Fatal pulmonary hypertension and resolving immune-complex glomerulonephritis in mixed connective tissue disease

Jones, Michael B.; Osterholm, Richard K.; Wilson, R. B.; Martin, Franklin H.; Commers, James R.; Bachmayer, Jay D.

doi:10.1016/0002-9343(78)90806-9

Cited by 79 publications

(19 citation statements)

References 26 publications

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“…In contrast, there are also some cases, including our case, which are accompanied with the amelioration of renal disease [3,10,16,21]. As shown in table II.…”

Section: Discussionmentioning

confidence: 67%

Renal Involvement in Mixed Connective Tissue Disease

et al. 1985

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5 cases with the compatible serological criteria of mixed connective tissue disease described earlier are presented. In 1 of them with a moderate degree of proteinuria, the renal biopsy disclosed membranous nephritis. However, despite the absence of overt clinical renal disease in the other 4 cases, biopsies disclosed membranous nephritis in 1 and mild mesangial proliferative glomerulonephritis in the remaining 3 cases. In the follow-up of these 4 cases, 2 subsequently developed abnormal urinalysis. Electron microscopic examinations demonstrated electron-dense deposits in glomeruli, and 4 of these patients also had microtubular structures in the endothelial cytoplasm. Contrarily to the original concept, our findings suggest that mixed connective tissue disease also induces immune complex disease.

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“…In contrast, there are also some cases, including our case, which are accompanied with the amelioration of renal disease [3,10,16,21]. As shown in table II.…”

Section: Discussionmentioning

confidence: 67%

Renal Involvement in Mixed Connective Tissue Disease

et al. 1985

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“…The diagnosis of mixed connective tissue disease in this patient was based on typical clinical findings and serology (1)(2)(3)(4)(5)(6)(7)(8)10,(21)(22)(23)). Raynaud's phenomenon, diffuse arthralgias, pericarditis, esophageal motility disorder, and mild restrictive lung disease were found in this case.…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary manifestations of mixed connective tissue disease are also frequent and include restrictive ventilatory defects, decreased diffusion capacity for carbon monoxide (DLco), interstitial infiltrates, and pulmonary hypertension (2,6-9,lO). Less common features recently emphasized are renal, gastrointestinal, central nervous system, and myocardial conduction system involvement (10)(11)(12)(13). Myocarditis with MCTD has previously been described in pediatric patients (9), but not in the adult.…”

Section: Common Clinical Features Of Mctd Includementioning

confidence: 99%

Myocarditis in mixed connective tissue disease

Whitlow¹,

Gilliam²,

Chubick³

et al. 1980

Arthritis & Rheumatism

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A young black woman with clinical and serologic features of mixed connective tissue disease (MCTD) developed myocarditis with congestive heart failure and ventricular ectopic activity. Despite treatment with steroids and cyclophosphamide, progressive myocarditis resulted in death 20 months after cardiomegaly first developed. Necropsy findings in the myocardium included multiple areas of extensive lymphocytic infiltration and patches of fibrosis. Myocarditis has not previously been described in the adult with MCTD but may be an important complication in patients with a high titer of antibody to nuclear ribonucleoprotein.

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“…Hitherto we have reported that some patients of collagen diseases had a high grade intimal thickening in the form of "scleroderma kidney", showing an abrupt elevation of systemic blood pressure and dying from cerebral hemorrhage or uremia (Sawai et al 1981). In many MCTD cases, typical hypertensive pulmonary vascular lesions were observed (Jones et al 1978;Graziano et al 1983;Ueda et al 1984;Hosoda et al 1987). In the present series of cases, no correlation was found between pulmonary and systemic vascular changes, especially of interlobular arteries of kidney, except in Case 14 where death was due to pulmonary hypertension; there was also edematous intimal thickening in the arteries of the kidney.…”

Section: Interstitiummentioning

confidence: 98%