A 20-year-old woman with a history of delivery at age 12.
She suffered from fatigue and swelling of her ankles.
There was anemia (hemoglobin: 9.5 g/dl), ESR 27: mm/h, uremia: 40 mg/dl, creatinine: 1.28 mg/dl, proteinuria: 241 mg/dl and triglycerides: 87-12.
TSH: > 100 uUI/ml, low T3 and T4 and positive antithyroid antibodies.
In primary care, symptoms were interpreted as secondary to anemia and hypothyroidism and iron and thyroxine were prescribed.
One month later, fatigue persisted despite normalization of TSH, no other symptoms had appeared but anemia had increased.
ESR was 106 mm/h, creatinine: 5.2 mg/dl, creatinine clearance (CrCl): 17.0 ml/min/1.73 m2, urea: 109 mg/dl, haematuria persisting 1.700 mg/dl.
Blood pressure 110/60 mmHg
She was hospitalized.
On physical examination, there were no other signs of silence and ankle edema.
Chest X-ray, sinuses and abdominal ultrasound were normal, with no renal alterations.
He suffered a rapidly progressive glomerulonephritis (RPGN).
Antinuclear and anti-DNA antibodies, immunoglobulin quantification, rheumatoid factor, C’3 and C’4 were negative or normal.
ANCA (IFI) were positive in 1/640 with perinuclear pattern ( ELISA was not performed for anti-MPO or anti-PR3 antibodies).
Methylprednisolone 1 g/for 3 times and cyclophosphamide 500 mg intravenously were administered, continuing with prednisone 60 mg/day and cyclophosphamide 100 mg/day orally.
A renal biopsy was performed, which showed, by optical coherence examination, 29 glomeruli, 23 of which were obsolete, compromised by crescent fibrocellular glomeruli in 17% and fibrocellular lesions in 52% of the glomeruli
Three glomeruli showed increasing cell predominance (10%).
Lymphocytic lymphocytic inflammatory infiltrate was present in the tubulocytic interstitial zone.
The vessels had a normal structure.
The tissue for immunofluorescence with 3 glomeruli, which had fibrin forming images of crescents.
There was no glomerular reactivity for C3, C1q, IgA or IgG.
The glomerulus examined by electronic means was preserved; its architecture was preserved; there were deposits and dense deposits in it and it only showed extensive pedicelar disappearance.
Chronic, active, creamy glomerulonephritis was diagnosed.
1.
Three months later, the patient's general condition and renal function had improved (creatinine: 2.0 mg/dl; proteinuria: m 1.270 mg/24 h) receiving prednisone, cyclophosphamide, enalapril, thyroxine and losartan.
One year later, creatinine: 1.6 mg/dl; proteinuria: 1.290 mg/24 h.
Two years later she reported amenorrhea, hot flashes, and sweating. She had estrogen toxicity, which was confirmed by low levels of cyclophosphamide and elevated gonadotropins.
Cyclophosphamide was changed to azathioprine 50 mg/day.
Menstruals didn't come back.
During this period there were no clinical manifestations of involvement of other organs or systems, outside those mentioned.
Ten years after diagnosis she presented genital bleeding.
Sorptive pelvic ultrasound showed a 15-week pregnancy.
Enalapril was discontinued maintaining azathioprine and prednisone.
Blood pressure was normal, there was no hematuria, CrCl: 58 ml/min/1.73 m2, creatinine: 1.5 mg/dl and proteinuria increased to 2.960 mg/24 h.
The pregnancy was normal and without edema, proteinuria increased to 4.440 mg/24 h at 33 weeks, associated with a reduction in CrCl to 50.9 ml/min/1.73 m2 with elevation.
A spontaneous delivery occurred at 37 weeks without complications.
The newborn weighed 2,440 g and had no malformations or other evident pathologies.
Two weeks later, proteinuria increased to 11,870 mg/24 h, CrCl: 50 ml/min/1.73 m2 and there was no hematuria.
It was indicated to suspend breastfeeding to restart the antiproteinuric therapy but the patient did not accept it, leaving the controls.
Reappeared 7 months after delivery breastfeeding.
Proteinuria was 12,700 mg/24 h, CrCl: 46.6 ml/min/1.73 m2 and creatinine: 2.0 mg/dl. Enalapril 20 mg/12 h and azathioprine 100 mg/day were indicated.
One year after delivery the situation had worsened, uremia: 94 mg/dl, creatinine: 3.1 mg/dl, CrCl: 41 ml/min/1.73 m2 and proteinuria: 4.850 mg/24 h.
The determination of ANCA by IF was positive at 1/640 and the determination by ELISA showed specificity of antibodies by myelooperoxidase.
Azathioprine was switched to cyclophosphamide and the corticosteroid dose was increased.
This reduced proteinuria to sub-nephrotic ranges over the next 3 months.
