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Clinical Journal of the American Society of Nephrology
Glomerular disease associated with nephrotic syndrome has rarely been recognized as a distinct complication of allogeneic hematopoietic cell transplantation. Case reports in the English and Japanese literature since have described variable glomerular histology, comprising mainly membranous glomerulonephritis MGN in almost two thirds and minimal change disease MCD in nearly one quarter of patients. Review of the literature reveals a close temporal relationship between the development of nephrotic syndrome shortly after cessation of immunosuppression and the diagnosis of chronic graft- versus -host disease GVHD.
MCD occurred earlier after hematopoietic cell transplantation, was diagnosed sooner after medication change, and exhibited a better prognosis in comparison with MGN. Glomerular lesions after hematopoietic cell transplantation may therefore represent the renal manifestation of GVHD. Further studies are warranted to delineate the pathogenesis of this complication.
The emergence of hematopoietic cell transplantation HCT for malignant and particularly so-called benign hematopoietic indications has resulted in the increasing recognition of acute and chronic renal failure as a complication.
In close temporal proximity to the HCT, well-described conditions such as medication-induced nephrotoxicity, tumor-lysis syndrome, septic-ischemic tubular necrosis, and drug- or virus-induced hemorrhagic cystitis may occur 1. Delayed decline of kidney function is usually caused by radiation nephropathy, calcineurin inhibitor nephrotoxicity as a result of graft- versus -host disease GVHD prophylaxis, and hemolytic-uremic syndrome 1.
The term bone marrow transplant nephropathy 2 , 3 has thus far been used to refer to late renal complications after HCT reminiscent of hemolytic-uremic syndrome, attributed to total body irradiation and nephrotoxic therapies.
This lesion is characterized by mesangiolysis, mesangial matrix expansion, glomerular capillary widening, endothelial injury and dropout, and subendothelial space widening secondary to deposition of fibrin and newly formed basement membrane 2 , 3. GVHD is a serious complication of allogeneic HCT, which can affect skin, eyes, mouth, serous membranes, liver, gastrointestinal and respiratory tracts, and the musculoskeletal, hematopoietic, and immune systems.
The pathophysiology is complex and consists of cytokine activation of antigen-presenting cells after radiochemotherapy, which leads to T cell proliferation and differentiation into effector cells. These cells secrete cytokines that mediate apoptosis, tissue injury, and a positive inflammatory feedback loop 5. Despite increasing knowledge about GVHD, nephrotic syndrome NS has not been studied extensively, albeit systematically, in this context. Case reports describe various pathologic glomerular changes after HCT.
Herein, we report two typical cases followed at our institution, as well as two patients with less frequent features, and we review previous case reports to identify common characteristics among patients. A white man patient 1 , born in , underwent an allogeneic peripheral blood progenitor cell transplantation PBT after cyclophosphamide and busulfan conditioning therapy from his HLA-identical brother at the Hospital of the University of Pennsylvania HUP in September for first acute myelogenous leukemia AML M2 relapse.
His course was complicated by development of acute skin GVHD despite cyclosporine A CsA prophylaxis as well as chronic lung GVHD, consisting of changes reminiscent of bronchiolitis obliterans organizing pneumonia and pleural effusions, in March The patient was treated with prednisone and CsA, which were tapered and discontinued in February In July , the patient was found to have new, biopsy-proven chronic GVHD with skin, lung, and pericardial involvement.
At the same time, the patient was also noted to have NS, characterized by anasarca, hypoalbuminemia 1. Secondary to steroid resistance, tacrolimus and mycophenolate mofetil were added in November His creatinine remained relatively stable at 0. Glomerular lesions in patients after hematopoietic cell transplantation. A and B Patient 1: No definitive basement membrane spikes were present on histology A , but the electron microscopy B showed epimembranous immune complex deposition.
The immunofluorescence not shown confirmed granular immune deposition for IgG along the capillary loops. In addition, occasional mesangial and subendothelial deposits and rare focal segmental glomerulosclerosis were also noted not shown.
C and D Patient 2: Mild mesangial proliferative glomerulopathy. Histology C showed very mild mesangial proliferation. Mild interstitial chronicity was also present not shown. Immunofluorescence not shown was negative. The patient was admitted to HUP in July with dyspnea on exertion, foamy urine, facial edema, and a rash on his face and on both arms for 1 wk.
He was found to have NS with anasarca, hypoalbuminemia albumin 1. Tests such as hepatitis B and C serologies, complement C3 and C4 levels, ANA, ANCA, serum and urine protein electrophoresis, and antistreptolysin titer were within normal limits, and blood cultures were negative. A kidney biopsy revealed rare mesangial proliferative glomerulonephritis Figure 1, C and D. A skin punch biopsy of the arm showed new chronic GVHD. At 4 wk, the forearm skin lesions had disappeared, and only trace lower leg edema was present creatinine 1.
Prednisone was tapered, but in April , the patient had a relapse of NS with peripheral edema, nephrotic-range proteinuria, and hypoalbuminemia. In August , a chest computed tomography scan revealed new lymphadenopathy indicating possible recurrence of HL, and the patient is currently receiving chemotherapy. In patient 1, the NS appearance in July coincided with GVHD involvement of a new organ as well as worsening in previously affected organs after discontinuation of CsA and prednisone in February The mesangial proliferative glomerulopathy Figure 1C seen in patient 2 was characterized by extensive foot process effacement Figure 1D and mild interstitial chronicity.
This interstitial chronicity precluded the histopathologic diagnosis of classic minimal change disease MCD. The mesangial proliferative glomerulonephritis may then have been the sole acute histologic correlation of NS, thereby constituting an MCD variant. The association is rare, and the pathophysiology is unknown. A delay of up to 19 mo has been described, in one case report 9 , between onset of NS, which spontaneously remitted, and diagnosis of recurrent HL.
However, the glomerulopathy typically occurs within 6 mo before a diagnosis of recurrent HL is made. In our case, the patient was in complete remission after HCT for 19 mo, and therefore 13 mo after first development of NS. Although a minimal possibility remains that the NS occurrence was related to the HL relapse, the close proximity between the cessation of CsA prophylaxis and the development of new skin GVHD along with NS 2 to 3 wk later is highly suggestive of a causal association.
In addition to patients 1 and 2, we noted two patients in our institution without clear-cut relationships between the NS presentation and HCT, chronic GVHD, or medication change.
Although these cases do not illustrate supposedly typical relationships, their inclusion within the scope of a descriptive analysis is warranted as they demonstrate NS occurrences after HCT. Otherwise, bias would be introduced by overestimation of tight links between NS and potentially inciting events. It is conceivable that some cases do not exhibit possibly typical risk factors before NS presentation despite a connection to HCT, probably dependent on the underlying histologic correlate and its progression pattern.
Both additional patients received a diagnosis of focal segmental glomerulosclerosis FSGS , which may be either a diagnosis that is linked directly to the HCT or a secondary phenomenon after a different glomerular insult. He presented with nephrotic-range proteinuria in May creatinine 2.
A renal biopsy revealed global glomerulosclerosis in 13 of 15 glomeruli, while one of the two remaining glomeruli had a small segmental area of sclerosis. Electron microscopy showed focal areas of podocyte foot process effacement, and moderate fibrosis and tubular atrophy were seen in the interstitium.
Currently, the patient has CKD stage 4, and he may receive a preemptive living-related donor kidney transplantation from his bone marrow donor in the future In November , she developed facial swelling and pedal edema, and laboratory tests were consistent with nephrotic syndrome creatinine 0.
A renal biopsy in December showed global sclerosis in two of 20 glomeruli, whereas others had varying degrees of hypercellularity with increased mesangial cells and diffuse foot process effacement. The patient received an intravenous methylprednisolone pulse therapy and oral prednisone thereafter without significant improvement.
A second renal biopsy, performed in January to assess chronicity, prognosis, and future treatment after 2 yr of glucocorticosteroid therapy, was consistent with fully developed FSGS global sclerosis in seven of 15 glomeruli, variable degrees of segmental sclerosis in the remaining glomeruli, focal visceral epithelial foot process effacement, moderately fibrotic interstitium, and tubular atrophy.
Ultimately, the patient may be a candidate for renal transplantation from her original bone marrow donor A literature search was conducted to place individual cases into perspective and to identify global characteristics of NS occurrence after HCT. Main case features were abstracted, and statistical analysis was performed with SPSS Percentages were calculated under exclusion of cases for which values were not reported, except where otherwise noted, as the number of missing values was usually low.
Forty-two previous, well-documented cases 11 — 43 that link allogeneic HCT and glomerular kidney disease have been published between and Main individual findings of the reviewed 46 cases are listed in complementary Table 2. Other publications 44 , 45 refer to additional cases, but the details are too scarce to take these patients into account for detailed analysis in this review.
In an additional case, NS developed after bone marrow nephropathy was found on renal histology, but the patient did not undergo biopsy again A cohort study 43 of nonmyeloablative HCT listed seven patients with NS, three of whom did not receive a histologic diagnosis and therefore were not included.
Two patients with a nephritic pattern as a result of ANCA-associated glomerulonephritis after autologous 47 and allogeneic 48 HCT were also excluded, and two pediatric case reports of membranoproliferative glomerulonephritis after autologous HCT 49 and of glomerular vasculopathy, resembling thrombotic microangiopathy, after cord blood transplantation 50 seemed unrelated as well.
Clinical characteristics of patients with NS and associated glomerular disease after allogeneic HCT a. Mesangial proliferative glomerulonephritis may be interpreted as an MCD variant 27 ; our study.
Other diagnoses vary and include FSGS that may be secondary to other types of glomerular lesions, mesangial IgA deposition with crescent formation, and diffuse proliferative and crescentic glomerulonephritis that may be superimposed on a different glomerular disease.
Histopathologic findings, both for glomerular disease and for extraglomerular changes, are not straightforward in some studies. For example, in two cases here classified only descriptively as immune complex—mediated glomerulonephritis, the authors reported glomerular subendothelial and subepithelial electron-dense deposits despite negative immunofluorescence, without arriving at a distinct diagnosis Several histopathologic specimens 12 , 13 , 25 , 28 — 30 , 32 , 42 exhibit interstitial infiltrates of variable intensity, consisting of lymphocytes, monocytes, or macrophages.
Two studies 30 , 42 confirm a donor origin of mononuclear interstitial infiltrates. Rare findings include immune complex deposition in the tubular basement membrane along with a diagnosis of MGN The predominant underlying hematologic diagnosis is CML; acute myeloblastic leukemia and aplastic anemia are other common diagnoses.
It should be noted that a recent meta-analysis 51 has confirmed individual study results that chronic GVHD is more likely after PBT than after BMT, which may be explained by the transfer of a greater T cell dose.
The question of whether the continuing relative increase of PBT among HCT leads to a greater probability of NS as a complication is beyond the scope of this review because of the lack of an adequate epidemiologic approach with higher patient numbers. The NS presentation is consistently described as obvious, a feature that undermines the probability of lead time bias as a result of early proteinuria detection because of close monitoring after HCT.
ANA titers are mostly reported as negative or low data not shown , whereas less than one fifth of the cases 14 — 16 , 21 , 32 , 37 , 39 , 40 possess a titer of 1: It would be speculative to predict that patients who are older at the time of HCT are more prone to MGN than to MCD as a complication, thereby imitating the age distribution of so-called primary glomerulopathies.
The time course between cessation or tapering of immunosuppressive medication and onset of NS suggests the former as a risk factor. These proportions were calculated with all cases in the denominator, including those with missing values, and therefore may be falsely low; however, the true effect could also be obscured by recall bias, as case report authors may have overattributed NS occurrence to medication changes retrospectively. Some of the references do not describe details about GVHD examinations or medication changes; therefore, a higher percentage of cases than reported may support the associations outlined above.
On the basis of these relationships, the interpretation of glomerular disease as the manifestation of renal GVHD is conceivable. The seemingly good prognosis has to be weighed against the lack of comparable long-term follow-up time, on which the case report authors often do not comment. Successful treatment options include glucocorticosteroids and CsA. Other agents that have been used in the past for GVHD and glomerular disease, respectively, seem to have therapeutic potential as well, such as cyclophosphamide, tacrolimus, mycophenolate mofetil, and rituximab.
The significance of the incongruity remains intangible as the question continues to be unsettled whether a steroid-CsA combination for extensive GVHD offers survival benefits over steroids alone or just ameliorates steroid side effects 52 — A cohort study 43 identified nonmyeloablative HCT as a risk factor for NS, because seven of consecutive patients from to developed this complication as opposed to none of patients after myeloablative HCT, with comparable rates of chronic GVHD during the same period.
Follow-up of larger cohorts is mandatory to define potential NS risk factors definitively, such as presence or history of GVHD, immunosuppression change, conditioning regimen, and degree of mixed hematopoietic chimerism.
This fact underscores the lack of tubulitis in the reviewed biopsy specimens, although one separate case report 56 links tubulitis to GVHD after steroid and CsA withdrawal in the absence of glomerular changes.
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Jan. Rituximab for adult primary FSGS and MCD Steroid-resistant and steroid- dependent ne- with steroid-dependent nephrotic syndrome. 6 Jul  reported that rituximab treatment did not increase the risk of infections in highly .. infection in 2 patients receiving rituximab for refractory myositis. in children with steroid-dependent minimal change nephrotic syndrome. However, frequent relapses, steroid dependence, steroid resistance, and side in 5 adult patients with multirelapsing/steroid-dependent nephrotic syndrome.