Pulmonary function in children with systemic lupus erythematosus.

Abstract

Background -Abnormalities of pulmonary function have been found in children with systemic lupus erythematosus (SLE) even in the absence of clinical or radiographic evidence of pulmonary involvement. It is unknown whether these abnormalities represent an early sign of progressive lung disease or whether they are associated with disease activity. Methods -After a mean of 4 5 years, respiratory function (forced vital capacity (FVC) and single breath gas transfer factor (TLCO)) and disease activity were reexamined in 13 of 15 previously studied children with SLE. Disease activity was assessed by a validated index of SLE activity (SLE activity measure (SLAM)). Results -In spite of the high prevalence of abnormalities of respiratory function at the baseline investigation, no chest radiographic abnormalities or overt clinical signs of lung disease were found at baseline, in the interval between the two investigations, or at the re-evaluation in any patient. From baseline to the second investigation the mean value of SLAM decreased and there was a trend toward an improvement in FVC and TLCO. TLCO was more severely impaired than FVC, being found as an isolated abnormality in a high percentage ofpatients (45% at baseline and 35% at follow up). There was a relationship between baseline TLCO and disease activity, expressed as a SLAM score. Moreover, there was a correlation between the changes in the SLAM score from baseline to the second investigation and the corresponding changes in the TLCO value, but not with the corresponding changes in the FVC value. Conclusions -In this series of patients the decrease in SLE activity from the first to the second investigation was associated with an improvement in pulmonary function. The presence of early isolated functional abnormalities was not associated with subsequent development of lung disease. (Thorax 1996;51:424-428)

Echocardiography and pulmonary function testing in childhood onset systemic lupus erythematosus

“…The frequency of abnormalities that we found in our 26 patients with clinically inapparent lung disease was 35%. The very high frequency found in the study of Cerveri et al, 14 was in part related to the narrower reference range for normality. In contrast to other studies, Cerveri et al, 14 demonstrated in their small group of patients a relationship between disease activity and PFT abnormalities.…”

“…The very high frequency found in the study of Cerveri et al, 14 was in part related to the narrower reference range for normality. In contrast to other studies, Cerveri et al, 14 demonstrated in their small group of patients a relationship between disease activity and PFT abnormalities. Similar to studies in adult patients, 9,36 ± 38 the most common PFT abnormalities reported in children include a restrictive pattern of lung mechanics andaor an impaired diffusion capacity, this was re¯ected in our patient population, where 40% of patients had impaired FVC, and 38% had impaired DLCO.…”

Respiratory complications of the rheumatological diseases in childhood

“…Research on the role of PFTs and DLCO in JSLE in the paediatric population is limited, but one study that looked at a cohort of patients with JSLE who were diagnosed before the age of 16 years found that 20% of patients (who were <20 years of age at the time of testing) had a significantly reduced DLCO 22. It has also been suggested that decreased DLCO may be secondary to underlying inflammatory activity due to disease, which may give artificially significant readings 23. However, respiratory monitoring using PFTs still has a role in the paediatric setting, to monitor for early signs of respiratory decline.…”

Psychiatric Illness of Systemic Lupus Erythematosus in Childhood: Spectrum of Clinically Important Manifestations

“…Наи-более часто поражаются оболочки сердца и миокард: пери-кардит, миокардит, значительно реже в последние годы -эндокардит (вальвулит митрального, крайне редко -аор-тального или трехстворчатого клапанов) или его исходы в виде уплотнения створок либо специфичный для СКВ бородавчатый эндокардит Либмана-Сакса. Данные ЭхоКГ нередко демонстрируют наличие перикардиально-го выпота даже у тех детей, которые не имеют клинических признаков поражения сердца [52], поэтому рекомендуется использовать базовую ЭхоКГ и ЭКГ у каждого пациента с СКВ.…”

“…Плохая переносимость физической нагрузки может свидетельствовать о наличии легочного и/или сердечного поражения в рамках СКВ, которое нередко на ранних эта-пах заболевания протекает бессимптомно [51,52,[54][55][56][57]. Периодический мониторинг для оценки вовлечения и/или прогрессирования поражения этих органов входит в оцен-ку активности СКВ с помощью валидированных шкал ак-тивности (SLEDAI, ECLAM, BILAG) и учитывается при определении индекса повреждения (SDI).…”