Association between the Lung Immune Prognostic Index and mortality in patients with idiopathic inflammatory myopathy-associated interstitial lung disease

Objective:To explore the association between the Lung Immune Prognostic Index(LIPI)and 1-year all-cause mortality in patients with idiopathic inflammatory myopathy related interstitial lung disease(IIM-ILD).Methods:Patients who were diagnosed with IIM-ILD at West China Hospital,Sichuan University from January 2008 to December 2021 were retrospectively included and categorized into three groups based on LIPI.Univariable and multivariable Cox proportional hazards models were conducted to explore potential association between the LIPI and patients'mortality.Results:A total of 1116 patients were screened,and 830 were included in this study.The multivariable Cox analysis showed that,compared with patients with poor LIPI,the hazard ratio(HR)for all-cause 1-year mortality was 0.22(95%CI 0.05-0.93,P=0.04)for patients in the good LIPI group(LDH<250 IU/L and dNLR<3).After excluding patients lost to follow-up within one year,a similar result was found for LIPI(HR 0.20,95%CI 0.05-0.86;P=0.03).Conclusions:Good LIPI was independently associated with decreased risk of all-cause 1-year mortality in patients with IIM-ILD.This easy-to-obtain index might be served as a potential marker for assessing the prognosis of IIM-ILD.

EMG STUDY IN THE DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS OF LIPID STORAGE MYOPATHY

Clinical, electromyographic and pathological features were studied in 18 patients with lipid storage myopathy (group I ) and 18 patients with polymyositis and dermatomyositis (group II). The results showed a remarkable lower spontaneous activity(SA) incidence (14% ) in group I than that (55% ) in group II; 46% and 34% short-duration motor unit potentials (MUAPs) with polyphasic potentials and 74% and 71% short-duration MUAPs without polyphasic potentials respectively; the percentages of increased polyphasic MUAPs were same in the two groups. The reduced or pathologic interference patterns accounted for 61 % in the group I and 50% in group II. Increased CPK, LDH and HBD were also found in both of them. It is suggested that the lipid storage myopathy may be diagnosed when patients have muscle weakness and myalgia with short-duration and low-amplitude and polyphasic MUAPs without or with occasional spontaneous activities, and increased CPK, LDH and HBD.

Pain and Fatigue Perceptions of Patients with Systemic Autoimmune Myopathies before and during the COVID-19 Pandemic Period

Objective: To assess general pain and fatigue and their association with the disease status of patients with systemic autoimmune myopathies (SAMs), in terms of the impact of the COVID-19 pandemic on these parameters. Methods: This is a cross-sectional and prospective cohort study that included 72 patients with SAMs who were matched by age and gender with 67 healthy individuals. The patients engaged in an interview via a validity survey to measure disease status, and assess a visual analog scale (VAS) for pain and fatigue in two periods: before (phase I) and during (phase II) of the Brazilian COVID-19 pandemic. For cross-sectional analysis, patients’ data from phase I were compared to healthy individuals’ information, whereas for prospective analysis, data of the patients who were assessed in phase II were compared to the same patients’ data from phase I. Results: The patients had significantly more pain perception, comparable fatigue perception and fatigue severity, when compared to the healthy individuals. During the COVID-19 pandemic period, the disease activity, pain and fatigue perceptions, and fatigue severity remained unchanged. Despite this, the pain and fatigue perceptions correlated significantly with patients’ VAS, the patient health outcome, and fatigue severity. In addition, the pain perception correlated to creatine phosphokinase, whereas fatigue perceptions correlated to physicians' VAS. Conclusions: The study showed that patients with SAMs have significantly increased pain perceptions compared to healthy individuals. During the COVID-19 pandemic period, the pain and fatigue perceptions remained unchanged in patients with SAMs but they correlated to several disease status parameters.

Phenotypical statin-associated immune-mediated necrotizing myositis with histological features of inclusion body myositis

Introduction:Statin-associated immune-mediated necrotizing myositis(IMNM)is a rare but distinct idiopathic inflammatory myopathy(IIM)that requires early recognition and intervention to prevent irreversible muscle damage.It is typically characterized by active statin use,elevated creatinine kinase(CK)levels,proximal muscle weakness,and at times,a positive 3-hydroxy-3-methyl-glutaryl-coenzyme A reductase(HMGCR)antibody.Treatment includes immediate discontinuation of the statin and may include corticosteroids,intravenous immunoglobulin(IVIG),and/or immunosuppressive therapy.Inclusion body myositis(IBM),another distinct IIM,also presents with elevated CK levels but with insidious onset of distal upper and proximal lower extremity weakness and is typically refractory to treatment.Case Description:A 64-year-old female patient presented with proximal muscle weakness,elevated CK levels,and a positive HMGCR antibody in the setting of statin use with muscle pathology suggestive of both statinassociated IMNM and IBM.She responded to subcutaneous methotrexate and a slow prednisone taper over several months,however,will require close monitoring for symptoms associated with either disease.Conclusion:In conclusion,we report a case of muscle weakness with muscle pathology demonstrating both statin-associated IMNM and IBM.This case highlights the importance of understanding the clinical and pathological features of statin-associated IMNM and IBM.