Study: Prognosis and treatment of myositis-associated severe
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The aim of this study was to determine the prognosis, clinical course, and current management of severe interstitial lung disease (ILD) associated with myositis that shows high mortality rates.

Patients with extreme ILD associated with polymyositis (PM) or dermatomyositis (DM) who needed mechanical ventilation and methylprednisolone pulse therapy (more than 1g/day use of methylprednisolone) were found among a total of approximately 66 million inpatient admissions.

-- 155 patients with PM and 394 with DM who fulfilled the above criteria were identified. The median age of patients was 65 years old; DM patients were significantly younger than PM patients (64 vs. 68 years old).

--The numbers of patients who were treated with calcineurin inhibitors, intravenous cyclophosphamide and Polymyxin B immobilized fiber column direct hemoperfusion (PMX-DHP) were 403, 318 and 78 respectively.

--All these treatments were given significantly more frequently to the patients with DM compared with those with PM. The uses of other treatment options were much less frequent.

--The median hospital days of initiating methylprednisolone pulse therapy, calcineurin inhibitors, mechanical ventilation, intravenous cyclophosphamide, PMX-DHP, and in-hospital death among patients with DM were 2, 4, 7, 8, 17, and 36, respectively.

--In-hospital mortality was significantly higher in patients with DM than in those with PM (76.6% vs. 56.8%).

In conclusion, despite aggressive and timely treatments, the mortality rate of patients with myositis-associated serious ILD requiring mechanical ventilation is extremely high.