Clinicopathologic characteristics, classification and outcome in lupus nephritis patients with thrombotic microangiopathy / 中华肾脏病杂志

ABSTRACT

Objective To investigate the clinicopathologic characteristics, classification and outcome in lupus nephritis(LN)patients with thrombotic microangiopathy(TMA). Methods LN patients with TMA proven by renal biopsy, from January 2000 to February 2009 in our hospital were enrolled. They were classified as poly-immunocomplex deposit group(n =35)and pauci-immunocomplex deposit group(n=25). Their clinicopathologic features and outcome were analyzed retrospectively. Results(l)The incidence of TMA in lupus nephritis was 9.2%(n=62), which presented severe hypertension, prominently elevated serum creatinine, anemia, thrombocytopenia, and was also the poorest prognosis of all the vascular lesion types. The incidence of death/end stage renal disease(ESRD)was 25.0%, with a mortality rate of 13.6%.(2)According to immunocomplex deposit in renal tissue, LN complicated with TMA could be classified as "poly immunocomplex deposit subtype" and "pauci-immunocomplex deposit subtype". The former presented better response to steroid and immunosuppressant therapy, in spite of more active clinicopathologic manifestations. The incidences of death/ESRD of poly subtype and pauci subtype were 8.8% and 32.0% respectively. Conclusions TMA presenting severe manifestations and the poorest prognosis is not rare in LN. LN with TMA may be classified as poly-immunocomplex deposit subtype and pauci-immunocomplex deposit subtype. This classification may be helpful in prognosis because the latter shows bad response to steroid-immunosuppressant therapy.