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The Cochrane Database of Systematic... Jul 2019Morphea (morphoea) is an immune-mediated disease in which excess synthesis and deposition of collagen in the skin and underlying connective tissues results in hardened...
BACKGROUND
Morphea (morphoea) is an immune-mediated disease in which excess synthesis and deposition of collagen in the skin and underlying connective tissues results in hardened cutaneous areas. Morphea has different clinical features according to the subtype and stage of evolution of the disease. There is currently no consensus on optimal interventions for morphea.
OBJECTIVES
To assess the effects of treatments for people with any form of morphea.
SEARCH METHODS
We searched the following databases up to July 2018: the Cochrane Skin Specialised Register, CENTRAL, MEDLINE, Embase, LILACS, and five trial registers. We checked the reference lists of included studies for further references to relevant randomised controlled trials.
SELECTION CRITERIA
Randomised controlled trials of topical, intralesional, or systemic treatments (isolated or combined) in anyone who has been clinically diagnosed by a medical practitioner with any form of morphea. Eligible controls were placebo, no intervention, any other treatment, or different doses or duration of a treatment.
DATA COLLECTION AND ANALYSIS
We used standard methodological procedures expected by Cochrane. The primary outcomes were global improvement of disease activity or damage assessed by a medical practitioner or by participants, and adverse effects. Secondary outcomes were improvement of disease activity and improvement of disease damage. We used GRADE to assess the quality of the evidence for each outcome.
MAIN RESULTS
We included 14 trials, with a total of 429 randomised participants, aged between 3 and 76 years. There were juvenile and adult participants; over half were female, and the majority had circumscribed morphea, followed by linear scleroderma. The settings of the studies (where described) included a dermatologic centre, a national laboratory centre, paediatric rheumatology and dermatology centres, and a university hospital or medical centre.The studies evaluated heterogenous therapies for different types of morphea, covering a wide range of comparisons. We were unable to conduct any meta-analyses. Seven studies investigated topical medications, two evaluated intralesional medications, and five investigated systemic medications. The study duration ranged from seven weeks to 15 months from baseline.We present here results for our primary outcomes for our four key comparisons. All of these results are based on low-quality evidence.The included studies were at high risk of performance, detection, attrition, and reporting bias.Global improvement of disease activity or damage after treatment may be higher with oral methotrexate (15 mg/m², maximum 20 mg, once a week, for 12 months or until disease flare) plus oral prednisone (1 mg/kg a day, maximum of 50 mg, in a single morning dose, for three months, and one month with gradually decreased dose until discontinuation) than with placebo plus oral prednisone in children and adolescents with active morphea (linear scleroderma, generalised morphea or mixed morphea: linear and circumscribed) (risk ratio (RR) 2.31, 95% confidence interval (CI) 1.20 to 4.45; number needed to treat for an additional beneficial outcome (NNTB) 3; 1 randomised controlled trial (RCT); 70 participants, all juvenile). This outcome was measured 12 months from the start of treatment or until flare of the disease. Data were not available separately for each morphea type. There may be little or no difference in the number of participants experiencing at least one adverse event with oral methotrexate (26/46) or placebo (11/24) (RR 1.23, 95% CI 0.75 to 2.04; 1 RCT; 70 participants assessed during the 12-month follow-up). Adverse events related to methotrexate included alopecia, nausea, headache, fatigue and hepatotoxicity, whilst adverse events related to prednisone (given in both groups) included weight gain (more than 5% of body weight) and striae rubrae.One three-armed RCT compared the following treatments: medium-dose (50 J/cm²) UVA-1; low-dose (20 J/cm²) UVA-1; and narrowband UVB phototherapy. There may be little or no difference between treatments in global improvement of disease activity or damage, as assessed through the modified skin score (where high values represent a worse outcome): medium-dose UVA-1 phototherapy versus low-dose UVA-1 group: MD 1.60, 95% CI -1.70 to 4.90 (44 participants); narrowband UVB phototherapy versus medium-dose UVA-1 group: MD -1.70, 95% CI -5.27 to 1.87 (35 participants); and narrowband UVB versus low-dose UVA-1 group: MD -0.10, 95% CI -2.49 to 2.29 (45 participants). This RCT included children and adults with active morphea (circumscribed morphea, linear scleroderma (with trunk/limb variant and head variant), generalised morphea, or mixed morphea), who received phototherapy five times a week, for eight weeks. Outcomes were measured at eight weeks from the start of treatment.Safety data, measured throughout treatment, from the same RCT (62 participants) showed that treatment with UVA-1 phototherapy may cause mild tanning compared to narrowband UVB: narrowband UVB versus medium-dose UVA-1: RR 0.03, 95% CI 0.00 to 0.42; 35 participants; narrowband UVB versus low-dose UVA-1: RR 0.03, 95% CI 0.00 to 0.41; 45 participants. However, there may be no difference in the number of participants reporting mild tanning when comparing medium and low dose UVA-1 phototherapy (RR 1.00, 95% CI 0.91 to 1.10; 44 participants). Transient erythema was reported in three participants with narrowband UVB and no participants in the low- or medium-dose UVA-1 groups.
AUTHORS' CONCLUSIONS
Compared to placebo plus oral prednisone, oral methotrexate plus oral prednisone may improve disease activity or damage in juvenile active morphea (linear scleroderma, generalised morphea or mixed morphea: linear and circumscribed), but there may be a slightly increased chance of experiencing at least one adverse event.When medium-dose UVA-1 (50 J/cm²), low-dose UVA-1 (20 J/cm²), and narrowband UVB were compared against each other in treating children and adults with active morphea (circumscribed morphea, linear scleroderma, generalised morphea and mixed morphea), there may be little or no difference between these treatments on global improvement of disease activity or damage. UVA-1 phototherapy may cause more mild tanning than narrowband UVB, but there may be no difference between medium- and low-dose UVA-1 phototherapy. These results are based on low-quality evidence.Limitations of data and analyses include risk of bias and imprecision (small number of participants or events and wide confidence intervals). We encourage multicentre RCTs to increase sample size and evaluate, with validated tools, different treatment responses according to the subtypes of morphea and age groups.
Topics: Adolescent; Adult; Aged; Child; Child, Preschool; Female; Humans; Male; Methotrexate; Middle Aged; Phototherapy; Prednisone; Randomized Controlled Trials as Topic; Scleroderma, Localized; Young Adult
PubMed: 31309547
DOI: 10.1002/14651858.CD005027.pub5 -
Journal of Clinical Medicine Sep 2021Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Current treatment paradigms rely on low levels of... (Review)
Review
Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup de sabre. The databases Scopus, PubMed, and EBSCO were searched for all reports discussing the treatment of localized scleroderma en coup de sabre. The keywords en coup de sabre, "facial linear scleroderma", and "morphea linearis", combined with "treatment" or "therapy" were used as search terms. A total of 34 articles analyzed treatment outcomes for patients with localized scleroderma en coup de sabre including 4 retrospective cohort studies, 2 prospective cohort studies, 4 case series, and 24 case reports, representing a total of 69 patients (38 children and 31 adults). Methotrexate was the most commonly investigated treatment (26 patients) with a highest response rate (26/26, 100%). Other treatments included systemic glucocorticosteroids (nine patients), followed by UVA1 (four patients), mycophenolate mofetil (two patients), hydroxychloroquine (five patients), abatacept (two patients), tocilizumab (three patients), cyclosporine (one patient), interferon gamma (one patient), PUVA therapy (two patients), NB-UVB therapy (one patient), and pulsed dye laser (one patient). Reconstructive and surgery treatment was successfully used for lesions with settled disease activity to improve the cosmetic aspect of the lesions. Conclusion: methotrexate is the most often-studied treatment and reported good clinical outcomes in children and adults with localized scleroderma en coup de sabre.
PubMed: 34640533
DOI: 10.3390/jcm10194517 -
Autoimmunity Reviews Jun 2022We conducted a systematic review, on behalf of the EULAR Study Group on Microcirculation in Rheumatic Diseases (EULAR SG MC/RD), to investigate the value of nailfold... (Review)
Review
Standardised interpretation of capillaroscopy in autoimmune idiopathic inflammatory myopathies: A structured review on behalf of the EULAR study group on microcirculation in Rheumatic Diseases.
OBJECTIVE
We conducted a systematic review, on behalf of the EULAR Study Group on Microcirculation in Rheumatic Diseases (EULAR SG MC/RD), to investigate the value of nailfold videocapillaroscopy (NVC) in idiopathic inflammatory myopathies (IIM).
METHODS
Three electronic databases were systematically searched to find all relevant manuscripts reporting NVC outcomes in IIM patients. Articles were assessed based on study design, population, NVC methodology and description of NVC results. To allow comparison between the articles, all NVC results were interpreted according to standardised capillaroscopic terminology, as previously consented by the EULAR SG MC/RD and the Scleroderma Clinical Trials Consortium (SCTC) Group on Capillaroscopy.
RESULTS
Of the 653 identified records; five were retained after critical appraisal on title, abstract and manuscript level. A marked difference in NVC was observed between (juvenile) dermatomyositis [(j)DM] versus polymyositis, healthy controls and systemic sclerosis patients. In addition, reduced capillary density and scleroderma pattern seem to be associated with active disease in (j)DM, while immunosuppressive treatment appears to reduce NVC abnormalities.
CONCLUSION
This is the first systematic review investigating NVC in IIM, interpreting the results according to an international consented standardised manner, as proposed by the EULAR SG MC/RD and SCTC Group on Capillaroscopy. We can conclude that NVC presents a promising asset in the diagnosis of (j)DM. Moreover, NVC could be a biomarker for organ involvement and follow-up. Large multicentre prospective standardised studies are further needed to definitely describe associations with clinical and laboratory parameters in the different IIM subtypes.
Topics: Autoimmune Diseases; Capillaries; Dermatomyositis; Humans; Microcirculation; Microscopic Angioscopy; Myositis; Nails; Prospective Studies; Rheumatic Diseases; Scleroderma, Localized; Scleroderma, Systemic
PubMed: 35421608
DOI: 10.1016/j.autrev.2022.103087 -
Journal of Clinical Medicine Jul 2021Morphea, also known as localized scleroderma (LoS), comprises a set of autoimmune sclerotic skin diseases. It is characterized by inflammation and limited thickening and... (Review)
Review
Morphea, also known as localized scleroderma (LoS), comprises a set of autoimmune sclerotic skin diseases. It is characterized by inflammation and limited thickening and induration of the skin; however, in some cases, deeper tissues might also be involved. Although morphea is not considered a life-threatening disease, the apparent cosmetic disfigurement, functional or psychosocial impairment affects multiple fields of patients' quality of life. Therapy for LoS is often unsatisfactory with numerous treatments that have only limited effectiveness or considerable side effects. Due to the advances in the application of lasers and their possible beneficial effects, the aim of this study is to review the reported usage of laser in morphea. We present a systematic review of available literature, performed with MEDLINE, Cinahl, Central, Scopus, Web of Science, and Google Scholar databases. We identified a total of twenty relevant studies (MEDLINE = 10, Cinahl = 1, Central = 0, Scopus = 2, Web of Science = 5, Google Scholar = 2) using laser therapy for LoS. Eight studies were focused on the use of PDL, six on fractional lasers (CO and Er:YAG), four on excimer, and two on either alexandrite or Nd:YAG.
PubMed: 34362192
DOI: 10.3390/jcm10153409 -
Annals of the Rheumatic Diseases Aug 2019In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and...
In 2012, a European initiative called Single Hub and Access point for paediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile localised scleroderma (JLS) is a rare disease within the group of paediatric rheumatic diseases (PRD) and can lead to significant morbidity. Evidence-based guidelines are sparse and management is mostly based on physicians' experience. This study aims to provide recommendations for assessment and treatment of JLS. Recommendations were developed by an evidence-informed consensus process using the European League Against Rheumatism standard operating procedures. A committee was formed, mainly from Europe, and consisted of 15 experienced paediatric rheumatologists and two young fellows. Recommendations derived from a validated systematic literature review were evaluated by an online survey and subsequently discussed at two consensus meetings using a nominal group technique. Recommendations were accepted if ≥80% agreement was reached. In total, 1 overarching principle, 10 recommendations on assessment and 6 recommendations on therapy were accepted with ≥80% agreement among experts. Topics covered include assessment of skin and extracutaneous involvement and suggested treatment pathways. The SHARE initiative aims to identify best practices for treatment of patients suffering from PRDs. Within this remit, recommendations for the assessment and treatment of JLS have been formulated by an evidence-informed consensus process to produce a standard of care for patients with JLS throughout Europe.
Topics: Administration, Oral; Adolescent; Child; Combined Modality Therapy; Consensus; Disease Management; Dose-Response Relationship, Drug; Drug Administration Schedule; Drug Therapy, Combination; Europe; Evidence-Based Medicine; Female; Humans; Male; Methotrexate; Phototherapy; Practice Guidelines as Topic; Prednisone; Prognosis; Scleroderma, Localized; Severity of Illness Index; Treatment Outcome
PubMed: 30826775
DOI: 10.1136/annrheumdis-2018-214697 -
Danish Medical Journal Jan 2017Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations:... (Review)
Review
INTRODUCTION
Diabetes mellitus and the prediabetic state are associated with a number of skin manifestations. This study is a systematic review of the following manifestations: acanthosis nigricans (AN), skin tags (ST), diabetic dermopathy (DD), rubeosis faciei (RF), pruritus (PR), granuloma annulare (GA), necrobiosis lipoidica (NL), scleroedema diabeticorum (SD) and bullosis diabeticorum (BD). These conditions possibly relate to underlying diabetogenic mechanisms. Our aim was to determine whether skin signs are feasible as cutaneous markers for the prediabetic or diabetic state.
METHODS
Data were collected from the databases PubMed, Embase and Cochrane. Articles were excluded if the populations presented with comorbidities or received treatment with drugs affecting the skin. Also, animal studies, studies with poor methodology and pilot studies were excluded.
RESULTS
Among the 34 included original articles, an association with diabetes was shown as follows: in eight articles with AN, five articles with ST, three articles with GA, two articles with NL, PR and SD respectively and in one article with RF. Three papers indirectly showed an association of DD with diabetes. Association between bullous skin lesions and diabetes was only documented by case reports and case series.
CONCLUSION
The results indicate a benefit of diabetes screening in individuals presenting with AN, ST or BD. Further studies are required to enlighten a possible association with RF, GA, SD or NL. Until such studies are available, it is advisable to screen individuals with the skin lesions presented by measuring their glycated haemoglobin.
Topics: Acanthosis Nigricans; Biomarkers; Blister; Blood Glucose; Diabetes Complications; Facial Dermatoses; Glycated Hemoglobin; Granuloma Annulare; Humans; Necrobiosis Lipoidica; Prediabetic State; Pruritus; Scleroderma, Localized; Skin Diseases
PubMed: 28007053
DOI: No ID Found -
Systematic Reviews Mar 2019Peer-led support groups play an important role in supporting people with chronic diseases. They may be particularly important for people with rare diseases who typically...
BACKGROUND
Peer-led support groups play an important role in supporting people with chronic diseases. They may be particularly important for people with rare diseases who typically do not have access to professional support options that focus on their disease-specific needs. Many peer-led support groups in rare diseases, however, are not sustained, and many patients do not have access to support groups. Training and education for peer support group leaders could address barriers to initiating and sustaining groups, but there is little evidence on the effectiveness of support group leader training programs. A previous systematic review evaluated the effects of training programs for peer leaders of support groups for people with medical illness on leader and support group outcomes, but it identified only one randomized controlled trial (RCT) that compared high- and low-resource training programs for cancer support group leaders. The trial did not find evidence that the high-resource program was more effective, but was limited by a small sample size and serious methodological limitations. To meet the needs of people living with the rare autoimmune connective tissue disease scleroderma, the Scleroderma Patient-centered Intervention Network has partnered with patient organizations to develop the Scleroderma Support group Leader EDucation Program, and a full-scale RCT to test the effectiveness of the program is planned. To verify the need for such a trial, we updated the previous systematic review.
UPDATED EVIDENCE
Review methods for the update were unchanged from the initial review. The updated database search yielded 1504 unique citations in addition to the 9757 assessed for eligibility in the previous review. All additional citations identified in the updated search were excluded at the title and abstract review stage.
CONCLUSIONS
Our systematic review update found that there is presently insufficient evidence on the effectiveness of training and support programs for peer leaders of disease-based support groups, highlighting the need for well-designed and rigorously conducted RCTs to examine the effects of training for peer leaders of support groups, especially in a rare disease context. The Scleroderma Patient-centered Intervention Network's trial of the Scleroderma Support group Leader EDucation Program will serve as such a trial.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO CRD42018096369.
Topics: Education, Nonprofessional; Humans; Leadership; Peer Group; Randomized Controlled Trials as Topic; Scleroderma, Localized; Scleroderma, Systemic; Self-Help Groups
PubMed: 30836989
DOI: 10.1186/s13643-019-0981-0 -
ACR Open Rheumatology Mar 2022The study objective was to conduct a systematic literature review (SLR) of ultrasound of the skin in patients with systemic sclerosis (SSc) to establish the degree to...
Examination of the Validity of Skin Ultrasound to Quantitate Skin Involvement for Multicenter Clinical Trials in Patients with Systemic Sclerosis (SSc): A Systematic Literature Review (SLR).
OBJECTIVE
The study objective was to conduct a systematic literature review (SLR) of ultrasound of the skin in patients with systemic sclerosis (SSc) to establish the degree to which ultrasound of the skin has been validated, using the Outcome Measures in Rheumatology (OMERACT) Filter.
METHODS
We conducted an SLR of publications between 1950 and 2018, using PubMed and Cochrane library, to examine ultrasound validity to quantitate SSc skin involvement. Inclusion criteria were as follows: (1) in English; (2) used the 1980 or 2013 classification criteria for SSc criteria; (3) either a randomized controlled trial, an observational study, or a case study including more than 15 patients; (4) subjects 18 years of age or older; (5) for mixed patient populations, SSc results were separable; and (6) the ultrasound machine was clearly described. Exclusion criteria were as follows: (1) not in English; (2) data did not record at least one of the validation criteria; (3) subjects aged less than 18 years; (4) subjects had disease other than SSc (eg, localized scleroderma or scleroderma-like disease); (5) a letter to the editor or an editorial; and (6) involved a modified Rodnan skin score of less than 2. Descriptive statistics were generated for each criterion.
RESULTS
From an initial 292 citations, 14 articles (1,055 patients) met inclusion and exclusion criteria. The status of validation for ultrasound was evaluated by using the OMERACT criteria of truth, discrimination, and feasibility (in turn divided into nine different criteria). Face, criterion, content, construct, reliability, and responsiveness criteria were met, and the feasibility criterion was partially met, whereas discrimination and reproducibility criteria were not met.
CONCLUSION
Based on an SLR through December 31, 2018, ultrasound of the skin met some but not all validation criteria for use in clinical trials.
PubMed: 34918494
DOI: 10.1002/acr2.11357