Michet, Clement J.

AbstractInflammatory processes, such as an infection or drug reaction, can cause antineutrophil cytoplasmic autoantibody (ANCA)‐associated vasculitis (AAV). Although quite rare, AAV may occur with SARS‐coronavirus disease 2019 (COVID‐19) antigenic exposure, either from infection or immunization. We present two cases of AAV: one that developed after COVID‐19 infection presenting as diffuse alveolar haemorrhage and another that developed shortly after vaccination, presenting as granulomatous pulmonary nodules. Both patients improved with supportive care and immunosuppressive therapies. This adverse event appears to be a very rare complication of COVID‐19 infection or vaccination. Early diagnosis of AAV is important because immunosuppressive therapy may improve patient outcomes.

ObjectiveTo identify longitudinal predictors of discordance between patients with rheumatoid arthritis (RA) and their health care providers, where patient global assessment of disease activity is substantially higher than provider global assessment.MethodsThis retrospective case–control study included 102 cases with positive discordance (i.e., ≥25 mm between patient and provider global assessments) and 102 controls without discordance who were matched for age, sex, RA duration, and Clinical Disease Activity Index (CDAI) score. Data were collected at the baseline visit (date of diagnosis or earliest available visit), the index visit (participation in a previous cross‐sectional study), and at up to 11 additional visits before the index visit. Data included patient characteristics, disease activity measures, Disease Activity Score in 28 joints (3‐variable) using the C‐reactive protein level (DAS28‐CRP), and medications. Data were analyzed by using linear and logistic regression models with smoothing splines for nonlinear trends.ResultsOverall, the mean age was 63 years, 75% of patients were female, and the mean RA duration was 10 years. Compared with controls, cases had higher rates of discordant visits during the 4 years before the index visit, and they had a higher CDAI score and DAS28‐CRP earlier in the disease course. Cases more frequently had antinuclear antibodies, nonerosive disease, prior depression, or prior use of antidepressants or fibromyalgia medications. Disease‐modifying medication use was not different between cases and controls.ConclusionThe findings inform new hypotheses about the relationships of disease activity and antinuclear antibodies to the later occurrence of positive discordance among patients with RA.

ObjectiveTo evaluate the incidence and prevalence of juvenile idiopathic arthritis (JIA) in Olmsted County, Minnesota in 1994–2013 and trends in juvenile rheumatoid arthritis (JRA) in 1960–2013.MethodsCases of arthritis in 1994–2013 were identified by diagnosis code with medical chart review to confirm diagnosis separately for JIA and JRA. Overall incidence rates with 95% confidence intervals (95% CIs) were age and sex adjusted to the 2010 US white population. Comparisons were made with an earlier (1960–1993) cohort from this same population.ResultsSeventy‐one incident cases of JIA in 1994–2013 were identified, with an overall age‐ and sex‐adjusted incidence rate of 10.3 per 100,000 (95% CI 7.9–12.7). Forty‐two (59%) were female, with an incidence of 12.4 per 100,000 (95% CI 8.6–16.2), as compared to 8.3 per 100,000 (95% CI 5.2–11.3) in males. The most common subtype was oligoarthritis (63%). The mean ± SD age at diagnosis was 8.2 ± 5.3 years. The prevalence of JIA on January 1, 2000 and January 1, 2010 was 51.0 per 100,000 (95% CI 25.2–76.8) and 57.6 per 100,000 (95% CI 31.0–94.5), respectively. When the annual incidence of JRA was compared over time from 1960 to 2013, there was no significant change in incidence overall; however, the incidence decreased among females (P = 0.003). A cyclic pattern of incidence was observed, with peaks approximately every 10 years. Similar to the findings with regard to incidence, prevalence did not change overall, but decreased among females (P = 0.048). There were 4 deaths in the cohort of JRA patients diagnosed in 1960–2013; the standardized mortality ratio was 1.50 (95% CI 0.41–3.83).ConclusionIncidence of juvenile arthritis overall in Olmsted County, Minnesota has not changed significantly in the past 53 years. A consistent cyclic pattern was noted.

ObjectiveTo determine trends in the incidence and clinical presentation of ankylosing spondylitis (AS), the incidence of cardiovascular disease (CVD), and cardiovascular (CV) risk factors among patients with AS and compare the observed incidence of CVD with that predicted by the Framingham Risk Score (FRS).MethodsA population‐based inception cohort of residents of Olmsted County, Minnesota age ≥18 years who fulfilled Modified New York Criteria for AS in 1980–2009 was assembled. Clinical features at presentation were recorded. Age‐ and sex‐adjusted incidence rates and survival were estimated. Incident CVD and CV risk factors were identified. The 10‐year CVD risk was calculated using the FRS. Standardized incidence ratios (ratios of observed CVD in AS to that predicted by the FRS) were calculated.ResultsEighty‐six patients were diagnosed with AS over the study period with an age‐ and sex‐adjusted incidence of 3.1 per 100,000 (95% confidence interval [95% CI] 2.5–3.8). The mean age at diagnosis was 35 years (range 19–69 years). Inflammatory back pain, seen in 90% of patients, was the most common presenting manifestation. The 10‐year mean ± SD cumulative incidence of CVD was 15.8% ± 6.1%, three times higher than the predicted events based on the FRS (standardized incidence ratio 3.01, 95% CI 1.35–6.69; P = 0.007). Overall survival was similar to the general population.ConclusionAS occurs in approximately 3 persons per 100,000 per year. Clinical features, extraarticular manifestations, and interval from symptom onset to diagnosis have remained constant in this population over the study period. The CVD risk in these patients is higher than expected and underestimated by the FRS.

Objective Epidemiologic studies comparing the incidence and prevalence of systemic lupus erythematosus (SLE) and isolated cutaneous lupus erythematosus (CLE) are few. Olmsted County, Minnesota provides a unique setting for such a study owing to resources of the Rochester Epidemiology Project. We sought to describe and compare the incidence and prevalence of SLE and CLE from 1993–2005. Methods SLE cases were identified from review of medical records and fulfilled the 1982 American College of Rheumatology classification criteria. CLE cases included patients with classic discoid lupus erythematosus, subacute CLE, lupus panniculitis, and bullous lupus erythematosus. Age‐ and sex‐adjusted incidence and prevalence were standardized to the 2000 US white population. Results The age‐ and sex‐adjusted incidence of SLE (2.9 per 100,000; 95% confidence interval [95% CI] 2.0–3.7) was similar to that of CLE (4.2 per 100,000; 95% CI 3.1–5.2, P = 0.10). However, the incidence of CLE was 3 times higher than SLE in men (2.4 versus 0.8 per 100,000; P = 0.009). The age‐ and sex‐adjusted prevalence of CLE on January 1, 2006 was higher than that of SLE (70.4 versus 30.5 per 100,000; P < 0.001). The prevalences of CLE and SLE in women were similar, but the prevalence of CLE was higher in men than in women (56.9 versus 1.6 per 100,000; P < 0.001). The incidence of CLE rose steadily with age and peaked at 60–69 years. Conclusion The incidences of CLE and SLE are similar, but CLE is more common than SLE in men and in older adults. These findings may reflect differences in genetic or environmental etiology of CLE.

We present a case of systemic granulomatous disorder/Blau syndrome. A patient was seen at our clinic with a diagnosis of Juvenile Idiopathic Arthritis (JIA). He was diagnosed with polyarticular JIA when he was two years old, at that time primary manifestations included inflammation of the hand and wrist joints bilaterally, later he developed ocular symptoms, which were attributed to JIA. He had liver, skin, pulmonary manifestations, and diagnostic workup including biopsy revealed granulomatous inflammation of these sites. During the diagnostic workup, he had worsening of ocular complaints, retinal exam showed panuveitis with multifocal choroiditis. These ocular findings are not seen in JIA, this, along with his other systemic manifestations, led us to revisit the diagnosis. Laboratory testing for genetic mutation for Blau syndrome was done and came back positive. Now all of his systemic findings were placed under one umbrella of systemic granulomatous syndrome/Blau syndrome. Due to worsening of ocular manifestations, he was started on Adalimumab with marked improvement of ocular and systemic manifestations and is followed by team that consists of Rheumatologist, Ophthalmologist, and Gastroenterologist.