Pulmonary hypertension in polymyositis-dermatomyositis: clinical and hemodynamic characteristics and response to vasoactive therapy

Minai, OA

doi:10.1177/0961203309102822

Cited by 34 publications

(19 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…epoprostenol, subcutaneous treprostinil and nebulised iloprost) may be considered in patients with dermatomyositis-or polymyositis-associated with pulmonary hypertension [120][121][122]. Treatment should be reserved, however, to those rare patients with group 1 PAH with no significant ventilatory impairment resulting from ILD, whereas treatment is not recommended in those with group 3 pulmonary hypertension considered secondary to ILD [119].…”

Section: Treatmentmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

View full text Add to dashboard Cite

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

show abstract

Section: Treatmentmentioning

confidence: 99%

Idiopathic inflammatory myopathies and the lung

et al. 2015

View full text Add to dashboard Cite

show abstract

“…It has long been recognised as a manifestation of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE) [1,2]; however, it can occur in other connective tissue diseases, including SSc-SLE overlap syndrome [3], mixed connective tissue disease (MCTD) [4][5][6], inflammatory myositides (dermatomyositis and polymyositis) [7,8], Sjögren's syndrome [9] and rheumatoid arthritis [10]. In patients suspected of having PAH, Doppler echocardiography has been recommended to detect pulmonary hypertension (PH) and evaluate for left ventricular systolic and diastolic dysfunction, leftsided ventricular enlargement or valvular heart disease [11].…”

mentioning

confidence: 99%

Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

Johnson

Granton²

2011

Eur Respir Rev

View full text Add to dashboard Cite

Pulmonary arterial hypertension (PAH) is a severe manifestation of systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). Due to improvements in the understanding of the pathogenesis of these diseases, improved methodological rigour in the conduct of epidemiological studies and the advent of successful therapies, our understanding of SSc-PAH and SLE-PAH has evolved considerably. In this review we will review the current evidence regarding the prevalence, prognostic factors and survival estimates for SSc-PAH and SLE-PAH. In doing so, we will compare and contrast these two diseases, highlight clinically useful features, discuss methodological limitations of existing data, and draw attention to areas where research is needed.

show abstract

“…In inflammatory myositis [9], and in ASS in particular, the occurrence of PH has never been systematically evaluated and its description rests only upon isolated case reports [10][11][12]. PH comprises many causes, including pulmonary arterial hypertension (PAH), left heart disease, chronic lung diseases, chronic thromboembolism and others [13].…”

Section: Introductionmentioning

confidence: 99%

Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival

et al. 2013

View full text Add to dashboard Cite

Antisynthetase syndrome is characterised by the association of interstitial lung disease and myositis with different anti-tRNA-synthetase antibodies. The occurrence, aetiology and prognosis of pulmonary hypertension have not yet been evaluated.Among 203 consecutive patients, transthoracic echocardiogram and right heart catheterisation results were retrospectively analysed in the light of clinico-biological, morphological and functional parameters. Definitions of pulmonary hypertension were based on the European Society of Cardiology/European Respiratory Society 2009 guidelines, with severe pulmonary hypertension being defined by a mean pulmonary arterial pressure .35 mmHg.Pulmonary hypertension was suspected by transthoracic echocardiogram in 47 (23.2%) cases, corresponding to pulmonary hypertension ''possible'' (n527, 13.3%) or ''likely'' (n520, 9.9%). Right heart catheterisation was performed in 21 patients, excluding pulmonary hypertension in five and confirming pre-capillary pulmonary hypertension in 16 (7.9%). Although related to interstitial lung disease in all cases, pre-capillary pulmonary hypertension was severe in 13 (81.3%) patients (mean¡SD pulmonary arterial pressure 46¡9 mmHg), frequently associated with low cardiac index (mean¡SD 2.3¡0.8 L?min -1 ?m -2 ) and high forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio (2.5¡0.6). Pulmonary hypertension was significantly associated with a lower survival rate (p,0.001), with a 3-year survival rate of 58%.The occurrence of pulmonary hypertension in antisynthetase syndrome is significant and dramatically worsens the prognosis. Although systematically associated with interstitial lung disease, pulmonary hypertension was usually severe, suggesting a specific pulmonary vascular involvement. @ERSpublications PH in antisynthetase syndrome significantly worsens the prognosis, suggesting a specific pulmonary vascular involvement

show abstract

Pulmonary hypertension in polymyositis-dermatomyositis: clinical and hemodynamic characteristics and response to vasoactive therapy

Cited by 34 publications

References 13 publications

Idiopathic inflammatory myopathies and the lung

Idiopathic inflammatory myopathies and the lung

Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

Pulmonary hypertension in antisynthetase syndrome: prevalence, aetiology and survival

Contact Info

Product

Resources

About