Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus

Quismorio, Francisco P.; Sharma, Om P.; Koss, Michael N.; Boylen, Thomas; Edmiston, A.; Thornton, Phyllis J.; Tatter, Dorothy

doi:10.1016/0049-0172(84)90015-5

Cited by 142 publications

(93 citation statements)

References 31 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…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.…”

Section: Introductionmentioning

confidence: 99%

“…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).…”

Section: Case Reportmentioning

confidence: 99%

See 1 more Smart Citation

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

et al. 1994

View full text Add to dashboard Cite

Anautopsied case of systemic lupus erythematosus with pulmonary hypertension is reported. A 29-year-old womanwith a seven-year history of polyarthralgia, butterfly rash, nephrotic syndromeand Raynaud's phenomenonwas admitted because of progressive dyspnea on exertion. Tests for antinuclear antibody, anti-cardiolipin antibody and lupus anticoagulant were positive. Echocardiographic examination revealed right ventricular hypertrophy and a moderate pericardial effusion. Estimated systolic pulmonary arterial pressure was 53 mmHg.Despite treatment with corticosteroids including pulse methylprednisolone therapy, lipo-PGEi and warfarin, she died of progressive congestive heart failure. Postmortem examination of the pulmonary vasculature revealed findings consistent with plexogenic pulmonary arteriopathy, without evidence ofvasculitis, fibrinoid necrosis, or thromboemboli. (Internal Medicine 33: 540-542, 1994)

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Case Reportmentioning

confidence: 99%

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

et al. 1994

View full text Add to dashboard Cite

show abstract

“…In addition, complement and immunoglobulin deposits are found in some patients suggesting that immune deposits may be involved in the pathogenesis. (Quismorio et al, 1984) Several aspects need to be considered when it comes to treating SLE associated PAH. All patients should receive long term anticoagulation especially those with aPL antibodies.…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

Pulmonary Manifestations of Systemic Lupus Erythematosus

Gari¹,

Telmesani²,

Alwithenani³

2012

Systemic Lupus Erythematosus

View full text Add to dashboard Cite

“…Üstelik sistemik lupus eritematozus (SLE) ve Sjögren gibi PAH'a yol açan durumlarda da akciğerlerde antikor-kompleman birikimleri gös-terilmiştir (7,8). PAH'la giden bu gibi klinik tablolarda sistemik otoimmünitenin varlığı akciğer dolaşımının da immün süreçten etkilenmiş olabileceğini düşündürmektedir (9).…”

unclassified

“…B hücreleri üzerinde düzenleyici veya kontrol edici olarak tanımlanan T reg hücrelerin aktivitesinde azalma veya kaybolma olması, mast vb hücrelerinden kendiliğinden reaktif olan B hücrelerine uyarıların gitmesine yol açarak B hüc-relerinin apoptozisden korunmasına ve patolojik antikor salgıla-maları için uyarılmasına yol açar (23 (27,28). PAH'un eşlik ettiği SLE ve Sjögren olgularında pulmoner arter duvarlarında antikor ve kompleman birikimi gösterilmiştir (8,9). Skleroderma ve AFA sendromunda endotele karşı gelişen oto-antikorların, endotelin apostozisini uyararak sistemik hastalık gelişiminin esas tetikleyicisi olabileceği öne sürülmüştür (29,30).…”

unclassified

The etiopathogenesis of pulmonary hypertension: inflammation, vascular remodeling

Kayıkçıoğlu

2010

Anadolu Kardiyol Derg

View full text Add to dashboard Cite

ABS TRACTPulmonary arterial hypertension (PAH) is a progressive disease marked by increased pulmonary artery resistance leading to right heart failure with a high mortality. PAH is histologically characterized by endothelial and smooth muscle cell proliferation, medial hypertrophy, inflammation, and thrombosis in situ. Elevated pulmonary vascular resistance is the result of an imbalance between locally produced vasodilators and vasoconstrictors, in addition to vascular wall remodeling. Recent evidence demonstrates that inflammatory processes are involved in the generation of pulmonary vascular remodeling leading to PAH. Viral infections or similar immune-modulators trigger auto-antibody generation by endothelial injury and indirectly lead to PAH. All these immune-modulators associated with PAH, are also known to decrease the regulatory subgroups (CD4) of T cells. With a recognition of important role of inflammation in the development of PAH, anti-inflammatory agents and anti-cancer drugs are accepted as potential specific targets for PAH treatment. Though clinical studies are not enough, anti-inflammatory agents seem to be promising in the treatment of this devastating disease. ( ÖZETPulmoner arteriyel hipertansiyon (PAH), artmış pulmoner vasküler direnç nedeniyle sağ kalp yetersizliğine ilerleyen, mortalitesi yüksek bir hastalıktır. PAH histolojik olarak endotel ve düz kas hücre proliferasyonu, mediyal hipertrofi, inflamasyon ve trombozla karakterizedir. Pulmoner vaskü-ler direnç artışı, pulmoner vasküler yatakta vazodilatatör ve vazokonstriktör mekanizmaların dengesizliğinden ve vasküler yeniden şekillenme sonucunda gelişmektedir. Güncel klinik kanıtlar inflamatuvar süreçlerin pulmoner vasküler yeniden şekillenme gelişiminde rol alarak, PAH'na yol açtığını göstermektedir. Viral enfeksiyonlar veya benzeri immünomodülatörler endotel hasarını tetikleyerek oto-antikor gelişimi ve dolayısı ile de PAH'na yol açmaktadır. Tüm bu PAH'la ilişkili immünomodülatörlerin T hücrelerinin düzenleyici alt gruplarını azalttığı (CD4) bilinmektedir. PAH gelişiminde inflamasyonun rolünün ortaya konması ile anti-inflamatuvar ajan veya anti-kanser ilaçların PAH tedavisinde yeri olabileceği düşünül-mektedir. Ancak henüz bu konuda yeterli çalışma olmamakla beraber, prognozu hala kötü olan bu hastalıkta anti-inflamatuvar tedavi yeni bir umut gibi gözükmektedir. (

show abstract

Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus

Cited by 142 publications

References 31 publications

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

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

Pulmonary Manifestations of Systemic Lupus Erythematosus

The etiopathogenesis of pulmonary hypertension: inflammation, vascular remodeling

Contact Info

Product

Resources

About