Rheum2Learn: Inflammatory Myopathies
Case Correlation to ACGME Core Competency Requirements
Patient Care
- Identify the characteristic clinical manifestations of the muscle disease of both dermatomyositis and polymyositis to be found on a detailed history and physical examination and utilize appropriate evaluation strategies, including laboratory, radiographic, neuromuscular studies, and pathology for diagnosis and management of both the muscle disease and visceral involvement
- Create a differential diagnosis of an elevated creatine kinase, and be able to employ the appropriate studies to differentiate the inflammatory myopathies from other causes
- Recognize the appearance of the rashes and skin manifestations characteristic of dermatomyositis (including heliotrope rash, shawl and V-sign, Gottron's papules, and mechanic's hands)
- Identify and recognize the examination findings of nailfold capillary abnormalities
- Identify and recognize calcinosis and its association with inflammatory muscle disease
- Differentiate inclusion body myositis from the other inflammatory myopathies and understand how the clinical presentation and treatment differ
- Recognize when therapy is needed and be able to initiate appropriate treatment
- Recognize and institute referral for subspecialty care when further diagnostic or therapeutic intervention is indicated
- Explain the risks and benefits of medications commonly used for treatment including corticosteroids and the potential role for other immunosuppressive therapy
- Identify interstitial lung disease as a common manifestation of myositis, including CT findings and mechanic's hands
- Recognize the association of an underlying malignancy and be able to institute appropriate malignancy screening
- Recognize the association of myositis overlap syndromes, including other systemic connective tissue disorders
- Identify the clinical features regarding muscle involvement in the inflammatory myopathies
- Describe and recognize the common rashes associated with dermatomyositis: heliotrope, Gottron's papules, V-sign/shawl sign and mechanic's hands
- Describe and recognize the systemic features that may be present in patients with an inflammatory myopathy with an emphasis on lung involvement
- Identify the most common autoantibodies associated with myositis and their clinical significance, particularly that of the anti-Jo-1 (anti-histidyl tRNA synthetase) antibody and the anti-synthetase syndrome
- Recognize the association of myositis with malignancy
- Describe the differences between polymyositis and dermatomyositis
- Describe the histological features which distinguish inclusion body myositis from polymyositis, dermatomyositis and steroid myopathy
- Recognize the association of myositis and the human immunodeficiency virus
- Generate a differential diagnosis for the presentation of muscle weakness and an elevated creatine kinase
- Recognize the diagnostic modalities available (including EMG,MRI and muscle biopsy) which can be utilized as part of the evaluation of an elevated creatine kinase and/or muscle weakness
- Describe the relationship and potential overlap of myositis with other systemic connective tissue diseases
- Distinguish steroid-induced myopathy from an inflammatory myopathy
- Recognize the different drug-related myopathies (including but not limited to the myopathies that can be seen with HIV therapies, statins, and colchicine) and compare their clinical features with those of the autoimmune, inflammatory myopathies
- Recognize the usefulness of corticosteroids in the treatment of the inflammatory myopathies
- Relate at least three immunosuppressive therapies(other than corticosteroids) that are used in the treatment of the inflammatory myopathies, either as steroid-sparing agents or for recalcitrant disease, and identify their potential toxicities
- Explain to the patient and family the need for close monitoring of the patient and details of follow up
- Discuss the potential complications and course of the disease, in a way that incorporates patients' perspectives
- Explain the importance of age appropriate cancer screening in inflammatory myopathies
- Discuss activity restrictions and the role of physical therapy in myositis treatment
- Explain the benefit and potential toxicity of glucocorticoid therapy
- Discuss the options for treatment with immunosuppressive agents and corticosteroids along with the potential benefit, toxicity and monitoring requirements
- Explain the rationale for a malignancy evaluation
- Explain the rationale for pulmonary monitoring
- Summarize the information to be provided to a specialist to whom one might refer a patient with suspected inflammatory muscle disease
- Display integrity and honesty in discussing patient care issues and management.
- Insure patient privacy.
- Promote patient autonomy in clinical and therapeutic decisions.
- Acknowledge and include family and social support as designated by the patient.
- Provide adequate time and accessibility to address patient concerns.
- Communicate in a timely fashion with other members of the patient's health care team including the primary care physician.
- Serve as the patient's advocate.
- Utilize web-based resources to supplement and update current knowledge base and to explore patient-specific problems
- Incorporate evaluation and feedback into practice and management
- Utilize errors and complications to improve understanding and future management
- Set learning goals in diagnostic strategies and management
- Identify barriers to accessing optimal medical care for each individual patient and utilize alternative resources when available to overcome these barriers.
- Engage and incorporate the input of all medical providers including other physicians, the nursing staff, and physical and occupational therapists as necessary.
- Serve as a source of learning and education for other members of the health care team for the patient.
- Utilize the existing health care system to support and establish patient care goals.
- Incorporate considerations of cost and risk-to-benefit ratios in clinical evaluations, monitoring and therapeutic decisions, for the individual patient.
- Recognize the impact of both the diagnostic and therapeutic interventions on the health care system, both locally and globally.