背景:肾脏是原发性干燥综合征(pSS)的常见颗粒外部位之一,以慢性肾小管间质性肾炎(TIN)最常见的病理类型。pSS的肾脏受累通常表现为慢性TIN并伴有1型或2型肾小管酸中毒(RTA)。很少有报道将肾脏受累描述为pSS中具有III型RTA的急性TIN。
■一名37岁女子因口干而入院,干眼,进行性肌肉无力持续了17个月.入院前两个月,患者的血钾水平为1.7mmol/L。
方法:进一步的测试证实了pSS和III型RTA。肾活检显示急性TIN和薄基底膜肾病(TBMN)。
方法:应用全剂量皮质类固醇(1mg/kg/天)和环磷酰胺(100mg/天)。
结果:患者的肌酐水平在3个月的随访期间下降了0.28mg/dL(1.18-0.90mg/dL)。
结论:我们报道了一例pSS相关肾损伤患者,表现为急性TIN,3型RTA和TBMN。这种情况增加了对pSS相关肾损伤的罕见表现的认识。在pSS相关的急性TIN中,环磷酰胺联合全剂量糖皮质激素可能取得良好的疗效.
BACKGROUND: The kidney is one of the common extraglandular sites involved in primary Sjögren syndrome (pSS), with chronic tubulointerstitial nephritis (TIN) the most common pathology type. Renal involvement in pSS often presents as chronic TIN accompanied by type 1 or 2 renal tubular acidosis (RTA). Description of renal involvement as acute TIN with type III RTA in pSS has been rarely reported.
UNASSIGNED: A 37-year-old woman was admitted with complaints of dry mouth, dry eyes, and progressive muscle weakness for 17 months. Two months before admission, the patient had a blood potassium level of 1.7 mmol/L.
METHODS: Further tests confirmed pSS and type III RTA. Renal biopsy demonstrated acute TIN and thin basement membrane nephropathy (TBMN).
METHODS: Full-dose corticosteroid (1 mg/kg/day) and cyclophosphamide (100 mg/day) were applied.
RESULTS: The creatinine levels of the patient decreased 0.28 mg/dL (1.18-0.90 mg/dL) during 3-month follow-up.
CONCLUSIONS: We reported a patient with pSS-associated kidney injury, presenting as acute TIN with type 3 RTA and TBMN. This
case increases the awareness of a rare manifestation of pSS-associated kidney injury. In pSS-associated acute TIN, cyclophosphamide combined with full-dose corticosteroids may achieve good outcomes.