title: [A retrospective clinical analysis of 16 patients with scleroderma renal crisis].
abstract: OBJECTIVE
  To analyze the clinical features, laboratory tests, treatments and outcome of patients with scleroderma renal crisis (SRC).
  
  
  METHODS
  We retrospectively reviewed the clinical and laboratory data of 16 patients with scleroderma renal crisis in Peking Union Medical College Hospital from May 2004 to May 2013. The treatment and outcome of SRC patients were also retrospectively analyzed.
  
  
  RESULTS
  There were a total of 16 SRC patients including 5 male patients and 11 females. The median age at SRC onset was (49.9 ± 12.3) years. It usually took 3.2 years from the diagnosis of systemic sclerosis (SSc) to SRC attack. Ten SRC patients belonged to diffuse cutaneous systemic sclerosis (dcSSc), and 6 patients were limited cutaneous systemic sclerosis (lcSSc). Among SRC patients, 16/16 were negative of anti-centromere antibodies (ACAs). All these 16 patients had hypertension and renal insufficiency, including 8 dialysis dependent after the onset of SRC and 7 with thrombotic microangiopathy. There were 3 patients receiving renal biopsy. The pathological findings were mainly summarized as intimal thickening and stenosis of renal arterioles. Among 13 patients with long-term followed-up, 11 patients received angiotensin converting enzyme inhibitors (ACEI), 5 patients died, 2 patients were dialysis dependent. Only 1 patient stopped dialysis after the combination treatment of ACEI and endothelin receptor antagonist. Another 5 patients didn't need dialysis.
  
  
  CONCLUSIONS
  SRC usually occurred at the early course of SSc. dcSSc was more frequent than lcSSc. ACAs were rarely found in SRC patients. The immediate and sufficient use of ACEIs was still the cornerstone of SRC treatment. Future studies are needed to evaluate the efficacy of endothelin receptor antagonist in the treatment of SRC.
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences,Beijing 100032, China.
authors.forename: Jiaxin
authors.initials: J
authors.lastname: Zhou
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences,Beijing 100032, China. Email:Xudong74@hotmail.com.
authors.forename: Dong
authors.initials: D
authors.lastname: Xu
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences,Beijing 100032, China.
authors.forename: Yong
authors.initials: Y
authors.lastname: Hou
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences,Beijing 100032, China.
authors.forename: Qian
authors.initials: Q
authors.lastname: Wang
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences,Beijing 100032, China.
authors.forename: Mengtao
authors.initials: M
authors.lastname: Li
authors.affiliation: Department of Rheumatology, Peking Union Medical College Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences,Beijing 100032, China.
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Zeng
mesh_terms: D058186:Acute Kidney Injury / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use*; D000974:Antibodies, Antinuclear / Q000627:therapeutic use*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D006977:Hypertension, Renal / Q000209:etiology* / Q000401:mortality / Q000628:therapy; D007674:Kidney Diseases; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis; D051437:Renal Insufficiency; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000628:therapy*; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors; D000974:Antibodies, Antinuclear