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Vasculitis are autoimmune diseases that produce necrotizing inflammation of small vessels and tissue damage. In Uruguay, according to the Uruguayan registry of glomerulopathies (RUG), it is one of the secondary glomerulopathies with the highest incidence and frequency. Transplantation is a good therapeutic option for the patient with vasculitis because mortality and the probability of recurrence is lower than in dialysis. The recurrence rate is close to 10%. De novo vasculitis is an extremely rare disease. The 2019 study by Buglioni et al is the one that analyzed the largest number of cases, 10. The number of women and men was equal and they had a variety of underlying diseases. They occurred at any time after transplantation during the first year or up to 8 years later. All patients were under immunosuppression. None of them had systemic manifestations. ANCAs were negative in all but 1 patient.
Descriptive and retrospective case series study of renal transplant patients diagnosed with de novo pauciimmune glomerulonephritis post-transplantation in the INU center between 1980 and 2023. The aim of the study was to analyze the clinical and histopathological characteristics, evolution and treatment received by these patients.
Of the 2000 transplantations performed between 1980 and 2023, 5 patients (0.25%) developed de novo pauciimmune glomerulonephritis, with an early diagnosis in 4 of them, median 15 days post-transplantation. Only one patient was diagnosed later at day 1576. The median age at diagnosis was 42 years (from 25 to 71 years).
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
PostTR time at diagnosis
13 days
56 days
17 days
11 days
1576 days
Cause of indication PBR
IRI
Deterioration fx, Pu
Deterioration fx.
Deterioration fx
Creatinine at the moment of PBR
6.47
2.95
2.43
7.4
3.04
Pru/mHu
2.8/yes
1.5/yes
0.88/yes
2.4/yes
0.15/yes
Renal involvment
No
yes, lungs
yes, skin
ACA
Negative
glomerular affectation
9/12
6/14
1/15
3/5
10/80
Treatment
Ciclophosfamide, metilprednisolona, plasmaféresis y gammaglobulina.
Ciclofosfamida, metilprednisolona, plasmaféresis y gammaglobulina.
Ciclofosfamida, metilprednisolona.
Ciclofosfamida, metilprednisolona
5 month
46 month
11 month
72 month
Creatinine 2.1 mg/dl
Creatinine 2.06 mg/dl
Creatinine 6 mg/dl
Ingreso a TRR
Creatinine 1.77 mg/dl
Creatinina 4 mg/dl
No Pu or mhu
no dato
no Pu or mhu
Pauciimmune GN in post-transplantation is a rare pathology; in our cohort it represented 0.25% of the total patients, but in others it was 0.001%.
The presentation can be immediate or late years after transplantation. In 4 of our patients the presentation was immediate after transplantation. Only one patient had a late presentation 4 years later. There is no predilection for sex, age or race.
In terms of clinical presentation, three of our patients had only renal involvement and two of them had systemic involvement, one cutaneous and one pulmonary. In the case of Buglioni's cohort, 9/10 patients had no systemic involvement and the remaining patients had no data.
ANCA were negative in all patients. In Buglioni's series 9/10 were ANCA negative. These findings contrast with vasculitis in native kidneys where 85% are positive. It could be suggested that in transplantation there is a different pathogenic mechanism.
Regarding histology, all cases showed extracapillary proliferation with negative immunoflorescence. The percentage of crescents was high in 3 of 5 cases. The degree of glomerular proliferation and renal insufficiency are lower than when the pathology is present in native kidneys.
As for the treatment there are no guidelines for the management. Decisions were taken according to each clinical case. The response to treatment in the cases that received plasmapheresis, cyclophosphamide and methylprednisolone was good with recovery of function and without relapses. Those who received only cyclophosphamide had a bad evolution and had to return to renal replacement therapy.