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8 lut 2023 · Although interstitial lung disease (ILD) is a leading cause of morbidity and mortality in patients with inflammatory myopathies, the current definition and diagnostic criteria of autoimmune myositis remain inadequate to capture the large proportion of patients with lung-dominant disease.
The average myositis patient often visits five doctors over three-and-a-half years before receiving an accurate diagnosis. Of those who die early in the disease course, the most frequent cause is not the myositis itself, but infection due to suppressed immunity as a result of treatment.
The acute form of ILD, also called rapidly progressive ILD, showing an acute worsening of dyspnea with a widespread alveolar abnormality on the chest radiological findings, has been recognized as a serious life-threatening condition with the poorest prognosis in PM-/DM-/CADM-ILD [11,15,16].
6 cze 2014 · The clinical characteristics of the patients with myositis-associated ILD (39 men and 75 women; median age, 56 years) are summarized in Table 1. The numbers of patients diagnosed with PM, DM, and CADM were 30, 41, and 43, respectively.
Use of AZA in the treatment of ILD was mentioned in published series 18,19 describing the characteristics and prognosis of patients with myositis-related ILD. Friedman et al 44 reported 10 patients with antisynthetase syndrome without apparent myositis.
The classification and treatment of patients with myositis-associated ILD remains challenging. A standardized therapeutic approach to these patients is lacking, and prospective studies in the field are needed to determine optimal treatment regimens.
1 cze 2022 · Several poor prognostic factors for myositis-ILD have been reported, including male gender, older age at disease onset, CADM, cutaneous involvement (rash), fever, skin ulceration, periungual erythema, elevated creatine kinase, raised ferritin >500 ng/mL, chest high-resolution computed tomography (HRCT) patterns (absence of ground-glass ...
