Inflammatory muscle involvement in systemic vasculitis: A systematic review

Abstract Introduction Inflammatory muscle disease is a rare but well-recognised manifestation of systemic vasculitis. It can present as focal myositis or symmetrical proximal muscle involvement seen in polymyositis and dermatomyositis. Investigative findings in inflammatory myositis include raised creatine kinase, abnormal electromyography and muscle biopsy. MR scans can identify muscle abnormality, oedema, fibrosis and the extent of muscle involvement, and are routinely requested to evaluate patients with suspected or definite myositis. They also guide us to the appropriate muscles to biopsy. MR scans are not diagnostic of myositis and treating clinicians must be aware of its mimics. Our case demonstrates one such mimic. Case description A 59-year-old male patient under care of the renal physicians for ANCA-associated vasculitis complained of bilateral thigh pain. He had completed IV cyclophosphamide therapy (CYCLOPs protocol) for vasculitis affecting lungs, kidneys, and ENT. He was taking 30mg prednisolone and 100mg azathioprine daily. He denied muscle weakness, paraesthesia, or sensory symptoms. There was no muscle wasting, fasciculation, or sensory loss. Deep tendon reflexes were normal. 8-months prior, he was investigated for right hip pain and paraesthesia with MR spine and right hip, and neurophysiological studies. Investigations confirmed lumbar disc prolapse with neurophysiology in keeping with right L4 denervation changes. He had been treated with physiotherapy and daily pregabalin, duloxetine, and co-codamol. Renal physicians requested MR thigh, which was reported as muscle oedema over the long length of the right vastus medialis and lateralis, suggesting myositis. Rheumatology referral for myositis related to vasculitis was made. He was PR3+ve-3IU/ml (improved), ESR-2, CRP-4.6mg/L, CK-94U/L. EGFR remained stable at 66ml/min. Clinical features were not supportive of myositis and systemic vasculitis was in remission. The decision was made to discuss scans at the rheumatology- radiology meeting before considering a change in immunosuppression or muscle biopsy. Radiologists felt that findings of localised muscle group involvement suggested a neurogenic cause rather than primary muscle inflammation. They had reviewed MR lumbar spine done previously which had identified degenerative changes with right foraminal stenosis at L3-4 and L4-5 disc space. Electrophysiology showed chronic neurogenic changes of moderate severity in predominately L4 innervated muscles – vastus medialis and adductor magnus of the right lower limb. Findings were supportive of chronic right L4 radiculopathy and not a vasculitic neuropathy or myositis. Hence immunosuppression was left unchanged. He responded well to physiotherapy and did not require spinal surgical intervention. Discussion Our patient developed bilateral thigh pain in the setting of vasculitis. MR imaging was suggestive of myositis. Differential diagnoses included myositis related to vasculitis, primary focal myositis followed by vasculitic neuropathy, or denervation changes due to nerve entrapment. MRI is an excellent imaging modality for skeletal muscle due to its soft tissue resolution. It helps in narrowing differentials in skeletal muscle pathology, clinicians are able to identify individually affected muscle or muscle groups, give information about active muscle inflammation and/or damage. They can assist clinicians in targeting muscle for biopsy. Pattern and extent of muscle involvement can provide valuable clue to peripheral nerve or root lesion. Though MR suggested myositis, clinical assessment was not typical of myositis. Our patient had no systemic features of active vasculitis, which are concurrently present in 80% of vasculitic neuropathies. Neurophysiology in vasculitic neuropathy typically shows evolving multifocal axonal neuropathy with reduced compound muscle action potential amplitudes. His electrophysiology was of chronic denervation in the L4 region and explained the patient’s clinical symptoms and MR findings. For skeletal muscle pathology, three key patterns of MRI signal intensity include mass lesions, fatty infiltration and muscular oedema. In this patient, recognising unilateral muscle oedema localised to the vastus medialis and lateralis (innervated by L4) was key to diagnosis. Other muscles innervated by L4 nerve root are iliopsoas, tibialis anterior and posterior. Neurophysiology did demonstrate changes in iliopsoas muscle. In this patient, the muscle atrophy common to lower motor neurone lesions was not present. This is possible when partial denervation or reinnervation occurs, changes noted on neurophysiological studies in this patient. Our case shows MR evidence of myositis may not always equate to inflammatory myositis. In this patient, awareness of the radiological mimics of myositis avoided a muscle biopsy or further immunosuppression. Key learning points Myositis is an uncommon but recognised manifestation of systemic vasculitis. Chronic denervation can cause muscle abnormalities that appears similar to myositis on MR scan of the affected muscle. As such Clinicians need to be aware of this similarity and in patients with localised or focal myositis. Knowledge of the muscles innervations may help to establish a neurogenic aetiology where muscles are solely affected. Muscle atrophy can be notably absent in denervated muscle if the nerve is partially denervated or regenerating. Conflict of interest The authors declare no conflicts of interest.

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The prevalence and impact of depression in primary systemic vasculitis: a systematic review and meta-analysis

Rheumatology International ◽

10.1007/s00296-020-04611-7 ◽

2020 ◽

Vol 40 (8) ◽

pp. 1215-1221

Author(s):

Bradley Pittam ◽

Sonal Gupta ◽

Ashar E. Ahmed ◽

David M. Hughes ◽

Sizheng Steven Zhao

Keyword(s):

Systematic Review ◽

Systemic Vasculitis ◽

Meta Analysis ◽

Primary Systemic Vasculitis

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Biological therapy for systemic vasculitis: A systematic review

Seminars in Arthritis and Rheumatism ◽

10.1016/j.semarthrit.2013.07.010 ◽

2014 ◽

Vol 43 (4) ◽

pp. 542-557 ◽

Cited By ~ 28

Author(s):

Lucía Silva-Fernández ◽

Estíbaliz Loza ◽

Víctor M. Martínez-Taboada ◽

Ricardo Blanco ◽

Íñigo Rúa-Figueroa ◽

...

Keyword(s):

Systematic Review ◽

Biological Therapy ◽

Systemic Vasculitis

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AB0514 CALF PAIN, KEY POINT IN THE DIAGNOSIS OF POLYARTERITIS NODOSA

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.6578 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1554.2-1554

Author(s):

J. S. Peinado Acevedo ◽

M. Calle ◽

A. Medina ◽

D. Jaramillo Arroyave ◽

A. Vanegas ◽

...

Keyword(s):

Polyarteritis Nodosa ◽

Systemic Vasculitis ◽

Clinical Manifestations ◽

Hbv Infection ◽

Renal Infarction ◽

Laboratory Findings ◽

Cross Sectional ◽

Muscle Involvement ◽

Severity Scores ◽

Calf Pain

Background:polyarteritis nodosa (PAN) is a primary systemic vasculitis that is becoming a rare disease in part by the decrease in hepatitis B virus (HBV) infection due to widespread vaccination. It is characterized by a full vast constellation of nonspecific clinical manifestations, which sometimes delays and makes it difficult to diagnose. Still, muscle involvement is a feature that could guide the clinician.Objectives:to describe the main clinical and laboratory characteristics of patients with PAN and to confirm the frequency of muscle involvement.Methods:retrospective cross-sectional descriptive study of 23 adult patients diagnosed with PAN between January 2011 and December 2018 in two high complexity hospitals in Medellin-Colombia.Results:twenty-three patients met ACR 1990 classification criteria for PAN, 52% were men with a median age of 51 (IR 36-60), 78.3% were newly diagnosed, and only two patients (8.7%) had HBV infection. General symptoms (found in 95% of the patients), cutaneous (82%), and articular (56%) were the most frequent manifestations. Among systemic symptoms, myalgia, especially calf pain, was the most common characteristic (78.3%), followed by weight loss (73.9%), fatigue (69.3%), and fever (59.3%). Laboratory findings and severity scores are shown in the table. Angiography was performed in 27.3% of patients, finding splanchnic (renal, hepatic and splenic) microaneurysms (17.4%), stenosis (13%), and renal infarction (4.3%). Fourteen patients (61%) had at least one positive biopsy documenting medium-sized artery vasculitis, mainly skin, muscle, nerve, or both; 9 (39%) had normal or inconclusive biopsy findings. All patients received high daily doses of prednisolone (50 ± 16 mg); 52.2% required cyclophosphamide, 30.4% azathioprine, 17.4% methotrexate, 8.7% rituximab, 4.3% dapsone and 4.3% plasmapheresis; acetylsalicylic acid was given to half of the patients and only one required antiviral therapy for HBV. With treatment, 87% improved; 22.7% had an infection, and 8.7% of patients died.Conclusion:myalgia was the main characteristics of our PAN patients, especially in calves, and its presence in patients with other general, skin or articular symptoms should raise the suspect of this vasculitis.References:[1]Karadag O, Jayne DJ. Polyarteritis nodosa revisited: a review of historical approaches, subphenotypes and a research agenda. Clin Exp Rheumatol. 2018;36 Suppl 111(2):135–142.[2]Pagnoux C, Seror R, Henegar C, et al. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616–626.TableCharacteristicPAN patients (n=23)CRP (mean and SD in mg/dl)6.3 ± 8.51ESR (mean and SD in mm/h)84 ± 38CPK (median and IR in U/L) normal value < 18076 (66)FFS (mean)1BVAS (median and IR)17 (7)PAN: polyarteritis nodosa; CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; CPK: creatine phosphokinase; FFS: five factor score; BVAS: Birmingham Vasculitis Activity Score; SD: standard desviation; IR (interquartile range)Disclosure of Interests:None declared

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The effects of low-ratio n-6/n-3 PUFA on biomarkers of inflammation: a systematic review and meta-analysis

Food & Function ◽

10.1039/d0fo01976c ◽

2021 ◽

Author(s):

Yali Wei ◽

Yan Meng ◽

Na Li ◽

Qian Wang ◽

Liyong Chen

Keyword(s):

Systematic Review ◽

Fatty Acid ◽

Polyunsaturated Fatty Acid ◽

Meta Analysis ◽

Chain Polyunsaturated Fatty Acid ◽

Inflammation Markers ◽

Pufa Supplementation ◽

Long Chain

The purpose of the systematic review and meta-analysis was to determine if low-ratio n-6/n-3 long-chain polyunsaturated fatty acid (PUFA) supplementation affects serum inflammation markers based on current studies.

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Parent Education in Studies With Nonverbal and Minimally Verbal Participants With Autism Spectrum Disorder: A Systematic Review

American Journal of Speech-Language Pathology ◽

10.1044/2019_ajslp-19-00007 ◽

2020 ◽

Vol 29 (2) ◽

pp. 890-902

Author(s):

Lynn Kern Koegel ◽

Katherine M. Bryan ◽

Pumpki Lei Su ◽

Mohini Vaidya ◽

Stephen Camarata

Keyword(s):

Systematic Review ◽

Autism Spectrum Disorder ◽

Parent Education ◽

Autism Spectrum ◽

Verbal Communication ◽

Fidelity Of Implementation ◽

Spectrum Disorder ◽

Education Programs ◽

Minimally Verbal ◽

Parent Education Programs

Purpose The purpose of this systematic review was to identify parent education procedures implemented in intervention studies focused on expressive verbal communication for nonverbal (NV) or minimally verbal (MV) children with autism spectrum disorder (ASD). Parent education has been shown to be an essential component in the habilitation of individuals with ASD. Parents of individuals with ASD who are NV or MV may particularly benefit from parent education in order to provide opportunities for communication and to support their children across the life span. Method ProQuest databases were searched between the years of 1960 and 2018 to identify articles that targeted verbal communication in MV and NV individuals with ASD. A total of 1,231 were evaluated to assess whether parent education was implemented. We found 36 studies that included a parent education component. These were reviewed with regard to (a) the number of participants and participants' ages, (b) the parent education program provided, (c) the format of the parent education, (d) the duration of the parent education, (e) the measurement of parent education, and (f) the parent fidelity of implementation scores. Results The results of this analysis showed that very few studies have included a parent education component, descriptions of the parent education programs are unclear in most studies, and few studies have scored the parents' implementation of the intervention. Conclusions Currently, there is great variability in parent education programs in regard to participant age, hours provided, fidelity of implementation, format of parent education, and type of treatment used. Suggestions are made to provide both a more comprehensive description and consistent measurement of parent education programs.

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Characteristics of the Frequency-Following Response to Speech in Neonates and Potential Applicability in Clinical Practice: A Systematic Review

Journal of Speech Language and Hearing Research ◽

10.1044/2020_jslhr-19-00322 ◽

2020 ◽

Vol 63 (5) ◽

pp. 1618-1635

Author(s):

Céline Richard ◽

Mary Lauren Neel ◽

Arnaud Jeanvoine ◽

Sharon Mc Connell ◽

Alison Gehred ◽

...

Keyword(s):

Systematic Review ◽

Cochrane Library ◽

Neurodevelopmental Outcomes ◽

Frequency Following Response ◽

Ovid Medline ◽

Published Evidence ◽

Mesh Terms ◽

Potential Applicability ◽

Clinical Potential

Purpose We sought to critically analyze and evaluate published evidence regarding feasibility and clinical potential for predicting neurodevelopmental outcomes of the frequency-following responses (FFRs) to speech recordings in neonates (birth to 28 days). Method A systematic search of MeSH terms in the Cumulative Index to Nursing and Allied HealthLiterature, Embase, Google Scholar, Ovid Medline (R) and E-Pub Ahead of Print, In-Process & Other Non-Indexed Citations and Daily, Web of Science, SCOPUS, COCHRANE Library, and ClinicalTrials.gov was performed. Manual review of all items identified in the search was performed by two independent reviewers. Articles were evaluated based on the level of methodological quality and evidence according to the RTI item bank. Results Seven articles met inclusion criteria. None of the included studies reported neurodevelopmental outcomes past 3 months of age. Quality of the evidence ranged from moderate to high. Protocol variations were frequent. Conclusions Based on this systematic review, the FFR to speech can capture both temporal and spectral acoustic features in neonates. It can accurately be recorded in a fast and easy manner at the infant's bedside. However, at this time, further studies are needed to identify and validate which FFR features could be incorporated as an addition to standard evaluation of infant sound processing evaluation in subcortico-cortical networks. This review identifies the need for further research focused on identifying specific features of the neonatal FFRs, those with predictive value for early childhood outcomes to help guide targeted early speech and hearing interventions.

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Treating Velopharyngeal Inadequacy Using Bilateral Buccal Flap Revision Palatoplasty

Perspectives of the ASHA Special Interest Groups ◽

10.1044/2019_pers-sig5-2019-0005 ◽

2019 ◽

Vol 4 (5) ◽

pp. 878-892

Author(s):

Joseph A. Napoli ◽

Linda D. Vallino

Keyword(s):

Systematic Review ◽

Obstructive Sleep Apnea ◽

Sleep Apnea ◽

Cleft Lip ◽

Cleft Lip And Palate ◽

A Priori ◽

Sufficient Evidence ◽

Obstructive Sleep ◽

Before And After ◽

Buccal Flap

Purpose The 2 most commonly used operations to treat velopharyngeal inadequacy (VPI) are superiorly based pharyngeal flap and sphincter pharyngoplasty, both of which may result in hyponasal speech and airway obstruction. The purpose of this article is to (a) describe the bilateral buccal flap revision palatoplasty (BBFRP) as an alternative technique to manage VPI while minimizing these risks and (b) conduct a systematic review of the evidence of BBFRP on speech and other clinical outcomes. A report comparing the speech of a child with hypernasality before and after BBFRP is presented. Method A review of databases was conducted for studies of buccal flaps to treat VPI. Using the principles of a systematic review, the articles were read, and data were abstracted for study characteristics that were developed a priori. With respect to the case report, speech and instrumental data from a child with repaired cleft lip and palate and hypernasal speech were collected and analyzed before and after surgery. Results Eight articles were included in the analysis. The results were positive, and the evidence is in favor of BBFRP in improving velopharyngeal function, while minimizing the risk of hyponasal speech and obstructive sleep apnea. Before surgery, the child's speech was characterized by moderate hypernasality, and after surgery, it was judged to be within normal limits. Conclusion Based on clinical experience and results from the systematic review, there is sufficient evidence that the buccal flap is effective in improving resonance and minimizing obstructive sleep apnea. We recommend BBFRP as another approach in selected patients to manage VPI. Supplemental Material https://doi.org/10.23641/asha.9919352

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