A 20-year-old male smoker and ex-consumer of cocaine and cannabis, who came to the hospital due to macroscopic findings and acute renal failure due to hematuria (AFRAO).
Twelve days before admission, the patient presented asthenia, anorexia, vomiting and dysuria, so she went to her bedside physician and was diagnosed with urinary infection, receiving ciprofloxacin treatment.
Four days later the patient developed fever of 38 oC, macroscopic hematuria and subjective decrease in diuresis.
Physical examination revealed the following data: blood pressure: 140/90 mmHg, temperature: 37oC.
General deterioration, cutaneous-mucosal dryness and bi-ventilation.
On admission, ARFO was observed, which required hemodialysis in the first 24 hours.
Forty-eight hours later he presented hemoptoic sputum, followed by two episodes of generalized tonic-clonic seizures.
Laboratory data at admission showed: Hto, 25.5%, Hb, 8.9 g/l, leukocytes, 16,700/mm3, platelets, 344,000/mm3, VSG, 11.8 mg/dl albumin,
Proteinogram, immunoglobulins and light chains kappa/lambda were normal.
Serology of hepatotropic virus, HIV, as well as liver function tests revealed no abnormalities.
Blood and urine toxic determinations, including cocaine metabolites, were negative.
The immunological study, consisting of determinations of ANA, ANCA, antiphospholipid antibodies, anticardiolipin antibodies, as well as complement levels, was negative.
However, anti-GBM antibodies were positive (72.5 ug/ml, normal range <10 U).
Urine analysis (24 h) showed proteinuria of 850 mg. There was intense hematuria in the sedimentary fluid.
A chest X-ray showed bilateral alveolar-interstitial infiltrate suggestive of alveolar hemorrhage.
Renal ultrasound showed kidneys of normal size, with increased cortical echogenicity.
Electroencephalogram, brain magnetic resonance imaging (MRI) and cerebral angio-MRI were normal.
Four days after admission a percutaneous renal biopsy was performed, in which an extracapilary glomerulonephritis (100% of semilunar) was observed, with collapse of the glomerular villous, moderate foci of interstitial necrosis
Immunofluorescence showed linear deposits of immunoglobulin G (IgG) around the glomerular basal membrane.
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The patient was diagnosed with SGP associated with probable CNS vasculitis ANCA negative.
On admission, the patient was treated with 3 boluses of 500 mg methylprednisolone for 3 consecutive days.
Subsequently, oral cyclophosphamide was associated with doses of 1.5 mg/kg/day and 15 sessions of plasmapheresis.
He also received treatment with valproic acid.
Respiratory and neurological symptoms disappeared with conservative treatment, but unfortunately renal function remained unchanged, resulting in hemodialysis program discharge.
Twenty-five days after admission, the patient was discharged with negative anti-GBM antibody titers, which allowed a slow reduction in immunosuppression without relapse data.
Cyclophosphamide was discontinued 3 months after admission, treating the patient with low doses of steroids (2.5 mg/day) and undergoing hemodialysis.
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Subsequent payment
Six months after discharge, after leaving the hypotensive treatment, the patient was readmitted for a hypertensive emergency (blood pressure 220/120 mmHg and right temporal intraparenchymal hematoma requiring surgical drainage).
At that time, serial determinations of anti-GBM antibodies were negative and, despite the severity of the lesions, the patient did not present neurological deficit at discharge.
Twenty months later, he received a kidney transplant from a cadaver donor, in addition to treatment with tacrolimus, mycophenolate mofetil and prednisone.
The evolution of the graft has been satisfactory and has not presented recurrence of the disease.
