Clinically amyopathic dermatomyositis associated with cutaneous ulcerations: a case-based review.

Introduction and importance: Dermatomyositis (DM) is an autoimmune myopathy primarily affecting both muscles and skin. When muscle weakness is not clinically apparent, but characteristic skin lesions are present, the condition is referred to as clinically amyopathic dermatomyositis (CADM). Case presentation: The authors present the case of a 52-year-old female with a typical DM rash, interstitial pneumonia, and multiple skin ulcers. The skin biopsy was consistent with DM, and there were no signs of muscle involvement. Myositis-related and myositis-specific autoantibodies were also negative. Significant improvement was not observed until the patient received successive monthly pulses of methylprednisolone and the introduction of methotrexate. This treatment regimen allowed for the complete tapering of prednisone and resulted in sustained disease control. Clinical discussion: In addition to the case presentation, a narrative literature review was conducted using the MEDLINE database, and an evidence-based treatment flowchart is proposed. CADM is a subtype of DM, related to higher incidences of interstitial lung disease, skin vasculopathy and malignancy. When ulcers or interstitial pneumonia are present, treatment should be early and aggressive. Active screening for neoplasms is recommended, particularly within the first 5 years. Conclusion: The authors presented a case of seronegative CADM featuring skin vasculopathy, successfully treated with consecutive methylprednisolone pulses. Our literature review emphasized the importance of focused CADM management trials, highlighting the need for further research.

Integrating the autoimmune connective tissue diseases for the medical student: A classification proposal based on pathogenesis and clinical phenotype.

It is hard for medical students to recognize and understand the clinical presentation of systemic connective tissue diseases (SCTDs). In this study, we aimed to review the immune mechanisms of the main SCTDs and to propose a classification system focused on the student and based on each immune dysfunction's clinical phenotype. The search involved the MEDLINE database and included the terms "systemic lupus erythematosus," "antiphospholipid syndrome," "inflammatory myopathies," "rheumatoid arthritis," "Sjögren's syndrome" or "systemic sclerosis" and "pathogenesis," and "immunology" or "mechanism of disease." Systemic lupus erythematosus (SLE) is a prototypic immune-complex disease with a tendency toward vascular injury. Antiphospholipid syndrome (APS) is a diffuse immune-mediated thrombotic vasculopathy. In inflammatory myopathies (IMs), muscle inflammation leading to muscle weakness is the cardinal manifestation. Rheumatoid arthritis (RA) is a unique form of erosive and destructive polyarthritis. Sjögren's syndrome (SS) causes sicca symptoms due to infiltration of the exocrine glands. Disseminated fibrosis in systemic sclerosis (SSc) is caused by vascular injury with excessive fibroblast activation. After the review, we created a focus group involving all the authors to group the diseases according to their pathogenesis and clinical phenotype. Our group agreed that SCTDs can be divided in 3 groups based on the preferential clinical presentation and immune dysfunction: 1) vasculopathic features (SLE and APS), 2) tissue inflammation (IMs, RA, and SS), and 3) tissue fibrosis (SSc). In synthesis, we suggest that clustering SCTDs in groups based on clinical phenotype and presumptive immune dysfunction instead of ordering autoantibodies randomly can help students understand the diseases.

Peripheral Neuropathy in Systemic Autoimmune Rheumatic Diseases-Diagnosis and Treatment.

Peripheral neuropathy (PN) is frequently observed in systemic rheumatic diseases and is a challenge in clinical practice. We aimed to review the evidence on the subject and proposed a comprehensive approach to these patients, facilitating diagnosis and management. We searched the MEDLINE database for the terms (and its respective Medical Subject Headings (MeSH) terms): "peripheral neuropathy" AND "rheumatic diseases" OR "systemic lupus erythematosus", "rheumatoid arthritis", "Sjogren syndrome", and "vasculitis" from 2000 to 2023. This literature review focuses on the diagnostic workup of PNs related to systemic lupus erythematosus, Sjögren's syndrome, rheumatoid arthritis, and systemic vasculitis. For every type of PN, we provide a pragmatic flowchart for diagnosis and also describe evidence-based strategies of treatment.

Physical exercise for dermatomyositis and polymyositis: a systematic review and meta-analysis.

Trials regarding physical exercise in dermatomyositis (DM) and polymyositis (PM) are heterogeneous. We aimed to summarize and critically analyze the available evidence to support the hypothesis that exercise is safe and improves strength and aerobic capacity. We performed a systematic review of clinical trials regarding physical exercise in dermatomyositis and polymyositis, without time restriction. We included studies from MEDLINE, EMBASE, SciELO, and Web of Science, published in English, Portuguese, or Spanish, and reporting outcomes related to safety, muscle performance, or aerobic capacity. The certainty of evidence was evaluated in accordance with the GRADE methodology. Meta-analysis was carried using pooled standardized mean differences (SMD) with 95% confidence interval as effect measure. We included 19 studies and 298 patients. The certainty of evidence was downgraded due to unbalanced confounding variables. The meta-analysis demonstrated improvements in strength (SMD [95% CI] = 0.61 [0.37-0.85], P < .00001) and aerobic capacity (SMD [95% CI] = 0.82 [0.29-1.34], P = .002), with no difference in creatine phosphokinase levels (SMD [95% CI] = - 0.23 [- 0.5-0.03], P = .08) after the interventions. No exacerbation was reported, and results were favorable in all stages of disease and ages, but might be different in the future with new classification criteria for PM and the inclusion of other idiopathic inflammatory myopathies. Novel approaches such as blood flow restriction training and aquatic plyometric exercises were promising. Physical exercise in DM/PM patients of all ages is probably safe and moderately improves muscle strength and aerobic capacity.

Exercise training attenuates insulin resistance and improves ß-cell function in patients with systemic autoimmune myopathies: a pilot study.

INTRODUCTION/OBJECTIVES: To assess the effects of exercise training on insulin resistance and ß-cell function in patients with systemic autoimmune myopathies (SAMs). METHOD: This quasi-experimental, prospective study includes 9 patients with SAMs (six with dermatomyositis, two with antisynthetase syndrome, and one with polymyositis). Patients were submitted to a 12-week, twice a week, exercise training program comprising aerobic and resistance exercises. Baseline and after the intervention, we evaluated disease status, aerobic capacity, muscle strength, body composition, insulin resistance, and ß-cell function parameters. RESULTS: The patients have a mean age of 46.7 years and stable disease. No clinical or laboratory parameter impairment was observed after the intervention. Compared with baseline, aerobic capacity, muscle strength, and function increased after 12 weeks (P < 0.05), while no changes were observed for body composition. Data from the oral glucose tolerance test showed that exercise did not change glucose area under the curve (AUC), whereas insulin and C-peptide AUC decreased significantly (P < 0.05). Furthermore, Matsuda index and HOMA2 percentage (both surrogates of insulin resistance) also improved (P < 0.05). CONCLUSION: Exercise training improved aerobic capacity, muscle strength, and muscle function in patients with SAMs. In addition, exercise training led to an attenuation of insulin resistance and improvements in ß-cell function parameters. These data indicate that exercise training can mitigate metabolic impairments, attenuating the cardiovascular risk in SAMs.Key Points• Exercise training improved aerobic capacity, muscle strength, and function without disease impairment• Exercise training was capable of improve insulin resistance and ß-cell function in patients with SAM• These results suggest that exercise can mitigate metabolic impairments in patients with SAM, attenuating the cardiovascular risk.

Intravenous human immunoglobulin and/or methylprednisolone pulse therapies as a possible treat-to-target strategy in immune-mediated necrotizing myopathies.

To evaluate the relevance of immunoglobulin (IVIg) and/or methylprednisolone pulse therapies in immune-mediated necrotizing myopathy (IMNM). Secondarily, to analyze the muscle damage measured by late magnetic resonance images (MRI). This retrospective study included 13 patients with defined IMNM (nine patients positive for the anti-signal recognition particle and four patients positive for hydroxyl-methyl-glutaryl coenzyme A reductase) who were followed from 2012 to 2018. International Myositis Assessment and Clinical Studies Group (IMACS) scoring assessed the response to a standardized treat-to-target protocol with disease activity core-set measures and late magnetic resonance imaging (MRI). The patients had a mean age of 53.5 years and were predominantly female and of white ethnicity. Median symptom and mean follow-up durations were 4 and 39 months, respectively. All patients received IVIg and/or methylprednisolone pulse therapies. All IMACS core-set measurements improved significantly after initial treatment. Nine patients achieved complete clinical response and among them 2 had complete remission. Eleven patients had discontinued glucocorticoid use by the end of the study. Only 2 patients had moderate muscle atrophy or fat replacement observed by MRI, with the remainder presenting normal or mild findings. Our patients with IMNM treated with an aggressive immunosuppressant therapy had a marked improvement in all IMACS core-set domains. Moreover, the MRI findings suggest that an early treat-to-target approach could reduce the odds of long-term muscle disability. Methylprednisolone and/or IVIg pulse therapies aiming at a target of complete clinical response are potential treatment strategies for IMNM that should be studied in future prospective studies.

Feasibility, safety and efficacy of exercise training in immune-mediated necrotising myopathies: a quasi-experimental prospective study.

OBJECTIVES: To evaluate the feasibility, safety and efficacy of exercise training in patients with immune-mediated necrotising myopathies (IMNM). METHODS: Eight consecutive sedentary patients with IMNM (5 anti-signal recognition particle and 3 anti-hydroxy-methyl-glutaryl coenzyme A reductase) were engaged in this study. Disease status was based on International Myositis Assessment and Clinical Studies Group (IMACS) core set measures. Physical performance was evaluated by cardiopulmonary exercise test, repetition maximum (RM) protocol, handgrip dynamometry, sit-to-stand (STS) and timed up-and-go (TUG) tests. All these parameters were measured at baseline and after a 12-week, twice-a-week, supervised exercise training comprising aerobic and strength exercises. RESULTS: Patients (aged 61 years on average) were very disabled at the beginning of the disease (mean duration of 17.7 months), but after being aggressively treated with a treat-to-target approach, they presented only mild symptoms that were well-controlled with oral immunosuppression and low disease status scores by the time of the exercise intervention. No disease relapsing, worsening of the IMACS set scores or adverse events were observed throughout the training period. Patients also increased aerobic capacity (e.g. time to achieve anaerobic threshold and time to achieve exhaustion), muscle strength (e.g. 1RM bench press) and function (e.g. STS test). CONCLUSIONS: Supervised exercise training did not impair disease status and seemed to be feasible, safe and effective in patients with IMNM. Moreover, exercise training increased aerobic capacity, muscle strength and function, suggesting that this could be a novel potential coadjuvant therapy in IMNM.