Overview
Overlap myositis is a form of idiopathic inflammatory myopathy (IIM) characterized by the combination of features of polymyositis or dermatomyositis with clinical manifestations of another systemic autoimmune connective tissue disease, such as systemic lupus erythematosus, systemic sclerosis (scleroderma), rheumatoid arthritis, Sjögren syndrome, or mixed connective tissue disease. It is also referred to as myositis overlap syndrome or overlap inflammatory myopathy. This condition primarily affects the musculoskeletal system, causing proximal muscle weakness and elevated muscle enzymes (such as creatine kinase), but also frequently involves other organ systems including the skin, lungs (interstitial lung disease), joints (arthritis or arthralgia), and the gastrointestinal tract (dysphagia, esophageal dysmotility). Raynaud phenomenon, mechanic's hands, and sclerodactyly may also be present depending on the associated connective tissue disease. Overlap myositis is commonly associated with specific myositis-specific or myositis-associated autoantibodies, including anti-PM-Scl, anti-U1-RNP, anti-Ku, and anti-synthetase antibodies (such as anti-Jo-1). The presence of these antibodies often helps define the clinical phenotype and may guide prognosis. Interstitial lung disease is a particularly important complication that can significantly affect morbidity and mortality, and screening with pulmonary function tests and high-resolution CT imaging is recommended. Treatment of overlap myositis typically involves immunosuppressive therapy. First-line treatment generally includes systemic corticosteroids (such as prednisone), often combined with steroid-sparing immunosuppressive agents such as methotrexate, azathioprine, mycophenolate mofetil, or calcineurin inhibitors (tacrolimus, cyclosporine). In refractory cases, intravenous immunoglobulin (IVIG), rituximab, or other biologic agents may be considered. Physical rehabilitation and monitoring for organ-specific complications, particularly pulmonary and cardiac involvement, are essential components of long-term management. Prognosis varies depending on the severity of organ involvement and response to treatment, but overlap myositis may carry a better muscular prognosis compared to some other IIM subtypes, though extramuscular manifestations can be challenging.
Also known as:
Clinical phenotype terms— hover any for plain English:
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
Treatments
No FDA-approved treatments are currently listed for Overlap myositis.
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Specialists
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Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Overlap myositis.
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Common questions about Overlap myositis
What is Overlap myositis?
Overlap myositis is a form of idiopathic inflammatory myopathy (IIM) characterized by the combination of features of polymyositis or dermatomyositis with clinical manifestations of another systemic autoimmune connective tissue disease, such as systemic lupus erythematosus, systemic sclerosis (scleroderma), rheumatoid arthritis, Sjögren syndrome, or mixed connective tissue disease. It is also referred to as myositis overlap syndrome or overlap inflammatory myopathy. This condition primarily affects the musculoskeletal system, causing proximal muscle weakness and elevated muscle enzymes (such as
How is Overlap myositis inherited?
Overlap myositis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Overlap myositis typically begin?
Typical onset of Overlap myositis is adult. Age of onset can vary across affected individuals.