Mini review of diagnostic and therapeutic approaches in myositis-associated interstitial lung disease

Myositis-associated interstitial lung disease (ILD) presents a significant challenge in terms of diagnosis and management due to its heterogeneity and potential for rapid progression. This review outlines the current strategies for diagnosing and managing myositis-associated ILD, focusing on mild to moderate and severe or rapidly progressive cases. Initial diagnosis involves comprehensive clinical evaluation, high-resolution computed tomography (HRCT), pulmonary function tests (PFTs), and serological testing for myositis-specific antibodies and myositis-associated antibodies. Management of mild to moderate ILD includes using corticosteroids and steroid-sparing agents, supported by oxygen therapy, pulmonary rehabilitation, and preventive vaccinations. Regular monitoring through PFTs, HRCT, and clinical assessments is essential to ensure effective management and detect disease progression. In cases of severe or rapidly progressive ILD, aggressive treatment with high-dose corticosteroids, additional immunosuppressive agents such as cyclophosphamide, rituximab, and calcineurin inhibitors, and the use of anti-fibrotic agents like Nintedanib and Pirfenidone are crucial. Supportive care measures, including oxygen therapy and mechanical ventilation, may be necessary for patients with severe respiratory failure. Implementing structured algorithms for diagnosis and management helps streamline the process and improve patient outcomes. Continuous research and advancements in immunology and pharmacology are essential for developing more effective treatments for this challenging condition.

کلیدواژه ها:

myositis-associated interstitial lung disease ، rapidly progressive interstitial lung disease ، myositis-associated antibodies ، myositis-specific antibodies ، Pulmonary Function Tests