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Objective: To compare cancer incidence, type, and survival between patients with idiopathic inflammatory myopathies (IIMs) in Western Australia (WA) and the general population., Methods: Administrative health data for hospitalized patients with incident IIM (n = 803, 56.5% female, median age 62.0 yrs), classified by a validated algorithm as polymyositis (PM; 36.2%), dermatomyositis (DM; 27.4%), inclusion body myositis (IBM; 17.1%), overlap myositis (OM; 10.7%), and other IIM (8.6%), were linked to WA cancer and death registries for the period of 1980 to 2014. Cancer incidence rates (CIRs) before and after IIM diagnosis as well as cancer mortality were compared with age-, sex-, and calendar year-matched controls (n = 3225, 54.9% female, median age 64 yrs) by rate ratios (RRs) and Kaplan-Meier survival estimates., Results: The prediagnosis CIR was similar for patients with IIM and controls (6.57 vs 5.95; RR 1.11, 95% CI 0.88-1.39) and for patients evolving to DM (n = 220) or other IIM subtypes (6.59 vs 6.56; RR 1.01, 95% CI 0.38-3.69). During follow-up, CIR was higher for all DM (4.05, 95% CI 3.04-5.29), with increased CIR for lung cancer vs controls (1.05 vs 0.33; RR 3.18, 95% CI 1.71-5.47). Cancer post diagnosis shortened life span by 59 months for patients with IIM (103 vs 162 months, P < 0.01), but reduced survival rates were observed only in patients with DM and IBM., Conclusion: Cancer risk was not increased prior to IIM, but CIR for lung cancer was increased following DM diagnosis. As cancer reduced survival only in patients with DM and IBM, these data support a strategy of limited cancer screening in IIM., (Copyright © 2024 by the Journal of Rheumatology.)

Objective: To clarify the prevalence, risk factors, outcome, and outcome-related factors of hemophagocytic lymphohistiocytosis (HLH) in patients with dermatomyositis (DM), polymyositis (PM), or clinically amyopathic dermatomyositis (CADM)., Methods: Data of patients with DM, PM, or CADM who were admitted to the First Affiliated Hospital of Zhejiang University from February 2011 to February 2019 were retrospectively collected. Patients diagnosed with HLH constituted the case group. A 1:4 case-control study was performed to identify risk factors for HLH in patients with DM, PM, or CADM through comparison, univariate, and multivariate logistic regression analysis. Intragroup comparison was made among patients with HLH to identify factors influencing unfavorable short-term outcome., Results: HLH was a rare (4.2%) but fatal (77.8%) complication in patients with DM, PM, or CADM. The retrospective case-control study revealed that higher on-admission disease activity (p = 0.008), acute exacerbation of interstitial lung disease (AE-ILD, p = 0.002), and infection (p = 0.002) were risk factors for complication of HLH in patients with DM, PM, or CADM. The following intragroup comparison showed that higher on-admission disease activity (p = 0.035) and diagnosis of CADM (p = 0.039) might influence the short-term outcome of patients with HLH. However, no risk factor was identified after false discovery rate correction., Conclusion: In this study, secondary HLH was a fatal complication, with higher on-admission disease activity, AE-ILD, and infection working as risk factors. The underlying role of infection and autoimmune abnormality in HLH in connective tissue disease was subsequently noted. Clinical factors influencing the short-term outcome of patients with secondary HLH require further study.

Objective: To examine the reproductive pattern of women with idiopathic inflammatory myopathy (IIM) compared to the general population., Methods: Population-based, nationwide registers were used to identify offspring of women with IIM and comparators., Results: Women with IIM in general had similar reproductive patterns as the comparators, whereas in those diagnosed between 26 and 45 years of age, there was an overall trend for fewer children as well as a higher proportion of nulliparity and a lower fertility rate in women with dermatomyositis than their comparators., Conclusion: Reproductive attention should be paid to patients with IIM diagnosed during the childbearing period.

Objective: Evidence suggests an increased risk of cardiovascular (CV) diseases, including acute coronary syndrome (ACS), in idiopathic inflammatory myopathies (IIM). The aim of this study was to investigate the risk of ACS in an incident IIM cohort compared to the general Swedish population., Methods: A cohort of 655 individuals with incident IIM and 6813 general population comparators were identified from national registries. IIM subjects were diagnosed from 2002 to 2011. Followup started at IIM diagnosis and corresponding date in the general population. ACS, CV comorbidities, and CV risk factors were defined using International Classification of Diseases codes. Incidence rates including 95% CI were calculated. Cox proportional hazards models were used to compare the risk of ACS in patients with IIM and the general population. The competing risk of death was accounted for using competing risk regression models., Results: The incidence rate of ACS in IIM was higher than in the general population, particularly within the first year of diagnosis and in older individuals. The overall ACS incidence rate in IIM was 15.6 (95% CI 11.7-20.4) per 1000 person-years, with an HR of 2.4 (95% CI 1.8-3.2) compared with the general population. When accounting for the competing risk of death, the risk of ACS in IIM remained increased with a cumulative incidence of 7% at 5 years compared to 3.3% in the general population., Conclusion: IIM individuals are at higher risk of ACS, particularly within the first year after diagnosis.

Objective: To determine the prevalence, distribution, and clinical manifestations of arthritis in a cohort of patients with idiopathic inflammatory myopathies (IIM). Associations with autoantibody status and HLA genetic background were also explored., Methods: Consecutive patients with IIM treated in a single center were included in this cross-sectional study (n = 106). History of arthritis, 68-joint and 66-joint tender and swollen joint index, clinical features of IIM, and autoantibody profiles were obtained by clinical examination, personal interview, and review of patient records. High-resolution genotyping in HLA-DRB1 and HLA-DQB1 loci was performed in 71 and 73 patients, respectively., Results: A combination of patients' medical history and cross-sectional physical examination revealed that arthritis at any time during the disease course had occurred in 56 patients (53%). It was present at the beginning of the disease in 39 patients (37%) including 23 cases (22%) with arthritis preceding the onset of muscle weakness. On physical examination, 29% of patients had at least 1 swollen joint. The most frequently affected areas were wrists, and metacarpophalangeal and proximal interphalangeal joints. Twenty-seven out of the 29 anti-Jo1-positive patients had arthritis at any time during the course of their illness; this prevalence was significantly higher compared to patients without the anti-Jo1 autoantibody (p < 0.0001). No association of arthritis with individual HLA alleles was found., Conclusion: Our data suggest that arthritis is a common feature of myositis. It is frequently present at the onset of disease and it may even precede muscular manifestations of IIM. The most common presentation is a symmetrical, nonerosive polyarthritis affecting particularly the wrists, shoulders, and small joints of the hands. We have confirmed a strong association of arthritis with the presence of the anti-Jo1 antibody.

Objective: The prevalence of organ complications in scleroderma (systemic sclerosis; SSc) varies by definition used. This study was done to determine the frequency of several features of SSc., Methods: A search of Medline-Ovid/Embase, PubMed, and Scopus databases from 1980 to November 30, 2011, was conducted to identify relevant articles with at least 50 patients with SSc extracting prevalence of each organ complication. Study quality was assessed using the STROBE (Strengthening The Reporting of OBservational studies in Epidemiology) checklist. Pooled prevalence was calculated using the random effects method. Heterogeneity was quantified using I(2)., Results: A total of 5916 articles were identified (913 from Medline-Ovid/Embase, 1009 from PubMed, and 3994 from Scopus); 5665 were excluded, leaving 251 articles for full-text review, with 69 included. Where available, frequencies were also included from the Canadian Scleroderma Research Group. Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, 95% CI 14-17), estimated pulmonary artery pressure > 40 mm Hg (18%, 95% CI 14-21), pulmonary arterial hypertension by right heart catheterization (15%, 95% CI 12-17), forced vital capacity (FVC) < 70% predicted (15%, 95% CI 12-17), FVC < 80% predicted (17%, 95% CI 12-21), myositis (13%, 95% CI 10-17), inflammatory arthritis (12%, 95% CI 9-16), Sjögren overlap (13%, 95% CI 10-16), and digital ulcers (DU; 15%, 95% CI 10-20); and 15% of DU have complications (amputations 12%, 95% CI 8-16, and hospitalizations 13%, 95% CI 6-21). Scleroderma renal crisis is uncommon but occurs in almost 15% (12%, 95% CI 5-19) of cases of disseminated cutaneous SSc. There is no 15% rule within skin and gastrointestinal tract for SSc., Conclusion: The "15%" rule for frequency of significant organ involvement in SSc is helpful.

Objective: To determine the prevalence of systemic sclerosis (SSc) overlap syndrome and autoantibody profile in a large single-center cohort., Methods: SSc diagnoses, subsets, and autoantibody profiles were obtained from clinical records of patients attending the Centre for Rheumatology, Royal Free Hospital, between September 1999 and February 2007., Results: In total, 332 (20%) of 1700 patients with SSc had overlap syndrome. This comprised myositis (42.8%), rheumatoid arthritis (RA; 32%), Sjögren's syndrome (SS; 16.8%), and systemic lupus erythematosus (SLE; 8.4%). Antinuclear antibody was positive in 96.6% of patients. Anticentromere antibody (ACA) was exclusively present in limited cutaneous SSc (lcSSc) overlap cases (22%), and more common in SSc/SS overlap (44.7%), whereas no difference was found in the prevalence of Scl-70 autoantibody between lcSSc and diffuse cutaneous SSc overlap groups. U1RNP was more frequent in SSc/SLE (44%), while Ro antibody was more likely to be found in SSc/SS (29.8%). ACA was absent and anti-Scl-70 was infrequent in SSc/myositis; polymyositis-scleroderma antibody was more frequent in this group (33.1%). About 50% of patients had raised rheumatoid factor (RF), with no difference between overlap groups irrespective of RF titer. In contrast, anticyclic citrullinated peptide antibody was more frequent in patients with RA features., Conclusion: About one-fifth of SSc cases had overlap features. There were distinct serological features that may predict specific clinical presentation and disease course.

Objective: Little information exists regarding the economic burden related to inflammatory myopathies. Our objective was to estimate health services costs in a large, unselected, population-based sample of patients with inflammatory myopathies., Methods: We identified subjects with polymyositis and dermatomyositis from administrative healthcare databases (covering all beneficiaries, about 7.5 million) in Quebec province, Canada. Average estimates of health services costs (physician visits, diagnostic tests and procedures, outpatient surgeries and procedures, acute care hospitalizations) for 2003 were calculated by multiplying health service use levels by the appropriate unit prices, determined from government fee schedules and other sources. Multiple linear regression analyses were performed to establish whether specific factors (age, sex, disease duration, region of residence, socioeconomic status, type of myositis, disease severity) were associated with cost., Results: We identified 1102 subjects with inflammatory myopathy from January 1, 1989, to January 1, 2003. About two-thirds were women (68.9%); average age at case ascertainment was 57.4 years (SD 18.4). The average cost of all reimbursed health services, in 2008 Canadian dollars, was $4099 per patient (SD $9639). Costs increased with age, and were highest early in the disease course. Greater disease severity (defined as the need for prior hospitalization for myositis) was also a strong predictor of both physician costs and total costs., Conclusion: These results indicate significant economic burden related to inflammatory myopathies, with important demographic predictors. Our estimates suggest that the health services costs in inflammatory myopathies may equal, or exceed, those of other serious diseases, such as rheumatoid arthritis and systemic sclerosis.

Objective: In 2005 a large outbreak of Salmonella hadar occurred in Spain following the consumption of commercial precooked roast chicken. We estimated the incidence and risk factors for reactive arthritis (ReA) and other musculoskeletal sequelae in the patients of this outbreak in 2 health departments of Castellon province., Methods: A prospective cohort study of the patients and their families was carried out. Clinical infection with Salmonella was considered as the exposure factor. The cohort was studied for ReA symptoms using a telephone questionnaire. Telephone interviews or medical examinations of subjects with musculoskeletal symptoms were conducted by a rheumatologist. Robust Poisson regression models were used in the analysis., Results: From the cohort of 262 people, 248 (94.7%) participated in the telephone survey, 155 with clinical salmonellosis (infected), 78 noninfected, and 15 with some symptoms but not clinical salmonellosis. One hundred one infected patients (65%) reported musculoskeletal symptoms, compared to 19 noninfected (24%) (adjusted relative risk = 2.60, 95% CI 1.73-3.90). Of the infected group, 16 ReA (incidence 10%, 95% CI 6.0-16.2), 7 enthesopathies, and 2 arthralgias were detected, and zero in the noninfected group. The risk factors for ReA were age, weight loss, and duration of diarrhea. Antibiotic treatment for the infection protected against symptoms of peripheral or axial arthritis (adjusted relative risk = 0.73, 95% CI 0.55-0.98)., Conclusion: The incidence of ReA and musculoskeletal symptoms after the infection was high. The use of antibiotics for S. hadar infection offered some protection against musculoskeletal symptoms.

Objective: To determine the incidence of idiopathic inflammatory myopathies (IIM) in Victoria, Australia, and look for evidence of space-time or spatial clustering., Methods: Cases of IIM diagnosed between 1989 and 1991 were identified by muscle biopsy and hospital discharge diagnosis review. Diagnosis was verified by medical record review and included if Bohan and Peter criteria for definite or probable disease were met. The pair-wise Euclidean distances between cases' residences were computed using grid references, and temporal distances were calculated between biopsy dates. The Mantel test for space-time clustering was computed. Each patient was also characterized by statistical local area (SLA) according to place of residence. For each SLA, the expected annual incidence of IIM was calculated, based upon its population distribution, and these were compared to the observed annual incidence. Confidence intervals for the true rate ratio (RR) for each SLA were calculated assuming a Poisson distribution, and the level of heterogeneity in the data was examined by calculation of a chi-squared for homogeneity., Results: Ninety-four cases met inclusion criteria for an annual incidence of 7.4 (95% CI 6.0-9.0) per million person-years. No space-time clustering was found (z = -0.434, p = 0.665), but there was evidence of spatial clustering. A total of 67 observed cases were distributed among 58 urban SLA. Four SLA had a greater than expected incidence of myositis (95% Poisson based CI excluded 1), accounting for 20 of the observed cases., Conclusion: The incidence of IIM in Australia is higher than most previous population based estimates. The finding of spatial clustering supports the hypothesis that environmental factors may be important in the pathogenesis of these diseases.

Objective: To study the incidence of systemic connective tissue diseases (CTD) and of systemic onset juvenile arthritis (S-JA) in children aged 0 to 15 years., Methods: A nationwide, prospective, hospital based series of new patients from Finland collected during a 4-year period and supplemented with data from the National Hospital Discharge Register. The population at risk was 1.02 million. Classification criteria developed for adult CTD and the American Rheumatism Association criteria for JA were used., Results: The annual incidence rates found were as follows: systemic lupus erythematosus 0.37, polymyositis/dermatomyositis (PM/DM) 0.30, mixed connective tissue disease 0.10, scleroderma 0.05, and S-JA 0.47/100,000. Girls outnumbered boys in the whole series of CTD (24:9) and also in S-JA (11:8), but in PM/DM the sex distribution was even., Conclusion: CTD and S-JA are rare before the age of 16 years. From the present incidence figures and those recently published for JA the incidence of CTD is estimated to be between 4 and 7% of that of JA, and S-JA accounts for only 2 to 4% of all JA.

One hundred and four patients with primary Sjögren's syndrome (SS) were evaluated for the presence (29%) or absence of Raynaud's phenomenon (RP). The clinical course of RP was, in general, benign and caused no vascular sequelae. In patients with primary SS having RP, nonerosive arthritis, vasculitis and pulmonary fibrosis were significantly more frequent than in those without RP. Myositis also appeared more frequently associated with RP, but the difference reached statistical significance only when combined in meta-analysis with 2 other comparable series. There were no differences in the autoantibody profiles of the 2 groups.

Inclusion body myositis is characterized by an insidious onset, progressive indolent course, and is generally felt to be refractory to standard therapy for myositis. We reviewed the charts of 32 patients with muscle biopsy findings suggestive of inclusion body myositis. The average time from symptom onset to diagnosis was 37 months, but initially 40% were incorrectly diagnosed. Twenty-eight patients (88%) were classified as definite or probable inclusion body myositis and were treated with various combinations of prednisone and immunosuppressive agents. Sixty-eight percent of those treated experienced a decrement in function and muscle strength. Three patients exhibited longterm improvement while 12 patients experienced delayed progression, defined by short term improvement in strength or a stable functional class, All of these patients received therapy, 5 in the form of methotrexate and prednisone. All untreated patients deteriorated clinically. In summary, (1) inclusion body myositis is a clinically distinct entity which is frequently misdiagnosed initially. (2) While clinical improvement with therapy is rare, our observations support recent reports that therapy may be associated with a slower rate of clinical progression. (3) Optimal therapy remains uncertain, but the use of low dose methotrexate and prednisone may warrant further study.

A black woman developed chronic myelopathy, polymyositis and synovitis. Although native of a nonendemic area the possibility of human T lymphotropic virus I association was considered. She tested positive and was serendipitously found to have been positive 16 years before. Risk factors were a transfusion and work as a serology technician. Sjögren's syndrome, alveolitis, gammopathy and immune dysfunction are other rheumatic manifestations of this virus.

The incidence of hospital diagnosed polymyositis-dermatomyositis (PM-DM) among residents of Pittsburgh and Allegheny County, PA was determined from 1963 to 1982. Medical records of all Allegheny County hospitals were searched for patients diagnosed PM-DM, and each accepted case was reviewed and verified by a physician, according to specified criteria. Direct age adjusted incidence rates were computed for race and sex, as new cases/million population/year, with the 1970 Allegheny County population as the standard. One hundred seventy-seven cases were identified during this 20-year survey for a total annual incidence of 5.5/million population. Incidence more than tripled during 1973-1982 compared to the first decade of study, with the greatest increase occurring in black women. Overall, the female to male incidence ratio was 2.2:1, but during the childbearing years (ages 15-44) this ratio increased to greater than 5:1 in both races. Detection of PM-DM is increasing, as shown by temporal trends. Although our series has significantly more adult PM and less DM and overlap cases than other published studies, annual incidence is similar to that reported in other locales.

In an attempt to delineate and compare the clinical, laboratory and epidemiologic features of polymyositis (PM) and dermatomyositis (DM) in children, the records of all 19 hospitals in Memphis and Shelby County, Tennessee, were surveyed over the 25-year period, 1948-1972. Seventeen documented cases of PM and 26 of DM were identified over the total period. PM was not diagnosed prior to 1962, while DM was found throughout the study. A significant female excess was observed in the PM group and in the combined patients 10-19 years of age. The only major differences found between PM and DM were the more acute and severe muscle involvement in DM and its cutaneous manifestations. Juvenile PM is a definite entity which should be differentiated from other myopathies because of its favourable response to corticosteroid therapy.

Utilizing case-control methods, we surveyed 322 persons who experienced the onset of polymyositis-dermatomyositis (PM-DM) during calendar years 1985 and 1986, in an effort both to test specific hypotheses, and to generate new hypotheses regarding antecedent risk associations for the disease. Data obtained by recall of events during the 12 months before disease onset in cases, and an identical time period in sex matched sibling controls, suggest that important questions for further study include the roles of antecedent heavy muscular exertion and emotional stress as risk factors for PM-DM. Furthermore, it appears that vaccinations, toxic exposures and symptoms of allergic phenomena, throat infection, and upper respiratory infection are either neutral or are negatively associated with risk of PM-DM, findings which also raise important new etiologic questions, or perhaps lay old ones to rest. Malignancy was infrequently noted.