Pulmonary hypertension and systemic lupus erythematosus

Abstract

A combination of systemic lupus erythematosus (SLE) and fatal pulmonary hypertension occurred in a patient who, to our knowledge, had the highest pulmonary artery pressure (120/65 mm Hg) reported without any clinical or autopsy findings of pulmonary interstitial disease or vasculitis. The gradual development of pulmonary hypertension over years is a rare complication in patients with SLE.

Pulmonary Hypertension in a Patient with Marian’s Syndrome

“…Pul monary hypertension has been reported in various diseases with a common denomina tor, such as connective tissue disorders and vasculopathies, including systemic lupus erythematosus [3], dermatomyositis [4], CREST syndrome [1], rheumatoid arthritis [5] and mixed connective tissue disease [6],…”

Pulmonary Hypertension in a Patient with Marian’s Syndrome

“…Pul monary hypertension has been reported in various diseases with a common denomina tor, such as connective tissue disorders and vasculopathies, including systemic lupus erythematosus [3], dermatomyositis [4], CREST syndrome [1], rheumatoid arthritis [5] and mixed connective tissue disease [6],…”

Pulmonary Hypertension in Systemic Lupus Erythematosus: A Report of an Autopsied Case.

“…PH is also found in rare occasions in rheumatoid arthritis, dermatomyositis or primary Sjogren's syndrome. The first report of isolated SLEcases with demonstrable PH appeared in 1962 (13), and only recently several articles have been published (1)(2)(3)(4)(5)(6)(7)(8)(9). Recent studies using Doppler echocardiography and right heart catheterization indicate that the incidence of PH is 14%in SLE (14).…”

“…Factors implicated in the pathogenesis ofplexogenic arteriopathy in SLEinclude: [ 1] frequent or permanent vasoconstriction, [2] vasculitis, and [3] …”

“…Pulmonary hypertension (PH) is a rare complication in SLE; only a few reports of such a complication have been published until recently (1)(2)(3)(4)(5)(6)(7)(8)(9). Wepresent here an autopsied case of SLEassociated with PH, Raynaud's phenomenonand a lupus anticoagulant.…”

Primary Pulmonary Hypertension: A Facet of A Diffuse Angiopathic Process?

“…Primary pulmonary hypertension (PPH) is a disease of unexplained etiology that be longs to a group of disorders affecting pri marily the precapillary lung vessels, leading to an increase of pressure and resistance in the pulmonary arterioles and to right heart hypertrophy and failure [12], The existence of PPH in patients with several systemic dis eases of the connective tissue [2,7,9,10], as well as the existence of liver or skin an giopathies in patients and animals with druginduced PPH [II. 13], raises the possibili ty that the latter is not always localized to the pulmonary vasculature, but may be a part of a generalized angiopathic process.…”

Pulmonary Hypertension in a Patient with Marian’s Syndrome

“…Pul monary hypertension has been reported in various diseases with a common denomina tor, such as connective tissue disorders and vasculopathies, including systemic lupus erythematosus [3], dermatomyositis [4], CREST syndrome [1], rheumatoid arthritis [5] and mixed connective tissue disease [6],…”

Pulmonary Hypertension in Systemic Lupus Erythematosus: A Report of an Autopsied Case.

“…PH is also found in rare occasions in rheumatoid arthritis, dermatomyositis or primary Sjogren's syndrome. The first report of isolated SLEcases with demonstrable PH appeared in 1962 (13), and only recently several articles have been published (1)(2)(3)(4)(5)(6)(7)(8)(9). Recent studies using Doppler echocardiography and right heart catheterization indicate that the incidence of PH is 14%in SLE (14).…”

“…Factors implicated in the pathogenesis ofplexogenic arteriopathy in SLEinclude: [ 1] frequent or permanent vasoconstriction, [2] vasculitis, and [3] …”

“…Pulmonary hypertension (PH) is a rare complication in SLE; only a few reports of such a complication have been published until recently (1)(2)(3)(4)(5)(6)(7)(8)(9). Wepresent here an autopsied case of SLEassociated with PH, Raynaud's phenomenonand a lupus anticoagulant.…”

Primary Pulmonary Hypertension: A Facet of A Diffuse Angiopathic Process?

“…Primary pulmonary hypertension (PPH) is a disease of unexplained etiology that be longs to a group of disorders affecting pri marily the precapillary lung vessels, leading to an increase of pressure and resistance in the pulmonary arterioles and to right heart hypertrophy and failure [12], The existence of PPH in patients with several systemic dis eases of the connective tissue [2,7,9,10], as well as the existence of liver or skin an giopathies in patients and animals with druginduced PPH [II. 13], raises the possibili ty that the latter is not always localized to the pulmonary vasculature, but may be a part of a generalized angiopathic process.…”

Pulmonary Hypertension in a Patient with Marian’s Syndrome

“…Pul monary hypertension has been reported in various diseases with a common denomina tor, such as connective tissue disorders and vasculopathies, including systemic lupus erythematosus [3], dermatomyositis [4], CREST syndrome [1], rheumatoid arthritis [5] and mixed connective tissue disease [6],…”

Pulmonary Hypertension in Systemic Lupus Erythematosus: A Report of an Autopsied Case.

“…PH is also found in rare occasions in rheumatoid arthritis, dermatomyositis or primary Sjogren's syndrome. The first report of isolated SLEcases with demonstrable PH appeared in 1962 (13), and only recently several articles have been published (1)(2)(3)(4)(5)(6)(7)(8)(9). Recent studies using Doppler echocardiography and right heart catheterization indicate that the incidence of PH is 14%in SLE (14).…”

“…Factors implicated in the pathogenesis ofplexogenic arteriopathy in SLEinclude: [ 1] frequent or permanent vasoconstriction, [2] vasculitis, and [3] …”

“…Pulmonary hypertension (PH) is a rare complication in SLE; only a few reports of such a complication have been published until recently (1)(2)(3)(4)(5)(6)(7)(8)(9). Wepresent here an autopsied case of SLEassociated with PH, Raynaud's phenomenonand a lupus anticoagulant.…”

Primary Pulmonary Hypertension: A Facet of A Diffuse Angiopathic Process?

“…Primary pulmonary hypertension (PPH) is a disease of unexplained etiology that be longs to a group of disorders affecting pri marily the precapillary lung vessels, leading to an increase of pressure and resistance in the pulmonary arterioles and to right heart hypertrophy and failure [12], The existence of PPH in patients with several systemic dis eases of the connective tissue [2,7,9,10], as well as the existence of liver or skin an giopathies in patients and animals with druginduced PPH [II. 13], raises the possibili ty that the latter is not always localized to the pulmonary vasculature, but may be a part of a generalized angiopathic process.…”