Clement J. Michet

Relapsing polychondritis. Survival and predictive role of early disease manifestations.

To define the natural history of relapsing polychondritis, the probability of survival and causes of death were determined in 112 patients seen at one institution. By using covariate analysis, early clinical manifestations were identified that predicted mortality. The 5- and 10-year probabilities of survival after diagnosis were 74% and 55%, respectively. The most frequent causes of death were infection, systemic vasculitis, and malignancy. Only 10% of the deaths could be attributed to airway involvement by chondritis. Anemia at diagnosis was a marker for decreased survival in the entire group. There was an interaction between other disease variables and age in determining their impact on outcome. For patients less than 51 years old, saddle-nose deformity and systemic vasculitis were the worst prognostic signs. For older patients, only anemia predicted outcome. The need for corticosteroid therapy did not influence survival.

Trends in the incidence and mortality of systemic lupus erythematosus, 1950–1992

OBJECTIVE To describe trends in systemic lupus erythematosus (SLE) incidence and mortality over the past 4 decades. METHODS Using the Rochester Epidemiology Project resources, medical records were screened to identify all Rochester, Minnesota residents with any SLE-associated diagnoses, discoid lupus, positivity for antinuclear antibodies, and/or false-positive syphilis test results determined between January 1, 1980 and December 31, 1992. Medical records were then reviewed using a pretested data collection form in order to identify cases of SLE according to the American College of Rheumatology 1982 revised criteria for SLE. Drug-induced cases were excluded. All identified SLE patients were followed up until death, migration from the county, or October 1, 1997. These data were combined with similar data from the same community obtained between 1950 and 1979, and trends in the SLE incidence and mortality over time were calculated. RESULTS Of the 430 medical records reviewed, 48 newly diagnosed cases of SLE (42 women and 6 men) were identified between 1980 and 1992. The average incidence rate (age- and sex-adjusted to the 1970 US white population) was 5.56 per 100,000 (95% confidence interval [95% CI] 3.93-7.19), compared with an incidence of 1.51 (95% CI 0.85-2.17) in the 1950-1979 cohort. The age- and sex-adjusted prevalence rate as of January 1, 1993 was approximately 1.22 per 1,000 (95% CI 0.97-1.47). Survival among SLE patients was significantly worse than in the general population (P = 0.017 compared with the 1980-1992 cohort, and P < 0.0001 compared with the 1950-1979 cohort, by log-rank test). Cox proportional hazards modeling demonstrated a statistically significant improvement in the survival rate over time (P = 0.035). CONCLUSION Over the past 4 decades, the incidence of SLE has nearly tripled, and there has been a statistically significant improvement in survival. These findings are likely due to a combination of improved recognition of mild disease and better approaches to therapy.

2012 provisional classification criteria for polymyalgia rheumatica: a European League Against Rheumatism/American College of Rheumatology collaborative initiative

The objective of this study was to develop EULAR/ACR classification criteria for polymyalgia rheumatica (PMR). Candidate criteria were evaluated in a 6-month prospective cohort study of 125 patients with new onset PMR and 169 non-PMR comparison subjects with conditions mimicking PMR. A scoring algorithm was developed based on morning stiffness >45 minutes (2 points), hip pain/limited range of motion (1 point), absence of RF and/or ACPA (2 points), and absence of peripheral joint pain (1 point). A score ≥4 had 68% sensitivity and 78% specificity for discriminating all comparison subjects from PMR. The specificity was higher (88%) for discriminating shoulder conditions from PMR and lower (65%) for discriminating RA from PMR. Adding ultrasound, a score ≥5 had increased sensitivity to 66% and specificity to 81%. According to these provisional classification criteria, patients ≥50 years old presenting with bilateral shoulder pain, not better explained by an alternative pathology, can be classified as having PMR in the presence of morning stiffness>45 minutes, elevated CRP and/or ESR and new hip pain. These criteria are not meant for diagnostic purposes.

Epidemiology of Systemic Lupus Erythematosus and Other Connective Tissue Diseases in Rochester, Minnesota, 1950 Through 1979

The incidence and prevalence rates of connective tissue disease syndromes in Rochester, Minnesota, from 1950 through 1979 are reported. The incidence of definite systemic lupus erythematosus (SLE) has not increased since 1960. The incidence of SLE in the elderly population was higher than that in previous reports. Rates of SLE and discoid lupus erythematosus were approximately equal. Other diagnoses (in decreasing order of frequency) were suspected lupus erythematosus, scleroderma, drug-induced lupus, and overlapping connective tissue disease syndromes. The 10-year survival of patients with definite SLE was decreased, and the survival of patients with suspected SLE was the same as that of the general population.

The lifetime risk of adult‐onset rheumatoid arthritis and other inflammatory autoimmune rheumatic diseases

OBJECTIVE Understanding of the personal risks for rheumatoid arthritis (RA) and other rheumatic diseases remains poor, despite advances in knowledge with regard to their pathogenesis, therapeutics, and clinical impact, in part because the personal lifetime risk of developing these diseases is unknown. This study was undertaken to estimate the lifetime risk of RA, as well as other inflammatory autoimmune rheumatic diseases, including systemic lupus erythematosus, psoriatic arthritis, polymyalgia rheumatica (PMR), giant cell arteritis, ankylosing spondylitis, and Sjögrens syndrome, and to provide an overall estimate of the risk of developing inflammatory autoimmune rheumatic disease over a lifetime. METHODS Using the incidence rates obtained from our population-based studies of rheumatic diseases among residents of Olmsted County, Minnesota, and mortality rates from life tables for the general population, we estimated the sex-specific lifetime risk of rheumatic disease. RESULTS The lifetime risk of RA developing in US adults was 3.6% for women and 1.7% for men, and the lifetime risk of rheumatoid factor-positive RA was 2.4% for women and 1.1% for men. The second most common inflammatory autoimmune rheumatic disease was PMR, with a lifetime risk of 2.4% for women and 1.7% for men. The overall lifetime risk of inflammatory autoimmune rheumatic disease was 8.4% for women and 5.1% for men. CONCLUSION One in 12 women and 1 in 20 men will develop an inflammatory autoimmune rheumatic disease during their lifetime. These results can serve as useful guides in counseling patients regarding their lifetime risk of these conditions and have important implications regarding disease awareness campaigns.

Ocular and Systemic Findings in Relapsing Polychondritis

The ocular and systemic findings in 112 Mayo Clinic patients with relapsing polychondritis were reviewed. The incidence of males and females was equal, with median age at diagnosis of 51 years and the median follow-up of 6 years. Most patients had several organ systems involved at the time of the diagnosis. Twenty-one patients had ocular symptoms at the onset, and 57 developed ocular symptoms during their course. Major ocular complications included proptosis, lid edema, episcleritis/scleritis, corneal infiltrates/thinning, iridocyclitis, retinopathy, and optic neuritis. The major system involvement included otorhinolaryngeal, respiratory, arthritic, renal, cardiovascular, dermatologic, and neurologic diseases. Generally, laboratory studies were not helpful in making the diagnosis but were valuable in monitoring the disease. Based on the experience in these cases, the indications for the various therapeutic modalities are offered.

Idiopathic Symptomatic Osteoarthritis of the Hip and Knee: A Population-Based Incidence Study

We identified all residents of Rochester, Minnesota, who sought medical assistance for the first time in 1985 because of symptomatic osteoarthritis of the hip or knee that was unrelated to a specific disease. Of these residents, 98 (59 women and 39 men) had 122 symptomatic joints (95 knees and 27 hips), for age- and sex-adjusted incidence rates of 205 new patients and 255 newly affected joints per 100,000 person-years. The incidence of osteoarthritis of the hip was greater in women than in men, whereas the sex ratio for occurrence of osteoarthritis of the knee approached unity. Rates at both sites increased steadily with aging in men but plateaued after the menopause in women; similar patterns were seen whether or not grade 1 disease was included. Projected nationally, these first population-based data indicate that as many as approximately half a million new symptomatic cases of idiopathic osteoarthritis of the knee and hip may arise annually in the white population of the United States. With increasing longevity, osteoarthritis may exact an even greater toll in the future.

Dual-energy CT for the diagnosis of gout: an accuracy and diagnostic yield study

Objectives To assess the accuracy of dual-energy CT (DECT) for diagnosing gout, and to explore whether it can have any impact on clinical decision making beyond the established diagnostic approach using polarising microscopy of synovial fluid (diagnostic yield). Methods Diagnostic single-centre study of 40 patients with active gout, and 41 individuals with other types of joint disease. Sensitivity and specificity of DECT for diagnosing gout was calculated against a combined reference standard (polarising and electron microscopy of synovial fluid). To explore the diagnostic yield of DECT scanning, a third cohort was assembled consisting of patients with inflammatory arthritis and risk factors for gout who had negative synovial fluid polarising microscopy results. Among these patients, the proportion of subjects with DECT findings indicating a diagnosis of gout was assessed. Results The sensitivity and specificity of DECT for diagnosing gout was 0.90 (95% CI 0.76 to 0.97) and 0.83 (95% CI 0.68 to 0.93), respectively. All false negative patients were observed among patients with acute, recent-onset gout. All false positive patients had advanced knee osteoarthritis. DECT in the diagnostic yield cohort revealed evidence of uric acid deposition in 14 out of 30 patients (46.7%). Conclusions DECT provides good diagnostic accuracy for detection of monosodium urate (MSU) deposits in patients with gout. However, sensitivity is lower in patients with recent-onset disease. DECT has a significant impact on clinical decision making when gout is suspected, but polarising microscopy of synovial fluid fails to demonstrate the presence of MSU crystals.

Renal involvement in relapsing polychondritis.

Twenty-nine of the 129 patients with RP seen at the Mayo Clinic between 1943 and 1984 had renal involvement. These patients were older, had arthritis and extrarenal vasculitis more frequently, and had a significantly worse survival rate than those without renal involvement. Renal biopsies were obtained in 11 of these 29 patients. The predominant lesions were mild mesangial expansion and cell proliferation, and segmental necrotizing glomerulonephritis with crescents. Small amounts of electron-dense deposits, predominantly mesangial, were noted on electron microscopy. Immunofluorescence revealed faint deposition of C3 and/or IgG or IgM, predominantly in the mesangium. Autopsies were obtained in 13 of the 47 patients who had died. Information regarding the renal pathology was available in 10 of these 13 autopsies. At the time of the initial evaluation at the Mayo Clinic, 6 of these 10 patients had evidence of renal involvement. At autopsy, none of these 10 patients had evidence of active renal vasculitis or segmental necrotizing glomerulonephritis, but 8 of the 10 patients exhibited variable degrees of vascular and glomerular sclerosis, segmental mesangial proliferation, tubular loss, and interstitial lymphocytic infiltrates. These observations expand the limited information available in the literature, which is based on 11 previously published case reports of renal involvement in RP. In only a few of our patients and previously reported patients were the manifestations of the disease limited to the systems characteristically involved in pure RP. The frequent coexistence of other autoimmune and connective tissue diseases supports the role of immune mechanisms in the pathogenesis of this syndrome. Deposition of immune complexes is likely to play a role in the pathogenesis of the glomerular lesions associated with RP. Administration of corticosteroids alone is sufficient to induce a complete remission in some cases, while in others the addition of a cytotoxic agent is necessary to control the activity of the disease or to spare corticosteroid side effects and maintain a remission. Immunosuppression-related infectious complications and undetected relapses after discontinuation of immunosuppressive therapy are largely responsible for the morbidity and mortality observed in these patients.

Total Knee Arthroplasty: A Population-Based Study

Total knee arthroplasty (TKA) is being used increasingly for the management of chronic arthritis of the knee. In this report, we review the frequency of application of TKA in the population of Olmsted County, Minnesota, from 1971 through 1986. The utilization rate of TKA increased from 20.5 per 100,000 person-years for 1971 through 1974 to 60.8 per 100,000 for 1983 through 1986. Although rates were higher in women, they increased with advancing age in both sexes. Rates between the urban and rural populations of Olmsted County did not differ. The two most common underlying diseases that necessitated TKA were osteoarthritis and rheumatoid arthritis; they were the cause of more than 90% of all operations. By extrapolating the rates of TKA in Olmsted County to the total 1986 US population, we estimate a need for at least 143,000 TKAs annually at a direct cost of more than