A 29% with current major depression. The BDI and MMPI-D yielded higher estimates of 55% of the 44%, respectively. Agreement on the diagnosis among the four methods was significantly greater than chance. When compared with the C-DIS, the BDI was the most sensitive instrument and the BDI-A most specific.
Initial success with nerve sparing retroperitoneal lymph node dissections in patients with low stage nonseminomatous germ cell tumors of the testis has led to the application of these techniques to a select group of 40 patients treated after chemotherapy at our institution between March 1988 and November 1991. A minimum 1-year followup was available for 38 patients. There have been no abdominal relapses, and 34 of the 38 patients report normal ejaculation postoperatively. Nerve sparing techniques are applicable to select patients undergoing retroperitoneal lymph node dissection after primary chemotherapy for metastatic disease without increasing the chance of local recurrence. Emission and ejaculation are preserved in the majority of patients.
It may be concluded that both peripheral and central mechanisms may operate in the pathophysiology of both impaired muscle function and pain in FM. These mechanisms may in part be attributable to physical deconditioning and disuse of muscle secondary to the characteristic pain and fatigue so often seen in FM. Most likely the initiation of this condition is multifactorial and the combination of peripheral and central factors that constitute a vicious circle may perpetuate the condition into a chronic state.
Myelodysplastic syndromes are a group of clonal hematopoietic stem cell disorders leading to peripheral cytopenias and a high probability of leukemic transformation. Immunological abnormalities, both humoral and cell mediated immunity, as well as vasculitis, a "lupus-like syndrome," neuropathy, and arthritis have been described in association with myelodysplastic syndromes. We describe 3 cases of polymyalgia rheumatica (PMR) developing within 4 months of detection of cytopenia. The possible association of myelodysplastic syndromes and PMR has not been previously recognized.
A 19-year-old white woman with severe systemic lupus erythematosus developed symptomatic sacroiliitis early in her disease. Over the next 14 years she showed radiological progression of sacroiliitis. She had neither HLA-DR3 nor B27 antigens. The rare coexistence of SLE and ankylosing spondylitis may not be determined solely by genetic factors; sacroiliitis may be just an infrequent manifestation of SLE.