The Clinical Manifestation, Pathologic Feature And Long-term Prognosis Of Patients Of Sjogren’s Syndrome With Renal Damage

Objective:To analyze the clinical manifestation, feature of pathology and factors affecting the long-term prognosis of patients of primary Sjogren’s syndrome with renal damage.Methods:A retrospective analysis of 103 cases of primary Sjogren’s syndrome with renal damage [99 female,4 males, median age 36 years (7-68 years)] in clinical manifestations, Immunological indicators, pathologic feature, and 75 cases who followed for more than twenty months in treatment, long-term prognosis and the factors affecting the prognosis.Results:The renal damage mostly affected tubulointerstitium.93(90.29%) patients had urinary infiltration reduction,65 cases (63.11%) had renal tubular acidosis (RTA), including type Ⅰ 59 cases (57.28%), type Ⅱ2 case (1.95%) and type Ⅲ4 cases (3.88%). 64 cases (62.14%) existed hypokalemia,11 cases (10.68%) existed nephrogenic diabetes insipidus.70 cases (67.97%) had urine urine protein; 23 cases (22.33%) had microscopic hematuria, and 2 cases (1.94%) with gross hematuria; 30 cases’(29.13%) creatinine increased.68 cases (66.02%) existed hypergammaglobulinemia. The positive rate of serum autoantibody levels were as follows:ANA (79.61%), anti-SSA (78.64%), anti-SSB (48.54%), RF (33.01%), anti-cardiolipin antibodies (14.56%), anti-RNP (12.62%) and Smith antibody (8.74%). Renal biopsy were made in 89 cases, and interstitial nephritis occupied 98.87%.16 cases had mesangial proliferation and IgA deposition, while 10 cases existed membranous nephropathy.75 patients had long-term follow-up (median follow-up time of 54 months,62.76±35.64 months average).1/3 of patients took a large number of immunosuppressive agents. The long-term complications were kidney stone, neuropsychiatric symptoms and hematological malignancy. At the end of the follow-up,2 patients’creatinine multiplied(more than 1.24 mg/dl) and 1 patient reached ESRD. The factors associated with prognosis included the ages of onset(P=0.044), chronic degree of the tubular interstitiallesion(P=0.001), globulin level(P=0.018), IgG level(P=0.042) and the hemoglobin level(P=0.049).Conclusions:Patients in Primary Sjogren’s syndrome patients with renal impairment were female mostly; Urine concentration ability decline, RTA and hypokalemia were most common; Renal pathology was mainly interstitial nephritis, some patients may had less mesangial proliferative with IgA deposition and membranous nephropathy. The long-term prognosis was good,5-year,10-year survival rate of human was 100%, while 5-year survival rate of kidney was 95.3%,10-year survival rate was 88%; The main factors affecting long-term prognosis included the ages of onset, chronic degree of the tubular interstitiallesion, hyperglobulinemia, high IgG level and anemia.