Clinical Differences Between Interstitial Lung Disease Associated With Clinically Amyopathic Dermatomyositis and Classic Dermatomyositis

Mukae, Hiroshi; Ishimoto, Hiroshi; Sakamoto, Noriho; Hara, Shintaro; Kakugawa, Tomoyuki; Nakayama, Seiko; Ishimatsu, Yuji; Kawakami, Atsushi; Eguchi, Katsumi; Kohno, Shigeru

doi:10.1378/chest.08-2740

Cited by 180 publications

(125 citation statements)

References 27 publications

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“…The presence of anti-ARS autoantibodies seems relatively protective against rapidly progressive ILD, although this presentation has been reported [62,63]. The detection of anti-MDA5 autoantibodies identifies dermatomyositis patients with a high risk of developing rapidly progressive ILD, with a pooled sensitivity of 77% and a specificity of 86% in a meta-analysis of 631 dermatomyositis or polymyositis patients [64,65].…”

Section: Rapidly Progressive Ildmentioning

confidence: 99%

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Idiopathic inflammatory myopathies and the lung

et al. 2015

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Idiopathic inflammatory myositis (IIM) is a group of rare connective tissue diseases (CTDs) characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD) is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD. @ERSpublications Interstitial lung disease is a leading cause of morbidity in dermatomyositis/polymyositis

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Section: Rapidly Progressive Ildmentioning

confidence: 99%

“…+ ratio was greater in CADM than in those with classic dermatomyositis and rapidly progressive ILD [62,70,87]. Increased neutrophil counts in BAL were associated with a worse outcome in dermatomyositis-or polymyositis-ILD in two studies [47,63].…”

Section: Bal Cellular Profilementioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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“…Otherwise, DAD is uncommon but seems to portend an especially poor prognosis in patients with amyopathic PM (Figure 14, A and B). [87][88][89] Pulmonary capillaritis and pulmonary hypertension have rarely been reported in PM/DM. 90,91 Pleuritis, bronchiolitis, and vascular changes are distinctly uncommon in PM/DM and should prompt a search for other possible etiologies.…”

Section: Pathologic Findingsmentioning

confidence: 99%

Pleuropulmonary Pathology in Patients With Rheumatic Disease

Schneider

Gruden

Tazelaar

et al. 2012

Archives of Pathology &Amp; Laboratory Medicine

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Thoracic manifestations of rheumatic disease (RD) are increasingly recognized as a significant cause of morbidity and mortality worldwide. Rheumatologic underpinnings have been identified in a significant proportion of patients with interstitial lung disease. The 5 RDs most frequently associated with pleuropulmonary disease are (1) rheumatoid arthritis, (2) systemic lupus erythematosus, (3) progressive systemic sclerosis, (4) polymyositis/dermatomyositis, and (5) Sjögren syndrome. The onset of thoracic involvement in these diseases is variable. In some patients, it precedes the systemic disease or is its only manifestation. Moreover, there is a wide spectrum of clinical presentation ranging from subclinical abnormalities to acute respiratory failure. Histopathologically, the hallmark features of thoracic involvement by RD are inflammatory, targeting one or more lung compartments. The reactions range from acute to chronic, with remodeling by fibrosis being a common result. Although the inflammatory findings are often nonspecific, certain reactions or anatomic distributions may favor one RD over another, and occasionally, a distinctive histopathology may be present (eg, rheumatoid nodules). Three diagnostic dilemmas are encountered in patients with RD who develop diffuse lung disease: 1) opportunistic infection in the immunocompromised host, 2) drug toxicity related to the medications used to treat the systemic disease, and 3) manifestations of the patient's known systemic disease in lung and pleura. To confidently address the latter, the 5 major RDs are presented here, with their most common pleuropulmonary pathologic manifestations, accompanied by brief clinical and radiologic correlations.

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“…It has been reported predominantly in Asia, including Japan, Hong Kong, and Taiwan (Lee et al, 2002, Mukae et al, 2009 and is often resistant to intensive therapy including high dose corticosteroids and immunosuppressive agents, resulting in fatal respiratory failure.…”

Section: Interstitial Lung Disease Associated With Clinically Amyopatmentioning

confidence: 99%

“…Among patients with DM or PM, interstitial lung disease is a major cause of morbidity and mortality (Fathi et al, 2008, Love et al, 1991, Marie et al, 2002. In particular, patients with clinically amyopathic dermatomyositis sometimes develop rapidly progressive interstitial lung disease that remains unresponsive to intensive immunosuppresive therapy (Mukae et al, 2009). …”

Section: Introductionmentioning

confidence: 99%