Vascular disease in mixed connective tissue disease (MCTD).

نویسنده

  • H Kondo
چکیده

Sharp et al first described mixed connective tissue disease (MCTD)in 1972 as a distinct entity of connective tissue disease (1). MCTDis a disease entity characterized by overlap symptoms of systemic lupus erythematosus (SLE), systemic sclerosis (SSc) and polymyositis/dermatomyositis (PM/DM), as well as by the presence of antibody to U1RNRHowever, it has been suggested that rather than being regarded as a distinct disease entity, MCTDshould be designated as undifferentiated connective tissue syndrome (2). On the other hand, several recent reports showed that MCTDwas a distinct disease entity (3-5). Regarding a genetic markers, MCTD-associated HLAis distinct from the SLE-and SSc-associated HLAin Japanese and Caucasian patients (3, 6, 7). Furthermore, as a clinical feature of this disease, pulmonary hypertension (PH) is a characteristic organ involvement and is the most frequent cause of death, especially in Japan (8-10). In MCTDpatients with PH, the muscular type pulmonary arteries and arterioles exhibit fibrous intimal thickening and medial muscular hypertrophy. Someshow marked luminal stenosis or fibrin thrombi (9, 10). A considerable number of flexiform lesions are present in some cases (10). These findings are similar to those of PHin SSc, which consist of concentric intimal proliferation, medial hypertrophy and a variable degree of myxomatous degeneration ( 1 1). However, renal crisis with malignant hypertension and a high level of plasma rein activity is clinically very rare in MCTDas a SSc symptom. Renal crisis is the most severe organ involvement in SSc. The primary process is injury of the endothelial cells, which results in intimal thickening and intimal proliferation of renal intralobular and arcuate arteries. Inflammatory cells are conspicuously absent in the pathologic examination of these arteries. Renal crisis is clinically rare in MCTD,but Sawai et al (12) described renal histopathological findings of 25 autopsy cases of MCTD, in which obvious renal intimal thickening of interlobular artery was observed. In this issue of the Journal, Yamaguchi et al report an interesting case of MCTDwith renal crisis and PH (13).

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عنوان ژورنال:
  • Internal medicine

دوره 40 12  شماره 

صفحات  -

تاریخ انتشار 2001