■单个患者中存在三种不同的实体通常具有临床意义,并且大多是轶事。系统性硬化症(SSc)的重叠,干燥综合征(SS)ANCA相关的肾限制性血管炎以前仅报道过一次.
■一名61岁的女性在咨询后进行了2年的症状学评估,呈现纸板状的皮肤,sclerodactyly,有限的口腔开放,皮肤和眼睛干燥。她因进行性肾衰竭入院(血清肌酐,5.5mg/dL)。她的血清学检查显示抗SCL-70,抗Ro,反La,反MPO,和抗核抗体.进行肾活检,并确认SSc的组织学发现,SS,和ANCA相关性血管炎伴纤维性占优势的活动性毛细血管外肾小球肾炎(EUVAS-Berden硬化类),活动性肾小管间质性肾炎,局灶性肾小管损伤,和中度慢性动脉病变。建立了6个月剂量的甲基强的松龙和环磷酰胺治疗。在最后一次随访中,患者维持了2.6mg/dL的稳定血清肌酐水平,并减少了蛋白尿,没有红细胞增多症,也不需要肾脏替代治疗.
■系统性硬化症是一种罕见的自身免疫性疾病;尽管如此,与干燥综合征重叠相对常见,尽管它与ANCA血管炎的关联是轶事。诊断整合对肾脏病学家定义预后和特定治疗提出了挑战。
UNASSIGNED: The presence of three different entities in a single patient is usually of clinical interest and mostly anecdotal. The overlap of systemic sclerosis (SSc), Sjögren syndrome (SS), and ANCA-associated renal-limited vasculitis has been reported only once previously.
UNASSIGNED: A 61-year-old female was evaluated at consultation with 2 years of symptomatology, presenting cardboard-like skin, sclerodactyly, limited oral opening, and dry skin and eyes. She was admitted for progressive renal failure (serum creatinine, 5.5 mg/dL). Her serology work-up showed positive anti-SCL-70, anti-Ro, anti-La, anti-MPO, and antinuclear antibodies. Renal biopsy was performed and confirmed histological findings for SSc, SS, and ANCA-associated vasculitis with active extracapillary glomerulonephritis with fibrous predominance (EUVAS-Berden sclerotic class), active tubulointerstitial nephritis, focal tubular injury, and moderate chronic arteriolopathy. Treatment with 6 monthly doses of methylprednisolone and cyclophosphamide was established. At the last follow-up, the patient maintained a stable serum creatinine level of 2.6 mg/dL and had decreased proteinuria, no erythrocyturia, and no requirement for renal replacement therapy.
UNASSIGNED: Systemic sclerosis is a rare autoimmune disease; nevertheless, overlap with Sjögren syndrome is relatively common, although its association with ANCA vasculitis is anecdotal. Diagnostic integration presents a challenge for nephrologists to define the prognosis and a specific treatment.