Interstitial lung diseases associated with amyopathic dermatomyositis

Abstract

The aim of the present study was to clarify the clinical characteristics and prognosis of patients with interstitial lung disease (ILD) associated with amyopathic dermatomyositis (ILD-ADM).The study consisted of 14 consecutive patients with ILD-ADM. Patients were classified into two categories, acute/subacute and chronic forms, according to the clinical presentation of ILD. The clinical features, responsiveness to therapy, and prognosis between the two forms were compared.Nine ILD-ADM patients were categorised as the acute/subacute form, and five as the chronic form. Arterial oxygen tension was significantly lower in the acute/subacute ILD than chronic ILD patients. On high-resolution computed tomography, ground-glass opacities were frequently found in the two forms, but consolidation was more common in acute/subacute ILD than chronic ILD. Bronchoalveolar lavage analysis showed higher numbers of total cells and lymphocytes in acute/subacute ILD than chronic ILD. Histologically, the most common finding was nonspecific interstitial pneumonia in the two forms, while diffuse alveolar damage was only found in acute/ subacute ILD. Acute/subacute ILD was generally resistant to therapy, while chronic ILD responded well. Notably, the mortality of acute/subacute ILD was much higher than that of chronic ILD (67 versus 0%, respectively).In conclusion, interstitial lung disease associated with amyopathic dermatomyositis includes two different forms, the acute/subacute and chronic forms, with distinct prognoses.

Interstitial lung disease: are we missing formes frustes of connective tissue disease?

“…The limitations of the study by SUDA et al [36] include its retrospective design and small size. Although most patients had a clinical and radiological pattern that differs from IPF, only a minority underwent a lung biopsy (mostly due to the rapid onset of the lung disease).…”

“…In this regard, in the current issue of the European Respiratory Journal, SUDA et al [36] have addressed novel aspects of the clinical presentation of ILD in dermatomyositis. Hence, in a retrospective cohort of 14 patients with amyopathic dermatomyositis, they identified two distinct profiles according to the clinical onset.…”

Interstitial Lung Disease Associated with Clinically Amyopathic Dermatomyositis

“…However, CD4/8 ratio in bronchoalveolar lavage fluid was significantly increased in cyclosporine-resistant cases compared to alive ones. Suda et al also described that the ratio of CD4/8 lymphocytes was higher in acute/subacute intestitial lung disease than chronic intestitial lung disease in clinically amyopathic dermatomyositis, but the difference was not statistically significant (Suda et al, 2006). Increased CD4/8 ratio in bronchoalveolar lavage fluid suggests that CD8 + T cells may not be major pulmonary inflammatory cells causing lung injury in PM-DM.…”

“…NSIP observed in PM-DM patients means the better survival compared with patients with idiopathic pulmonary fibrosis (Douglas et al, 2001) and mortality is similar to that seen in idiopathic NSIP (Tansey et al, 2004). In intestitial lung disease with clinically amyopathic dermatomyositis, although the most common finding is NSIP (Suda et al, 2006), the intestitial lung disease often takes an aggressive course even when the radiological and histological features are consistent with NSIP (Miyazaki et al, 2005). Tiju et al reported that digital infarcts with microangiopathy may be a useful indicator for early intervention in intestitial lung disease associated with DM (Tjiu et al, 2004).…”

Interstitial Pneumonia Associated with Connective Tissue Disease: An Overview and an Insight

“…Patients with CADM have a greater risk of developing ILD, especially prone to rapidly progressive lung disease corresponding to DAD [111,112]. Anti-melanoma differentiation-associated gene 5 (MDA5) antibodies (also referred to as anti-CADM-140 antibodies) were identified in the serum from patients with CADM by firstly immunoprecipitation assays [113].…”