Male, 61 years old, white.
Macrohematuria and hypertension after oropharyngeal infection at age 21.
Biopsychiatric renal puncture (BRP) at 31 years in Australia (unknown result).
In December 2006, at age 57, peritoneal dialysis was initiated.
In April 2008, she received a kidney transplant from a cadaveric donor.
Creatininemia at discharge was 0.8 mg/dl and without proteinuria.
Immunosuppression: cyclosporine, mycophenolate mofetil (MMF) and prednisone.
Twenty-six months after transplantation, dry cough, fever, pain, and abdominal distention appear.
Erythematoviolaceous papules on the lower limbs and gluli were observed prior to admission.
Examination: Congestive pharynx.
1 to 3 mm papules, erythematous-violaceous, which did not disappear from compression on thighs, buttocks, and soles.
Epigastric pain
Inject without pain or enlargement.
1.
Laboratory data are summarized in Table 1.
binding to hepatitis B and C virus, human immunodeficiency virus, antinuclear antibodies and antineutrophil cytoplasmic antibodies.
Normal supplementation.
Faecal occult blood by positive immunologic method.
1.
The clinical presentation suggests HSP.
Skin biopsy was performed, with negative immunofluorescence.
There is a progressive increase in proteinuria, so BRP is performed showing proliferative glomerulonephritis intra and extracapilary with cellular and fibro glomerulonephritis, mostly segmental, which affects cellular and fibroglomeruli.
Necrosis foci and leukocytoclasia.
Mesangial deposits and predominant IgA pericapillary deposits.
According to the national guidelines (www.nefroprevention.org.uy), treatment is initiated with boluses of methylprednisolone 1 g/d x 3 and monthly cyclophosphamide 6, 15 mg/k.
Cyclosporine 2 mg/k/d was maintained and MMF was suspended.
The evolution is shown in Table 1.
At 22 months after the onset of HSP, serum creatinine was 1.21 mg %, urine proteinuria 0.32 g/d.
Moroni et al.1 and Han et al.2 describe a risk of recurrence of HSP, ranging from 0 to 61 %, with greater recurrence in a related living donor.
Thervet et al. described histological recurrence (IgA deposits) after one year of transplantation in 69 % of patients with immunoglobulin A nephropathy (IgA nephropathy).
Shimizu et al.3 described a case of post-transplant extracapilary IgA glomerulonephritis.
The presence of abdominal pain suggested atypical HSP.
Except for this doubtful diagnostic reference, the appearance of new post-transplant HSP has not been described.
In this case, the absence of purpura prior to transplantation suggests a de novo occurrence of HSP.
Araque et al. published in 1995 the first case of HSP in a patient with chronic renal failure, years after diagnosis of IgAN.
In our case, the previous history is suggestive of IgAN and currently presents an HSP.
Recurrence of HSP has a worse prognosis: 50% graft loss at 30 months compared to recurrence of IgAN (11 %)2.
HSP in adults is more severe and its prognosis is worse than in children.
The series reported by Pillebout et al.7 (250 cases) reported that 25% of adults achieve extreme renal failure.
Initial renal function and the degree of proteinuria are markers of poor prognosis, as well as glomerular necrosis and elements of chronicity7.
Our case showed an intense inflammatory component, cellular cells and necrosis in more than 50% of the glomeruli.
Soler et al. demonstrated that renal survival was lower in patients with Hairy Cells in renal biopsy at diagnosis.
Clinical and histological discordance regarding the need for BPR in all adult patients with minimal renal involvement in the context of HSP.
Regarding treatment, transplant immunosuppression did not prevent the appearance of HSP.
This is close to literature 2,9,10.
Although treatment in HSP is controversial, small series have shown benefit in the aggressive treatment of these patients.
However, the Pillebout study published in September 201011, after our patient was treated, suggests that cyclophosphamide does not offer additional benefit.
In this series extracapilary proliferation was observed in 37 to 27 % of patients in each treatment group.
Our patient had more severe active lesions.
Maintenance was performed with azathioprine, given the limited utility described for MMF in IgAN12,13.
There is a progressive reduction in proteinuria, which is currently less than 0.5 g/d, maintaining stable renal function.
The notable facts of this clinical case are:
1.
The de novo presentation of an HSP following transplantation despite medication.
2.
The need for early histological study, given the clinical and pathological discordance.
3.
The controversy about the treatment to be performed, knowing that the evolution of HSP in adults is not favorable, and that the implementation of a more aggressive treatment was recently questioned by the Pillebout group.
