Pulmonary hypertension in systemic sclerosis.

Trell, Erik; Lindström, C

doi:10.1136/ard.30.4.390

Cited by 53 publications

(11 citation statements)

References 27 publications

(19 reference statements)

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“…PAH is an important complication of connective tissue diseases, a group of disorders characterized by vascular injury, autoimmunity, tissue inflammation, and organ dysfunction [3, 4] including systemic sclerosis [5], systemic lupus erythematosus [6], mixed connective tissue diseases [7, 8], and to a lesser extent, rheumatoid arthritis, polymyositis/dermatomyositis, and Sjogren’s syndrome [9]. …”

Section: Introductionmentioning

confidence: 99%

Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature

et al. 2013

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Pulmonary arterial hypertension (PAH) is a progressive and life-threatening disease. Understanding of PAH prevalence remains limited, but PAH has been reported as a frequent complication in connective tissue diseases. This study estimated prevalence of PAH in patients with connective tissue diseases and prevalence of idiopathic PAH using a systematic review of the literature. We searched PubMed through May 19, 2012 for all studies on prevalence of PAH in patients with connective tissue diseases or prevalence of idiopathic PAH. To be included, studies had to be in English, have humans as subjects, and determine prevalence within a time interval of up to 2 years. Studies only investigating pediatric patients were excluded. Pooled prevalence estimates were calculated. Twenty studies were identified in the review. Seventeen of the 20 studies reported prevalence of PAH in connective tissue diseases and three reported prevalence of idiopathic PAH. The pooled prevalence estimate of idiopathic PAH was 12 cases per million population (95 % CI 5 cases per million to 22 cases per million) with estimates ranging from 5.9 cases per million population to 25 cases per million population. The pooled prevalence estimate of PAH in patients with connective tissue diseases was 13 % (95 % CI, 9.18 % to 18.16 %) with reported estimates ranging from 2.8 % to 32 %. Prevalence of PAH in patients with connective tissue diseases was substantially higher than that of idiopathic PAH based on pooled prevalence estimates. Comparisons of PAH prevalence in persons with connective tissue disease and idiopathic PAH using a large observational study would be helpful in better assessing relative prevalence.

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Section: Introductionmentioning

confidence: 99%

Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature

et al. 2013

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“…Although the CREST syndrome was first described in 1911 and again described in 1964, it was only in 1973 that oesophageal involvement was added to it.9-' The CREST syndrome is sometimes considered a more benign variant of PSS,2-4 but other authors emphasise that visceral involvement, including pulmonary hypertension, can occur.5 6 In a previously published report comparing CREST syndrome with PSS the CREST patients frequently had acrosclerosis; thus they had PSS by the major ARA criterion.7 Our study specifically excluded any patients from the CREST group who had hide-bound, sclerotic skin proximal to the MCPs, while all PSS patients met this major criterion. The PSS group was matched to the CREST patients with respect to age, sex, and, most importantly, duration of disease from first symptom.…”

Section: Discussionmentioning

confidence: 99%

Clinical and serological comparison of 17 chronic progressive systemic sclerosis (PSS) and 17 CREST syndrome patients matched for sex, age, and disease duration.

Fürst¹,

Clements²,

Saab³

et al. 1984

Annals of the Rheumatic Diseases

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SUMMARY This study compares the clinical and serological differences between 17 PSS and 17 carefully matched CREST patients. Patients were matched for sex, age by decade, and, importantly, disease duration (112± 9.2 vs. 12*0±9-3 years). Muscular and skin involvement were greater for the PSS groups (p<002) and pulmonary involvement was also greater (p<0O05), at least for non-smoking PSS patients. On the other hand no clinically significant differences were found between groups for other visceral involvement -including comparisons of gastrointestinal, cardiovascular, and renal involvement. There were also no laboratory differences except in anti-RNP antibody (p<0-04).

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“…Severe pulmonary hypertension is a well-recognized complication of a number of collagen vascular disorders, particularly the CREST syndrome (Salerni et ul., 1977;Smith et al, 1979;Trell and Lindstrom, 1971;Young and Mark, 1978). Although myocardial involvement, hypertension, and pulmonary fibrosis can also occur in scleroderma generally, and CREST in particular, pulmonary hypertension can occur independently (Botstein and LeRoy, 1981;Bulkley et al, 1976;Gaffrey et al, 1982;Salerni et al, 1977;Smith er al., 1979;Trell and Lindstrom, 1971;Young and Mark, 1978).…”

Section: Discussionmentioning

confidence: 99%

“…Although myocardial involvement, hypertension, and pulmonary fibrosis can also occur in scleroderma generally, and CREST in particular, pulmonary hypertension can occur independently (Botstein and LeRoy, 1981;Bulkley et al, 1976;Gaffrey et al, 1982;Salerni et al, 1977;Smith er al., 1979;Trell and Lindstrom, 1971;Young and Mark, 1978). Pulmonary hypertension frequently leads to progressive right heart failure and death (Salemi et a]., 1977).…”

Section: Discussionmentioning

confidence: 99%

Unloading the right ventricle in the crest syndrome variant of progressive systemic sclerosis (scleroderma)

Morrison

Goldman

Alepa

1984

Clinical Cardiology

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Summary: A patient with severe pulmonary hypertension secondary to the CREST syndrome variant of scleroderma is described. Acute reductions in both pulmonary artery pressure and total pulmonary resistance were seen with nifedipine and oxygen administration. Reductions in resistance were maintained for over one month with this combination. These results raise the possibility that some of the pulmonary hypertension seen in this condition is reversible.

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Pulmonary hypertension in systemic sclerosis.

Cited by 53 publications

References 27 publications

Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature

Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature

Clinical and serological comparison of 17 chronic progressive systemic sclerosis (PSS) and 17 CREST syndrome patients matched for sex, age, and disease duration.

Unloading the right ventricle in the crest syndrome variant of progressive systemic sclerosis (scleroderma)

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