Tholl U, Hartung K, Helmchen U, Anlauf M
Medizinische Klinik II, Zentralkrankenhaus Reinkenheide, Bremerhaven.
Dtsch Med Wochenschr. 1998 Dec 18;123(51-52):1541-6. doi: 10.1055/s-2007-1024220.
An 82-year-old woman with hypertension for 20 years developed a nephrotic syndrome with severe oedema followed by acute oliguric renal failure after a bout of bronchitis and a gastrointestinal infection. She also complained of xerostomia and dry eyes of recent onset.
Biochemical tests showed a serum creatinine level of 6.1 mg/dl, a 1:5120 antinuclear antibody (ANA) titre, and positive values for Ro(SS-A) and La(SS-B) antibodies. HLA-DR typing demonstrated HLA-DR3 (HLA-DRB10301) and DR13 (HLA-DRB113) antigens. Renal biopsy revealed minimal glomerular lesions with focal and segmental glomerulo-sclerosis as well as (hypertension-induced) benign nephrosclerosis and focal tubular atrophy with interstitial fibrosis.
After two hemofiltrations and concomitant administration of 100 mg prednisone renal function quickly improved and the proteinuria fell to 1 g/dl. At the same time the xerostomia improved. The nephrotic syndrome recurred 7 months later after the prednisone dose had been reduced to 10 mg/d, but after the dose had been raised to 50 mg/d and cyclosporin A (150 mg/d) had been added a lasting remission occurred and renal function became stable though impaired.
The relatively rare association of glomerular disease (here focal segmental glomerulosclerosis) with Sjögren's syndrome can, as in this case, be triggered by a viral infection. A genetic predisposition for Sjögren's syndrome is suggested by the demonstration of HLA-DR3 alleles. Administration of steroids is indicated for the treatment of the nephrotic syndrome and, in case of recurrence, can be combined with cyclosporin A. Both drugs also influence the symptoms of Sjögren's syndrome.
一名患有20年高血压的82岁女性，在患了一阵支气管炎和一次胃肠道感染后，出现了肾病综合征并伴有严重水肿，随后发展为急性少尿性肾衰竭。她还诉说近期出现口干和眼干。
生化检查显示血清肌酐水平为6.1mg/dl，抗核抗体（ANA）滴度为1:5120，Ro（SS - A）和La（SS - B）抗体呈阳性。HLA - DR分型显示存在HLA - DR3（HLA - DRB10301）和DR13（HLA - DRB113）抗原。肾活检显示肾小球病变轻微，伴有局灶节段性肾小球硬化，以及（高血压所致）良性肾硬化和局灶性肾小管萎缩伴间质纤维化。
经过两次血液滤过并同时给予100mg泼尼松后，肾功能迅速改善，蛋白尿降至1g/dl。与此同时，口干症状有所改善。7个月后，泼尼松剂量减至10mg/d时肾病综合征复发，但在剂量增至50mg/d并加用环孢素A（150mg/d）后，病情持续缓解，肾功能虽仍受损但趋于稳定。
肾小球疾病（此处为局灶节段性肾小球硬化）与干燥综合征相对罕见的关联，在本病例中可由病毒感染引发。HLA - DR3等位基因的存在提示了干燥综合征的遗传易感性。对于肾病综合征的治疗，建议使用类固醇，复发时可联合环孢素A。这两种药物也会影响干燥综合征的症状。
