Idiopathic Inflammatory Myositis

Özet

Idiopathic inflammatory myositis (IIM) is a systemic autoimmune disease with heterogeneous subgroups, mainly characterized by muscle pain and muscle weakness caused by muscle inflammation. IIM diagnoses are based on clinical, laboratory, radiological, electrophysiological, and histopathological findings. IIM can be divided into subgroups in tight of new myositis-specific autoantibodies (MSA), histopathological developments, and classification criteria. These are dermatomyositis (DM), immune-mediated necrotizing myopathy (IMNM), overlap myositis (OM), inclusion body myositis (ICM), amyopathic dermatomyositis (ADM), polymyositis (PM), and cancer-associated myositis. Different subgroups have different clinical, histopathological findings, autoantibody profiles, prognosis, and treatment responses. 30-40% of patients achieve clinical remission with treatment, but 10% have recurrent disease. Mortality is usually due to cardiac (22%), pulmonary complications (22%), infections (15%),and malignancy (11%). IIM is related to an increased risk of heart disease, but the prevalence remains unclear. Approximately 70% of patients may have only subclinical symptoms. The three main causes of cardiac mortality in IIM patients are congestive heart failure, myocardial infarction, and arrhythmias. Evaluation of cardiac function at the time of diagnosis and during follow-up in every patient with myositis is important for early diagnosis and treatment, even in patients with remission.

İdiyopatik inflamatuar miyozit (İİM), esas olarak kas iltihabının neden olduğu kas ağrısı ve kas güçsüzlüğü ile karakterize, heterojen alt gruplara sahip sistemik bir otoimmün hastalıktır. İİM tanıları klinik, laboratuvar, radyolojik, elektrofizyolojik ve histopatolojik bulgulara dayanmaktadır. İİM, yeni miyozite özgü otoantikorlar (MSA), histopatolojik gelişmeler ve sınıflandırma kriterlerine göre alt gruplara ayrılabilir. Bunlar dermatomiyozit (DM), immün aracılı nekrotizan miyopati (İMNM), overlap miyozit (OM), inklüzyon cisimcikli miyozit (İKM), amiyopatik dermatomiyozit (ADM), polimiyozit (PM) ve kanserle ilişkili miyozittir. Farklı subgrupların farklı klinik, histopatolojik bulguları, otoantikor profilleri, prognoz ve tedavi yanıtları vardır. Hastaların %30-40'ı tedavi ile klinik remisyona ulaşır, ancak %10'unda relaps görülür. Mortalite genellikle kardiyak (%22), pulmoner komplikasyonlar (%22), enfeksiyonlar (%15) ve malignite (%11) nedeniyledir. İİM kalp hastalığı riskinin artmasıyla ilişkilidir, ancak yaygınlığı belirsizliğini korumaktadır. Hastaların yaklaşık %70'inde yalnızca subklinik semptomlar olabilir. İİM hastalarında kardiyak mortalitenin üç ana nedeni konjestif kalp yetmezliği, miyokard enfarktüsü ve aritmilerdir. Miyozitli her hastada tanı anında ve takip sırasında kardiyak fonksiyonun değerlendirilmesi erken tanı ve tedavi için önemlidir.

Referanslar

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Sayfalar

243-252

Gelecek

23 Haziran 2025

Lisans

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