Acute respiratory failure due to diaphragmatic weakness revealing a polymyositis

Dauriat, Gaëlle; Stern, Jean Baptiste; Similowski, Thomas; Herson, S.; Belmatoug, Nadia; Marceau, Armelle; Fantin, Bruno; Fournier, Michel; Mal, Hervé

doi:10.1016/s0953-6205(02)00015-8

Cited by 9 publications

(7 citation statements)

References 9 publications

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“…than 50% of normal. 26 Reduced maximal respiratory pressures are hallmarks of myopathic restrictive disease, 109,130,131 although their normality does not exclude respiratory muscle involvement.…”

Section: Clinical and Laboratory Datamentioning

confidence: 99%

“…Respiratory muscle inflammation is associated with reduced lung volumes when muscle strength is less The fibrosis is more conspicuous anteriorly, a feature of ventilator-associated damage than 50% of normal. 26 Reduced maximal respiratory pressures are hallmarks of myopathic restrictive disease, 109,130,131 although their normality does not exclude respiratory muscle involvement.…”

Section: Clinical and Laboratory Datamentioning

confidence: 99%

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Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

2011

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The lung is frequently involved in connective tissue diseases (CTDs), although the frequency of lung manifestations varies according to the type of CTD. Interstitial lung diseases (ILD) are frequently seen in CTDs, particularly systemic sclerosis (SSc), polymyositis/dermatomyositis (PM/DM) and rheumatoid arthritis (RA), accounting for a significant proportion of deaths. A large percentage of patients with CTD-associated ILD has limited and stable disease, not requiring treatment. However, a significant minority has severe and/or progressive disease, necessitating prompt initiation of treatment. CTD-ILD histological patterns include non-specific interstitial pneumonia (NSIP), usual interstitial pneumonia (UIP), organizing pneumonia (OP), diffuse alveolar damage (DAD) and lymphocytic interstitial pneumonia (LIP). NSIP is the most common pattern in all CTDs, except for RA, characterized by a higher frequency of UIP. ILD can present acutely or chronically, with acute presentations being more common in systemic lupus erythematosus and PM/DM. Idiopathic pulmonary fibrosis (IPF) is a progressively worsening ILD characterized by inflammation and fibrosis. The characteristic histological pattern of IPF is UIP. Interestingly, a UIP pattern is associated with a significantly better survival in CTD-related disease compared to the idiopathic variety. Prognosis in IPF is dismal, with a median survival since diagnosis of 2-3 years. No treatment regimen has been shown to improve survival in IPF. By contrast, although there have been only two randomized placebo-controlled trials investigating the effect of immunosuppressive treatment in SSc-associated ILD, clinical experience suggests that immunosuppressive drugs in CTD-related ILDs are capable of benefiting a significant proportion of patients, particularly those with certain histological patterns of disease. This review will essentially focus on CTD-associated ILD and will compare aspects of clinical presentation and management to those of IPF.

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Section: Clinical and Laboratory Datamentioning

confidence: 99%

Section: Clinical and Laboratory Datamentioning

confidence: 99%

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

2011

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“…Different elements of the respiratory system may be involved in patents with PM/DM: respiratory muscles dysfunction (168), interstitial lung disease, lung cancer, aspiration pneumonia in patients with pharyngolaryngeal muscles involvement, PHT (169). Cardiac involvement is common and may induce dyspnea and chest X‐ray abnormalities.…”

Section: Polymyositis and Dermatomyositismentioning

confidence: 99%

The respiratory system in connective tissue disorders

Crestani

2005

Allergy

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The connective tissue disorders (also called collagen vascular diseases) represent an heterogeneous group of immunologically mediated inflammatory disorders with a large variety of affected organs besides the lungs. The respiratory system may be involved in all its components: airways, vessels, parenchyma, pleura, respiratory muscles, etc. The frequency, clinical presentation, prognosis and response to therapy vary, depending on the pattern of involvement as well as on the underlying connective tissue disorders. The subject of this review is to describe the most frequent type of lung disorders observed in patients with connective tissue disease (CTD). We will focus on the most frequent CTD: systemic lupus erythematosus, rheumatoid arthritis, scleroderma, Sjogren's syndrome, dermatopolymyositis and mixed CTD.

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“…Acute respiratory distress or failure due to diaphragmatic dysfunction has been described in polymyositis (Schiavi et al,1984;Sano et al, 1994;Dauriat et al, 2002) congenital myotonic dystrophy (Yong et al, 2003), poliomyelitis and severe Guillain-Barré syndrome. However, diaphragmatic pathology as a cause of clinically unexplained death is uncommon, with only a few cases reported in the English literature to our knowledge.…”

Section: Discussionmentioning

confidence: 99%

Diaphragmatic Pathology: A cause of clinically unexplained death in the perinatal/paediatric age group

Sundararajan

Ostojic²,

Rushton³

et al. 2005

Med Sci Law

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Sudden unexpected death in infancy and childhood requires a 'full' post-mortem investigation. Guidance from the Royal College of Pathologists recommends sampling of all the major organs. However, the diaphragm does not feature in this or in most lists of routine histology. Our aim is to emphasize the importance of sampling the diaphragm for histological examination during autopsy. We describe three autopsy cases of clinically unexplained death in the perinatal and paediatric age group that showed significant pathology of the diaphragm. In Case 1, a previously healthy five-year-old girl collapsed suddenly and died four days later. In Case 2, an eight-month-old infant had repeated episodes of respiratory arrest that culminated in death. Autopsy demonstrated a predominantly diaphragmatic myositis. In Case 3 a female neonate had a respiratory arrest three days after birth and died less than a month later. Autopsy showed multiple large calcified necrotic fibres in the diaphragm. The diaphragm is seldom sampled at autopsy. In the first two cases a predominantly diaphragmatic myositis was either the direct or underlying cause of death. In the third case long-standing diaphragmatic pathology of uncertain cause may have contributed to the original respiratory arrest. Had the diaphragm not been examined histologically, the cause of death would have remained unascertained in these cases. In cases of sudden death in infancy and childhood, failure to reach a diagnosis may lead to undue suspicion falling upon the child's carers. This underscores the need for full histology at post-mortem in child deaths, including diaphragmatic sampling.

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Acute respiratory failure due to diaphragmatic weakness revealing a polymyositis

Cited by 9 publications

References 9 publications

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

Review Series: Aspects of Interstitial lung disease: Connective tissue disease-associated interstitial lung disease: How does it differ from IPF? How should the clinical approach differ?

The respiratory system in connective tissue disorders

Diaphragmatic Pathology: A cause of clinically unexplained death in the perinatal/paediatric age group

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