Incidence of physician-diagnosed primary Sjögren syndrome in residents of Olmsted County, Minnesota.

OBJECTIVES: To estimate the incidence of physician-diagnosed primary Sjögren syndrome (SS) among residents of Olmsted County, Minnesota, in the setting of usual medical care and to determine how often objective criteria are available in the medical records of such patients. PATIENTS AND METHODS: We reviewed all medical records of residents in Olmsted County with physician-diagnosed SS from 1976 to 1992 to determine whether they had undergone objective tests for keratoconjunctivitis sicca, salivary dysfunction, or serologic abnormality. Confounding illnesses were excluded. To identify misclassified cases, all records from patients with xerostomia or keratoconjunctivitis sicca were also reviewed. The average annual SS incidence rates were calculated by considering the entire population to be at risk. RESULTS: Of 75 patients with onset of SS during the study period, 53 had primary SS. All patients were white, 51 (96.2%) were women, and the mean +/- SD age was 59+/-15.8 years. The age- and sex-adjusted annual incidence was 3.9 per 100,000 population (95% confidence interval, 2.8-4.9) for patients with primary SS. Eleven patients (20.8%) with physician-diagnosed SS had no documentation of objective eye, mouth, or laboratory abnormalities. Objective evaluations performed most frequently were laboratory and ocular tests and least often were investigations of xerostomia. CONCLUSIONS: The average annual incidence rate for physician-diagnosed primary SS in Olmsted County is about 4 cases per 100,000 population. These data probably underestimate the true incidence because they are based on usual medical care of patients with SS in a community setting, rather than on a case-detection survey. In the future, a true incidence may be possible with a higher index of suspicion, greater attention to objective tests, and increased awareness of new classification criteria for SS. For epidemiological studies based on existing data, application of current criteria may not be feasible, and consensus on criteria for such studies would be useful.

Survivorship in a population based cohort of patients with Sjögren's syndrome, 1976-1992.

OBJECTIVE: Sjögren's syndrome (SS) has been associated with development of lymphoid malignancies and other significant medical complications, but the effect of SS on survival in a population based sample has not been reported. We analyzed survival in an incidence cohort of patients diagnosed with SS in residents of Olmsted County, Minnesota, USA, between 1976 and 1992. METHODS: All records of physician diagnosed SS were reviewed, as well as all records from patients diagnosed with xerostomia and keratoconjunctivitis sicca, and records of patients with rheumatoid arthritis (RA) and systemic lupus erythematosus. The case definition for SS required 2 of 3 criteria: keratoconjunctivitis sicca, xerostomia, or serologic abnormality. Confounding illnesses were excluded. All patients were white. RESULTS: Of the 74 cases of SS identified, 50 (67%) had primary SS and 24 (33%) secondary SS. An average of 7.2 years of followup was available for patients with primary SS and 9.9 years for patients with secondary SS. Compared with the general population, patients with SS had increased mortality (p = 0.04). When patients with primary and secondary SS were studied separately, increased mortality was found in patients with secondary SS (p < 0.005) but not primary SS (p = 0.86). CONCLUSION: In this population based cohort, patients with primary SS did not have increased mortality. However, mortality may have been increased in patients with secondary SS, the majority of whom had RA.

Lupus panniculitis: clinical perspectives from a case series.

OBJECTIVE: To review clinical and laboratory features of lupus panniculitis from a large group of patients. METHODS: Retrospective chart review of patients diagnosed with lupus panniculitis at a tertiary medical center from 1976 to 1993. RESULTS: Lupus panniculitis occurred most frequently in adult women. Skin lesions involved proximal extremities, trunk, face, and scalp. Only 4 of 40 patients fulfilled criteria for systemic lupus erythematosus (SLE), and, other than positive antinuclear antibodies, a paucity of other autoantibodies was seen. Average disease duration was 6 years (range 0-38). Treatment with antimalarial agents was undertaken in most cases. Disease related morbidity (disfigurement and disability) was relatively common, but death was rare. CONCLUSION: Lupus panniculitis is a chronic inflammatory disease of subcutaneous adipose tissue that can develop during the course of SLE, although most patients do not develop systemic lupus.

Expansion of unusual CD4+ T cells in severe rheumatoid arthritis.

OBJECTIVE: The repertoire of T cells in patients with rheumatoid arthritis (RA) is characterized by clonal expansion of selected CD4+ T cells, which are autoreactive and lack the expression of the functionally important CD28 molecule. The goal of this study was to determine the contribution of these unusual lymphocytes to the disease process. METHODS: RA patients (n = 108) and normal controls (n = 53) were examined for the expression of CD4+ CD28- T cells by 2-color fluorescence-activated cell sorter analysis. Clinical data were ascertained by retrospective chart review. RESULTS: The frequencies of CD4+ CD28- T cells displayed a bimodal distribution, defining carriers and noncarriers in normal subjects and RA patients. In longitudinal studies, the noncarrier and carrier phenotypes were stable over time. Carriers of CD4+ CD28- T cells accumulated in the RA population (64% versus 45%; P = 0.02). The expansion of CD4+ CD28- T cells correlated with extraarticular involvement, but not with disease duration, antirheumatic treatment, or severity of joint destruction. The patient subsets with nodular disease (P = 0.02) and rheumatoid organ disease (P = 0.04) had the highest proportion of CD4+ CD28- T cell carriers. The size of the CD4+ CD28- compartment correlated with extraarticular progression of RA (P = 0.001 in nodular RA, P = 0.003 in rheumatoid organ disease). CONCLUSION: The bimodality of distribution of CD4+ CD28- T cell frequencies is compatible with genetic control of the generation of these unusual T cells. In RA patients, CD4+ CD28- T cells are not an epiphenomenon of the disease process, but predispose patients to developing inflammatory lesions in extraarticular tissues.

The repertoire of CD4+ CD28- T cells in rheumatoid arthritis.

BACKGROUND: While oligoclonality of circulating CD4- CD8 and of CD8+ T cells is not uncommon, clonal dominance within the CD4 compartment is not frequently found in healthy individuals. In contrast, the majority of patients with rheumatoid arthritis (RA) have clonally expanded CD4+ T cell populations. Previous studies have demonstrated that these clonogenic CD4+ T cells do not express the CD28 molecule. To examine the correlation between CD28 expression and clonal proliferation, we have analyzed the T cell receptor (TCR) diversity of CD4+ CD28- T cells in normal individuals and in RA patients. MATERIAL AND METHODS: The size of the peripheral blood CD4+ CD28- compartment was determined in 30 healthy individuals and 30 RA patients by two-color FACS analysis. In 10 RA patients and five controls with more than 2.5% CD4+ CD28- T cells, TCR BV gene segment usage was analyzed with 19 BV-specific antibodies. Oligoclonality was assessed in sorted CD4+ CD28+ and CD28- T cells using TCR BV-BC-specific polymerase chain reaction and size fractionation. Clonal dominance was confirmed by direct sequencing. RESULTS: The CD4+ CD28- T cell compartment was expanded to more than 2.5% in 70% of the RA patients and 30% of the normal individuals. Compared with the CD4+ CD28+ T cells, the TCR BV gene segment usage among CD4+ CD28- cells was grossly skewed with the dominance of single BV elements. Molecular TCR analysis provided evidence for oligoclonality in 17 of 21 expanded BV elements. In two unrelated RA patients who shared both HLA-DRB1 alleles, the TCR beta-chain sequences of dominant clonotypes were highly conserved. CONCLUSIONS: Oligoclonality is a characteristic feature of CD4+ CD28- T cells which are expanded in some healthy individuals and in the majority of RA patients. The lack of CD28 expression is a common denominator of CD4+, CD8+, and CD4- CD8- T cells prone to develop clonal dominance. The limited TCR diversity of clonal CD4+ CD28- populations in RA patients suggests that these T cells recognize a limited spectrum of antigens. The fact that the majority of individuals with marked expansions and oligoclonality of CD4+ CD28- T cells are RA patients suggests a role for these unusual lymphocytes in the pathogenetic events leading to RA.

Splinter hemorrhages following arterial puncture.

Splinter hemorrhages can be a feature of the antiphospholipid syndrome. We describe a patient in whom splinter hemorrhages developed following radial artery puncture. The implications of this findings in patients with the antiphospholipid syndrome are discussed.

Septic arthritis in adults: clinical features, outcome, and intensive care requirements.

A review of adults with septic arthritis was undertaken to evaluate outcome of treatment and intensive care requirements in a community-based teaching hospital. During an 80-month period (1986-1992), 38 cases of septic arthritis were identified. Underlying joint disease was present in 84% of patients. Mean age was 68 years, with a range of 26 to 100 years and a median of 70 years. Patients did not always initially display signs of infection; fever was present in only 42%, and leukocytosis was present in 67%. Total in-hospital mortality was 26%, but the mortality attributed to septic arthritis was 13%. Polyarticular septic arthritis occurred in 26% of patients and carried a 40% mortality. Twenty-four percent of patients required transfer to the intensive care unit (ICU); they had a 67% mortality. Three of four patients with polyarticular septic arthritis requiring intensive care died. Average length of hospital stay for survivors receiving a full course of antibiotics was 35 days; it diminished to 14 days for 5 uncomplicated cases who received home IV antibiotics. Eighty-nine percent of survivors had return of function of the affected joints. Thirty-two percent required surgical intervention, and 5% were complicated by osteomyelitis. Septic arthritis remains a costly disease affecting primarily the elderly with underlying joint disease. Polyarticular septic arthritis and the need for ICU care portend a high mortality. The functional outcome of those who recovered was generally good.

Iguana-associated salmonellosis in a young adult.

PURPOSE: Review a case of Salmonella infection in a young adult related to handling of an infected iguana. METHODS: Case report. RESULTS: Most cases of Salmonella infection related to handling of reptiles have occurred in children. We report a case of Salmonella diarrhea in a 19-year-old male who kept a pet iguana. The iguana was asymptomatic, but Salmonella grew from stool specimens. CONCLUSIONS: Those who keep iguanas as pets, which are particularly attractive to adolescents and young adults, should be aware that iguanas frequently carry Salmonella. Those caring for adolescents and young adults should always inquire into the pet-keeping habits of their patients when illnesses develop.

Sickness in Spoon River: village health at the turn of the century.

Edgar Lee Masters published numerous poems, plays, and novels between 1900 and 1942; most go unread, with the exception of Spoon River Anthology, which is among the most popular works of American poetry of the twentieth century. This collection of poems tells of the lives of the inhabitants of a fictional American town--Spoon River, Illinois. Many of the poems consider sickness and health in the community, and the insight they offer into human responses to illness continues to be relevant today, contributing not only to the lasting popularity of Spoon River Anthology, but also the literary value of the work.