1996
DOI: 10.1183/09031936.96.09081760
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Pulmonary hypertension associated with POEMS syndrome

Abstract: Pulmonary hypertension (PH) is an uncommon but lethal complication of some connective tissue diseases. We describe two cases of PH associated with plasma cell dyscrasia, with polyneuropathy (P), organomegaly (O), endocrinopathy (E), M protein (P) and skin changes (S) described as POEMS syndrome.Two patients, one female and one male, were hospitalized for dyspnoea. Both had a history of POEMS syndrome a few years previously. PH was diagnosed from cardiac catherization, and complete investigations did not reveal… Show more

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Cited by 25 publications
(20 citation statements)
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“…1 The survival of patients with pulmonary hypertension and POEMS is worse than those with POEMS alone, with median survival time of 54 months in large case series. 4 Although reversibility of pulmonary hypertension has been reported with corticosteroids, [1][2][3][4][5] this is, to our knowledge, the first case reported in which PH has resolved following radical radiotherapy to bone lesions. It is important to consider associated syndromes when a diagnosis of PH is made, because treatment of the underlying condition may lead to improvement in PH, not just in POEMS but in other conditions, such as human immunodeficiency virus infection and systemic lupus erythematosus.…”
Section: Case Descriptionmentioning
confidence: 95%
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“…1 The survival of patients with pulmonary hypertension and POEMS is worse than those with POEMS alone, with median survival time of 54 months in large case series. 4 Although reversibility of pulmonary hypertension has been reported with corticosteroids, [1][2][3][4][5] this is, to our knowledge, the first case reported in which PH has resolved following radical radiotherapy to bone lesions. It is important to consider associated syndromes when a diagnosis of PH is made, because treatment of the underlying condition may lead to improvement in PH, not just in POEMS but in other conditions, such as human immunodeficiency virus infection and systemic lupus erythematosus.…”
Section: Case Descriptionmentioning
confidence: 95%
“…1,2 The suggested prevalence of associated PH is 27%-48%, although these figures were obtained using TTE to estimate pulmonary artery pressure. [3][4][5] In addition, thrombotic events can be associated with POEMS, with reports of an association with chronic thromboembolic pulmonary hypertension.…”
Section: Case Descriptionmentioning
confidence: 99%
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“…Cases of PH in patients with POEMS syndrome have been reported in the literature. [3][4][5][6][7][8][9] However, a comprehensive review of PH in POEMS syndrome is lacking. Therefore, we sought to characterize the prevalence, clinical correlates, response to treatment, and prognostic significance of PH in a retrospective study of a population of POEMS syndrome patients.…”
Section: Introductionmentioning
confidence: 99%
“…Therefore, it is essential to perform complementary tests in order to rule out other causes of pulmonary hypertension. (4,5,(10)(11)(12) In the case reported here, the patient presented all clinical criteria for the diagnosis of POEMS syndrome and was submitted to right heart catheterization in order to confirm the diagnosis of pulmonary hypertension. In addition, the remainder of the investigation ruled out other clinical conditions that might have been associated with the development of pulmonary hypertension.…”
Section: Case Reportmentioning
confidence: 99%