What should be the systemic review of a Juvenile Dermatomyositis (JDM) patient?

From the Guidelines

A comprehensive systemic review of a juvenile dermatomyositis (JDM) patient should include a thorough assessment of multiple organ systems, including skin, muscle, cardiopulmonary, gastrointestinal, and joint evaluations, as well as laboratory investigations and imaging studies, to guide appropriate treatment adjustments and improve patient outcomes. The assessment should begin with a thorough skin examination looking for characteristic findings such as Gottron's papules over the knuckles, heliotrope rash around the eyes, and malar rash, as well as assessing for calcinosis, which can develop in chronic disease 1. Evaluate muscle strength in proximal muscle groups using formal muscle testing, as proximal weakness is a hallmark of JDM. Perform a detailed cardiopulmonary assessment including auscultation and functional capacity evaluation, as interstitial lung disease and myocarditis can occur. Gastrointestinal evaluation should check for dysphagia, reflux, and abdominal pain that might indicate vasculopathy. Joint examination should assess for arthritis or contractures. Laboratory investigations should include muscle enzymes (CK, aldolase, AST, ALT, LDH), inflammatory markers (ESR, CRP), myositis-specific antibodies, and complete blood count. Regular monitoring of these parameters helps track disease activity and treatment response. Imaging studies like MRI of proximal muscles can detect inflammation even before clinical weakness becomes apparent. Pulmonary function tests should be performed if respiratory symptoms are present. This systematic approach ensures comprehensive evaluation of disease activity across all potentially affected organ systems and guides appropriate treatment adjustments, as recommended by the consensus-based guidelines for the management of JDM 1.

Some key points to consider in the systemic review of a JDM patient include:

• The importance of a multidisciplinary team, including a physiotherapist and specialist nurse, in the management of JDM 1
• The use of a safe and appropriate exercise program, monitored by a physiotherapist, to improve muscle strength and function 1
• The consideration of sun protection, including the routine use of sunblock on sun-exposed areas, to prevent further skin damage 1
• The potential use of immunosuppressive therapy, such as methotrexate or cyclosporin, in addition to corticosteroids, to control disease activity and prevent organ damage 1
• The need for regular monitoring of disease activity and treatment response, using a combination of clinical assessments, laboratory investigations, and imaging studies, to guide treatment adjustments and improve patient outcomes 1.

Overall, a comprehensive systemic review of a JDM patient requires a thorough and multidisciplinary approach, incorporating clinical assessments, laboratory investigations, and imaging studies, to guide appropriate treatment adjustments and improve patient outcomes.

From the Research

Systemic Review of JDM Patient Treatment

• The treatment of juvenile dermatomyositis (JDM) typically involves the use of glucocorticoids, such as prednisone, alone or in combination with other immunosuppressive drugs like methotrexate or cyclosporine 2.
• A study found that the combination of prednisone and methotrexate was more effective than prednisone alone in achieving clinical remission and reducing the time to treatment failure 3.
• The use of intravenous methylprednisolone, methotrexate, and other medications has increased over time, with patients diagnosed after 1997 receiving these treatments more frequently 4.
• A review of the clinical management of JDM highlights the importance of reducing corticosteroid exposure and notes that the combination of high-dose corticosteroids and methotrexate is a standard initial management approach 5.
• A Brazilian registry of JDM cases found that the most common treatment was a combination of prednisone and methotrexate, and that aldolase was a significant independent variable for definite JDM diagnosis 6.

Treatment Outcomes and Predictors

• A study found that the achievement of PRINTO JDM 50-70-90 response after 2 months of treatment, an age at onset greater than 9 years, and the combination therapy of prednisone and methotrexate increased the probability of achieving clinical remission 2.
• The time to clinical remission was shorter in patients treated with prednisone and methotrexate compared to those treated with prednisone alone or prednisone and cyclosporine 3.
• The use of methotrexate was associated with older age at diagnosis and anti-MJ autoantibodies, while antimalarial therapy was associated with anti-p155/140 autoantibodies and mild onset severity 4.

Medication Usage and Side Effects

• High-dose oral prednisone was the primary therapy given to 99% of patients, with the median time to half the initial oral prednisone dose being shorter in patients diagnosed after 1997 4.
• The combination of prednisone and cyclosporine was associated with a higher rate of adverse events, including skin and subcutaneous tissue disorders, gastrointestinal system disorders, and general disorders 3.
• Infections and infestations were significantly increased in patients assigned prednisone plus cyclosporine and prednisone plus methotrexate 3.