Matteson, Eric L.

AbstractBackgroundThe epidemiology of cutaneous sarcoidosis is not well‐characterized as only referral‐based studies are available.ObjectivesTo characterize the epidemiology of cutaneous sarcoidosis, with emphasis on annual incidence and clinical characteristics, from 1976 to 2013.MethodsInception cohorts of patients with incident isolated cutaneous sarcoidosis and incident systemic sarcoidosis with cutaneous involvement in 1976–2013 in Olmsted County, Minnesota, United States were identified based on comprehensive individual medical record review. Inclusion in the isolated cutaneous sarcoidosis cohort required physician diagnosis and skin biopsy showing non‐necrotizing granuloma. Inclusion in the systemic sarcoidosis with cutaneous involvement cohort required presence of systemic sarcoidosis and cutaneous lesions. Presence of systemic sarcoidosis was determined by physician diagnosis supported by histopathology of non‐necrotizing granuloma, characteristic radiologic features of intrathoracic sarcoidosis and exclusion of other granulomatous diseases. Cutaneous lesions were defined as either sarcoidosis‐specific or non‐specific.ResultsThere were 62 cases with sarcoidosis‐specific cutaneous lesions (36 cases of sarcoidosis‐specific cutaneous lesions and 26 cases of isolated cutaneous sarcoidosis) which corresponded to an incidence of 1.9 per 100 000 population. The female to male ratio was 2.1 : 1. Plaques, papules and subcutaneous nodules were the most commonly observed cutaneous lesions. There was no significant difference in cutaneous presentation between those who had isolated skin disease and those who had skin disease in association with systemic sarcoidosis. Prognosis of cutaneous sarcoidosis was favourable, as over 90% of patients had a good response to either glucocorticoids, hydroxychloroquine or tetracycline antibiotics. This study has a significant limitation, in that the studied population was predominantly Caucasians who generally have a lower prevalence of skin disease.ConclusionsThe incidence of sarcoidosis‐specific cutaneous lesions was about 1.9 per 100 000 population with female predominance. The cutaneous presentations were similar among those with and without systemic sarcoidosis.

BackgroundFactors associated with dissection from inflammatory aortic aneurysms may be different from those in the general population.ObjectiveThe aim of this study was to evaluate the risk factors for aortic dissection/rupture in patients with giant cell arteritis (GCA) and aortic aneurysms.MethodsA population-based incident cohort of patients with a diagnosis of GCA from 1950 to 2004 was used. All patients with aortic aneurysms diagnosed 1 year prior to GCA diagnosis or any time thereafter were included. Cox proportional hazard models were used to evaluate risk factors for aortic dissection/rupture.ResultsThe study included 33 patients (91% women) with GCA and aortic aneurysms. Mean age at diagnosis of aortic aneurysm was 83.6 years. There were 27 thoracic aneurysms and 19 abdominal aneurysms. Eight patients developed aortic dissection/rupture (both thoracic and abdominal aorta in 5 cases, thoracic aorta only in 2 cases, and isolated abdominal aorta in 1 case).Older age (hazard ratio [HR], 0.27 per 10 years; 95% confidence interval [CI], 0.09–0.86) and later calendar year at diagnosis of aortic aneurysm (HR, 0.29 per 10 years; 95% CI, 0.13–0.69) were associated with decreased risk of dissection/rupture. Size of the thoracic aneurysm (HR, 1.17; 95% CI, 0.69–1.99) was not associated with dissection/rupture. Histopathology showed active aortitis in 4 of 7 patients with aortic dissection/rupture compared with 0 of 7 patients with aortic aneurysm without dissection/rupture.ConclusionsAneurysm size was not a predictor of aortic dissection/rupture in this cohort of patients with GCA. The higher frequency of active aortitis in patients with dissection suggests that active inflammation may play a role.

Objective To investigate the risk of malignancy in patients with sarcoidosis in a population‐based cohort. Methods A cohort of Olmsted County, Minnesota, residents diagnosed with sarcoidosis between January 1, 1976 and December 31, 2013 was identified based on individual medical record review. For each sarcoidosis subject, 2 sex‐ and age‐matched comparator subjects without sarcoidosis were randomly selected. Cases and comparators were then cross‐indexed with the Mayo Clinic Cancer Registry, which collected data on every type of malignancy except for nonmelanoma skin cancer, for malignancy ascertainment. Results A total of 345 incident cases of sarcoidosis and 690 comparators were identified. There was no difference in the prevalence of malignancy at the index date between the 2 groups (4.3% among cases and 4.3% among comparators; P = 1.0). During followup, 36 patients with sarcoidosis and 91 subjects without sarcoidosis developed malignancy, with a cumulative incidence at 10 years of 3.8% and 7.1%, respectively. The difference corresponded to a hazard ratio (HR) of 0.72 (95% confidence interval [95% CI] 0.49–1.06). The cumulative incidences at 10 years for individual types of malignancy were also similar between the 2 groups, with nonsignificant HRs. However, subgroup analysis found that cases with extrathoracic involvement were at higher risk of incident hematologic malignancy compared with cases without extrathoracic involvement (HR 1.87 [95% CI 1.09–3.22]). Conclusion Risk of malignancy was similar among patients with sarcoidosis compared to nonsarcoidosis subjects. However, the risk of incident hematologic malignancy was significantly higher among patients with sarcoidosis with extrathoracic involvement compared to patients without extrathoracic disease.

ObjectiveTo compare health care planning models forecasting rheumatology workforce requirements in western countries.MethodsA systematic literature review was conducted through medical databases (Ovid MEDLINE, Embase, CINAHL, and Cochrane Library) and the grey literature. All articles reporting a rheumatology workforce model were included.ResultsThe search yielded 6,508 articles, and 14 publications (on 12 studies) were included. Workforce models were available for the US (n = 3), Canada (n = 3), the US plus Canada (n = 1), Germany (n = 2), Spain (n = 1), and the UK (n = 2). The number of rheumatologists required to serve a population of 100,000 people was calculated, with a range of 0.7 (UK, calculated for 1988) to 3.5 (Spain, calculated for 2021). Most models used a needs‐based approach (n = 6); 3 studies each applied a supply‐ or demand‐based method. The following variables were considered by ≥1 model: disease prevalence, patients' referral to rheumatologists, clinical visits/patient/year, population development, factors influencing performance of rheumatologists, patient flow/care sharing, and medical technologies/infrastructure development.ConclusionHeterogeneity in methods used, the period or calendar years for which the estimates were projected, and heterogeneity of variables evaluated led to disparate estimates, with results ranging from 0.7 to 3.5 rheumatologists per 100,000 population. An international initiative is needed to agree upon a common approach for a reliable estimation of manpower requirements in rheumatology.

ObjectiveIt is unclear whether isolated cutaneous lupus erythematosus (CLE) affects cardiovascular risk. We estimated the cumulative incidence and mortality of cardiovascular diseases in a population‐based CLE cohort and compared the risk with a matched non‐CLE cohort.MethodsAll incident cases of CLE in Olmsted County, Minnesota, between 1965 and 2005 were followed until December 2013. The cumulative incidence of cerebrovascular accidents (CVAs [including stroke and transient ischemic attack]), ischemic heart disease (IHD [including coronary artery disease, myocardial infarction, and angina pectoris]), heart failure, and peripheral arterial disease (PAD) was derived and compared to an age‐, sex‐, and calendar year–matched non‐CLE cohort using Cox models.ResultsThere were 155 patients with CLE (mean ± SD age at diagnosis 48 ± 16 years, 65% female, mean ± SD BMI 26.3 ± 7.1 kg/m2, 40% smokers, 9% with diabetes mellitus). During a median followup of 14.6 years, 41 CLE patients had cardiovascular events (15 patients with CVAs, 32 patients with IHD), with a 20‐year cumulative incidence of 31.6%. As compared to non‐CLE subjects, the risk of CVAs (smoking‐adjusted hazard ratio [HR] 2.97 [95% confidence interval (95% CI) 1.13–7.78]) and PAD (HR 2.06 [95% CI 0.99–4.32]) was increased in patients with CLE, but the risk of IHD was not increased (HR 0.94 [95% CI 0.57–1.54]). There was no increase in cardiovascular mortality (HR 1.68 [95% CI 0.76–3.75]). The magnitude of risk for any cardiovascular outcome was not significantly influenced by the extent of cutaneous involvement.ConclusionCLE may be associated with an increased risk of CVAs and PAD, but not IHD. Factors contributing to increased CVA risk in patients with CLE merit evaluation.

ROS deficiency promotes proinflammatory T cells in rheumatoid arthritis.

ObjectiveTo characterize the epidemiology of mixed connective tissue disease (MCTD) from 1983 to 2014.MethodsAn inception cohort of patients with incident MCTD in 1985–2014 in Olmsted County, Minnesota was identified based on comprehensive individual medical record review. Diagnosis of MCTD required fulfillment of at least 1 of the 4 widely accepted diagnostic criteria without fulfillment of classification criteria for other connective tissue diseases. Data were collected on demographic characteristics, clinical presentation, laboratory investigations, and mortality.ResultsA total of 50 incident cases of MCTD were identified (mean age 48.1 years and 84% were female). The annual incidence of MCTD was 1.9 per 100,000 population. Raynaud's phenomenon was the most common initial symptoms (50%), followed by arthralgia (30%) and swollen hands (16%). The diagnosis was frequently delayed with the median time from first symptom to fulfillment of criteria of 3.6 years. At fulfillment of criteria, arthralgia was the most prevalent manifestation (86%), followed by Raynaud's phenomenon (80%), swollen hands (64%), leukopenia/lymphopenia (44%), and heartburn (38%). Evolution to other connective tissue occurred infrequently with a 10‐year rate of evolution of 8.5% and 6.3% for systemic lupus erythematosus and systemic sclerosis, respectively. The overall mortality was not different from the general population with a standardized mortality ratio of 1.1 (95% confidence interval 0.4–2.6).ConclusionThis study was the first population‐based study of MCTD to provide a complete picture of epidemiology and clinical characteristics of MCTD. MCTD occurred in about 2 persons per 100,000 per year. Evolution to other connective diseases occurred infrequently and the mortality was not affected.