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Seminars in Arthritis and Rheumatism Apr 2022Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive... (Review)
Review
INTRODUCTION
Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive clinically amyopathic dermatomyositis (CADM) is frequently associated with rapidly progressive interstitial lung disease (RP-ILD) and high mortality rates. There is a lack of data on management of this often fatal condition. The aim of this systematic review was to evaluate current evidence that assesses the available management options and discuss the associated management challenges.
MATERIAL AND METHODS
This systematic review was conducted according to PRISMA guidelines. Online databases were searched from inception to April of 2021 using the search terms: "dermatomyositis" OR "amyopathic dermatomyositis" OR "clinically amyopathic dermatomyositis" AND "MDA-5″ OR "melanoma differentiation-associated gene-5″ OR "CADM-140″ AND "management" OR "treatment" OR "therapy" OR "therapeutics". Articles assessing the use of pharmacologic agents on 10 or more patients with MDA5-antibody positive CADM associated with ILD were included. Narrative or systematic reviews and meta-analyses were not eligible for inclusion.
RESULTS
A total of 15 eligible studies and 399 unique patients were selected. We identified only one open-label randomized controlled trial (RCT) that examined the management of anti-MDA5 antibody CADM/DM-ILD. Further, 3 cohort studies with prospective arms matched against historical controls, 10 retrospective cohort studies, and 1 retrospective case series were included. A combined therapeutic regimen of high-dose systemic glucocorticoids and other immunosuppressive agents such as calcineurin inhibitors and/or cyclophosphamide, administered early, appears to give the highest rates of survival in those with RP-ILD, while additional therapies such as plasma exchange can be added for refractory disease. Further, tofacitinib and rituximab might have a place in the therapeutic armamentarium of this challenging to treat condition. Early detection and treatment are of extreme importance, given the risk for rapid decline and high mortality in this subset of patients.
CONCLUSION
There are limited RCTs evaluating the treatment of ILD associated with MDA5-antibody positive CADM. Initiating a combined immunosuppressive therapeutic regimen early in the disease course improves overall morbidity and mortality. RCTs and larger prospective studies are needed to provide high-quality evidence to inform future treatment guidelines.
Topics: Autoantibodies; Dermatomyositis; Disease Progression; Humans; Immunosuppressive Agents; Lung Diseases, Interstitial
PubMed: 35134633
DOI: 10.1016/j.semarthrit.2022.151959 -
Clinical and Experimental Rheumatology Mar 2023Dermatomyositis (DM) is an idiopathic inflammatory myopathy that commonly manifests with proximal muscle weakness and is associated with extramuscular pathology,... (Review)
Review
Dermatomyositis (DM) is an idiopathic inflammatory myopathy that commonly manifests with proximal muscle weakness and is associated with extramuscular pathology, including characteristic skin lesions such as Gottron's papules and heliotrope rash, as well as lung, gastrointestinal, joint, and cardiac involvement. Systemic corticosteroids are a cornerstone of therapy, and more recently intravenous immunoglobulin (IVIG; OCTAGAM®) has been approved by the US Food and Drug Administration for the treatment of adults with DM. Both steroids and IVIG represent nonspecific anti-inflammatory therapy, and more targeted approaches are lacking. Transcriptomics has identified upregulation of interferon (IFN)-regulated genes as key features of both adult DM and juvenile DM (JDM). Accordingly, blocking IFN signalling through inhibition of the Janus kinase (JAK) pathway represents a potential treatment option for DM. Placebo-controlled trial data assessing the use of JAK inhibitors for the treatment of DM are limited; as such, a systematic literature review was undertaken to assess the evidence of JAK inhibitors in the treatment of patients with DM. Terms related to DM and JAK inhibitors were searched using PubMed, Embase, Web of Science, Scopus, and Dimensions to identify peer-reviewed publications reporting patients with DM who were treated with a JAK inhibitor. Baseline demographics, clinical characteristics, and treatment outcome data were extracted. A total of 48 publications reporting 145 unique patients (adult DM, n=84; JDM, n=61) were identified. Among cases of adult DM, 61 of 84 (73%) had refractory skin disease at baseline, and all (61 of 61) reported improvement in cutaneous symptoms. Of patients with adult DM, 16 of 84 (19%) had refractory muscle disease at baseline, and all (16 of 16) reported improvement in muscle symptoms. In patients with adult DM complicated by interstitial lung disease (ILD; n=33), 31 (94%) patients improved with JAK inhibitor treatment. Among cases of JDM with refractory skin disease at baseline (60 of 61), most patients (57 of 60; 95%) showed improvements in skin symptoms after JAK inhibitor treatment. Of patients with JDM with refractory muscle disease at baseline (36 of 61), most (30 of 36; 83%) reported improvement in muscle symptoms. Four patients with JDM and ILD experienced improvement in lung disease activity following treatment with a JAK inhibitor. Among both DM and JDM cases, all patients (17 with DM and 16 with JDM) who had elevated serum IFN and/or IFN-stimulated gene expression at baseline showed reduction in IFN or IFN gene expression. Although the conclusions that can be drawn from this analysis are limited because of the differences in assessments used across publications, overall treatment of patients with DM or JDM with a JAK inhibitor was associated with significant improvement of a wide range of DM manifestations, including skin lesions, muscle weakness, and ILD. Our systematic literature review suggests that JAK inhibitors may be a viable treatment option for DM/JDM, and randomised controlled trials are necessary to confirm these findings.
Topics: Adult; Humans; Dermatomyositis; Janus Kinase Inhibitors; Immunoglobulins, Intravenous; Muscular Diseases; Muscle Weakness; Lung Diseases, Interstitial
PubMed: 35766013
DOI: 10.55563/clinexprheumatol/hxin6o -
Autoimmunity Reviews Apr 2022Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic,... (Review)
Review
Many systemic immune-mediated diseases (SIDs) may involve the heart and present as myocarditis with different histopathological pictures, i.e. lymphocytic, eosinophilic, granulomatous, and clinical features, ranging from a completely asymptomatic patient to life-threatening cardiogenic shock or arrhythmias. Myocarditis can be part of some SIDs, such as sarcoidosis, systemic lupus erythematosus, systemic sclerosis, antiphospholipid syndrome, dermato-polymyositis, eosinophilic granulomatosis with polyangiitis and other vasculitis syndromes, but also of some organ-based immune-mediated diseases with systemic expression, such as chronic inflammatory bowel diseases. The aim of this review is to describe the prevalence, main clinical characteristics and prognosis of myocarditis associated with SIDs.
Topics: Churg-Strauss Syndrome; Granulomatosis with Polyangiitis; Humans; Myocarditis; Prevalence; Prognosis
PubMed: 34995763
DOI: 10.1016/j.autrev.2022.103037 -
The Cochrane Database of Systematic... Dec 2019Strength training or aerobic exercise programmes, or both, might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Strength training or aerobic exercise programmes, or both, might optimise muscle and cardiorespiratory function and prevent additional disuse atrophy and deconditioning in people with a muscle disease. This is an update of a review first published in 2004 and last updated in 2013. We undertook an update to incorporate new evidence in this active area of research.
OBJECTIVES
To assess the effects (benefits and harms) of strength training and aerobic exercise training in people with a muscle disease.
SEARCH METHODS
We searched Cochrane Neuromuscular's Specialised Register, CENTRAL, MEDLINE, Embase, and CINAHL in November 2018 and clinical trials registries in December 2018.
SELECTION CRITERIA
Randomised controlled trials (RCTs), quasi-RCTs or cross-over RCTs comparing strength or aerobic exercise training, or both lasting at least six weeks, to no training in people with a well-described muscle disease diagnosis.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures expected by Cochrane.
MAIN RESULTS
We included 14 trials of aerobic exercise, strength training, or both, with an exercise duration of eight to 52 weeks, which included 428 participants with facioscapulohumeral muscular dystrophy (FSHD), dermatomyositis, polymyositis, mitochondrial myopathy, Duchenne muscular dystrophy (DMD), or myotonic dystrophy. Risk of bias was variable, as blinding of participants was not possible, some trials did not blind outcome assessors, and some did not use an intention-to-treat analysis. Strength training compared to no training (3 trials) For participants with FSHD (35 participants), there was low-certainty evidence of little or no effect on dynamic strength of elbow flexors (MD 1.2 kgF, 95% CI -0.2 to 2.6), on isometric strength of elbow flexors (MD 0.5 kgF, 95% CI -0.7 to 1.8), and ankle dorsiflexors (MD 0.4 kgF, 95% CI -2.4 to 3.2), and on dynamic strength of ankle dorsiflexors (MD -0.4 kgF, 95% CI -2.3 to 1.4). For participants with myotonic dystrophy type 1 (35 participants), there was very low-certainty evidence of a slight improvement in isometric wrist extensor strength (MD 8.0 N, 95% CI 0.7 to 15.3) and of little or no effect on hand grip force (MD 6.0 N, 95% CI -6.7 to 18.7), pinch grip force (MD 1.0 N, 95% CI -3.3 to 5.3) and isometric wrist flexor force (MD 7.0 N, 95% CI -3.4 to 17.4). Aerobic exercise training compared to no training (5 trials) For participants with DMD there was very low-certainty evidence regarding the number of leg revolutions (MD 14.0, 95% CI -89.0 to 117.0; 23 participants) or arm revolutions (MD 34.8, 95% CI -68.2 to 137.8; 23 participants), during an assisted six-minute cycle test, and very low-certainty evidence regarding muscle strength (MD 1.7, 95% CI -1.9 to 5.3; 15 participants). For participants with FSHD, there was low-certainty evidence of improvement in aerobic capacity (MD 1.1 L/min, 95% CI 0.4 to 1.8, 38 participants) and of little or no effect on knee extension strength (MD 0.1 kg, 95% CI -0.7 to 0.9, 52 participants). For participants with dermatomyositis and polymyositis (14 participants), there was very low-certainty evidence regarding aerobic capacity (MD 14.6, 95% CI -1.0 to 30.2). Combined aerobic exercise and strength training compared to no training (6 trials) For participants with juvenile dermatomyositis (26 participants) there was low-certainty evidence of an improvement in knee extensor strength on the right (MD 36.0 N, 95% CI 25.0 to 47.1) and left (MD 17 N 95% CI 0.5 to 33.5), but low-certainty evidence of little or no effect on maximum force of hip flexors on the right (MD -9.0 N, 95% CI -22.4 to 4.4) or left (MD 6.0 N, 95% CI -6.6 to 18.6). This trial also provided low-certainty evidence of a slight decrease of aerobic capacity (MD -1.2 min, 95% CI -1.6 to 0.9). For participants with dermatomyositis and polymyositis (21 participants), we found very low-certainty evidence for slight increases in muscle strength as measured by dynamic strength of knee extensors on the right (MD 2.5 kg, 95% CI 1.8 to 3.3) and on the left (MD 2.7 kg, 95% CI 2.0 to 3.4) and no clear effect in isometric muscle strength of eight different muscles (MD 1.0, 95% CI -1.1 to 3.1). There was very low-certainty evidence that there may be an increase in aerobic capacity, as measured with time to exhaustion in an incremental cycle test (17.5 min, 95% CI 8.0 to 27.0) and power performed at VO max (maximal oxygen uptake) (18 W, 95% CI 15.0 to 21.0). For participants with mitochondrial myopathy (18 participants), we found very low-certainty evidence regarding shoulder muscle (MD -5.0 kg, 95% CI -14.7 to 4.7), pectoralis major muscle (MD 6.4 kg, 95% CI -2.9 to 15.7), and anterior arm muscle strength (MD 7.3 kg, 95% CI -2.9 to 17.5). We found very low-certainty evidence regarding aerobic capacity, as measured with mean time cycled (MD 23.7 min, 95% CI 2.6 to 44.8) and mean distance cycled until exhaustion (MD 9.7 km, 95% CI 1.5 to 17.9). One trial in myotonic dystrophy type 1 (35 participants) did not provide data on muscle strength or aerobic capacity following combined training. In this trial, muscle strength deteriorated in one person and one person had worse daytime sleepiness (very low-certainty evidence). For participants with FSHD (16 participants), we found very low-certainty evidence regarding muscle strength, aerobic capacity and VO peak; the results were very imprecise. Most trials reported no adverse events other than muscle soreness or joint complaints (low- to very low-certainty evidence).
AUTHORS' CONCLUSIONS
The evidence regarding strength training and aerobic exercise interventions remains uncertain. Evidence suggests that strength training alone may have little or no effect, and that aerobic exercise training alone may lead to a possible improvement in aerobic capacity, but only for participants with FSHD. For combined aerobic exercise and strength training, there may be slight increases in muscle strength and aerobic capacity for people with dermatomyositis and polymyositis, and a slight decrease in aerobic capacity and increase in muscle strength for people with juvenile dermatomyositis. More research with robust methodology and greater numbers of participants is still required.
Topics: Dermatomyositis; Exercise; Exercise Tolerance; Humans; Muscle Strength; Muscular Diseases; Muscular Dystrophies; Muscular Dystrophy, Facioscapulohumeral; Myotonic Dystrophy; Physical Fitness; Polymyositis; Randomized Controlled Trials as Topic; Resistance Training
PubMed: 31808555
DOI: 10.1002/14651858.CD003907.pub5 -
Dermatologic Therapy May 2021Refractory dermatomyositis (DM) is defined as cases that do not show improvement after initial treatment with two different immunosuppressives combined with... (Review)
Review
Refractory dermatomyositis (DM) is defined as cases that do not show improvement after initial treatment with two different immunosuppressives combined with corticosteroids with or without intravenous immunoglobulins. In recent years, few studies have reported a positive response to the use of Janus kinase inhibitors (JAK-inhibitors) for the treatment of refractory DM. A systematic literature review was performed for articles studying the use of JAK-inhibitors for the treatment of refractory DM. We identified 38 females and 15 males treated with JAK-inhibitors without serious side effects. Tofacitinib was the most frequently used JAK-inhibitor followed by ruxolitinib. Significant improvement in CDASI score, muscle strength, body weight, and skin lesions were reported in most of the studies. The duration of follow-up ranged from 1 to 15 months without relapse. Therefore, the use of JAK-inhibitors looks promising in the treatment of refractory DM and further high volume research may be required to validate the current concept. As only case reports and series were identified without direct comparison for review, there is a potential risk of bias. Despite these limitations, we believe that the result of this analysis allows a better understanding of treatment options for refractory DM and will help generate a hypothesis that can be further tested.
Topics: Dermatomyositis; Drug-Related Side Effects and Adverse Reactions; Female; Humans; Immunosuppressive Agents; Janus Kinase Inhibitors; Male
PubMed: 33713527
DOI: 10.1111/dth.14939 -
The Journal of Rheumatology Jan 2021Rheumatoid arthritis (RA) and other rheumatic diseases may present with ocular manifestations.The purpose of our work was to determine the prevalence and type of eye... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Rheumatoid arthritis (RA) and other rheumatic diseases may present with ocular manifestations.The purpose of our work was to determine the prevalence and type of eye involvement in RA and other connective tissue diseases through a metaanalysis and literature review.
METHODS
A systematic review of the literature was performed using Medline, Web of Science, and the Cochrane Library from their inceptions until January 7, 2019. Conjunctivitis, keratoconjunctivitis sicca, xeropthalmia, uveitis, eye hemorrhage, optic neuritis, papilledema, orbital disease, retinal artery/vein occlusion, macular edema, retinitis, chorioretinitis, scleritis, iridocyclitits, choroid hemorrhage, blindness, and amaurosis fugax were searched for prevalence in patients with RA, systemic lupus erythematosus (SLE), antiphospholipid syndrome (APS), dermatomyositis, polymyositis, systemic sclerosis, Sjögren syndrome (SS), undifferentiated connective tissue disease, giant cell arteritis, granulomatosis polyangiitis (GPA; formerly Wegener granulomatosis), systemic vasculitis, and sarcoidosis.
RESULTS
There were 3394 studies identified and 65 included. The prevalence of eye involvement was 18% in RA, 26% in GPA, 27% in giant cell arteritis, 27% in sarcoidosis, 31% in SLE, and 35% in APS. The most common manifestation was dry eye syndrome ("dry eye"; keratoconjunctivitis sicca) in most diseases analyzed, with an especially high frequency of 89% in SS. Anterior and posterior uveitis were the most common ocular complications in sarcoidosis, occurring in 16% (95% CI 3-28) and 6% (95% CI 3-9) of patients, respectively.
CONCLUSION
Eye involvement is present in approximately one-fifth of patients with RA, and a one-quarter to one-third of patients with connective tissue diseases (other than SS at 89%) and vasculitis.
Topics: Arthritis, Rheumatoid; Connective Tissue Diseases; Granulomatosis with Polyangiitis; Humans; Lupus Erythematosus, Systemic; Rheumatic Diseases
PubMed: 32358156
DOI: 10.3899/jrheum.190768 -
Treatment of calcinosis cutis in systemic sclerosis and dermatomyositis: A review of the literature.Journal of the American Academy of... Feb 2020We have limited data on the treatment of calcinosis cutis associated with systemic sclerosis and dermatomyositis. (Review)
Review
BACKGROUND
We have limited data on the treatment of calcinosis cutis associated with systemic sclerosis and dermatomyositis.
OBJECTIVE
To assess the efficacy and tolerance of available treatments for calcinosis cutis based on previously published studies.
METHODS
We performed a systematic review of studies published in Medline, Embase, and the Cochrane library during 1980-July 2018. The strength of clinical data was graded according to the modified Oxford Centre for Evidence-Based Medicine levels of evidence.
RESULTS
In all, 30 studies (288 patients) were included. Eleven therapeutic classes, surgery, and physical treatments were identified as potential treatment options for calcinosis cutis. On the basis of results of a small randomized controlled trial and 4 retrospective studies, low-dose warfarin should not be used for calcinosis cutis (level IB evidence). The results of several studies suggest diltiazem and bisphosphonates might be useful treatment options (level IV). Considering biologic therapies, rituximab has shown promising results in treating both dermatomyositis and systemic sclerosis, whereas tumor necrosis factor inhibitors might be useful for treating juvenile dermatomyositis (level IV). Intralesional sodium thiosulfate might be a promising alternative (level IV).
LIMITATIONS
Few included studies had a high level of evidence.
CONCLUSION
This study highlights the efficacy and tolerance profiles of available treatments for calcinosis cutis, with a focus on level of evidence.
Topics: Calcinosis; Dermatologic Surgical Procedures; Dermatomyositis; Diltiazem; Humans; Injections, Intralesional; Physical Therapy Modalities; Randomized Controlled Trials as Topic; Rituximab; Scleroderma, Systemic; Skin Diseases; Thiosulfates; Treatment Outcome
PubMed: 31302187
DOI: 10.1016/j.jaad.2019.07.006 -
Rheumatology (Oxford, England) Jun 2021To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to... (Meta-Analysis)
Meta-Analysis
OBJECTIVES
To identify clinical factors associated with cancer risk in the idiopathic inflammatory myopathies (IIMs) and to systematically review the existing evidence related to cancer screening.
METHODS
A systematic literature search was carried out on Medline, Embase and Scopus. Cancer risk within the IIM population (i.e. not compared with the general population) was expressed as risk ratios (RR) for binary variables and weighted mean differences (WMD) for continuous variables. Evidence relating to cancer screening practices in the IIMs were synthesized via narrative review.
RESULTS
Sixty-nine studies were included in the meta-analysis. DM subtype (RR 2.21), older age (WMD 11.19), male sex (RR 1.53), dysphagia (RR 2.09), cutaneous ulceration (RR 2.73) and anti-transcriptional intermediary factor-1 gamma positivity (RR 4.66) were identified as being associated with significantly increased risk of cancer. PM (RR 0.49) and clinically amyopathic DM (RR 0.44) subtypes, Raynaud's phenomenon (RR 0.61), interstitial lung disease (RR 0.49), very high serum creatine kinase (WMD -1189.96) or lactate dehydrogenase (WMD -336.52) levels, and anti-Jo1 (RR 0.45) or anti-EJ (RR 0.17) positivity were identified as being associated with significantly reduced risk of cancer. Nine studies relating to IIM-specific cancer screening were included. CT scanning of the thorax, abdomen and pelvis appeared to be effective in identifying underlying asymptomatic cancers.
CONCLUSION
Cancer risk factors should be evaluated in patients with IIM for risk stratification. Screening evidence is limited but CT scanning could be useful. Prospective studies and consensus guidelines are needed to establish cancer screening strategies in IIM patients.
Topics: Adenosine Triphosphatases; Age Factors; Antibodies, Antinuclear; Creatine Kinase; DNA-Binding Proteins; Deglutition Disorders; Dermatomyositis; Female; Guidelines as Topic; Humans; L-Lactate Dehydrogenase; Lung Diseases, Interstitial; Male; Myositis; Neoplasms; Publication Bias; Raynaud Disease; Risk; Sex Factors; Skin Ulcer; Tomography, X-Ray Computed; Transcription Factors
PubMed: 33599244
DOI: 10.1093/rheumatology/keab166 -
Frontiers in Immunology 2022Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases with various subtypes, myositis-specific antibodies, and affect multiple... (Meta-Analysis)
Meta-Analysis
OBJECTIVE
Idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of autoimmune diseases with various subtypes, myositis-specific antibodies, and affect multiple systems. The treatment of IIMs remains challenging, especially for refractory myositis. In addition to steroids and traditional immunosuppressants, rituximab (RTX), a B cell-depleting monoclonal antibody, is emerging as an alternative treatment for refractory myositis. However, the therapeutic response to RTX remains controversial. This meta-analysis aimed to systematically evaluate the efficacy and safety of RTX in patients with IIMs, excluding sporadic inclusion body myositis.
METHODS
PubMed, Embase, Cochrane Library, China National Knowledge Infrastructure, and WanFang Data were searched for relevant studies. The overall effective rate, complete response rate, and partial response rate were calculated to assess the efficacy of RTX. The incidences of adverse events, infection, severe adverse events, severe infection, and infusion reactions were collected to evaluate the safety of RTX. Subgroup analyses were performed using IIM subtypes, affected organs, continents, and countries. We also performed a sensitivity analysis to identify the sources of heterogeneity.
RESULTS
A total of 26 studies were included in the quantitative analysis, which showed that 65% (95% confidence interval [CI]: 54%, 75%) of patients with IIMs responded to RTX, 45% (95% CI: 22%, 70%) of patients achieved a complete response, and 39% (95% CI: 26%, 53%) achieved a partial response. Subgroup analyses indicated that the overall efficacy rates in patients with refractory IIMs, dermatomyositis and polymyositis, as well as anti-synthetase syndrome were 62%, 68%, and 62%, respectively. The overall efficacy rates for muscle, lungs, and skin involvement were 59%, 65%, and 81%, respectively. In addition, studies conducted in Germany and the United States showed that patients with IIMs had an excellent response to RTX, with an effective rate of 90% and 77%, respectively. The incidence of severe adverse events and infections was 8% and 2%, respectively.
CONCLUSION
RTX may be an effective and relatively safe treatment choice in patients with IIMs, especially for refractory cases. However, further verification randomized controlled trials is warranted.
Topics: Humans; Rituximab; Myositis; Polymyositis; Immunosuppressive Agents; Autoimmune Diseases
PubMed: 36578492
DOI: 10.3389/fimmu.2022.1051609 -
Seminars in Arthritis and Rheumatism Dec 2022To evaluate the role of myositis-specific autoantibodies (MSAs) in interstitial lung disease (ILD), management of idiopathic inflammatory myopathies (IIM) associated... (Review)
Review
OBJECTIVE
To evaluate the role of myositis-specific autoantibodies (MSAs) in interstitial lung disease (ILD), management of idiopathic inflammatory myopathies (IIM) associated ILD, and if there is a role for MSA specific management of ILD.
METHODS
A systematic review was performed examining how MSAs relate to ILD manifestations in IIM patients and comparing treatment outcomes with varying immunosuppressive regimens.
RESULTS
112 papers were included in this analysis. Patients with anti-aminoacyl tRNA synthetase (anti-ARS) and anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibodies had consistently higher rates of ILD than other MSA groups. Anti-ARS positive patients had higher rates of chronic ILD whereas anti-MDA5 positive patients had higher rates of rapidly progressive ILD (RP-ILD). The most common high-resolution computed tomography (HRCT) patterns for ILD in anti-ARS and anti-MDA5 positive patients were nonspecific interstitial pneumonia (NSIP) and unclassifiable respectively. Anti-transcription intermediary factor 1-gamma (anti-TIF1-γ), anti-Mi-2, anti-nuclear matrix protein 2 (anti-NXP-2), and anti-3-hydroxy-3-methylglutaryl coenzyme A reductase (anti-HMGCR) antibodies were associated with a decreased risk of ILD. Small sample sizes, a lack of head-to-head trials, and non-randomized designs prevented drawing meaningful conclusions with respect to immunosuppressive management.
CONCLUSION
Clear relationships exist with regards to the ILD manifestations of certain MSAs. Standard therapy for IIM associated ILD (IIM-ILD) is glucocorticoids with the addition of others immunosuppressives in patients with or at risk of RP-ILD as well as in refractory cases. Immunosuppressives should be preferentially used in MSA populations in which they have been studied and shown to be efficacious.
Topics: Humans; Myositis; Lung Diseases, Interstitial; Autoantibodies; Immunosuppressive Agents; Retrospective Studies
PubMed: 36116345
DOI: 10.1016/j.semarthrit.2022.152088