強皮症と栄養障害

強皮症と栄養障害に関とうするエビデンスはあまりなかったのですが、最近2つの論文が発表されました。強皮症では栄養障害はよく認めるという当然の結果ですが…。

強皮症は膠原病で慢性的な炎症を認めますので悪液質になりやすいだけでなく、嚥下障害や消化管機能障害を認めますので飢餓にもなりやすいので、栄養管理が重要です。強皮症に限らず、すべての膠原病で悪液質を認めることがありますし、機能障害を認める疾患も多いので、リハ栄養では重要な疾患です。

膠原病と栄養に関するエビデンスは関節リウマチが圧倒的に多いのですが、その他の膠原病疾患（SLE、皮膚筋炎など）でも栄養管理が大切であることが広まればと思います。

もっとも関節リウマチでの栄養管理の重要性さえ、日本ではまだあまり広がっていないのが現状です…。関節リウマチではるいそうや悪液質が多いということだけでも、リハ栄養で伝えていかないといけませんね。

①Lijana Krause, et al: Nutritional status as marker for disease activity and severity predicting mortality in patients with systemic sclerosis. Ann Rheum Dis published online May 28, 2010. doi: 10.1136/ard.2009.123273

124人中69人（55.7％）の強皮症患者が低栄養。50％がエネルギー必要量以下の摂取量。19.8％が基礎エネルギー消費量以下の摂取量。栄養指標は生命予後と関連。

ABSTRACT
Objective: To assess and analyse nutritional status in patients with systemic sclerosis (SSc) and identify possible associations with clinical symptoms and its prognostic value.
Methods: Body mass index (BMI) and parameters of bioelectrical impedance analysis (BIA) were assessed in 124 patients with SSc and 295 healthy donors and matched for sex, age and BMI for comparisons. In patients with SSc, BMI and BIA values were compared with clinical symptoms in a cross-sectional study. In a prospective open analysis, survival and changes in the
nutritional status and energy uptake induced by nutritional treatment were evaluated.
Results: Patients with SSc had reduced phase angle (PhA) values, body cell mass (BCM), percentages of cells, increased extracellular mass (ECM) and ECM/BCM values compared with healthy donors. Malnutrition was best
refl ected by the PhA values. Of the patients with SSc, 69 (55.7%) had malnutrition that was associated with severe disease and activity. As assessed by multivariate analysis, low predicted forced vital capacity and high N-terminal(NT)-proBNP values discriminated best between good and bad nutritional status. Among different clinical parameters, low PhA values were the best predictors for SSc-related mortality. BMI values were not
related to disease symptoms or mortality. Fifty per centof patients with SSc had a lower energy uptake related to their energy requirement, 19.8% related to their basal metabolism. Nutritional treatment improved the patients’nutritional status.
Conclusions: In patients with SSc, malnutrition is common and not identifi ed by BMI. BIA parameters reflect disease severity and provide best predictors for patient survival. Therefore, an assessment of nutritional status should be performed in patients with SSc.

②Baron M, et al: Malnutrition is common in systemic sclerosis: results from the Canadian scleroderma research group database. J Rheumatol. 2009 Dec;36(12):2737-43.

malnutrition universal screening tool(MUST)で栄養スクリーニング。
586人中18％の患者で低栄養のハイリスク群。すべての強皮症患者で栄養スクリーニングが行われるべき。（私は栄養アセスメントまでしっかり行うべきだと思っていますが）

Abstract
OBJECTIVE: Systemic sclerosis (SSc) is a multisystem disease associated with significant morbidity and increased mortality. Little is known about nutritional status in SSc. We investigated the prevalence and demographic and clinical correlates of nutritional status in a large cohort of patients with SSc.
METHODS: This was a cross-sectional multicenter study of patients (n = 586) from the Canadian Scleroderma Research Group Registry. Patients were assessed with detailed clinical histories, medical examinations, and self-administered questionnaires. The primary outcome was risk for malnutrition using the "malnutrition universal screening tool" (MUST). Multiple logistic regression was used to assess the relationship between selected demographic and clinical variables and MUST categories.
RESULTS: Of the 586 patients in the study, MUST scores revealed that almost 18% were at high risk for malnutrition. The significant correlates of high malnutrition risk included the number of gastrointestinal (GI) complaints, disease duration, diffuse disease, physician global assessment of disease severity, hemoglobin, oral aperture, abdominal distension on physical examination, and physician-assessed possible malabsorption. Among 14 GI symptoms, only poor appetite and lack of a history of abdominal swelling and bloating predict MUST. These factors accounted for 24% of the variance in MUST scores.
CONCLUSION: The risk for malnutrition in SSc is moderate and is associated with shorter disease duration, markers of GI involvement, and disease severity. Patients with SSc should be screened for malnutrition, and potential underlying causes assessed and treated when possible.