A 30-year-old woman was hospitalized in 2002 for polyarthritis and nephropathy (proteinuria, hematuria, creatinine 2.0 mg/dl, creatinine clearance 49 ml/min/1.73 m2).
Prednisone was prescribed, which was abandoned at 4 months due to appearance of tapered faces, as well as her medical controls.
A year later, he consulted due to arterial hypertension and was prescribed carvedilol.
There was renal failure (creatinine 1.7 mg/dl, creatinine clearance 54 ml/min/1.73 m2), proteinuria 400 mg/24 h, anemia, elevated ESR and ANCA(+) (perinuclear pattern).
The patient was hospitalized for renal biopsy (RBp) due to suspicion of crescentic glomerulonephritis, but the patient fled hours later and the RBp was not performed.
He returned 3 months later, hypertensive (160/100 mmHg), his renal function was in similar ranges, there was microscopic haematuria, leucocyturia, proteinuria (1,890 mg/day) and indirect ANCA(+) dilution.
As a RBp was not accepted having the clinical diagnosis of AN(+) cyclophosphamide, oral steroids 50 mg/day, corticosteroids 30 mg/day, and corticosteroids 40 mg/day were indicated (see section 4.5).
At the end of that year, her renal function had improved (creatinine clearance 62 ml/min/1.73 m2), there was no proteinuria (joint use of lysinopril and telmisartan) and she received 50 mg of cyclophosphamide.
At the third year of her disease (2004), she presented amenorrhea (cyclophosphamide toxicity) and reactivation of her vasculitis with marked worsening of renal function (creatinine: 4.0 mg/dl; creatinine clearance: 16 ml/min/1.73).
Neither PR3 nor MPO ELISA was determined.
A total of 60 mg/day of cyclophosphamide 150 mg/day was prescribed to reduce the severity of renal failure.
At the end of that year, creatinine clearance was: 65 ml/min/1.73 m2, proteinuria: 650 mg/24 h and there was no hematuria.
In the fourth year (2005), cyclophosphamide was changed by azathioprine, maintaining low doses of dextrose, telmisartan and lisinopril.
At the end of the year creatinine was 1.5 mg/dl, creatinine clearance: 68 ml/min/1.73 m2 and proteinuria: 480 mg/24 h.
At the fifth year he presented intercostal herpes zoster.
The following year he was normotensive, with stable renal function (creatinine clearance: 64 ml/min/1.73 m2) and proteinuria: 709 mg/24 h.
At the seventh year he did not come to controls.
He returned to the eighth year due to macroscopic hematuria and acute sinusitis.
While affirming to maintain the indicated therapy, the renal function had deteriorated (creatinine: 4.1 mg/dl,emia: 122 mg/dl), urine test: cylinders and fatty ureters, cerebellum
Antibiotics were prescribed and the patient was followed up in 1 week.
She returned 6 months later with notorious compromise of general status, renal failure (creatinine: 7.4 mg/dl, creatinine clearance: 12 ml/min/1.73 m2; urine output: 185 mg/dl).
Serology for B, C and HIV was negative.
A tunneled catheter was installed, chronic hemodialysis was started and an arteriovenous fistula (AVF) was built.
Against this backdrop.
Six months later, one week after starting to use the AVF for dialysis, the patient came to control in poor general condition, dysneic and intensely febrile and anemic.
There were pericardial smears, bilateral pleural effusion, leukocytosis (12,200 mm3), ESR: 140 mm/h and CRP: 354 mg/L (VN: < 9).
The following diagnostic hypotheses were raised: catheter-related sepsis, infective endocarditis, opportunistic infection, vasculitis reactivation and insufficient dialysis.
Blood cultures were taken in peripheral blood and by catheter (removal immediately), antibiotics were administered, dialysed daily and red blood cells were transfused.
In the following days his general condition improved, the fever fell slowly and the pleural fleets and effusions disappeared.
The hemocultive and urocultive were negative, the echocardiogram showed a pericardial effusion (no vegetations), the ANA were negative, complement C’3 and C’4: normal and the ANCA were negative by immunofluorescence.
The patient was in acceptable conditions and was discharged, although the etiology of her febrile condition and severe irritation was unknown.
Days later, the fever reappeared.
The thoracic-pelvis scanner performed looking for the cause of the fever showed marked abdominal aorta (low renal arteries), stenoses that progressed towards the arteries showed marked dilation of the iliac arteries.
There was also periaortic inflammatory tissue around the left subclavian artery that had proximal stenosis.
The kidneys were small.
The radiologist fixed a Takayasu's arteritis.
Corticosteroid and cyclophosphamide therapy was restarted.
1.
In the next 5 years he has been in good condition and no signs of reactivation of his disease have appeared.
During this period, despite the absence of ischemic signs in the lower and upper limbs, a iliac bypass was performed, with the aim of undergoing kidney transplantation.
During the intervention, the aortic segment was resected, which showed fibrosis of the adventitia fusion to the muscle (which was atrophic) and hyperplasia of the intima, compatible with sequelae of a distal arousal.
No granulomas, necrosis or giant cells were found.
