Pneumorrhachis and pneumomediastinum in connective tissue disease-related interstitial lung disease: case series from a tertiary care teaching hospital in South India

Sandhya, Pulukool; Keshava, Shyamkumar Nidugala; Danda, Debashish; Padhan, Prasanta; Mathew, John; Gibikote, Sridhar

doi:10.1007/s00296-011-1862-6

Cited by 13 publications

(9 citation statements)

References 19 publications

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“…∼6-8% of patients with polymyositis/dermatomyositis and ILD may develop a pneumomediastinum, leading to death in 35-66% of cases [3, 60,138]. The presentation of pneumomediastinum ranges from acute respiratory failure to fortuitous diagnosis (33% of cases) [3,139]. The predominant manifestations at onset are increased dyspnoea, cervical or facial swelling, subcutaneous emphysema, cervical pain and cough [3,140].…”

Section: Pleural Involvement Pneumomediastinummentioning

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: Pleural Involvement Pneumomediastinummentioning

confidence: 99%

“…Pneumomediastinum is sometimes complicated by asymptomatic pneumorrachis, i.e. epidural emphysema, diagnosed by HRCT [139]. Relapse of pneumodiastinum occurs in a quarter of cases [3].…”

Section: Pleural Involvement Pneumomediastinummentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

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“…In DM, spontaneous pneumomediastinum has been found in patients with history of interstitial pneumopathy [4][5][6][7][8][9][10][11][13][14][15][16][17][18][19][20] and may occur simultaneously with subcutaneous emphysema 4,6-10,12-15,17,19-21,23,25-40 and pneumothorax. [4][5][6]9,10,13,25,26,28,29,34,35,42,46 In our series, all cases had a history of interstitial pneumopathy, 55.5% had subcutaneous emphysema and 11.1% pneumothorax, at the time of the diagnosis of spontaneous pneumomediastinum.…”

Section: ■Discussionmentioning

confidence: 99%

Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review

Pallo

Shinjo

2018

Medical Express

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OBJECTIVES:To describe a case series of spontaneous pneumomediastinum in dermatomyositis and to review the literature. METHODS:This was a retrospective single-center case series, reporting 9 patients with pneumomediastinum and defined dermatomyositis, followed from 2005 to 2017. RESULTS: Median age of patients: 33 years; cutaneous and pulmonary involvement in all cases; constitutional symptoms in 88.8% of patients; involvement of the joints in 11.1%, gastrointestinal tract in 44.4%, and muscles in 77.7%; subcutaneous emphysema was observed in 55.5% and pneumothorax in 11.1%, respectively. Muscle weakness was observed in 77.7% of cases and with a median level of serum creatine phosphokinase of 124U/L. Drawing on results for our literature review, the overall analysis showed that the risk factors associated with spontaneous pneumomediastinum were: (a) a history of interstitial pneumopathy; (b) normal or low levels of muscle enzymes; (c) previous use of systemic glucocorticoid; (d) over 50% of patients had subcutaneous emphysema; (e) high mortality as a consequence of severity of the interstitial lung disease. CONCLUSIONS: Our case series revealed that pneumomediastinum is a rare complication in dermatomyositis that occurs in patients with a history of interstitial pneumopathy and may be accompanied by subcutaneous emphysema and pneumothorax.

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“…The etiologies of PR may be classified into nontraumatic, traumatic, and iatrogenic causes.Nontraumatic causes of PR include spontaneous pneumothorax and pneumomediastinum, infectious events, violent coughing because of asthma, forceful vomiting because of diabetic ketoacidosis and Boerhaave syndrome, abuse of the inhalation drug ecstasy, tumor invasion and postradiation changes, cardiopulmonary resuscitation, physical exercise, and ingestion of a foreign body. 1,3,8,[10][11][12] PR may be secondary to trauma including head trauma, chest trauma, and spinal, abdominal, and pelvic injuries. [13][14][15][16] Iatrogenic causes of PR are rare com-pared with the other causes; they include anesthetic and diagnostic techniques involving lumbar puncture, iatrogenic durotomy during surgical intervention to the spine, abdominal and lung surgery, endotracheal intubation, and craniotomy.…”

Section: Discussion Definitionmentioning

confidence: 99%

Symptomatic Pneumorrhachis

Tatarlı

Ceylan

Köktekir

et al. 2013

J Neurol Surg A Cent Eur Neurosurg

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Pneumorrhachis (PR) is the presence of air within the spinal canal, whether localized in the epidural or in the subarachnoid space. Evidence of intraspinal air, especially in the subarachnoid space, had been thought to be merely a radiological artifact of serious underlying pathology until it was proven that PRs can be related to neurologic symptoms ranging from radicular pain to serious neurologic deficits. The etiologies, pathomechanisms, and natural courses show differences from case to case, with the result that no consistent treatment strategies exist in the literature. Although the conservative treatment modalities seem to be more appropriate in nonsymptomatic cases, treatment strategies in symptomatic cases remain the subject of discussion. In this study, we present two symptomatic cases of PR arising from different causes and review the literature, focusing especially on the symptomatic cases and strategies for treating them.

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Pneumorrhachis and pneumomediastinum in connective tissue disease-related interstitial lung disease: case series from a tertiary care teaching hospital in South India

Cited by 13 publications

References 19 publications

Idiopathic inflammatory myopathies and the lung

Idiopathic inflammatory myopathies and the lung

Spontaneous pneumomediastinum in dermatomyositis: a case series and literature review

Symptomatic Pneumorrhachis

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