Idiopathic Inflammatory Myopathy with Diffuse Alveolar Damage

Abstract

Interstitial lung disease (ILD) in patients with myositis is defined by the presence of interstitial changes on radiographic examination. The reported prevalence of ILD varies from 0% to nearly 50%. However, only rarely has the pathological pattern of diffuse alveolar damage (DAD) associated with idiopathic inflammatory myopathy (IIM) been reported. We report five patients with IIM (one with dermatomyositis, one with polymyositis, and three with amyopathic dermatomyositis) and respiratory failure. Four underwent open lung biopsy with pathological proof of diffuse alveolar damage (DAD). Despite intensive immunosuppressive therapy, all of them died. In addition to the case reports, we discuss DAD in patients with IIM.

Interstitial lung diseases associated with amyopathic dermatomyositis

“…As ADM is a rare disease, previous studies of ILD-ADM included only one, or a few patients [7][8][9][10][11][12][13]. The present study investigated the highest number of patients with ILD-ADM so far.…”

“…To date, contradictory data have been reported in the prognosis of ILD-ADM. Previous studies, mainly from Asia, have demonstrated that ILD-ADM generally runs an aggressive course, leading to fatal respiratory failure [7][8][9][10][11][12]. In contrast, COTTIN et al [13] recently described a benign form of ILD-ADM.…”

“…There have been few reports of the HRCT findings of ILD-ADM [7,13,20]. According to these reports, ground-glass opacities, consolidation and irregular linear opacities were often seen in ILD-ADM.…”

“…Several studies have demonstrated that rapidly progressive interstitial lung disease (ILD) with a poor prognosis occurs in patients with ADM [7][8][9][10][11][12]. These patients were often resistant to intensive therapy, such as highdose corticosteroids plus immunosuppressive agents, resulting in fatal respiratory failure.…”

Signal recognition particle (SRP) positive myositis in a patient with cryptogenic organizing pneumonia (COP)

“…According to Tazelaar [18], polymyositis or dermatomyositis may be accompanied by either usual interstitial pneumonia (UIP), diVuse alveolar damage (DAD) or bronchiolitis obliterans organizing pneumonia (BOOP, now called COP). DiVuse alveolar damage is reported to have the worst prognosis and bronchiolitis obliterans organizing pneumonia (BOOP/COP) has the most favorable prognosis [10,15,19]. Anti-SRP antibodies, which are mainly polymyositis speciWc, may also be found in dermatomyositis or systemic sclerosis [5,9,13].…”