全身性紅斑狼瘡與假性腸阻塞：系統性回顧

Intestinal Pseudo-obstruction in Patients with Systemic Lupus Erythematosus: A Systematic Review

目的：全身性紅斑狼瘡（systemic lupus erythematosus, SLE）為一種多系統自體免疫疾病，腸胃道表現雖不常見，但具有重要的臨床意義。假性腸阻塞（intestinal pseudo-obstruction, IPO）屬於罕見且常被低估的併發症，延遲診斷常導致不必要的外科處置，顯示提升警覺與建立明確治療策略的必要性。本研究進行系統性回顧，以探討其臨床特徵、相關共病症、治療方式，以及預後因子。
方法：我們自PubMed檢索1974年1月至2024年5月間之「intestinal pseudo-obstruction」以及「systemic lupus erythematosus」文獻，納入符合ACR-1997或SLICC-2012分類標準且具臨床/影像IPO診斷者，共彙整149位患者。並將其預後分為完全緩解與復發/死亡兩類，進行預後因子分析。
結果：患者以年輕女性為主（年齡中位數31.5歲；女性占92.9%），腹部症狀至診斷的時間之中位數為30天。IPO常與其他共病症共存，包括輸尿管腎盂擴張（uretero-hydronephrosis）與膽道擴張（biliary dilatation）。歸納治療上，高劑量類固醇為誘導期基礎用藥，而早期合併cyclophosphamide與較佳預後顯著相關（30.8% vs 0%，p=0.03）；相反的，維持期使用azathioprine與較差預後相關（11.5% vs 42.9%，p=0.04）。其餘因子均無法預測預後。
結論：SLE-IPO常與其他器官共病症併發，若未積極治療，復發與死亡風險高。因此，及早辨識並積極地給予免疫抑制治療，可進而改善病人預後並避免外科手術。

Objective: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease in which gastrointestinal manifestations are uncommon but can be clinically significant. Intestinal pseudo-obstruction (IPO) is a rare, underrecognized complication. Delayed diagnosis often results in unnecessary surgical intervention, underscoring the need for greater awareness and clearer treatment strategies. We performed a systematic review to characterize the clinical spectrum, concomitant visceral smooth‑muscle involvement, treatments, outcomes, and prognostic factors of SLE‑related IPO.
Methods: We systematically searched PubMed (January 1974–May 2024) for“intestinal pseudo-obstruction”AND“systemic lupus erythematosus,”applying ACR‑1997 or SLICC‑2012 criteria plus clinical/radiologic IPO diagnosis, and pooled 149 patients (56 case reports; 61 PUMC; 32 SMU). Outcome categories were defined as: good = complete clinical response with durable stability; bad = recurrence or death. Mann–Whitney U and Fisher’s exact tests assessed prognostic factors (p <0.05).
Results: Patients were predominantly young women (median age 31.5 years; 92.9% female), with a median symptom duration of 30 days before diagnosis. IPO frequently co-occurred with other smooth muscle dysmotility syndromes, including uretero-hydronephrosis and biliary tract dilatation. High-dose glucocorticoids were the mainstay of induction therapy, but early use of cyclophosphamide was significantly associated with favorable outcomes (30.8% in good vs. 0% in bad outcome group, p=0.03). In contrast, maintenance azathioprine was associated with worse outcomes (11.5% in good vs. 42.9% in bad outcome, p=0.04). No demographic or serologic markers predicted prognosis.
Conclusion: SLE-related IPO often clusters with other smooth muscle involvement and carries a high risk of relapse or death without aggressive treatment. Early recognition and prompt initiation of high-dose corticosteroids with cyclophosphamide may improve long-term outcomes and prevent surgical complications. These findings support a more intensive immunosuppressive approach in managing SLEIPO.