Aim To investigate the risk factors for interstitial lung disease (ILD) and prognosis in patients with idiopathic inflammatory myopathy (IIM). Methods A retrospective longitudinal study was performed in patients diagnosed with IIM between January 2012 and December 2018. Results The study cohort included 91 men and 195 women who were classified as having dermatomyositis (DM, n = 183), polymyositis (PM, n = 77), or clinical amyopathic DM (CADM, n = 26). ILD was identified in 46.5% (n = 133) of patients with IIM. The independent risk factors for ILD were age at disease onset, presence of anti‐Ro‐52 antibody, Gottron's papules, elevated serum immunoglobulin M levels and hypoalbuminemia. Older age at disease onset, ILD, malignancy, and increased serum aspartate aminotransferase and neutrophil‐to‐lymphocyte ratio (NLR) were identified as the independent predictors for mortality, whereas elevated serum albumin level was associated with a better prognosis. A total of 73 deaths (25.5%) occurred after a median follow‐up time of 33 months. Infection (49.3%) was the leading cause of death. In the overall cohort, the 1‐year, 5‐year and cumulative survival rates were 83.2%, 74.2% and 69.4%, respectively. The receiver operating characteristic curve indicated that the optimal cut‐off value of NLR for predicting death in IIM was 6.11. Conclusion IIM patients have a poor prognosis with substantial mortality, especially in patients who have older age at onset, ILD, malignancy and higher NLR. Close monitoring and aggressive therapies are required in patients having poor predictive factors.
Objectives: In this study, we aimed to assess the value of therapeutic plasma exchange (TPE) in the treatment of rheumatic diseases and compare the safety of different replacement fluids used in TPE. Patients and methods: A total of 727 TPE procedures in 285 patients (57 males, 228 females; mean age: 39.7±15.4 years; range, 13 to 79 years) with rheumatic diseases between January 2011 and February 2019 were retrospectively analyzed. Data including demographic and clinical characteristics of the patients were recorded. Treatment response to TPE and adverse events were evaluated in all patients. Results: Indications for TPE included 13 different disorders, with the majority being systemic lupus erythematosus (up to 50%). The mean number of TPE sessions was 2.55±1.00 per patient and the mean exchange plasma volume was 2,270±256 mL per session. Combined plasma and albumin was the most frequently used replacement fluid (69.5%), followed by albumin and plasma in 20.5% and 10.0% of episodes, respectively. Up to 73.7% (210/285) patients achieved clinical improvement after TPE treatment. Adverse events occurred in 15.1% (110/727) of all the procedures, and allergic reaction (34.5%) was the most common event. The overall incidence rate of complication was similar among the three types of replacement fluids (p=0.214). Conclusion: Based on our study results, TPE is an invasive, but safe, useful and, sometimes, essential tool with an acceptable risk/benefit ratio for most rheumatic diseases. Albumin can be used as a feasible substitute for plasma in case of shortage of blood resources.
ObjectiveTo examine the efficacy of tacrolimus on top of glucocorticoids (GCs) in the management of idiopathic inflammatory myopathies-associated interstitial lung disease (IIM-ILD) and further assess the therapeutic benefit and safety of low-dose pirfenidone followed above treatments.MethodsThe retrospective study comprised 250 patients with IIM-ILD hospitalized in Tongji Hospital from 2014 to 2020. Demographic data, survival outcomes, and recurrence rates over the 1-year follow-up period were retrospectively analyzed. These patients were divided into two groups based on treatment with tacrolimus alone or other conventional immunosuppressants. Endpoints were compared by adjusted Cox regression model using inverse probability of treatment weighting to minimize treatment bias and potential confounders. For the prospective study, IIM-ILD patients treated with tacrolimus alone or tacrolimus combined with low-dose pirfenidone were enrolled from 2018 to 2020. Clinical characteristics, survival outcomes and multifarious assessment scales were followed up at baseline, 3, 6 and 12 months. The primary endpoint was 12-month survival rate and the secondary endpoints included respiratory-related events, adverse events, exacerbation in HRCT findings and laboratory parameters during therapy courses, and changes in respiratory function.ResultsFor the retrospective study, tacrolimus group (n=93) had a significantly higher survival rate (weighted HR=0.330, p=0.002) and a lower relapse rate (weighted HR=0.548, p=0.003) compared with patients treated with other types of immunosuppressant (n=157) after adjustment. The prospectively enrolled 34 IIM-ILD patients were treated with tacrolimus (n=12) or tacrolimus combined with low-dose pirfenidone (n=22). After 12 months of treatment with tacrolimus, patients in the prospective cohort showed significant improvements in cardio-pulmonary function, disease activity, muscle strength, and mental scale from baseline. Subgroup analysis indicated that patients with tacrolimus and pirfenidone combination therapy showed lower chest HRCT scores (p=0.021) and lower respiratory-related relapse rates than those in tacrolimus monotherapy group (log-rank p=0.0029). The incidence rate of drug-associated adverse events (AEs) was comparable between two groups and none of the patients discontinued the treatment due to severe AEs.ConclusionTacrolimus is well-tolerated and effective in the treatment of IIM-ILD. Furthermore, low-dose pirfenidone add-on treatment seems result in favorable improvements in pulmonary involvements for IIM-ILD patients.Clinical Trial Registrationhttp://www.chictr.org.cn, identifier ChiCTR2100043595.
Objective: To systemically investigate the prevalence and risk factors of monoclonal gammopathy (MG) in patients with autoimmune inflammatory rheumatic disease (AIIRD). Methods: A literature search was conducted using databases of PubMed, EMBASE and Web of Science for relevant studies from inception to July 31, 2021. The pooled prevalence, odds ratio (OR), weighted mean difference (WMD) and 95% confidence interval (CI) were calculated with Stata 16.0 using a random or fixed effects model. Results: In 17 included studies involving 6667 AIIRD patients, the pooled prevalence of MG in AIIRD patients was 7% (95%CI: 0.06-0.09). Compared to general populations, patients with Sjögren’s syndrome (pSS) possessed the highest risk for MG (OR 4.51; 95%CI: 3.39-5.74), followed by systemic lupus erythematosus (OR 3.99; 95%CI: 2.84-5.14), ankylosing spondylitis (OR 2.04; 95%CI: 1.11-2.97), and rheumatoid arthritis (OR 2.00; 95%CI: 1.79-2.22). Older age (WMD=5.17 years; 95%CI: 0.68-9.66), higher erythrocyte sedimentation rate (WMD=14.04 mm/H; 95%CI: 7.77-20.30), higher serum gammaglobulins level (WMD=1.92 mg/dL, 95%CI: 0.51-3.32) were associated with a greater risk of MG in AIIRD patients. Conclusions: Monoclonal gammopathy prevalence was higher in AIIRD patients, especially in pSS patients. Older age, higher ESR, and hypergammaglobulins were risk factors for MG in AIIRD patients.
Objective. To examine the efficacy of tacrolimus on top of glucocorticoids (GCs) in the management of idiopathic inflammatory myopathies-associated interstitial lung disease (IIM-ILD) and further assess the therapeutic benefit and safety of low-dose pirfenidone followed above treatments. Methods. IIM-ILD patients hospitalized in Tongji Hospital from 2014 to 2020 were included. Demographic data, survival outcomes, and recurrence rates over the 1-year follow-up period were retrospectively analyzed. In the retrospective study, patients were divided into two groups based on treatment with tacrolimus or other conventional immunosuppressants. For the prospective study, a total of 34 IIM-ILD patients either treatment-naïve or after a sufficient period of washout were enrolled from 2018 to 2020 and treated with tacrolimus alone (n=12) or in combination with low-dose pirfenidone (n=22). Clinical characteristics, survival outcomes and multifarious assessment scales were followed up at baseline, 3, 6 and 12 months. The primary endpoint was 12-month survival rate and the secondary endpoints included respiratory-related events, adverse events, exacerbation in HRCT findings and laboratory parameters during therapy courses, and changes in respiratory function.Results. A total of 250 IIM-ILD patients followed up for over 1 year were identified. Tacrolimus group (n=93) had a significantly higher survival rate (HR=0.428, p=0.018) and a lower relapse rate (HR=0.587, p=0.009) compared with patients with other types of immunosuppressant (n=157) after adjustment. The prospectively enrolled 34 IIM-ILD patients were treated with tacrolimus with or without combination with low-dose pirfenidone. After 12 months of treatment with tacrolimus, patients showed significant improvements in cardio-pulmonary function, disease activity, muscle strength, and mental scale. Sub-group analysis indicated that patients with tacrolimus+pirfenidone combination therapy showed better chest HRCT scores and lower respiratory-related relapse rates than those in tacrolimus only group (log-rank p=0.0029). The incidence rate of drug-associated adverse events (AEs) was comparable between two groups and none of the patients discontinued the treatment due to severe AEs.Conclusion. Tacrolimus is well-tolerated and effective in improving both myositis and lung involvements. Furthermore, low-dose pirfenidone add-on treatment seems could result in multidimensional improvements in IIM-ILD patients. Trial registration. Chinese clinical trial Register, http://www.chictr.org.cn, ChiCTR2100043595.
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