IgG4-related disease-rare but you should not forget it.

Immunoglobulin G4-related disease is a systemic immune-mediated disease with insidious evolution characterized by fibroinflammatory lesions over virtually any organ system. Despite the remarkable progression of knowledge, its etiology remains undefined. Due to its relapse-remitting pattern, it could accumulate irreversible damage, increasing comorbidities and mortality. This paper emphasizes key concepts for diagnosing and treating patients with this condition.

Effects of an educational intervention for rational cardiac enzyme requisitions in critically ill patients: a pre-post intervention study.

BACKGROUND: Although cardiac troponin is recommended as the biomarker of choice to evaluate myocardial injury, inappropriate low-value ordering practice is frequent, particularly routine ordering of creatine kinase-myocardial band (CK-MB) tests where troponin is available. OBJECTIVE: The aim of this study was to evaluate the impact of an educational intervention for rational request of cardiac biomarkers in the intensive care unit. METHOD: We conducted a quasi-experimental, pre-post implementation study of an educational program (expository-dialogue presentation and disclosure of a decision algorithm) for rational cardiac biomarker testing in adult critically ill patients. The study was divided into two 12-month periods: pre-intervention (September 2017-August 2018) and post-intervention (October 2018-September 2019). An interrupted time series with a segmented regression model was applied to analyze variation over time in CK-MB and troponin testing. RESULTS: We included 4429 patients: 2181 patients in the pre-intervention period and 2248 patients in the post-intervention period. A reduction in the concomitance of CK-MB and troponin testing was observed (concomitance in 1415 tests in the pre-intervention period vs 348 tests in the post-intervention period). The interrupted time series analysis demonstrated a noticeable immediate reduction in the concomitance of CK-MB with troponin after the intervention (-0.13 tests per patient, P = 0.0016) but not in the secular trend for the concomitance. The proportion of patients with the acute coronary syndrome as a discharge diagnosis was not different between the pre- and post-intervention period. CONCLUSION: Our pre-post interventional study demonstrated a significant decrease in the concomitance of CK-MB and troponin tests. A rational high-value ordering practice of cardiac biomarkers is possible in critically ill patients and might be suitable for educational interventions.

Leflunomide in Takayasu arteritis - A long term observational study.

OBJECTIVE: To evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. METHODS: An open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). RESULTS: The mean follow up time was 43.0±7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among TA patients who had changed leflunomide to another agent, two had an additional clinical relapse and needed to change therapy. CONCLUSION: Leflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

Leflunomide in Takayasu arteritis - A long term observational study. / Leflunomida na arterite de Takayasu - Estudo observacional de longo prazo.

OBJECTIVE: To evaluate the extended follow-up data on efficacy and toxicity of leflunomide therapy in Takayasu arteritis (TA) patients previously enrolled in the original open-label study of short-term effects of leflunomide in TA. METHODS: An open-label long-term longitudinal study was performed in TA patients who fulfilled the 1990 American College of Rheumatology criteria for TA and had participated in a previous study that evaluated short-term efficacy of leflunomide in TA. Complete follow-up information could be retrieved from 12 out of 15 patients enrolled in the original study. Disease activity was evaluated by Kerr's criteria and by the Indian Takayasu Activity Score 2010 (ITAS2010). RESULTS: The mean follow up time was 43.0±7.6 months and 5 (41.6%) TA patients remained on leflunomide therapy while 7 (58.3%) TA patients had to change to another therapy due to failure to prevent relapses in 6 patients and toxicity in one patient. No significant differences were found between patients who remained on leflunomide therapy and those who changed to another agent regarding age at study entry, time since diagnosis, prednisone daily dose at study entry, baseline ITAS2010, mean or maximum ESR and CRP, and cumulative prednisone dose at study end. Among two TA patients who had changed laflunomide to another agent, two had a clinical relapse and needed to change therapy. CONCLUSION: Leflunomide led to sustained remission in approximately half of patients at a mean time of 12 months and was well tolerated by TA patients.

Homocysteine levels in Takayasu arteritis -- a risk factor for arterial ischemic events.

OBJECTIVE: To evaluate homocysteine levels in patients with Takayasu arteritis (TA) and in controls, and to analyze associations between homocysteine levels and paraoxonase 1 (PON1) activity, cysteine levels, methotrexate use, disease activity, extent of arterial involvement, and ischemic events in patients with TA. METHODS: A cross-sectional study was performed with 29 patients with TA and 30 controls who underwent clinical evaluation and blood sample collection in the fasting state. RESULTS: Among patients with TA, active disease was observed in 9 (31.0%) and previous arterial ischemic events in 10 (34.5%). Therapy with methotrexate was prescribed to 9 (31.0%) patients and it was associated with folic acid in 8 cases. Median homocysteine level was higher in patients with TA [10.9 µmol/l, interquartile range (IQR) 9.6-14.8] than in controls (6.9 µmol/l, IQR 5.1-11.9; p < 0.001). No difference was found regarding mean homocysteine levels between those using methotrexate and those under other therapies (12.8 ± 5.3 µmol/l vs 12.1 ± 3.2 µmol/l, respectively; p = 0.662). TA patients with active disease presented lower homocysteine levels (10.4 ± 2.1 µmol/l) compared to TA patients in remission (13.1 ± 4.2 µmol/l) (p = 0.034). A significant correlation was found between cysteine and homocysteine levels in patients with TA (ρ = 0.676, p < 0.0001), while there was no correlation between homocysteine and PON1 activity (ρ = 0.214, p = 0.265). Median homocysteine levels were higher in patients with ischemic events (13.2 µmol/l, IQR 10.9-17.5) compared to patients with no ischemic events (9.8 µmol/l, IQR 8.7-14.7; p = 0.027) and were associated with arterial ischemia in patients with TA (OR 1.31, 95% CI 1.01-1.71, p = 0.041). CONCLUSION: Patients with TA presented higher homocysteine levels than controls and homocysteine was associated with an increased risk of arterial ischemic events in TA.