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FRI0346 Renal involvement in granulomatosis with polyangiitis
  1. N Bulanov1,
  2. E Makarov2,
  3. E Kuznetsova1,
  4. M Bulanova3,
  5. P Novikov1,
  6. S Moiseev1
  1. 1Sechenov First Moscow State Medical University
  2. 2Lomonosov Moscow State University, Moscow
  3. 3Vladimir Regional Clinical Hospital, Vladimir, Russian Federation


Objectives We assessed the frequency, clinical features and prognosis of renal involvement in granulomatosis with polyangiitis (GPA) and current treatment approaches.

Methods We performed a retrospective analysis of 234 patients with GPA, diagnosed according to Chapel-Hill Consensus Conference 2012 classification, 81 male and 153 female, aged 53 (41; 62) years. 54 patients (23.1%) had localized GPA. 174 (74.4%) were ANCA positive. Median follow up was 61.5 (32; 105) months. 103 patients (44%) had a history of renal involvement. Frequencies of proteinuria, hematuria, hypertension, rapidly progressive glomerulonephritis (RPGN), acute kidney injury (AKI) and chronic kidney disease (CKD) grades were analyzed.

Results In 25 patients (24.3% of 103) renal involvement developed at disease onset, the other 78 patients (75.7%) developed glomerulonephritis after a median of 3 (1; 12) months. Hematuria was present in 94 (91.3%), proteinuria in 85 patients (82.5%), nephrotic syndrome in 11 patients (10.7%), hypertension in 31 patients (30.1%). Rapidly progressive glomerulonephritis (RPGN) defined by the doubling of serum creatinine within ≤3 months developed in 29 patients (28.2% of 103). Myeloperoxidase-ANCA-positive patients (n=12) developed RPGN significantly more often than proteinase-3-ANCA-positive patients (n=78): 7 (58.3%) vs 22 (28.2%) (p=0.0499). 11 (10.7%) patients developed AKI, stage 3 being the most common (in 8 patients). 40 (38.8%) patients exhibited indolent renal disease course, i.e. without worsening of renal function. By the end of the follow-up, 33 (32.0%) patients were diagnosed with CKD grade 3b-5, among them 11 patients (10.7%) developed end-stage renal disease (ESRD). 6 patients (5.8%) died. As the first line induction therapy all patients received corticosteroids in combination with cyclophosphamide in 93 patients (90.3%), rituximab (RTX) in 3 patients (2.9%), mycophenolate mofetil in 7 patients (6.8%). RTX was used for remission induction in 27 (26.2%) patients due to refractory or recurrent disease. During follow-up, 74 patients (71.8%) developed one or more severe relapses with a relapse rate of 0.27 per patient-year. 50 (48.5%) patients had renal relapses (0.096 per patient-year).

Conclusions Prevalence of renal involvement in the studied group was lower than expected, most likely because of a large proportion of patients with localized GPA. Our study showed that RPGN was not the most common feature of renal involvement. Almost 40% of patients developed indolent course of kidney disease. Nevertheless one third of patients developed CKD G3B or worse by the end of the follow up. Despite effective induction therapy with high-dose corticosteroids, cytotoxic agents and/or RTX relapses, both renal and extrarenal, are frequent.

Disclosure of Interest None declared

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