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lupus-associated pulmonary hypertension
Monday 24 January 2011
Pulmonary hypertension (PH) in systemic lupus erythematosus (SLE) is associated with an unfavorable prognosis.
The presence of PH defines a subgroup of patients with a severe disease and increased mortality. Antiphospholipid antibodies and Raynaud’s phenomenon may contribute to the pathogenesis of PH.
Pathology
The small to large pulmonary arterial walls are markedly thickened.
Pulmonary hypertension in SLE without pulmonary parenchyma involvement is extremely rare.
Variants
pulmonary hypertension observed in identical twins with a similar clinical course (2004751)
See also
pulmonary involvemen in systemic lupus erythematosus (SLE)
- pleural effusions
- pleuritis
- recurrent bronchopneumonia
- severe pulmonary hypertension
References
Pulmonary hypertension in systemic lupus erythematosus: relationship with antiphospholipid antibodies and severe disease outcome. Cefle A, Inanc M, Sayarlioglu M, Kamali S, Gul A, Ocal L, Aral O, Konice M. Rheumatol Int. 2009 Dec 11. PMID: 20012052
Successful pregnancy in pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report. Streit M, Speich R, Fischler M, Ulrich S. J Med Case Reports. 2009 Jun 9;3:7255. PMID: 19830150
Fatal pulmonary hypertension in identical twins with systemic lupus erythematosus. Wilson L, Tomita T, Braniecki M. Hum Pathol. 1991 Mar;22(3):295-7. PMID: 2004751
