Pulmonary Hypertension in Polymyositis

Bunch, Thomas W.; Tancredi, Robert G.; Lie, J.T.

doi:10.1378/chest.79.1.105

Cited by 75 publications

(26 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The proportion of patients with IPAH with a positive vasodilator response (6%) was of a similar order to that in previous registries [29,30]. As observed elsewhere, survival in PAH-SSc was inferior to that of IPAH [18,34,35] and the number of cases of PAH-CTD associated with CTDs other than SSc was small [18,[36][37][38][39][40][41]. In contrast to the French registry, limited cutaneous SSc was far more common than the diffuse form (94% versus 67% of PAH-CTD-SSc) [29].…”

Section: Discussionsupporting

confidence: 64%

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

Hurdman¹,

Condliffe²,

Elliot³

et al. 2011

Eur Respir J

379

362

View full text Add to dashboard Cite

Pulmonary hypertension (PH) is a heterogeneous condition. To date, no registry data exists reflecting the spectrum of disease across the five diagnostic groups encountered in a specialist referral centre.Data was retrieved for consecutive, treatment-naïve cases diagnosed between 2001 and 2010 using a catheter-based approach. 1,344 patients were enrolled, with a mean follow-up of 2.9 yrs.The 3-yr survival was 68% for pulmonary arterial hypertension (PAH), 73% for PH associated with left heart disease, 44% for PH associated with lung disease (PH-lung), 71% for chronic thromboembolic PH (CTEPH) and 59% for miscellaneous PH. Compared with PAH, survival was inferior in PH-lung and superior in CTEPH (p,0.05). Multivariate analysis demonstrated that diagnostic group independently predicted survival. Within PAH, Eisenmenger's survival was superior to idiopathic PAH, which was superior to PAH associated with systemic sclerosis (p,0.005). Within PH-lung, 3-yr survival in sleep disorders/alveolar hypoventilation (90%) was superior to PH-lung with chronic obstructive pulmonary disease (41%) and interstitial lung disease (16%) (p,0.05). In CTEPH, long-term survival was best in patients with surgically accessible disease undergoing pulmonary endarterectomy.In this large registry of consecutive, treatment-naïve patients identified at a specialist PH centre, outcomes and characteristics differed between and within PH groups. The current system of classification of PH has prognostic value even when adjusted for age and disease severity, emphasising the importance of systematic evaluation and precise classification.

show abstract

Section: Discussionsupporting

confidence: 64%

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

Hurdman¹,

Condliffe²,

Elliot³

et al. 2011

Eur Respir J

379

362

View full text Add to dashboard Cite

show abstract

“…Pulmonary arterial hypertension Epidemiology Precapillary pulmonary hypertension associated with dermatomyositis was first described in 1956 [112], and was reported more recently in case reports [113,114] and short series [5]. The estimated frequency of pulmonary hypertension is 8% in patients with anti-ARS autoantibodies [5], and may be as high as 29% in patients with anti-PL7 autoantibodies [115].…”

Section: Discussionmentioning

confidence: 99%

“…Cutaneous features of SSc including Raynaud's phenomenon, digital ulcers, sclerodactyly and puffy fingers are frequently reported in patients with anti-ARS autoantibodies, and are more frequent in overt pulmonary hypertension [8,118]. Two case reports in overt and hypomyopathic polymyositis describe severe right ventricular hypertrophy and plexogenic severe intimal fibrosis consistent with pulmonary arteriopathy at autopsy [113,117]. Obliterative pulmonary vasculopathy (resulting from proliferation of endothelial and vascular smooth muscle cells) similar to that of SSc, but involving more proximal pulmonary arterioles, often coexists [117].…”

Section: Pathogenesis and Pathologymentioning

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

“…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

Pulmonary Hypertension in Polymyositis

Cited by 75 publications

References 4 publications

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

ASPIRE registry: Assessing the Spectrum of Pulmonary hypertension Identified at a REferral centre

Idiopathic inflammatory myopathies and the lung

Pulmonary hypertension in systemic sclerosis and systemic lupus erythematosus

Contact Info

Product

Resources

About