The patient is a 40-year-old Caucasian female diagnosed with inflammatory bowel disease type Crohn's disease (A1L3B2 from the Vienna classification, A2L3B2 from the 33 years of the Montreal classification).
She reported no drug allergies or other associated medical-surgical diseases.
She's an active smoker.
His mother has been diagnosed with systemic necrotizing vasculitis with renal and splenic involvement.
The patient came to the Emergency Department in 2005 for presenting watery diarrhea of five days of evolution, of more than five stools a day without pathological products.
It is accompanied by vomiting after ingestion, food content, and constant epigastric pain irradiated in a belt that does not change with ingestion or deposition.
There is no fever or other accompanying symptoms.
Physical examination revealed significant hypotension (78 mmHg) and edema with a foveal in the lower limbs as well as edema of the lower limbs. The rest of the examination was normal.
The patient was admitted with suspected moderate bud of her disease so intravenous treatment with corticosteroids was initiated at a rate of 1 mg/kg/24 h for induction of remission, combining low molecular weight heparin.
Endoscopic examination was performed by means of barium intestinal transit multiple zones of incomplete stenosis at the level of the loops of the terminal ileum and demonstrating involvement of the cecum and right colon.
The presence of CMV and Clostridium difficile superinfection was ruled out.
During his hospital stay there is a marked progressive malnutrition with loss of all nutritional parameters (total protein 2.6, albumin 1.1, cholesterol 107, triglycerides 98, RBP 5.1, prealbumin: < 1.25-OH vitamin D).
Calcitriol < 5 pg/ml.
TSH 3.43 mIU/l, FT4 1.08 ng/dl, FT3 2.23 pg/ml.
Vitamin B12 168 pg/ml, folic acid 1 ng/ml.
Iron 7ug/dl, transferrin 58 mg/dl, 167 ng/ml), so it is necessary to establish supplementary enteral nutrition and ferritin intake.
The patient developed torpid edema with progression of peripheral edema and hypotension until she was in a situation of hypotension, which was maintained, ruling out the echocardiogram performed for any cardiac cause.
There is evidence of hypoalbuminemia and severe hypoproteinemia accompanied by progressive worsening of renal function (creatinine 1.0 to 2.4 mg/dl in a few days), with marked proteinuria (urine = 19,000 mg / 9.0 mg total protein:
Location of acute renal failure associated with nephrotic syndrome and hypoalbuminemia, which cause hypotension and anasarca, furosemide and hydrochlorothiazide are initiated according to recommendations of the Nephrology Service.
Despite the therapeutic measures used, there was no clinical or renal improvement, so it was decided to perform a rectal biopsy when secondary amyloidosis was suspected in the context of CD.
Congo red stain of the sample obtained shows the presence of amyloid material, Congo red positive, resistant to permanganate.
After performing immunohistochemical study, it was demonstrated expression of amyloid A protein (AA) in the material deposited around the submucosal vessels, demonstrating the secondary character of this deposit.
Once the definitive diagnosis of secondary amyloidosis with renal involvement in the form of nephrotic syndrome and renal failure was obtained, treatment with colchicine was established, showing a decrease in the situation of anasarca and renal function.
As maintenance treatment of CD, azathioprine is administered, after determination of thiopurylmethyltransferase levels.
Since then, the patient maintains renal function within normal parameters and does not require hospitalization for underlying disease, continuing to receive treatment.
