The Clinical Course of Systemic Lupus Erythematosus

Abstract

In our case the participation of lipid deposits in the structural abnormalities of calcific aortic stenosis cannot be questioned. Nests of lipophages or foam cells and deposits of cholesterol crystals are apparent on the sur¬ face of the valve, in its substance, at its base, and in the endocardium proximal to it. These characteristic stigmas of atherosclerosis or xanthomatosis are surrounded by partially calcified fibrous tissue. The lesions in the valve resemble closely the classical atherosclerotic changes in the aorta and its main branches. This demonstration of xanthomatous lesions in the valve does not preclude the possibility that they were accompanied or preceded by inflammatory or proliferative damage from rheumatic fever. In studying this case considerable effort was ex¬ pended in evaluating this possibility. The criteria out¬ lined by Gross and his associates9 were followed in the examination of the heart. Many sections were taken from the valves, pericardium, endocardium, and various stra¬ tegic parts of the cardiac muscle. None of these examina¬ tions revealed any stigma of rheumatic fever. The only lesion that might be interpreted as indicative of rheu¬ matic disease was the network of small blood vessels noted grossly adjacent to one of the largest of the athero¬ matous patches on the mitral valve. Such vascular changes may accompany rheumatic fever but are also seen adja¬ cent to fresh xanthomatous lesions.The absence of any recognizable active lesions of rheu¬ matic fever is more remarkable when one considers the age of the patient. Rothschild, Kugel, and Gross 10 and Console 3I have found that Aschoff bodies can be shown in the hearts of 75 to 95% of patients who die of rheu¬ matic heart disease in the first two decades of life. It might be added that these authors have emphasized also the rarity of the more advanced degrees of deformity in younger persons with aortic stenosis. From our examina¬ tions it is not possible to classify the heart as rheumatic though even the most careful inspection cannot exclude completely the possibility of previous rheumatic damage.The lesions shown in the aortic valve of this young man must be regarded as extremely rare, and it may be that the circumstances of the case have little relevance to the cause of the usual calcific stenotic aortic valve.Considerable significance attaches, however, to the oc¬ currence of even one instance in which atherosclerotic or xanthomatous lesions are obtrusively displayed and no stigma of rheumatic fever can be found. Such a demonstration should lead to some caution in accepting too literally the belief that rheumatic fever is the only pathogenic factor of importance in the development of calcific aortic stenosis. It suggests an obligation to ex¬ amine diseased aortic valves, particularly in older sub¬ jects, for the presence of lipophages, cholesterol crystals, and other characteristic changes of atherosclerosis, and to use in this scrutiny the same meticulous care and dili¬ gence exercised by Karsner and Koletzky in their search ...

Patterns of disease activity in systemic lupus erythematosus

“…Ropes and other early investigators also reported that spontaneous remissions lasting a few months to several years occurred in 35-70% of untreated patients (16)(17)(18). More recent studies differ in reported remission rates, partly because of differences in the duration of followup and the definition of remission used, including the need to not be taking any medications, the absence of serologic activity, and the minimum duration of disease quiescence.…”

Pleural fibrosis mediates shrinking lungs syndrome in children

“…[1][2][3][4] Standard textbooks of pulmonary medicine relate the progressive dyspnea, small lung volume with elevated hemi diaphragms to ''an unspecified restriction in chest wall expansion'' generally ascribed to diaphragmatic weakness. 5 Investigators have hypothesized or measured defects in lung recoil, 1 phrenic nerve function 6 and diaphragmatic strength.…”

Serum heat-labile opsonins in systemic lupus erythematosus.

“…Evidence was obtained that a decrease in serum heat-labile opsonin concentration was in fact responsible for the defect. It was also shown that the defect in phagocytosis was not due to the presence of leukocytotoxic antibodies (6,7) in the deficient SLE sera by the successful restoration of opsonic capacity upon addition of normal serum in 12 of 16 experiments. The less than complete restoration observed with occasional sera of this group may have been due to consumption of critical complement components of the added normal serum during the 1 and 2-h phagocytosis periods by the active SLE sera present, since the latter are known to contain immune complexes and to be anti-complementary (31).…”

“…The increased susceptibility of patients with active systemic lupus erythematosus (SLE)1 to bacterial and mycotic infections has been well documented (1)(2)(3)(4)(5)(6)(7)(8). Al-though the introduction of corticosteroid therapy may currently be a contributing factor, infections constituted a frequent cause of death in the precorticosteroid era (1)(2)(3)(4)(5).…”