A 63-year-old woman with a history of psoriasis and hysterectomy for myomatosis.
Two of their 3 children developed insulin-dependent diabetes mellitus at 18 years of age and another ankylosing spondylitis.
For 2 months she had diarrhea, weight loss and late hematochezia and was hospitalized.
On physical examination, she was pale and severely ill, had nails that migrated, hair loss, tongue depapillated, and lower limb edema.
There was anemia with microcytosis, hypochromia and macrocytosis; iron and folic acid deficiency, elevated ESR, thrombocytosis, hypoalbuminemia, massive proteinuria (46.3 g/24 h) and renal dysfunction
Hematuria, leucocyturia and cylindruria were not observed in the urine sediment.
Clinically, CD and NS were diagnosed.
Panendoscopies showed marked signs of duodenal mucosa and biopsy showed characteristic alterations of CD.
Anti-tissue transglutaminase and anti-endomysium antibodies were positive, antinuclear, anti-DNA, ANCA and rheumatoid factor were negative.
The concentrations of IgG, IgA, IgM, C'3 and C'4 were normal.
Plasma protein electrophoresis showed no monoclonal peak and urine immunofixation did not demonstrate the presence of light chains.
Serology for HIV, virus B and C was negative.
Vitamin B12, folates, iron, red balloons and total parenteral nutrition (TCPN) were indicated.
Myelogram showed hyperplasia of the three series.
A non-contrast-enhanced scan showed a very small spleen (43 x 20 x 18 mm), subcutaneous cellular tissue edema, ascites and pleural effusion.
On the fifth day of admission she had a generalized seizure episode and phenytoin was prescribed.
Magnetic resonance imaging showed nodular millimetric defects in the subcortical white matter.
On the seventh day she presented acute pulmonary edema and fell into shock.
High doses of noradrenaline and dopamine were required.
Hydrazone was administered at 300 mg/day.
Cardiac enzymes (CK-MB and troponins) were persistently normal.
The echocardiogram showed dilation of the left atrium, left ventricle dilated with diffuse hypokinesia and left ventricular systolic and diastolic dysfunction with ejection fraction 32% (Simpson) and pulmonary hypertension.
A high fever appeared the next day, which led to suspicion of sepsis and dropped into anuria.
Antibiotic therapy was indicated with tazodone and pastepenem and peritoneal dialysis was installed.
Klebsiella pneumoniae was isolated from the blood cultures, presumed to originate in the paranasal sinuses, where the same germ was cultured.
On the following days, she received mechanical ventilation and peritoneal dialysis, receiving TCPN and catechiae.
On day 15, the peritoneum-dialysis was removed and continuous hemovenous (HFWC) was started.
The echocardiogram performed on day 17 of admission showed significant improvement in cardiac function with an ejection fraction of 75%.
After the tenth day of renal failure and 19 days after admission, the patient developed severe arterial hypertension (SA > 200 mmHg, DP > 130 mmHg), which led to the use of nitroglycerin, captopryl, am.
Simultaneously, there was a rapid anemization and decrease in platelet count from 850,000 to 110,000 mm3, requiring repeated transfusion of red blood cells.
A Coombs test was negative.
On day 30, mechanical ventilation was withdrawn and a CT scan showed nodular liver lesions that led to suspicion of an abdominal infection.
Fluconazole was prescribed.
On day 34, the patient developed digestive hemorrhage secondary to an ulcerative colitis which was endoscopically sclerotic.
On day 45, a surgical BR was performed that included 24 glomeruli.
The findings were compatible with a mesangioproliferative glomerulopathy pattern, without evidence of deposit of podium complexes with diffuse complement factors and with characteristics of a lesion secondary to pedothrombotic microcangiopathy.
Signs of extensive tubular disease were also found.
On day 49, HFWC was diagnosed and conventional hemodialysis was started, which was suspended 52 days after the onset of renal function improvement (36.7 ml/min/1.73 m2).
She was discharged on day 66 of hospitalization, receiving prednisone 30 mg/day as therapy for nephrotic syndrome and a gluten-free diet.
1.
During the 7 months after discharge due to persistent NS, being treated with prednisone, enalapril, losartan, atorvastatin and furosemide; oral cyclophosphamide 100 mg/ day was indicated.
This drug was used for 11⁄2 year associated with prednisone 5 mg/day, obtaining a partial remission of nephrotic syndrome.
At the last follow-up visit, at 46 months post-discharge, the patient's general condition was very good, her BMI was 27 kg/m2, there were no edemas, 24-h proteinuria 1,441 mg/ min creatinine clearance 301.73
