Unexpected recovery from longterm renal failure in severe diffuse proliferative lupus nephritis

Abstract

BackgroundSevere renal manifestation of systemic lupus erythematosus (SLE) is not uncommon and is associated with an indeterminate prognosis. Complete remission can be obtained, however, at least in the young when chronic lesions are absent and adequate anti-inflammatory therapy is immediately initiated.Case presentationWe report the unusual case of a 12-year-old girl who presented with severe oliguric renal failure, macrohematuria and skin rash. Renal biopsy revealed the diagnosis of severe diffuse proliferative glomerulonephritis (GN) with cellular crescents in 15 out of 18 glomeruli and full-house pattern in immunofluorescence indicating lupus nephritis IVB according to WHO, IV-G(A) according to ISN/RPS classification. The serological parameters confirmed the diagnosis of SLE and the patient was immediately treated with methylprednisolone, cyclophosphamide and immunoadsorption. Initially, despite rapid amelioration of her general condition, no substantial improvement of renal function could be achieved and the patient needed hemodialysis treatment for 12 weeks. Unexpectedly, in the further follow-up at first diuresis increased and thereafter also creatinine levels substantially declined so that hemodialysis could be discontinued. Today, 6 years after the initial presentation, the patient has normal renal function and a SLEDAI score of 0 under a continuous immunosuppressive therapy with Mycophenolate mofetil (MMF) and low dose steroid.ConclusionDespite the severity of the initial renal injury and the unfavourable renal prognosis the kidney apparently has a tremendous capacity to recover in young patients when the damage is acute and adequate anti-inflammatory therapy is initiated without delay.

Kidney outcomes for children with lupus nephritis

“…The decision of when is best to transplant therefore needs to be carefully balanced against the recognized increased risks of morbidity and mortality associated with long-term dialysis. It is of the authors’ opinion that a period of 6–12 months of remission should be achieved prior to transplantation to allow for systemic disease quiescence before major surgery and to ensure that there is no chance of kidney recovery, as delayed kidney improvement is reported [ 38 ]. This may require interim dialysis.…”

An Unusual Case of Crescentic Lupus Nephritis Presenting With Normal Renal Function

“…Other factors that have been predicted to affect the renal function are evidence of chronicity like glomerular sclerosis, fibrous crescents (unlike fibrocellular which are more active lesions), tubular atrophy and interstitial fibrosis [13]. The proliferative forms of lupus nephritis tend to have a relatively poorer renal function [14]. Of these, Class III (focal lupus nephritis) and IV (diffuse lupus nephritis) are usually considered to be the more severe forms and are aggressively treated [5].…”

Chronic Dialysis Discontinuation: A Systematic Narrative Review of the Literature in the New Millennium

“…While vascular diseases play a major role in the large series and in registry data, case reports, which are more prone to publication biases, mainly regard the more “charming” glomerular diseases and are mainly targeted at describing exceptional cases, highlighting the possibility of long-term recovery after various treatments, whether started in the pre-dialysis phase or performed during dialysis (29–37, 39–40) (Tab. II).…”

“…Table II summarizes the 13 case reports retrieved by our search strategy; they deal with one to two cases, and are reported from different sources (8 from Europe, 2 from Japan, 1 from Canada, and 2 from USA). The distribution of the case reports differs from that of the case series and registry data, highlighting the possibility of renal function recovery in immunologic or hematologic diseases, encompassing all the major glomerulopathies, from the rare ones, like Wegener's disease and atypical HUS, to the common ones, like IgA nephropathy or FSGS (29)(30)(31)(32)(33)(34)(35)39). However, in keeping with the important role of vascular diseases in this context, two cases (cholesterol emboli and renal artery stenosis) are also reported (38,41).…”