Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Antibodies to fibrin-bound tissue plasminogen activator (tPA) have been found in autoimmune diseases with vascular injury, such as systemic lupus erythematosus and scleroderma. The purpose of this study was to determine whether patients with primary pulmonary hypertension (PPH) have an immunogenetically determined response to fibrin-bound tPA. Antibodies to fibrin-bound tPA were determined in three patient groups: 45 adults with PPH, 41 children with PPH, and 40 children with anatomically large congenital pulmonary to systemic communications (PHT+shunt). The frequencies of the HLA class II (DRB1,3,4,5, and -DQB1) alleles in these three patient groups were compared with those of 51 healthy Caucasian control subjects. Fibrin-bound tPA antibodies were found in four of 45 (9%) adults with PPH, four of 41 (10%) children with PPH, and one of 40 (2.5%) children with PHT-shunt. HLA class II typing, which was available for seven of nine Caucasians with fibrin-bound tPA antibodies, revealed that six of seven (86%) patients typed HLA-DQ7 (DQB1*0301) and one typed HLA-DQ6. The 86% frequency of HLA-DQ7 in the antibody positive patients was significant compared with the 29% frequency in the healthy control subjects (p = 0.007, p corrected [pc] = 0.05, OR = 14.4). Of interest, these antibody-positive patients, although lacking antiphospholipid antibodies, shared an amino acid epitope, common to HLA-DQB1*06,07 and 08 subtypes, which was previously reported to be associated with the lupus anticoagulant. In conclusion, antibodies to fibrin-bound tPA and HLA-DQ7, and possibly the same epitope associated with the lupus anticoagulant, defined a small subset of children and adults with PPH.
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PMID:Primary pulmonary hypertension, tissue plasminogen activator antibodies, and HLA-DQ7. 900 24

Pulmonary hypertension (PH) is a common complication of connective tissue disease. While there are no reliable epidemiological data, the prevalence of systemic sclerosis (SSc) has been estimated as being 8-12%. Mixed connective tissue disease (MCTD) and systemic lupus erythematodes (SLE) are thought to have a lower prevalence. PH associated with SSc has a poor prognosis, a two-year survival rate of approximately 50%, if untreated. Systematic literature search for studies of PH and CTD between 02/2007 and 02/2008 found 38 articles, a selection of which is reviewed here. One epidemiological study showed that nowadays PH is together with interstitial pulmonary fibrosis the most common cause of death in patients with SSc. Before the introduction of angiotensin- converting enzyme inhibitors the most frequent cause of death was acute renal crisis. Investigations of the pathogenesis of PH in CTD revealed that in patients with a severe, treatment-resistant course there is frequent histological evidence of pulmonary veno-occlusive disease. A prospective study on diagnosis revealed that transthoracic echocardiography (TTE) is better than magnetic resonance imaging or pulmonary function tests especially in screening for PH in patients with SSc, because of its high specificity and good predictive value at higher pulmonary pressures. But because of the low sensitivity of TTE right heart catheterization is the gold standard for verifying PH also in patients with SSc. A therapeutic uncontrolled trial indicated that treatment with bosentan combined with sildenafil results in clinical stabilization, but patients with idiopathic PHT responded better.
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PMID:[Update in pulmonary hypertension associated with connective tissue diseases - a systematic literature review]. 1881 96