Fatal pulmonary hypertension in identical twins with systemic lupus erythematosus. 1991

L Wilson, and T Tomita, and M Braniecki
Department of Pathology, University of Kansas Medical Center, Kansas City 66103.

Identical twins were simultaneously diagnosed with systemic lupus erythematosus (SLE) at the age of 12. Later, both developed pulmonary involvement of SLE including pleural effusions, pleuritis, and recurrent bronchopneumonia. In their last year of life, their pulmonary condition deteriorated and they died of severe pulmonary hypertension at the age of 20. At autopsy, small to large pulmonary arterial walls were markedly thickened. There was no SLE renal involvement. Pulmonary hypertension in SLE without pulmonary parenchyma involvement is extremely rare, with only 18 such cases reported. This is the first report of pulmonary hypertension observed in identical twins with a similar clinical course. This case may suggest the possible genetic fate of pulmonary involvement of SLE.

UI MeSH Term Description Entries
D006976 Hypertension, Pulmonary Increased VASCULAR RESISTANCE in the PULMONARY CIRCULATION, usually secondary to HEART DISEASES or LUNG DISEASES. Pulmonary Hypertension
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D004200 Diseases in Twins Disorders affecting TWINS, one or both, at any age. Diseases in Twin,Twin, Diseases in,Twins, Diseases in,in Twin, Diseases,in Twins, Diseases
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults

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