Polymyositis
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Publication
Journal: Asia Pacific Allergy
December/12/2021
Abstract
Human herpesvirus 6 (HHV-6) was initially labeled as a human B lymphotropic virus because it was isolated in patients diagnosed with lymphoproliferative disorders. There are two variants of HHV-6: HHV-6A and HHV-6B. A considerable majority of recorded primary infections and reactivation events are primarily due to HHV-6B. We report a case of HHV-6 encephalitis reactivation in a 75-year-old Caucasian diabetic female with a past medical history of polymyositis treated with prednisone for a long time who presented with generalized weakness and drowsiness. She developed her symptoms after contact with her grandchildren, who recently had viral-like symptoms treated with antibiotics. Magnetic resonance imaging (MRI) of the brain without contrast showed 14 mm high transverse relaxation time (T2)/fluid-attenuated inversion recovery (FLAIR) signal intensity focus on the left temporal lobe, suspicious for primary versus metastatic neoplasm. Cerebrospinal fluid analysis found that protein concentration was 75 mg/dl, glucose concentration 55 mg/dl, white blood cell count was 22/mm3, with a lymphocytic predominance. Meningitis/encephalitis polymerase chain reaction (PCR) panel detected HHV-6. She was discharged after treatment with ganciclovir for 14 days. It is crucial to recognize HHV-6 infections in immunocompromised patients who present with a T2/FLAIR signal intensity focus in the left temporal lobe. In a hospital setting, rapid HHV-6 encephalitis testing is important to make a correct diagnosis to avoid any delay to prevent further morbidity and mortality.
Keywords: encephalitis; ganciclovir; hhv6; pcr; reactivation.
Publication
Journal: Frontiers in Neurology
December/8/2021
Abstract
Introduction: The phenomenon of coexisting autoimmune diseases (ADs) in patients with myasthenia gravis (MG) has attracted considerable attention. However, few studies have investigated the burden and potential clinical associations of ADs in Chinese MG cohorts. Methods: In this retrospective cross-sectional study, we reviewed the records of 1,132 patients with MG who were admitted to Huashan Hospital Fudan University from August 2013 to August 2020. Patients were excluded if they had incomplete medical records (n = 336). Results: Comorbid ADs were found in 92 of 796 Chinese patients with MG (11.6%), among which, hyperthyroidism (6.7%), hypothyrosis (2.6%), and vitiligo (0.8%) were predominant. Patients with MG with ADs were predominantly female, younger at the onset of MG symptoms, and had a lower frequency of thymoma. Compared to the general population, we found a significantly higher percentage of hyperthyroidism (8.5-fold increase, p < 0.001), hypothyrosis (2.6-fold increase, p < 0.001), vitiligo (1.3-fold increase, p < 0.001), rheumatoid arthritis (1.4-fold increase, p < 0.001), immune thrombocytopenic purpura (193.1-fold increase, p < 0.001), autoimmune hemolytic anemia (7.4-fold increase, p < 0.001), autoimmune hepatitis (5.1-fold increase, p < 0.001), and polymyositis (11.5-fold increase, p < 0.001) in patients with MG with ADs. Patients with MG with ADs presented a lower proportion of previous history of MC (0 vs. 5.6%, p < 0.05) than those without ADs. The proportion of MGFA Class I at onset in patients with MG with ADs was significantly higher than that in patients with MG without ADs (77.0 vs. 52.7%, p < 0.05). The proportion of MuSK-positive in patients with MG with ADs was significantly lower than that in patients with MG without ADs (0 vs. 4.8%, p < 0.05). Conclusion: In conclusion, we observed a higher frequency of concurrent ADs in a Chinese MG cohort. Furthermore, MG combined with ADs tended to have mild clinical presentation.
Keywords: autoimmune diseases; clinical characteristics; comorbidities; myasthenia gravis; percentage.
Publication
Journal: Ophthalmic Plastic and Reconstructive Surgery
December/6/2021
Abstract
Paraneoplastic extraocular muscle enlargement has been reported in a small number of patients with cancers including breast or lymphoma, usually presenting with bilateral multiple muscle involvement. Such myositis may be autoimmune. Furthermore, orbital inflammation is a recognized complication of immune-modulation therapy used to treat melanoma, such as ipilimumab. Extraorbital myositis has been described in myeloma, and polymyositis in melanoma. We present a case of bilateral, asymmetrical extraocular muscle enlargement with spontaneous resolution in a patient with simultaneous new diagnoses of metastatic malignant melanoma and multiple myeloma. A similar episode 7 months before diagnosis also resolved spontaneously. The authors believe this to be the first reported case of paraneoplastic orbital myositis associated with multiple myeloma or untreated malignant melanoma.
Publication
Journal: Frontiers in Immunology
December/5/2021
Abstract
Background: Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare but fatal complication in idiopathic inflammatory myopathy (IIM) patients. The clinical value of radiological manifestations and serum cytokines remain unknown in this systemic crisis. This study aims to investigate the clinical value of PET/CT scan and cytokine profiles in predicting and understanding sHLH in IIM patients.
Methods: Adult IIM patients who were admitted to the four divisions of the First Affiliated Hospital, Zhejiang University School of Medicine (FAHZJU) from January 1, 2017 to December 31, 2020 were reviewed. PET/CT scan, cytokine profiles, and other factors of patients who met the inclusion and exclusion criteria were collected and analyzed.
Results: Sixty-nine out of 352 IIM patients were finally enrolled into the study. Ten patients developed sHLH and 70.0% of them died within 6 months. After false discovery rate (FDR) correction and multivariate logistic regression analysis, increased serum interferon (IFN)-γ level (p = 0.017), higher spleen mean standard uptake value (SUVmean, p = 0.035), and positivity of anti-MDA5 antibody (p = 0.049) were found to be significantly correlated with development of sHLH in IIM patients. The combination of serum IFN-γ, spleen SUVmean, and anti-MDA5 antibody found a balanced and satisfying predictor with a cutoff value of 0.047 and AUC of 0.946. A moderate correlation was identified between ferritin and spleen SUVmean (p = 0.001, r = 0.380) as well as serum IFN-γ(p = 0.001, r = 0.398). Before FDR correction, higher bilateral lung SUVmean (p = 0.034) and higher colon/rectum SUVmean (p = 0.013) were also observed in IIM patients who developed sHLH. By narrowing down to IIM patients with sHLH, anti-MDA5-antibody-positive DM patients tended to suffer from unfavorable outcome (p = 0.004) in Kaplan-Meier survival analysis.
Conclusion: Increased serum level of IFN-γ, elevated splenic FDG uptake, and positivity of anti-MDA5 antibody were significantly correlated with development of sHLH in IIM patients. Lung and lower digestive tract might also be affected due to systemic immune activation in IIM patients with sHLH. In addition, splenic FDG uptake, in combination with serum IFN-γand anti-MDA5 antibody, was found valuable in predicting development of sHLH in IIM patients. Among IIM patients with sHLH, anti-MDA5-antibody-positive DM patients showed higher tendency for unfavorable outcome.
Keywords: PET/CT; cytokine profile; dermatomyositis; idiopathic inflammatory myopathy; polymyositis; secondary hemophagocytic lymphohistiocytosis.
Publication
Journal: Rheumatology
December/4/2021
Abstract
Objectives: To study the phenotype of macrophage infiltrates and their role in angiogenesis in different Idiopathic Inflammatory Myopathies (IIMs).
Methods: The density and distribution of the subpopulations of macrophages subsets (M1, inducible nitric oxide+, CD11c+; M2, arginase-1+), endomysial capillaries (CD31+, FLK1+), degenerating (C5b-9+), and regenerating (NCAM+) myofibers, were investigated by immunohistochemistry in human muscle samples of diagnostic biopsies from a large cohort of untreated patients (n: 81) suffering from anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (anti-HMGCR)+ Immune Mediated Necrotizing Myopathy (IMNM), anti-signal recognition particle (anti-SRP)+ IMNM, seronegative IMNM, Dermatomyositis, Polymyositis, Polymyositis with mitochondrial pathology, sporadic Inclusion Body Myositis, Scleromyositis, and anti-Synthetase Syndrome. The samples were compared with mitochondrial myopathy and control muscle samples.
Results: Compared with the other IIMs and controls, endomysial capillary density (CD) was higher in anti-HMGCR+ IMNM, where M1 and M2 macrophages, detected by confocal microscopy, infiltrated perivascular endomysium and expressed angiogenic molecules such as VEGF-A and CXCL12. These angiogenic macrophages were preferentially associated with CD31+ FLK1+ microvessels in anti-HMGCR+ IMNM. The VEGF-A+ M2 macrophage density was significantly correlated with CD (rS: 0.98; p: 0.0004). Western blot analyses revealed increased expression levels of VEGF-A, FLK1, HIF-1α, and CXCL12 in anti-HMGCR+ IMNM. CD and expression levels of these angiogenic molecules were not increased in anti-SRP+ and seronegative IMNM, offering additional, useful information for differential diagnosis among these IIM subtypes.
Conclusion: Our findings suggest that in IIMs, infiltrating macrophages and microvascular cells interactions play a pivotal role in coordinating myogenesis and angiogenesis. This reciprocal crosstalk seems to distinguish anti-HMGCR associated IMNM.
Keywords: M2 macrophages; Myositis; Necrotizing myopathy; Neovascularization.
Publication
Journal: Joint Bone Spine
December/2/2021
Abstract
Keywords: Dermatomyositis; Epidemiology; Expenditures; Hospitalization; Polymyositis.
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Journal: J Pharm Technol
December/2/2021
Abstract
Background: Currently, specific studies identifying how repository corticotropin injection (RCI) is used in rheumatologic conditions are lacking. This is a first step to familiarize the trends of demographics using RCI as well as other medication use. Objective: RCI may produce anti-inflammatory as well as immune-modulatory effects. The purpose of this study is to examine the demographics of those who use RCI and the change in medication use, specifically prednisone, after RCI initiation. Method: This study used the Symphony Health Solutions (SHA) Claims database from 2008 to 2015. International Classification of Disease, Ninth Revision, codes were used to identify rheumatologic conditions including rheumatoid arthritis, systemic lupus erythematosus, dermatomyositis, and polymyositis. Information including RCI dose and concomitant medication uses was also obtained. Results: A total of 2749 patients with rheumatologic conditions receiving RCI were investigated for demographic information, and a total of 1048 patients with rheumatologic conditions on RCI were examined for medication use. The use of nonsteroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs, and biologics overall decreased significantly in all 3 rheumatologic conditions except biologics in dermatomyositis/polymyositis. In addition, mean prednisone dose before and after RCI use significantly decreased one quarter (12 weeks) after RCI initiation. Conclusion: Claims-based study on RCI use indicates that RCI use might reduce use of prednisone, disease-modifying anti-rheumatic drugs, and other biologics. Further prospective study is needed.
Keywords: corticosteroids; databases; nonsteroidal anti-inflammatory drugs; rheumatoid arthritis; systemic lupus erythematosus.
Publication
Journal: Nature Reviews Disease Primers
December/2/2021
Abstract
Idiopathic inflammatory myopathies (IIM), also known as myositis, are a heterogeneous group of autoimmune disorders with varying clinical manifestations, treatment responses and prognoses. Muscle weakness is usually the classical clinical manifestation but other organs can be affected, including the skin, joints, lungs, heart and gastrointestinal tract, and they can even result in the predominant manifestations, supporting that IIM are systemic inflammatory disorders. Different myositis-specific auto-antibodies have been identified and, on the basis of clinical, histopathological and serological features, IIM can be classified into several subgroups - dermatomyositis (including amyopathic dermatomyositis), antisynthetase syndrome, immune-mediated necrotizing myopathy, inclusion body myositis, polymyositis and overlap myositis. The prognoses, treatment responses and organ manifestations vary among these groups, implicating different pathophysiological mechanisms in each subtype. A deeper understanding of the molecular pathways underlying the pathogenesis and identifying the auto-antigens of the immune reactions in these subgroups is crucial to improving outcomes. New, more homogeneous subgroups defined by auto-antibodies may help define disease mechanisms and will also be important in future clinical trials for the development of targeted therapies and in identifying biomarkers to guide treatment decisions for the individual patient.
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Journal: Journal of Palliative Care
November/25/2021
Abstract
Introduction: Skin disorders and neuropathy often occur as side effects of chemotherapy. We encountered a patient who was treated for drug-induced skin symptoms, but the symptoms did not improve, and he was eventually diagnosed as having dermatomyositis.
Case presentation: A 71-year-old man underwent chemotherapy with regorafenib in February 2020 for the postoperative recurrence of sigmoid colon cancer, but treatment was discontinued after about 2 months owing to the appearance of skin symptoms, which were thought to be side effects of regorafenib. Subsequently, his symptoms further worsened, and he was hospitalized 3 weeks after the appearance of the initial skin symptoms, and a palliative care team was asked to relieve his back pain caused by the drug-induced skin symptoms. Erythema was widely observed on the lower back and limbs, and he experienced needle stick-like pain. Furthermore, the patient demonstrated difficulty in lifting both upper limbs. As acetaminophen was effective for his pain, the dose was slowly increased with careful observation. The cause of the patient's muscle weakness was unclear, and after careful discussion of the possible causes among specialists in dermatology, neurology, and rheumatoid arthritis, a diagnosis of dermatomyositis associated with the malignant tumor was made about 10 days after his admission. The patient's symptoms gradually improved with steroid pulse treatment (methylprednisolone 1 g/day for 3 days) followed by high-dose gamma globulin treatment (2.5 g/day for 5 days), and the patient was discharged 48 days after admission.
Discussion: Because this patient was referred to a palliative care team for the purpose of relieving pain caused by skin symptoms associated with chemotherapy, a crucial point is the symptoms were treated as side effects of the chemotherapy from the beginning. As neuropathy can occur as a result of chemotherapy, the pain and muscle weakness could be explained at the time; however, the symptoms continued to worsen even after the chemotherapy was stopped. Because the symptoms were not typical of polymyositis/dermatomyositis, diagnosis of the patient was delayed, even though he was treated in each specialized department. Our present case indicates that paraneoplastic syndrome should always be kept in mind when treating cancer patients.
Keywords: chemotherapy; dermatomyositis; pain management; palliative care.
Publication
Journal: Nucleosides, Nucleotides and Nucleic Acids
November/23/2021
Abstract
Cytosolic 5'-nucleotidase IA (cN-IA) plays a central role in the regulation of the purine nucleotide pool in skeletal muscle, preferentially converting adenosine monophosphate to adenosine. cN-IA can act as an autoantigen in muscle diseases, including the paraneoplastic syndrome related to breast cancer (BC). As a result of myocyte damage, released cN-IA protein may trigger the production of anti-cN-IA antibodies (anti-NT5C1A). This work aimed to develop an effective method to measure cN-IA activity in the serum and analyze it in BC patients. Our study demonstrated that serum cN-IA activity was decreased in BC patients and we assumed it is due to the presence of specific autoantibodies. We found correlations between cN-IA activity and parameters of inflammatory muscle damage. Thus, cN-IA is worth further attention to clarify its usefulness as a biomarker of BC-associated polymyositis.
Keywords: Cytosolic 5′-nucleotidase 1A; anti-NT5C1A autoantibody; breast cancer; cN-IA; polymyositis.
Publication
Journal: Journal of Community Hospital Internal Medicine Perspectives
November/21/2021
Abstract
Dermatomyositis (DM) and polymyositis (PM) are both immune-mediated inflammatory myopathies known to occur in paraneoplastic syndromes associated with a new diagnosis of malignancy, most commonly breast, ovarian, lung, pancreatic, stomach, colorectal, and Non-Hodgkin's lymphoma1 in DM and breast, lung, bladder cancer, and Non-Hodgkin's lymphoma in PM. 2,3,4 While inflammatory markers such as creatine kinase (CK) may be elevated with either DM or PM, marked elevation is rare. Herein, we report a case of newly diagnosed pancreatic cancer presenting with inflammatory myopathy and marked CK elevation. We review the frequency of PM as a paraneoplastic syndrome, the association with marked CK elevation, and the association with pancreatic cancer.
Keywords: Paraneoplastic myopathy; myopathy; pancreatic cancer; paraneoplastic myositis; paraneoplastic syndrome.
Publication
Journal: Frontiers in Medicine
November/21/2021
Abstract
Purpose: To determine the association between a history of clinically diagnosed dengue infection and the risk of systemic autoimmune rheumatic diseases (SARDs). Methods: Using claims data from the 1997-2013 Taiwanese National Health Insurance Research Database, we included 74,422 patients who were diagnosed with SARDs and 297,688 patients without SARDs who were matched (in a 1:4 ratio) for age, sex, year of SARDs index date, and city of residence. The associations between the development of SARDs and a history of dengue infection (International Classification of Diseases, Ninth Revision, Clinical Modification code 061) were investigated using conditional logistic regression analysis shown as odds ratios (ORs) with 95% confidence intervals (CIs) after adjusting for potential confounders. Results: We included 17,126 patients with systemic lupus erythematosus (SLE), 15,531 patients with Sjogren's syndrome (SS), 37,685 patients with rheumatoid arthritis (RA), 1,911 patients with systemic sclerosis (SSc), 1,277 patients with dermatomyositis (DM), and 892 patients with polymyositis (PM). SLE (OR, 4.55; 95% CI, 2.77-7.46; p <0.001) risk was significantly associated with a history of dengue infection. However, no statistically significant association was found between dengue infection and SS (OR, 1.41; 95% CI, 0.88-2.26; p = 0.155), RA (OR, 1.03; 95% CI, 0.70-1.50; p = 0.888), SSc (OR, 1.97; 95% CI, 0.38-10.29; p = 0.420), DM (OR, 0.54; 95% CI, 0.04-7.27; p = 0.641), or PM (OR, 2.08; 95% CI, 0.23-18.79; p = 0.513). Conclusion: This study revealed that a history of dengue infection was significantly associated with the risk of SLE, but not SS, RA, SSc, DM, or PM.
Keywords: claims data; dengue; history; risk; systemic autoimmune rheumatic disease (SARD).
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Journal: Medical Journal of Malaysia
November/21/2021
Abstract
Polymyositis is rarely associated with Graves' disease. A 22- year-old woman was admitted for progressively worsening proximal muscle weakness of both upper and lower extremities. One month prior to admission, she was diagnosed with thyrotoxicosis and prescribed carbimazole 10mg twice daily. Neurological examination confirmed proximal myopathy and blood investigations revealed marked elevation of muscle enzymes, particularly creatine kinase. Electromyography demonstrated myopathic changes while right quadriceps muscle biopsy showed only traces of inflammatory myopathy. She was treated with pulsed intravenous methylprednisolone followed by tapering doses of oral prednisolone, which was eventually down-titrated to 5mg daily during subsequent clinic visits. The initial clinical improvement that she exhibited did not persist despite being rendered euthyroid. She was readmitted approximately one year later with the same complaint. A second course of intravenous methylprednisolone brought about clinical improvement as well as reduction of creatine kinase levels. A diagnosis of polymyositis was then made, for which she was managed with oral prednisolone 20mg daily in combination with gradual up-titration of azathioprine. She continued to show clinical and biochemical improvements during follow-ups. Polymyositis should be considered in the diagnostic workup of proximal myopathy in a patient with Graves' disease, especially in the setting of markedly raised muscle enzymes.
Publication
Journal: Arthritis and Rheumatology
November/21/2021
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Journal: Arthritis and Rheumatology
November/21/2021
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Journal: Clinical and Experimental Rheumatology
November/18/2021
Abstract
Objectives: We performed a retrospective and prospective observational study to investigate whether the T lymphocyte activation antigen dipeptidyl peptidase 4 (DPP4)/CD26 is expressed in the skeletal muscle of patients with idiopathic inflammatory myopathies (IIM) and whether its expression offers clues to understand the events taking place in the tissue.
Methods: CD26 expression in the muscle, evaluated by immunofluorescence, was assessed in 32 patients with IIM and 5 healthy controls and compared among patients with dermatomyositis (DM), immune-mediated necrotising myopathy (IMNM), inclusion body myositis (IBM), and polymyositis (PM). The relationship of CD26 expression and localization with clinical, serological and histological features was determined.
Results: CD26 is selectively expressed in the skeletal muscle of patients with IIM. The highest levels of CD26 are found in the skeletal muscle from patients with DM and in particular in those characterized by tissue necrosis and vascular inflammation. CD26 expression is associated with decreased muscle performance and independently predicts the number of treatments before reaching disease stabilization or improvement (odds ratio, OR=1.2, p<0.05).
Conclusions: CD26 is expressed in the IIM skeletal muscle and may represent a target of molecular intervention for patients with treatment-refractory myositis.
Publication
Journal: Rheumatology
November/17/2021
Abstract
Objectives: We aimed to review whether polymyositis (PM) and dermatomyositis (DM) patients have an increased cardiovascular (CV) risk, including ischaemic heart disease (IHD), cerebrovascular accidents (CVA), and venous thromboembolism (VTE).
Methods: We searched PubMed, Embase, and the Cochrane database for relevant studies from inception to February 2021.
Results: Twenty-two studies comprising 25,433 patients were included. With PM/DM versus general populations, the risk was significantly increased for CV events (relative risk (RR)=2.37, 95%CI:1.86-3.02). The RR of CV events for males with PM/DM was higher than for females (RR = 1.43; 95%CI:1.17-1.74). PM/DM patients followed for one to five years had a significantly higher CV risk than those followed for five to ten years (RR = 3.51, 95%CI:1.95-6.32). The risk was increased for North Americans (RR = 4.28, 95%CI:2.57-7.11), Europeans (RR = 2.29, 95%CI:1.58-3.31), and Asians (RR = 2.03, 95%CI:1.41-2.90). Our meta-analysis found that the elevated CV event risk was related to PM (RR = 2.35, 95%CI:1.51-3.66) and DM (RR = 2.55, 95%CI:1.66-3.93). Subgroup analyses showed that the risk was significantly increased for IHD (RR = 1.76, 95%CI:1.40-2.21), CVA morbidity (RR = 1.31, 95%CI:1.03-1.67), and ischaemic stroke (IS) (RR = 1.47, 95%CI:1.26-1.73), with no statistically significant increased risk of haemorrhagic stroke mortality (RR = 1.43, 95%CI:0.92-2.21). The CV event risk was increased for VTE (RR = 4.60, 95%CI:3.17-6.66), deep venous thrombosis (DVT) (RR = 5.53, 95%CI:3.25-9.39), and pulmonary embolism (PE) (RR = 5.26, 95%CI:2.62-10.55).
Conclusion: This meta-analysis found that PM/DM patients had a ∼2.37 times increased CV risk, particularly males diagnosed in the previous five years. PM/DM may be an independent risk factor for developing IHD, IS, DVT, and PE.
Keywords: cerebrovascular accidents; dermatomyositis; ischaemic heart disease; meta-analysis; polymyositis; venous disease; venous thromboembolism.
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Journal: Current Pharmaceutical Design
November/15/2021
Abstract
Inflammatory myopathies, in short, myositis, are heterogeneous disorders that are characterized by inflammation of skeletal muscle and weakness of arms and legs. Research over the past few years has led to a new understanding regarding the pathogenesis of myositis. The new insights include different pathways of the innate and adaptive immune response during the pathogenesis of myositis. The importance of non-inflammatory mechanisms such as cell stress and impaired autophagy has been recently described. New target-specific drugs for myositis have been developed and are currently being tested in clinical trials. In this review, we discuss the mechanisms of action of pharmacological standards in myositis and provide an outlook of future treatment approaches.
Keywords: Inflammatory myopathies; anti-synthetase syndrome; biologic agents; dermatomyositis; disease mechanisms; immunosuppression; inclusion body myositis; myositis; necrotizing myopathy; polymyositis; treatment.
Publication
Journal: BMC Pulmonary Medicine
November/14/2021
Abstract
Background: Many patients with polymyositis (PM) or dermatomyositis (DM) have circulating myositis-specific antibodies (MSAs). Interstitial lung disease (ILD) is a common manifestation of PM/DM, and it can even precede the onset of characteristic muscle or skin manifestations. Furthermore, there appear to be some patients with ILD and circulating MSAs who do not develop muscle or skin disease even after prolonged follow-up. We sought to determine whether ILD is equally or more common than myositis or dermatitis at the time of initial detection of MSAs.
Methods: We identified all patients found to have circulating MSAs at our institution over a 4-year period and assessed for the presence of lung, muscle, and skin disease at the time of initial detection of MSAs. Among those found to have ILD, we compared demographic and clinical features, chest CT scan findings, and outcomes between those with PM/DM-associated ILD and those with ILD but no muscle or skin disease.
Results: A total of 3078 patients were tested for MSAs, and of these 40 were positive. Nine different MSAs were detected, with anti-histidyl tRNA synthetase (anti-Jo-1) being the most common (35% of MSAs). Among patients with positive MSAs, 86% were found to have ILD, compared to 39% and 28% with muscle and skin involvement, respectively (p < 0.001). Fifty percent of all MSA-positive patients had isolated ILD, with no evidence of muscle or skin disease. Those with isolated ILD were more likely to be older and have fibrotic changes on chest CT, less likely to receive immunomodulatory therapy, and had worse overall survival.
Conclusions: In this study we found that individuals with circulating MSAs were more likely to have ILD than classic muscle or skin manifestations of PM/DM at the time of initial detection of MSAs. Our findings suggest that the presence of ILD should be considered a disease-defining manifestation in the presence of MSAs and incorporated into classification criteria for PM/DM.
Keywords: Dermatomyositis; Interstitial lung disease; Myositis-specific antibodies; Polymyositis.
Publication
Journal: Joint Bone Spine
November/13/2021
Abstract
Objective: Aromatase inhibitors (AIs) are widely used in the adjuvant therapy setting in patients with estrogen receptor-positive breast cancer. Rheumatic side effects of AIs have been reported, including bone loss, arthralgias, myalgias, and tenosynovitis. There is emerging evidence that AIs are linked to new-onset autoimmune diseases. Here, we describe three novel cases of inflammatory myopathies that occurred during AI therapy.
Methods: In total, three patients with inflammatory myopathy in the setting of AI therapy were treated in the Section of Rheumatology, MD Anderson Cancer Center. All the patients were retrospectively chart reviewed. We obtained verbal consent from the patients for use of their cases for teaching and publication purposes and only de-identified data have been used.
Results: None of our patients from these three cases had a history of autoimmune disease and all had undergone recent cancer screenings, indicating no re-occurrence of breast cancer and no evidence of new cancer. The completion or discontinuation of AI therapy was associated with the resolution of the myopathies in all three cases.
Conclusions: This case series suggests a link between AIs and the new onset of inflammatory myopathy. If a patient develops an inflammatory myopathy while on an AI, the AI therapy should be considered the possible trigger. If the myopathy cannot be controlled with immunosuppression, then discontinuation of the AI should be considered.
Keywords: Inflammatory myopathy; aromatase inhibitors; breast cancer; dermatomyositis; fasciitis; polymyositis.
Publication
Journal: Critical Reviews in Clinical Laboratory Sciences
November/11/2021
Abstract
Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders affecting primarily muscles, but other organs can be involved. This review describes the clinical features, diagnosis and treatment for IIMs, namely polymyositis (PM), dermatomyositis (DM), sporadic inclusion body myositis (sIBM), immune-mediated necrotizing myopathy (IMNM), and myositis associated with antisynthetase syndrome (ASS). The diagnostic approach has been updated recently based on the discovery of circulating autoantibodies, which has enhanced the management of patients. Currently, validated classification criteria for IIMs allow clinical studies with well-defined sets of patients but diagnostic criteria to guide the care of individual patients in routine clinical practice are still missing. This review analyzes the clinical manifestations and laboratory findings of IIMs, discusses the efficiency of modern and standard methods employed in their workup, and delineates optimal practice for clinical care. Α multidisciplinary diagnostic approach that combines clinical, neurologic and rheumatologic examination, evaluation of electrophysiologic and morphologic muscle characteristics, and assessment of autoantibody immunoassays has been determined to be the preferred approach for effective management of patients with suspected IIMs.
Keywords: Polymyositis (PM); antisynthetase syndrome (ASS); dermatomyositis (DM); immune-mediated necrotizing myopathy (IMNM); sporadic inclusion body myositis (sIBM).
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Journal: Caspian Journal of Internal Medicine
November/10/2021
Abstract
Background: As a global health pandemic, the novel severe acute respiratory syndrome-coronavirus 2 (SARS- CoV2) outbreak began in December 2019 which rapidly spread to more than 200 countries. Respiratory complications and fever are the most obvious symptoms. Sometimes the neurological features are superimposed on the main disease and complicate patient's status.
Case presentation: We describe 6 patients with COVID-19 and concomitant quadriparesia who underwent electrodiagnosis using EMG/NCS and results indicated 3 axonal variants of Guillain-Barré syndrome (GBS), including; 2 cases AMAN (acute motor axonal neuropathy), 1 case AMSAN (acute motor and sensory axonal neuropathy), three myopathies, including one combination of CIN/CIM (critical illness neuropathy/critical illness myopathy), one CIM and one acute polymyositis in these cases.
Conclusion: Early diagnosis of the neuromuscular disorders of coronavirus could help for correct planning in the treatment of COVID-19 patients. Since GBS and inflammatory myopathies have an autoimmune basis, the immunotherapies such as IVIG, steroids, plasma exchange and other novel treatments as hemoperfusion can promise better and faster recovery in respiratory function and neuromuscular activity among COVID-19 patients who have musculature paralysis concomitantly. However, all these treatments are challenging and further clinical trials should be done to confirm the efficacy and safety of mentioned therapies.
Keywords: Acute motor axonal neuropathy; COVID-19; Corona virus; Critical illness myopathy; Critical illness neuropathy; GBS; Quadriparesis.
Publication
Journal: Rheumatology
November/9/2021
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Journal: Neurology India
November/7/2021
Abstract
Polymyositis is a group of muscle disease characterised by progressive muscle inflammation and predominantly muscle weakness. It usually presents subacutely with proximal weakness and mild diffuse muscular pain. Some patients have atypical presentation like early respiratory difficulty, Motor neuron disease (MND), or isolated dysphagia which leads to delay in diagnosis and treatment. We present one such case.
Keywords: Interstial lung Disease (ILD); myasthenia gravis; polymyositis; weakness.
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