NICE has accredited the process used by BSR to create its clinical guidelines. The term began on 27 February 2012 and the current renewed accreditation is valid until 28 January 2023. More information on accreditation can be viewed at www.nice.org.uk/accreditation. Idiopathic inflammatory myopathy (IIM) is a multi-system autoimmune condition characterised by muscle inflammation (myositis), interstitial lung disease (ILD), and skin manifestations with an incidence of up to 19 per 1,000,000 person-years in adults and up to 4 per 1,000,000 person-years in children. Estimated UK prevalence (for adult-onset IIM) is 10, 000 [1, 2]. No rigorously produced evidence-based guidelines for IIM spanning juvenile and adult-onset disease exists. Assimilating key research relating to management and formation of practical evidence-based recommendations will aid clinicians and help optimize management and outcomes. The target readership is clinicians caring for patients with IIM, including paediatric and adult rheumatologists, neurologists, dermatologists, respiratory physicians, oncologists, gastroenterologists, and cardiologists. Rheumatology and neurology nurses, physiotherapists, occupational therapists, podiatrists, speech and language therapists, specialist rheumatology pharmacists, and psychologists will also find these recommendations relevant. Diagnosis, classification, and investigation of suspected IIM are not addressed. Inclusion body myositis is not covered. The project was led by an executive committee (J.B.L., A.G.S.O., H.C., N.M., L.M.). A multidisciplinary working group was convened with input from rheumatologists (D.I., H.G., H.T., N.Mc., A.P., P.G., S.T., H.C., P.K., P.M.M., A.G.S.O.), paediatric rheumatologists (C.Pi., C.Pa., N.Ma., K.W., L.M., T.A.), neurologists (J.B.L., M.H.), a paediatric neurologist (S.Sp.), a nurse (P.L.), a pharmacist (O.A.), a dermatologist (S.M.), paediatric dermatologist (R.M.), physiotherapists (V.C., S.M., W.J.G.), and a former GP/Specialty Doctor in Rheumatology (J.J.). Lay (patient and relative) input was also received throughout the process (P.T., Y.T., J.S., P.R.). The guideline production process was informed by a EULAR Recommended Methodologist (P.M.M.) and literature searches were carried out by experts at the Centre for Rheumatic Diseases, Kings College London (J.G., K.B., M.Y.). This guideline was developed in line with the BSR Creating Clinical Guidelines Protocol using AGREEII (Appraisal of Guidelines for Research and Evaluation II) methodology. Starting March 2018, the executive committee and working group agreed the guideline scope and created key questions structured using the PICO (patient or population, intervention, comparison, outcome) format. Each question was subdivided into focused clinical questions during the evidence review and recommendation formulation process. Using key questions as a basis, a literature search was undertaken using Ovid (see ‘Search terms’ in Supplementary Material S1, available at Rheumatology online). Search results and additional manually identified references up to October 2020 were included. Evidence published after October 2020 was not included as this was the cut-off for eligibility. A potential limitation of this guideline is that relevant literature may have been published since October 2020; data or information from these studies could not be included in the recommendation formation process. Published peer reviewed clinical studies relating to any IIM subtype except inclusion body myositis were included. Case reports/series were limited to those describing outcomes for three or more subjects. Review articles, editorials, conference proceedings, and existing clinical guidelines were excluded. Non-English language papers were excluded unless a translation was published. Basic science studies without clear clinical applicability were excluded. Abstracts of papers were reviewed by two authors to determine eligibility against these criteria (PRISMA flow diagram shown in Supplementary Fig. S1, available at Rheumatology online). The full text of each eligible paper was reviewed by two assessors using Grading of Recommendations, Assessment, Development, and Evaluations (GRADE) methodology. Each reference was categorized as high (A), moderate (B) or low/very low (C) quality. A third assessor resolved disagreements. A total of 213 papers were used to form recommendations. See Supplementary Table S1 (available at Rheumatology online) for details of evidence base contributing to recommendations. Draft recommendations were created and categorized as applicable to all patients, adult-specific or paediatric-specific. The process outlined by the Scottish Intercollegiate Guidelines Network (SIGN) [3] was used to summarize the quality of body of evidence for each recommendation: high (A), moderate (B), low (C) or very low (D), according to GRADE methodology. Content, wording, strength of recommendation (strong = 1, conditional = 2), and quality of supporting evidence for each recommendation were subjected to a formal consensus building process using a combination of face-to-face meetings and online surveys. Strength of agreement (SoA) for finalized recommendations was determined using a simple binary voting system for each voter and is presented as a percentage. Authors were free to abstain from voting on areas where they did not feel clinically competent, with the percentage reflecting voters. Only recommendations with a SoA >80% were included in the guideline. Requirement for updates will be considered by the BSR Standards, Audit, and Guidelines Working Group and according to principles outlined in the BSR Creating Clinical Guidelines Protocol. Recommendations are followed by parentheses detailing GRADE and SoA details (strength of recommendation, quality of body of evidence, SoA). 1-High dose glucocorticoids should be used to treat active muscle inflammation at time of treatment induction (1, B, 100%). 1a-Adult-specific. Oral prednisolone at a dose of 0.5–1 mg/kg/day, usually 40–60 mg, is recommended (1, B, 100%). 1b-Paediatric-specific. Oral prednisolone at a dose of 1–2 mg/kg/day or intravenous methylprednisolone pulses 30 mg/kg/day, maximum 1 g daily i.v. dose is recommended (1, B, 100%). 1c-Intravenous methylprednisolone is to be considered, especially when there are concerns about gastrointestinal absorption. Use of intravenous methylprednisolone may allow increased therapeutic effect and less toxicity compared with oral glucocorticoid (2, B, 96%). 2-Oral prednisolone should be tapered according to clinical response (1, B, 100%). 3-Disease modifying anti-rheumatic drugs should be used to reduce muscle inflammation, achieve clinical remission and reduce steroid burden (1, C, 100%). 3a-Paediatric-specific. Early, complete control of muscle weakness and inflammation should be sought in juvenile-onset IIM, with the aim of improving outcomes and reducing disease-related complications (1, B, 100%). 3b-Paediatric-specific. A combination of high dose glucocorticoid and methotrexate should be used as first-line treatment in most cases (1, B, 100%). 3c-Paediatric-specific. A combination of prednisolone and methotrexate, as opposed to prednisolone and ciclosporin, should be used for the treatment of juvenile-onset IIM as this has a more favourable side effect profile (1, B, 100%). 3d-Paediatric-specific. Mycophenolate mofetil is to be considered as a treatment option to improve skin and muscle disease (2, C, 100%). 3e-Adult-specific. Methotrexate, azathioprine, tacrolimus, ciclosporin, and mycophenolate mofetil are to be considered for the treatment of active myositis and long-term maintenance of disease remission (2, C, 96%). 4-Intravenous immunoglobulin should be considered as a treatment of severe and/or refractory muscle inflammation (1, B, 100%). 5-Management of IIM should include a safe and appropriate exercise programme led and monitored by a specialist physiotherapist and/or a specialist occupational therapist to improve quality of life and function (1, B, 100%). 6-Rituximab is to be considered as a treatment option for refractory myositis and may be particularly effective in (2, A, 100%): Juvenile-onset disease Patients with a positive myositis autoantibody profile Patients with lower burden of disease damage 7-Cyclophosphamide should be considered as a treatment option for severe and/or refractory IIM (1, B, 100%). 8-Adult-specific. Abatacept is to be considered as a treatment option in refractory adult IIM (2, B, 100%). Glucocorticoids are crucial for myositis remission induction and maintenance. Glucocorticoid dose should be weaned when disease activity, considered across all domains, substantially improves, usually after around 6 weeks of treatment initiation. Available evidence precludes evidence-based recommendations regarding rate of glucocorticoid dose reduction. Whilst dosages per kilogram are included in recommendations for juvenile onset disease, it is important to note that ceiling doses may apply. Steroid-free remission can be facilitated using DMARDs and/or additional immunosuppressive/immunomodulatory treatments. Evidence exists to support use of conventional synthetic DMARDs (csDMARDs) (tacrolimus, azathioprine, methotrexate, ciclosporin, mycophenolate mofetil) alongside glucocorticoids early in the disease course to induce and maintain remission, although conflicting results exist in some cases [4–10]. Evidence does not exist to allow recommendation of specific csDMARDs as first-/second-/third-line for adults. DMARDs should be prescribed and monitored according to existing age-appropriate BSR guidelines [11, 12]. Exercise is safe and effective for people with IIM and can improve quality of life and function. Specialist physiotherapy and occupational therapy input is important for management of patients with IIM and should be considered in service planning to ensure appropriate access for all patients. Evidence exists allowing recommendation of use of ‘second-line’ treatments, such as CYC, rituximab (RTX), IVIG and abatacept, for patients with persistent active disease despite glucocorticoid and csDMARD therapy. A prospective, double-blind, randomized, placebo-controlled phase III study, completed after the cut-off date for evidence inclusion, has demonstrated efficacy of IVIG [13]. CYC is an option for severe and/or refractory IIM. Route of administration should be considered since intravenous (i.v.) CYC (intermittent pulses), compared with oral CYC, is associated with fewer side effects. CYC is usually administered by i.v. infusion [14], reducing risk of leucopenia, haemorrhagic cystitis, and gonadal toxicity [15, 16]. RTX and IVIG are options for management of active IIM (e.g. myositis, dysphagia, refractory skin disease) refractory to glucocorticoid/csDMARD-based immunosuppression. In England, RTX and IVIG can only be used according to NHS England (NHSE) commissioning stipulations and should be prescribed in conjunction with a specialist centre [17, 18]. NHSE guidance does not apply in Wales, Northern Ireland or Scotland. In Scotland, the National Plasma Products Expert Advisory Group (NPPEAG) indicates IVIG as appropriate for patients with resistant or aggressive disease [19]. A single prospective delayed-start study has demonstrated the benefit of abatacept in adult-onset IIM [20]. Future studies are required to confirm efficacy. At time of recommendation consensus forming there was insufficient evidence to recommend anti-TNF-α therapy for treatment of myositis. There was also insufficient evidence to recommend use of Janus kinase (JAK) inhibitors in IIM treatment; however, published case series are promising and future clinical trials may provide a stronger evidence base [21–23]. 1-Rituximab is to be considered for the treatment of skin disease refractory to glucocorticoid/csDMARD-based immunosuppression (2, B, 100%). 2-IVIG should be considered for the treatment of skin disease refractory to glucocorticoid/csDMARD-based immunosuppression (1, B, 100%). 3-Sun avoidance and regular use of high factor broad spectrum sun cream is to be considered to reduce likelihood of a disease flare affecting skin or muscle (2, C, 100%). 4-Paediatric-specific. Systemic immunosuppressive drugs are to be considered for the treatment of ongoing skin disease activity, including reduced nailfold capillary density (2, C, 100%). 5-Paediatric-specific. An early increase in treatment is to be considered in patients with persistent skin disease to aid remission and reduce development of calcinosis (2, C, 100%). Inadequate evidence exists to allow recommendation of topical agents to treat IIM-specific skin manifestations; however, topical tacrolimus and glucocorticoids could be considered alongside dermatology input. Evidence relating to treatment of IIM-related skin manifestations is limited; however, studies indicate the ability of both IVIG and RTX to treat skin manifestations refractory to glucocorticoid/csDMARD-based immunosuppression. Nailfold capillary abnormalities in children with IIM can reflect systemic disease activity and should be considered when making treatment decisions [24]. Studies indicate sun exposure is associated with cutaneous and non-cutaneous DM and JDM disease flares [25]. Sun avoidance may thus form part of the management strategy for DM/JDM. 1-Paediatric-specific. Routine assessment of pulmonary function, including measurement of diffusing capacity or transfer factor of the lung for carbon monoxide (DLCO or TLCO) in juvenile-onset IIM should be performed, as pulmonary function abnormalities are frequent and may be asymptomatic (1, B, 100%). 2-Adult-specific. Interstitial lung disease should be screened for in high-risk patients (1, B, 100%). 3-Adult-specific. In the treatment of rapidly progressive interstitial lung disease (RP-ILD): Induction therapy with high dose steroids is to be considered (2, C, 96%). The use of ciclosporin or tacrolimus, alongside steroids, is to be considered in patients with RP-ILD (2, C, 96%). Cyclophosphamide or rituximab therapy is to be considered early, potentially as part of the induction regimen (2, C, 96%). 4-Adult-specific. In the treatment of chronic IIM-associated interstitial lung disease: Immunosuppression using steroids with or without a single DMARD (azathioprine, ciclosporin, tacrolimus, mycophenolate) is to be considered (2, C, 100%). Rituximab or cyclophosphamide is to be considered in treatment-resistant patients (2, C, 100%). IIM-related ILD management should be carried out alongside ILD-specialist respiratory physicians. ILD risk is increased with anti-synthetase syndrome, presence of an anti-synthetase-associated autoantibody, anti-melanoma differentiation-associated protein 5 autoantibody positivity, and scleroderma overlap. ILD screening methods include plain chest X-ray radiography, pulmonary function tests (including DLCO), and where indicated, high resolution CT scanning. Insufficient evidence exists to advise ILD screening frequency. Insufficient evidence exists to form recommendations regarding pharmacological management of IIM-associated ILD in paediatric patients. 1-Adult-specific. A bone health assessment should be performed, regardless of glucocorticoid therapy, and appropriate management instigated (1, B, 100%). Fracture risk consideration in IIM is important given glucocorticoid use, female preponderance, and average age of onset for adult disease [26]. Fragility fracture risk assessment should be carried out in accordance with NICE guidance at time of diagnosis and whenever risk factors change [27]. Glucocorticoid weaning, once remission is attained, may reduce fragility fracture risk. Studies, although limited by small populations, suggest JDM is associated with increased vertebral fracture risk, even before substantial corticosteroid exposure [28]. 1-Paediatric-specific. Juvenile-onset IIM should be managed by paediatric specialists as it differs from adult-onset IIM in several ways, including greater presence of subcutaneous calcification, less disease damage, lack of association with cancer, increased risk of and autoantibody (1, C, time to diagnosis is associated with disease early to a specialist service is to be considered (2, C, 100%). should be into when using that muscle function, and quality of life (1, B, 100%). 4-Paediatric-specific. should for of disease is associated with increased risk of (1, C, 5-Paediatric-specific. Patients with juvenile-onset IIM should be for calcinosis (1, C, 100%). appropriate such as the and should be used to muscle function, and quality of life There is in patients with disease compared with those with JDM Patients should be screened for disease and associated with increased risk of calcinosis include age at disease particularly disease onset in to diagnosis or to treatment more severe disease, disease and presence of protein Clinical and plain X-ray can be used to should be for myositis (1, B, 100%). and can disease and and may help treatment of results should be carried out in the of the clinical should not be used to disease 1-Paediatric-specific. Routine screening for is not in juvenile-onset IIM (1, B, 100%). 2-Adult-specific. The risk of should be considered in all patients and screening should be particularly considered in those with the risk factors (1, B, 100%): age at onset to immunosuppressive therapy disease onset for There is an association adult-onset IIM and Evidence to effective screening is limited indicates the of CT of the and for patients, such as positive patients. and can be considered in patients. In with adult-onset IIM, juvenile onset IIM is not associated with cancer, with literature only of case Routine screening in juvenile-onset IIM is not unless is to should be to disease is (1, B, 100%). should be managed in conjunction with specialists (1, B, 96%). is required as patients may be at risk of disease flare (1, C, 96%). should be managed alongside specialists to lower increased risk of such as and and during although indicates IIM control is associated with outcomes should be once disease remission is using with according to the BSR guideline on drugs in and 1-Adult-specific. Patients should a regular risk assessment (1, C, 100%). and management of risk factors is to be considered, including or abnormalities (2, C, 100%). IIM is associated with an increased incidence of and disease an for to reduce risk however, insufficient evidence exists to advise screening frequency. and glucocorticoid treatment are considered for the in of patients with JDM Studies have identified risk factors in JDM patients that may to increased risk of early in 1-Adult-specific. Patients should screening for damage and are to be considered (2, B, 100%). 2-Adult-specific. should be used as the for screening and (1, B, 100%). for in patients with juvenile-onset IIM with and is to be considered (2, C, 100%). myositis is associated with increased and risk. damage may indicate some particularly can also be and from potentially is including and reduced rate have been in people with IIM. assessment of is to be considered in all patients (2, C, assessment and of speech and language is to be considered in those with (2, C, 100%). therapy for active disease and resistant to is to be considered (2, C, 100%). is quality of and is associated with and can be may not be by muscle weakness is increased with or should for and early of speech and language when is as an for IVIG treatment by NHSE IVIG and including csDMARDs azathioprine, ciclosporin, tacrolimus, mycophenolate CYC, and RTX have been to improve of and/or and should be (1, C, and quality of life should be using an age-appropriate (1, B, 100%). quality of life (e.g. skin steroid should be (1, C, 96%). 4-Paediatric-specific. quality of life in children include muscle and and should be managed (1, C, exercise and/or should be across all of disease activity with the aim of improving (1, B, 96%). given by a specialist physiotherapist and/or a specialist occupational therapist to improve strength should be considered, to the of strength on of daily and quality of life (2, C, 96%). are in of quality of life in both adult and juvenile-onset IIM. Evidence a of IIM-specific factors that can such as active disease, increased and muscle strength can be using such as the and the specialist physiotherapy and/or occupational therapy should be for concerns such as low and and as early where can be is to be considered when clinical associated and risk factors may according to (2, C, 96%). to be at increased risk of disease, increased risk, and more at risk of juvenile are in children with JDM in and Supplementary Fig. (available at Rheumatology online) an of recommendations. There should be to in the Use of the Table available at Rheumatology online) is This guideline the limited evidence base available for IIM, with of or of treatments. Recommendations are on trials are crucial to promising treatments. outcomes especially to or of IIM on health and quality of life should not be Patients and should be in for future IIM UK and the for Rheumatology guideline on the management of and adult patients with inflammatory has followed NICE accredited in its development and The authors to BSR for support throughout each of formation of the guideline. The authors also to UK for that formation of the guideline. The authors to part in voting during the consensus A.G.S.O., H.C., and led guideline formation throughout all A.G.S.O., H.C., N.M., and the guideline was reviewed and by all and led the review process. Y.T., and (patient and relative) input throughout the process. authors to the consensus building and recommendation process and the and are by the National for Research Research Centre The in this are those of the authors and not those of the National the National for or the of production of these guidelines received support from UK and an Clinical in was by a National for Research England Clinical Research an Clinical in Rheumatology by NHS and the for Rheumatic and was by the Research in the form of a Clinical Research was also received from the for Rheumatology to and associated with a face-to-face consensus building authors have of in line with BSR (see Supplementary available at Rheumatology online). has received from and has received from and in a that has received from in a that has received from and has received support from and has received from and has received from and has received support from and has received from and has received from and authors have of relevant data produced during the guideline development process are presented in this or in the Supplementary data are available at Rheumatology
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Oldroyd et al. (2022) studied this question.